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Abstracts Presented at the 22nd Joint Meeting of the International Society of Dermatopathology, February 27–28, 2019, Hilton Crystal City at Washington Reagan National Airport, Arlington, Virginia, USA

The American Journal of Dermatopathology: November 2019 - Volume 41 - Issue 11 - p e148–e155
doi: 10.1097/DAD.0000000000001430

Oral Abstract Presentations, Wednesday, 02/27/2019, 8:00–10:00 AM

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Pushing and Loss of Elastic Fibers in Melanocytic Lesions Is Highly Specific for Melanoma and Is Rare in Melanocytic Nevi

A. Stillhard, S. Cazzaniga, L. Borradori, and H. Beltraminelli

Dermatology Department, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.

Background: Histologically, the differentiation between nevus and melanoma is straightforward in the vast majority of cases. Although several criteria to distinguish nevi from melanoma have been proposed, there are few melanocytic lesions (ML), for which a diagnosis is very difficult or even impossible.

Methods: We performed a retrospective study analyzing 14 histological characteristics in 150 mL (30 melanoma and 120 nevi: 30 compound/dermal, 30 junctional, 30 dysplastic, 30 blue).

Results: Eight characteristics, that is, regression, inflammation, loss of rete ridges, atrophy of the epidermis, UV-elastosis, loss of elastic fibers (EF) in the ML, loss of EF in fibrosis, pushing of EF, showed a significant difference between nevi an melanoma. Pushing and loss of EF in the ML, and loss of EF in fibrosis are frequently associated with melanoma, and are only rarely seen in benign ML.

Conclusions: We have identified 8 morphological characteristics which are helpful to differentiate melanocytic nevi from melanoma.

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Two Cases of Subcutaneous Sweet Syndrome With Histiocytoid Features

Mary Brady, MD, Eric Hossler, MD, and Jaqueline Junkins-Hopkins, MD

Departments of Dermatology and Dermatopathology, Geisinger Health System, Danville, PA.

Cutaneous lesions of Sweet syndrome (SS) are histologically characterized by dermal neutrophils. Subcutaneous histiocytoid sweet syndrome (SHSS) is a rare variant of SS consisting of myeloperoxidase-positive mononuclear histiocytoid cells, representing immature cells of myeloid lineage, which are localized in the subcutaneous fat. Two such cases are presented.

Case Descriptions: Patient 1 is a 50-year-old female with a history of IgG lambda monoclonal paraproteinemia with a 5-year history of recurrent, tender lesions on the trunk and extremities associated with fever, malaise and arthralgias. Patient 2, a healthy 13-year-old female, presented with intermittent, asymptomatic firm lesions on the lower extremities, occasionally associated with arthralgias or preceding upper respiratory infection. Biopsies from both patients revealed a lobular panniculitis predominantly composed of myeloperoxidase-positive mature and immature neutrophils with histiocytoid morphology. Features suggestive of leukemia cutis were not identified.

SHSS is a distinct variant of SS with unique histopathologic features and similar associations with systemic conditions, including hematologic disorders, malignancy, medications, and autoimmune and inflammatory conditions. This should prompt a thorough investigation and surveillance.

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Granulomatous Skin Eruption in Common Variable Immunodeficiency

Olivia Crum, BA,* Emma Johnson, MD,† and Nneka Comfere, MD†,‡

*Mayo Clinic School of Medicine; †Department of Dermatology; and ‡Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN.

Patient History: A 58-year-old female with a 10-year history of common variable immunodeficiency (CVID) presented with worsening ulcerative and painful skin eruption. Clinical exam revealed diffuse tender, violaceous-to-purple-hued, plaques and subcutaneous nodules with superficial erosions, most consistent with a granulomatous process. Lesional biopsy revealed dermal palisaded granulomas composed of epithelioid histiocytes and scattered multinucleated giant cells with surrounding lymphocytic inflammation and zones of interstitial collagen degeneration. GMS and Fite stains were negative for microorganisms. Tissue cultures were negative.

Diagnosis: Reactive granulomatous dermatitis associated with common variable immunodeficiency syndrome.

Treatment: Granulomatous disease involving the lungs, spleen, liver and/or skin occurs in 8–22% of patients with CVID. Treatment of granulomas in CVID presents a particular challenge, as some form of immune suppression may be required, which is undesirable in the presence of an existing immunodeficiency. Studies on treatment options and response to treatment are limited. Reported treatments have included glucocorticoids, hydroxychloroquine, cyclosporine, mycophenolate mofetil, and TNF-alpha antagonists. A retrial of a TNF-alpha inhibitor was recommended in this case.

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Prominent Transepidermal Melanin Deposition is a Distinguishing Histopathologic Feature of Melasma

F. Hafeez,* D. A. Mata,* C. G. Lian,* and E. G. Poulos EG†,‡

*Brigham and Women’s Hospital; †University of Miami Miller School of Medicine; and ‡Global Pathology, Miami Lakes, FL.

Melasma and postinflammatory hyperpigmentation (PIH) represent the most common causes of acquired hyperpigmentation. Though these diagnoses can be difficult to distinguish clinically, validated histopathological criteria to differentiate these lesions in the literature are sparse to none. This study’s purpose was to compare melasma and PIH across several histologic features, using both H&E and Fontana-Masson staining. From July 4, 2010, to July 23, 2018, cases of previously-diagnosed melasma and PIH were sought from the pathology laboratories of Aurora Diagnostics Global Pathology (Miami Lakes, FL) and BWH (Boston, MA). Clinical impression, site of biopsy, clinical photographs, and/or medical records were evaluated to ensure the validity of the histological diagnosis; cases with questionable diagnoses were excluded. In total, 30 biopsies (27 patients) of melasma and 37 biopsies (35 patients) of PIH were obtained. Using FM staining, cases of melasma were found to demonstrate a greater degree of transepidermal melanin deposition and basal layer melanin deposition compared to cases of PIH (P < 5.0 × 10−6 and P = 0.007, respectively).

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Late Onset Eccrine Angiomatous Hamartoma With Myxoid Features: A Report of 2 Cases

Koorosh Haghayeghi,* Leslie Robinson-Bostom,† Gladys Telang,† Martin Weinstock,† and Eric Karasko†

Departments of *Pathology and †Dermatology, Brown University/Rhode Island Hospital, Providence, RI.

Eccrine angiomatous hamartoma (EAH) is a rare benign lesion with admixed proliferations of eccrine and vascular constituents. This rare entity is classically described as solitary painful congenital lesion of the extremities in children. However, atypical presentations have been identified. We report 2 adult-onset cases with unusually prominent myxoid features. The first patient is a 55-year-old man with 5-year history of a boggy blue nodule on the proximal lower leg which was refractory to intralesional steroids for presumed myxedema. Biopsy revealed intermixed vascular and hypertrophic eccrine coils within a myxoid stroma compatible with EAH. The second patient is a 45-year-old man with 3-year history of a painful ill-defined nodule on his medial thigh. Biopsy revealed classic EAH with prominent myxoid features. Both lesions were surgically excised. Despite its indolent nature, spontaneous regression of EAH is rare. In cases of progressive enlargement or pain, surgical extirpation remains the definitive treatment. These cases illustrate the variability in clinicopathologic features of EAH, arising in an unusual demographic.

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Perplexing Cases of Panniculitis: Expanding the Differential Diagnosis of Panniculitis

Jennifer L. Hanson, MD,* Kabeer K. Shah, DO,*,† Valentina Logunova, MD,* Sindhuja Sominidi Damodaran, MD,* and Alina G. Bridges, DO*,†

Departments of *Dermatology and †Lab Medicine and Pathology, Mayo Clinic, Rochester, MN.

Winner Best Oral Abstract.

We present 4 cases of panniculitis to expand the differential to include infection, inflammatory reaction to autologous fat injection, and drug reactions to chemotherapy and checkpoint inhibitors. Novel cases of panniculitis will be described including (1) infectious panniculitis resulting from disseminated enterovirus infection; (2) panniculitis mimicking cellulitis and inflammatory breast cancer resulting from autologous fat transfer for breast reconstruction; (3) chemotherapy induced panniculitis mimicking erythema nodosum or a neutrophilic dermatosis and (4) checkpoint inhibitor-induced panniculitis. Our unusual cases broaden the knowledge of the etiologies of panniculitis and emphasize the importance of careful clinicopathologic correlation to arrive at an accurate diagnosis for optimal patient management.

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Interobserver Reliability in Distinguishing Xanthogranuloma From Reticulohistiocytoma

Brian S. Hoyt, MD,* Konstantinos D. Linos, MD,* Shaofeng Yan, MD, PhD,* Shabnam Momtahen, MD,* Aravindhan Sriharan, MD,* Youdinghuan Chen,† Tien-Anh N. Tran, MD,‡ and Robert E. LeBlanc, MD*

*Department of Pathology and Laboratory Medicine, Dartmouth-Hitchcock Medical Center; Lebanon, NH and Dartmouth Geisel School of Medicine; Hanover, NH; †Department of Epidemiology, Dartmouth Geisel School of Medicine, Hanover, NH; and ‡Florida Hospital Center for Diagnostic Pathology, Orlando, FL.

The Histiocyte Society recently stated that reticulohistiocytoma (RH) “is simply a xanthogranuloma (XG) in which oncocytic macrophages and ground-glass giant cells dominate.” Given the considerable histologic overlap between these entities, we retrospectively reviewed 62 cases of XG or RH from 2 institutions to determine the interobserver reliability in distinguishing them. Each case was examined independently by 5 board-certified dermatopathologists. In a majority of cases (36 cases, 58 percent), at least one reviewer indicated that the case had overlapping features of both XG and RH. Overall, there was moderate interobserver diagnostic concordance (mean κ = 0.63, median McNemar’s P < 0.001) underscoring the significant histologic overlap and supporting the possibility that XG and RH are the same entity.

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Signet-Ring Squamous Cell Carcinoma In Situ: Report of a Rare Entity and a Review of the Literature

Yuan Yu Michael Huang, MD,* Sihem Khalifa, MD,† and Matthew Kuhar, MD*,†

Departments of *Dermatology and †Pathology & Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN.

Signet-ring cells demonstrate eccentric nuclei located near the cellular border due to displacement by large cytoplasmic vacuoles. They are classically seen in adenocarcinomas but can be seen other neoplasms as well. The signet-ring cell variant of cutaneous squamous cell carcinoma (SCC) is an extremely rare variant of SCC, with only 14 cases previously reported in the literature. We report a case of a 59-year-old woman presenting with a slowly growing pink scaly papule on the right lateral posterior arm that showed focal conventional squamous cell carcinoma in situ (SCCIS) and a predominant adjacent cell population with compelling signet ring cell morphology. Immunohistochemical studies confirmed the diagnosis and excluded other more common entities with signet-ring cytology. To our knowledge, we report the first case of signet-ring variant SCCIS. A review of the 14 previously described signet-ring variant SCC was also performed and compared with our case in hopes of further understand this rare histological diagnosis.

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Combined Prognostic Significance of Defective Mismatch Repair and BRAFV600E Mutation in Melanoma

Israel Kasago, Saleh Najjar, Ammoura Ibrahim, David Jones, and John Andrew Carlson

Albany Medical Center, Albany, NY.

Introduction: MSI (Microsatellite instability) is a frequent event in melanoma. However, the combined prognostic role of MSI and BRAFV600E mutation in melanoma is unclear. In this study, we examined the combined prognostic value of mismatch repair (MMR) protein expression and BRAFV600E mutation in melanoma.

Methods: We correlated MLH1, MSH2, MSH6 and PMS2 expression with BRAFV600E mutation status, histopathological predictors and survival data in a series of 55 primary melanomas and 93 metastatic melanomas.

Results: The percentage of tumor cells with MSH6 nuclear positivity (PTNP) of more than 80% was noted in 94/143 (66%) and intense MSH6 was noted in 76/143 (53%). Increased MSH6 PTNP and intense MSH6, both correlated with poor overall survival (χ2 = 22.47, P = 0.0001, χ2 = 9.30, P = 0.0096), respectively. Increased MSH6 PTNP correlated with increased recurrence rate (χ2 = 13.64, P = 0.0034). BRAFV600E mutation status revealed no statistically significant differences in overall survival.

Conclusions: Mismatch repair status is an independent prognostic biomarker and reflex mismatch repair assessment by immunohistochemistry can be used as a powerful predictor of overall survival and recurrence in melanoma.

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Atypical Generalized Enteroviral Eruption in an Adult: Not Limited to “Hand, Foot And Mouth” Disease

Aleksandar Krbanjevic, MD, PhD,* Dorothy Rodenbeck, MD,† Jerry Feldman, MD,† and Marylee Braniecki, MD*

*Department of Pathology, University of Illinois at Chicago; and †Department of Dermatology, Cook County Health & Hospitals System.

Hand-foot-and-mouth-disease (HFMD) is a highly infectious viral illness caused by the Enterovirus genus (most commonly Coxsackie A16, as well as Enterovirus 71 and other less common strains). It typically presents with painful mouth sores and a blistering rash on the hands, feet and buttocks. HFMD usually affects children less than 10 years old and is transmitted via respiratory droplets or fecal-oral route. HFMD develops within 3–6 days of infection with a fever followed by 1–3 mm erythematous macules which evolve into painful vesicles. Only 11% of adults develop cutaneous lesions. We share a case of an atypical presentation of HFMD in a 34-year-old Caucasian male. After a 1-day prodrome of fever and sore throat, he suffered an abrupt onset of pruritic dusky vesiculopapules on his palms and soles with spread to his extremities, perioral face and ears, plus a confluent morbilliform morphology on his trunk. He notably had no changes in his oral mucosa. Skin biopsies showed interepidermal balloon cell necrolysis with marked papillary dermal edema and perivascular mononuclear infiltrates. Lesional swab demonstrated amplified Enteroviral RNA. Although it may have a self-limited course, we present this case as to raise awareness that cutaneous Enteroviral eruption in adults may manifest as an “atypical HFMD,” with a generalized papulovesicular skin eruption involving the palms and soles, but without oral involvement.

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Haematoxylin-Eosin Staining Pattern of Acral Stratum Corneum Corresponds to Cristae Profundae in Acral Melanocytic Lesions

Y. A. Liu and R. I. Crawford

University of British Columbia, Vancouver, BC, Canada.

Background: It is challenging to differentiate between acral lentiginous melanoma (ALM) and acral melanocytic nevi (AMN). Dermatoscopic findings of parallel-ridge-pattern in ALM and parallel-furrow-pattern in AMN are reflected in histology; melanocytes in ALM congregate in the cristae profundae intermedia (CPI) and melanocytes in AMN congregate in the cristae profundae limitans (CPL). We aim to identify the CPI and CPL in haematoxylin-eosin sections of acral melanocytic lesions irrespective of orientation, and evaluate their melanocytic proliferation patterns.

Methods: We identified 22 patients with AMN and 8 patients with ALM. 40×-magnification limitation was created to isolate the acral stratum corneum for CPI/CPL identification. Complete re-evaluation was performed for the dominant melanocytic proliferation pattern.

Results: 30/37 acral melanocytic lesions demonstrated haematoxylin-eosin difference between the CPI and CPL. 13/13 cases of ALM showed diffuse melanocytic proliferation involving both CPI and CPL. 22/24 cases of AMN showed melanocytic proliferation isolated to the CPL.

Conclusions: Stratum corneum H&E staining can help identify CPI and CPL in all orientations. Melanocytic proliferation involving both CPI and CPL can help distinguish ALM from AMN.

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Malignant Granular Cell Tumor Presenting With Distinct Benign and Sarcomatous Cell Populations

Lisa M. Marinelli, BS, Andrew L. Folpe, MD, and Ruifeng Guo, MD, PhD

Division Anatomic Pathology, Mayo Clinic, Rochester, MN.

Granular cell tumor (GCT) is an uncommon neoplasm with Schwannian differentiation and distinct granular cytoplasm. The vast majority of GCT are benign. Malignant GCT is an extremely rare high-grade sarcoma associated with aggressive clinical behavior. Herein we report a case of histopathological malignant GCT. The patient is a 44 year old male with a left arm mass (>3 cm). Biopsy showed 2 morphologically distinct cell populations. All of the cells contained granular cytoplasm and stained positively for S100 and CD68 (weak) and negatively for Melan A and pan-cytokeratin. The first population, composed of nests of ovoid granular cells with a low nuclear to cytoplasmic ratio and small monotonous nuclei, was consistent with conventional GCT. The second population, composed of fascicles of large sarcomatoid spindled cells with prominent nucleoli, nuclear pleomorphism, and high mitotic activity (up to 11/10 hpf), supports the diagnosis of malignant GCT associated with overlying conventional GCT. The overlying epithelium demonstrated reactive pseudoepitheliomatous hyperplasia (PEH) mimicking that of well-differentiated squamous cell carcinoma. Given the rendered diagnosis of malignant GCT, the patient underwent wide local excision with clinical follow-up (8 months). There is no recurrence or metastasis thus far. Further clinical follow-up is needed.

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Histopathologic Comparison of Cutaneous Adverse Events in Immune Checkpoint Blockade Therapy

Saisindhu Narala, Macartney Welborn, Shelby Kubicki, Osama Hashmi, Sana Zahirrudin, and Anisha B. Patel

MD Anderson Cancer Center, Houston, TX.

Immune checkpoint inhibitors (ICIs), which target cytotoxic T-lymphocyte-associated antigen-4 (anti-CTLA4) or programmed cell death (anti-PD1), have been increasingly used for a wide range of malignancies. They are associated with significant cutaneous adverse events (CAEs) and a greater understanding of their pathophysiology is warranted. This study is the first to analyze biopsy H&E sections (8 patients on anti-PD1 therapy and 19 patients on anti-CTLA4 therapy) for categorization of epidermal reaction pattern and inflammatory cell types with an in-depth clinical review. Presence of eosinophils was associated with anti-CTLA4 (P < 0.05). The small sample size limited statistical significance, but a few trends were noted. Lichenoid dermatitis was associated with anti-PD1 (P = 0.09), ICI dosage decrease or discontinuation (75% vs. 40%, p=0.12), and systemic steroid use (75% vs. 31%, P = 0.09). Time to rash onset for CAEs with vesicles was shorter (22 vs. 48 days, P = 0.17), and for CAEs with dyskeratosis was longer (66 vs. 27 days, P = 0.22). A prospective study is warranted to further correlate histopathologic features with clinical CAE course, helping guide CAE management more efficiently.

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Hyaline Cell-Rich Apocrine Mixed Tumor With Cytologic Atypia

Chika Ohata

Department of Dermatology, Kurume University School of Medicine, Kurume, Fukuoka, Japan.

Hyaline cell-rich apocrine mixed tumor is relatively rare, and it often possesses atypical cells. Despite the presence of atypical cells, other histopathological features such as well circumscription, smooth border, predominance of bland cells, and no mitotic figures lead to classify these tumors as benign. In addition, no recurrence or metastasis has been reported even when cytologic atypia is identified. An 80-year-old woman presented with a 2-year-history of a nodule on the face. Histopathological examination of the totally excised lesion revealed a well-circumscribed nodular lesion with smooth border in the dermis and subcutis. The lesion consisted of an epithelial component and a myxoid stroma. The epithelial components were arranged in large and small aggregations with or without tubular structures, which occasionally showed apocrine secretion. Most epithelial cells were plasmacytoid hyaline cells with homogeneous, deeply eosinophilic cytoplasm, and signet-ring type cells were occasionally seen. Some plasmacytoid hyaline cells had abundant eosinophilic cytoplasm and large oval to polygonal nuclei with indistinct nucleoli, and a few extremely large atypical cells presented large bizarre nuclei with small nucleoli. No mitotic figures were found. The lesion did not recur for 14 months after excision.

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Cutaneous Involvement in Multiple Myeloma: A Series of 10 Cases

Gauri Panse, MD

Department of Dermatology & Pathology, Yale School of Medicine, New Haven, CT.

Cutaneous involvement by multiple myeloma (MM) is relatively uncommon. The aim of this study was to study clinicopathologic and immunohistochemical features in a series of MM with secondary cutaneous involvement. Ten cases of MM involving skin were retrospectively reviewed. Patients ranged from 61 to 89 years (7 males, 3 females). Sites of involvement included head & neck (n = 3), trunk (n = 5) & extremities (n = 2). All patients showed evidence of MM on bone marrow biopsy preceding secondary cutaneous involvement. Histopathologic examination revealed a diffuse (n = 6) or interstitial (n = 4) dermal infiltrate of plasma cells. Three cases revealed a predominantly plasmablastic infiltrate, wherein the plasma cells were atypical and poorly differentiated. One case demonstrated amyloid deposition surrounding the neoplastic infiltrate. The plasma cells were either kappa-restricted (6/10) or lambda-restricted (4/10). By immunohistochemistry, the cells were CD138+ (10/10), MUM-1+ (5/5) and CD20− (9/9). CD79a was either diffuse (1/7), partial (3/7) or negative (3/7), while CD56 was positive in 6/9 and negative in 3/9 cases. Follow-up (8 cases, range 5–67 months) revealed disease related death in 6/8 cases.

Conclusions: Three of the 10 cases of MM in skin showed poorly differentiated morphology that could potentially mimic cutaneous lymphomas, carcinoma or melanoma. Occasional cases of MM in skin may demonstrate amyloid deposition. In patients with known MM, CD138 positivity can be helpful for confirming the diagnosis.

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LEF-1 and ß-Catenin Expression in Acral Lentiginous Melanoma

Nisha Ramani, Phyu P. Aung, Jun Gu, Steven Sfamenos, Priya Nagarajan, Michael T. Tetzlaff, Jonathan L. Curry, Doina Ivan, Wen-Jen Hwu, Victor G. Prieto, and Carlos A. Torres-Cabala

The University of Texas, MD Anderson Cancer Center, Houston, TX.

Introduction: The role of Wnt/ß-catenin signaling pathway, in which ß-catenin and lymphoid enhancer-binding protein-1 (LEF-1) act as the main molecules, is crucial for the development of melanocytes. Activation of this pathway is a valuable biomarker for prognosis and a therapy target in a variety of tumors; however, little is known of its role in acral lentiginous melanoma (ALM).

Materials and Methods: Thirty-five ALM (26 primary and 9 metastatic) and 24 controls (20 non-ALM melanoma and 4 acral nevi) were tested by immunohistochemistry for ß-catenin and LEF-1. Intensity (0, 1+, 2+, and 3+) and number of positive cells (%) were recorded and H score was calculated.

Results: ALM median age was 55.5 years (18–93) with M:F of 1.3:1. ALM stages were: MIS (2), pT1 (1), pT2a (10), pT2b (1), pT3a (1), pT3b (5), pT4a (3) and pT4b (12). Cytoplasmic/membranous ß-catenin was detected in 33/35 (94.28%) and nuclear LEF-1 in 24/35 (68.5%). In non-ALM ß-catenin was seen in 14/18 (77.7%) and LEF-1 in 12/18 (66.6%). Acral nevi expressed ß-catenin in 4/4 (100%); 2/4 (50%) were positive for LEF-1, with low H-score.

Conclusions: LEF-1 expression is frequent in ALM and appears to be low in acral nevi. Nuclear ß-catenin labeling was not detected, suggesting that LEF-1 may act independently of nuclear localization of ß-catenin. Further evaluation of Wnt/ß-catenin signaling pathway for therapy and prognosis of ALM is warranted.

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Acneiform-Like Presentations of Folliculotropic Mycosis Fungoides

Christie Riemer,* Gabriel Sciallis,* Marian McEvoy,* Roger Weenig,† and Nneka Comfere*,‡

*Department of Dermatology, Mayo Clinic, Rochester, MN; †Associated Skin Care Specialists P.A., Fridley, MN; and ‡Department of Laboratory Medicine and Pathology, Rochester, MN.

Folliculotropic mycosis fungoides (FMF) is a variant of cutaneous T-cell lymphoma, that has clinical overlap with a variety of inflammatory follicular unit disorders. We describe 4 clinical and histopathologic presentations of FMF masquerading as acneiform entities: hiadrenitis suppurativa, furunculosis and acne vulgaris (ages 33–65 years, 3:1 female to male). Clinical presentations included open/closed comedones, inflammatory pustules/papules/nodules, follicular papules with keratotic plugging, cysts and scarring involving the face and intertriginous areas. All patients failed to respond to standard therapies including topical/oral antibiotics, isotretinoin, topical/intralesional corticosteroids and excision. All lesional skin biopsies showed perifollicular CD4-postive T-lymphocytes with pilotropism, with additional varied findings of intra-follicular mucinous deposition, foreign body granulomatous inflammation, acute inflammation and necrosis. None had concurrent systemic disease. We present these cases to illustrate the clinical and histopathologic spectrum of FMF that may strikingly resemble acneiform disorders. We highlight the importance of histopathologic and immunohistochemical evaluation for diagnostic confirmation of presumed acne unresponsive to standard therapies.

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Cutaneous Xanthogranuloma in Children and Adults: A 29-Year Experience

Behzad Salari, MD and Louis P. Dehner, MD

Department of Pathology, Washington University in St. Louis, MO.

Background: Juvenile xanthogranuloma (JXG) is an uncommon non-Langerhans cell histiocytosis. We aimed to provide an update on histologic and immunophenotypic pattern of this entity.

Methods: A retrospective review of cases with the pathologic diagnosis of “xanthogranuloma” were performed from our archives from 1989 to 2018. All skin and skin with underlying soft tissue lesions were included. Patients with concurrent extracutaneous lesions were not included in final analysis.

Results: A total of 392 patients were identified with the median age of 6.8 years and a male predominance (57.7%). Uniform positivity was noticed for all patients with available vimentin (n = 45), CD68 (n = 99), and CD163 (n = 6) stains. Other immunohistochemistry studies showed the following expression: factor XIIIa, 62/67 (93%); CD31, 4/10 (40%); CD4, 4/6 (67%); S-100 protein, 24/81 (30%); and CD1a, 1/55 (2%). Comparing group A (age <20 years; median 2.1 years) vs. group B (age ≥ 20 year; median 43.0 years) showed the following reactivity: factor XIIIa, 93.2% versus 87.5%; CD31 57.1% versus 0%; CD4, 75% versus 50%; S-100 protein, 28.1% versus 35.3%; and CD1a, 1.8% versus 0%.

Conclusions: In this large comprehensive age cohort of cutaneous JXG patients, we demonstrated immunophenotypic expression of the lesion regardless of age. Although low in numbers, CD163 shows promising results in diagnosis of JXG and CD31 failed to show consistent results. Histologic pattern findings will be discussed.

“Dermatopathology Trainee World Cup”, Wednesday 02/28/2019, 10:36 AM–12:36 PM

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Molluscoid Sweet Syndrome: A Rare Clinical Variant

Christine Ahn,* Sean McGregor,† Lindsay Strowd, MD,† and Omar Sangüeza*

Departments of *Pathology and †Dermatolog, Wake Forest School of Medicine, Winston Salem, NC.

3rd Place Winner DP Trainee World Cup.

Sweet syndrome is a reactive neutrophilic dermatosis with varied clinicopathologic presentations. We describe 2 cases of molluscoid Sweet syndrome, a rare variant with only one prior case report.

Case 1: A 50-year-old woman with a history of renal disease and bacterial endocarditis presented with lip erosions and umbilicated papules on the face and trunk. The clinical differential diagnosis included cryptococcosis, penicilliosis, and molluscum contagiosium. Skin biopsy and tissue culture were performed. Histology revealed papillary edema with a robust dermal neutrophilic infiltrate. PAS, GMS, mucicarmine and tissue culture were negative. Antifungal therapy was discontinued, and lesions rapidly resolved on corticosteroid therapy.

Case 2: A 41-year-old man presented with umbilicated papules and hemorrhagic vesicles on the dorsal fingers. Skin biopsy demonstrated papillary edema with neutrophilic infiltrate in the dermis. PAS, GMS, mucicarmine stains and tissue culture were negative. The patient improved with corticosteroid therapy.

Molluscoid lesions are nearly pathognomonic of infection, particularly in immunosuppressed patients. However, molluscoid Sweet syndrome is a rare clinical variant that clinicians and pathologists should recognize to prevent treatment delay.

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A Geographic Inflammatory Dermatitis in a Patient With Anti-fibroblast Growth Factor 3 Antibody Small Fiber Neuropathy

Connor Flint, MD,* Ann Bell, MD,† and Nicholas Logemann, DO*

*United States Navy; and †United States Army, Walter Reed National Military Medical Center, Bethesda, MD.

Fibroblast Growth Factor Receptor 3 (FGFR3) is a protein that interacts with Fibroblast growth factors to affect mitogenesis and cell differentiation. Mutations in this gene have been linked to defects in long bone growth and development, and the formation of nevi in multiple genodermatoses. Antibodies to FGFR3 have not previously been described within the scope of Dermatology and appear only rarely in other subspecialty literature. When it does, it is in regard to sensory neuropathy as well as a potential treatment modality in some cancers expressing FGFR3. We present a patient with anti-FGFR3 antibodies causing an extremely painful small fiber neuropathy, who presented with geographic hyperpigmentation in the distribution of a prior inflammatory dermatitis. She represents a novel dermatologic manifestation of a disease process previously only characterized in neurology literature.

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Diffuse Dermal Angiomatosis With Clinical Features Simulating Calciphylaxis in the Setting of End-Stage Renal Disease

Diana S. Braswell, MD, Rony A François, MD, PhD, Maria Isabel Longo, MD, PhD, and Kiran Motaparthi, MD

Department of Dermatology, University of Florida, Gainesville, FL.

Diffuse dermal angiomatosis (DDA) is localized form of reactive angioendotheliomatosis (RAE). DDA Herein, we present a case of a DDA in the setting of end-stage renal disease (ESRD). A 63-year-old woman with ESRD presented with several painful, indurated nodules with purpura and ulceration on the thighs. An initial punch biopsy demonstrated a proliferation of vascular channels of various size and lined by endothelia without cytologic atypia, dissecting collagen through the reticular dermis and subcutis. A wider incisional biopsy was promptly recommended given the clinical context. However, histology once again demonstrated a similar reactive vascular proliferation; HHV-8 immunostain was negative, and special stains for calcium (Von Kossa) and elastin (Verhoeff-Van Gieson) failed to identify features of calciphylaxis. Considered a reactive vascular proliferation in response to tissue hypoxia, DDA is well-described in association with peripheral vascular disease, macromastia and obesity, severe atherosclerosis, and smoking. DDA may also be seen in the setting of ESRD and may clinically simulate causes of retiform purpura and ulceration, including vasculitides, vasculopathies, and calciphylaxis.

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Metastatic Malignant Melanoma of Unknown Primary Mimicking an Epithelioid Malignant Peripheral Nerve Sheath Tumor: Utility of Next Generation Sequencing

Dena Elkeeb, MD, MS, Lauren Ritterhouse, MD, PhD, Thomas Krausz, MD, and Oluwakemi Onajin, MD

Departments of Dermatology and Pathology, University of Chicago, Chicago, IL.

1st Place Winner DP Trainee World Cup.

We present a case of a metastatic malignant melanoma of unknown primary mimicking an epithelioid malignant peripheral nerve sheath tumor (MPNST). An 80-year-old male presented with a one-month history of a rapidly enlarging nodule on the left posterior shoulder. Skin biopsy revealed a well-circumscribed dermal proliferation composed of nests and fascicles of spindled and epithelioid atypical cells with rhabdoid features. The neoplastic cells were strongly positive for S100 and SOX 10, retained INI1 nuclear staining, and negative for Melan-A and HMB-45. A CT-scan revealed multiple lung nodules and biopsy showed similar histopathological findings to the skin nodule. Next generation sequencing of the lung nodule demonstrated high mutational burden including NF1 mutation, and numerous CC>TT dinucleotide substitutions consistent with ultraviolet radiation mutation signature; thereby, favoring malignant melanoma. Spindle cell melanoma and MPNST are difficult to distinguish due to similar histopathologic features. Our case highlights the utility of next generation sequencing in the diagnosis of malignant melanoma.

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A Case of Cutaneous Spindle Cell Adenolipoma With Eccrine and Apocrine Differentiation

Maya Farah, MD and Hye Jin Chung, MD, MMS

Dermatopathology Section, Department of Dermatology, Boston University School of Medicine, Boston, MA.

Cutaneous spindle cell adenolipoma (SCAL) is a recently described rare variant of lipoma with 11 cases reported to date. We report a consultation case of a 77-year-old male presenting with a nodule on the right nasolabial fold, diagnosed as apocrine fibroadenoma or sebaceous hyperplasia by an outside pathologist. The specimen revealed an ill-defined dermal tumor composed of mature adipocytes, bland spindle cells, and dilated eccrine and apocrine glands and ducts in a fibromyxoid stroma. The spindle cells were positive for CD34 and negative for S100 and SOX10. These findings are consistent with those of SCAL. The pathogenesis of this entity is controversial and includes a hamartomatous process, development from adipose tissue surrounding eccrine glands, or glands entrapment within a growing lipoma. In the present case, the glandular component is extensive and includes both eccrine and apocrine differentiation, which has not been previously described and further supports a hamartomatous nature. Awareness of this rare entity is helpful to prevent confusion with other look-alike primary and metastatic cutaneous lesions.

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Lepromatous Leprosy: An Elusive Mimicker Mistaken for Polyarteritis Nodosa & Disseminated Tuberculosis

Danielle Fasciano, DO,* David Ullman, MD,* Matthew Innes, MD,† David Dorn, MD,* Vishnu Reddy, MD,* Elizabeth Ergen, MD,† and Peter Pavlidakey, MD*,†

*Department of Pathology, UAB, Birmingham, AL; and †Department of Dermatology, UAB, Birmingham, AL.

Leprosy is a chronic infectious disease caused by Mycobacterium leprae and presents as skin lesions and peripheral neuropathies with upper respiratory mucosa involvement. We present a case of a 36-year-old immunocompromised female whom was diagnosed polyarteritis nodosa vasculitis (PAN) in Trinidad and returned back to the US with pleuritic chest pain, fever, chills, fatigue, epistaxis and cough. Physical examination revealed a diffuse rash. Pancytopenia prompted a bone marrow biopsy, revealing acid fast bacteria, suspicious for disseminated tuberculosis. Histologic examination of the skin lesions revealed disseminated Fite-positive microorganisms within the dermis and nerves. She developed purpura fulminans with disseminated intravascular coagulation and was placed on palliative care. PCR results shortly thereafter revealed the presence of M. leprae DNA. This case demonstrates an unusual presentation of leprosy with bone marrow involvement. This case also cautions that leprosy may be misdiagnosed as a vasculitis, specifically PAN, as there is overlap in the clinical presentation of each disease.

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Small Vessel Vasculitis: A Rare Side Effect of TNF-alpha Inhibitors

Sarah Heaton, MD* and Michael Royer, MD†

*Walter Reed National Military Medical Center, Bethesda, MD; and †Joint Pathology Center, Silver Spring, MD.

We present a case of small vessel vasculitis occurring in 48 year old male being treated with adalimumab. The patient presented with a 4-day history of painful, crusted gray macules with surrounding erythema on the lower extremities. Additionally, there were small papules on the patient's arm that were reportedly identical to the early lesions from his legs. Biopsy from the leg revealed epidermal necrosis, and superficial and deep perivascular dermal inflammation consisting of lymphocytes, neutrophils and eosinophils. Within the reticular dermis, several vessels contained intimal inflammation with thrombi and surrounding erythrocyte extravasation. Biopsy from the arm revealed subtle changes suggestive of vasculitis. TNF-alpha inhibitors are commonly used to treat various autoimmune and inflammatory skin conditions and are generally well tolerated. However, various new onset inflammatory dermatitides have been reported as side effects, including vasculitis. Most cases of cutaneous vasculitis occurring in patients taking TNF-alpha inhibitors presents as palpable purpura with leukocytoclastic vasculitis on histology. Other types of small vessel vasculitis, as in our case, have rarely been reported.

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An Unusual Presentation of Pyemotes Ventricosus Dermatitis Presenting With Umbilicated Papules Mimicking Pox Virus Infection

Ania Henning, MD, Helen Torok, MD, and Joshua Weaver, MD

Department of Pathology & Laboratory Medicine, Summa Health System, Akron City, OH.

2nd Place Winner DP Trainee World Cup.

Pyemotes ventricosus mites are an uncommon cause of pruritic dermatitis seen most commonly in occupational exposure, prominently found in professionals such as farmers, landscapers, and factory workers who work with grains, wheat, dried beans or grasses. The clinical description of the rash has typically been described as papular, erythematous, with a central vesicular lesion. We describe a case of Pyemotes dermatitis with an atypical clinical presentation. A 30-year-old male presented with pruritic, umbilicated papules, which involved his right lateral trunk and upper thigh leading to the submitted clinical impression of molloscum contagiosum. A biopsy of the skin was taken and fragments of arthropod consistent with Pyemotes ventricosus were identified within umbilicated indentations of skin. The patient subsequently admitted to the onset of the rash immediately after carrying bales of straw while supporting each bale with his right side. The possibility of Pyemotes dermatitis mimicking a Pox virus-like eruption should be considered when encountering an unusual umbilicated papular eruption in the appropriate patient with occupational exposure.

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Pseudoepitheliomatous Hyperplasia Associated With Basal Cell Carcinomas: A Histopathologic Case Series

Kristofer Holte and Asok Biswas

Department of Pathology, Western General Hospital and the University of Edinburgh, Edinburgh, United Kingdom.

Background: Pseudoepitheliomatous hyperplasia (PEH) is characterised by epidermal hyperplasia with squamatisation of adnexal epithelium. An association between PEH and BCC is poorly recognised; to date only 2 case series have been published, totalling 71 patients.

Methods: We retrospectively reviewed the clinicopathologic features (>20 variables) of 31 cases of PEH associated with BCC.

Results: The BCC subtypes were: nodular 26, infiltrative 3, mixed nodular/infiltrative 2. The extent of PEH was greater than previous reports with 13 cases having >66% involvement by tumor area. 12 cases demonstrated ulceration involving either the BCC or PEH component. PEH in ulcerated tumours was less extensive, more mitotically active and more likely to be clinically misdiagnosed as squamous cell carcinoma (SCC). The presence of squamous eddies and keratin pearls was independent of ulceration.

Conclusions: The co-existence of BCC and PEH is more than a histologic curiosity. Failure to recognise this association could potentially lead to misdiagnosis of a SCC particularly in superficial biopsies.

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Primary Cutaneous Ewing Sarcoma

Andrea Jurgens, MD, Dawn Davis, MD, Jorge Torres-Mora, MD, and Andre M. Oliveira, MD, PhD

Mayo Clinic Dermatology, Rochester, MN.

Ewing sarcoma is a primitive neuroectodermal tumor that typically originates in the bone with rare instances of primary cutaneous examples reported. Clinically, these present as solitary, superficial lesions mimicking benign cutaneous lesions that are primarily diagnosed with aspiration cytology, immunohistochemistry, and molecular genetic analyses. We report a case of a 28 year old female with a rapidly growing subcutaneous nodule on the left forehead. Histological examination revealed sheets of small, round blue cells. Immunoperoxidase studies revealed that the tumor cells were positive for CD99, weakly positivity for ERG while negative for AE1/AE3, CD3, CD20, TDT, calretinin, WT-1, desmin, SOX10 and TLE-1. Although the fusion transcripts and EWSR1-ERG were not detected by PCR, molecular cytogenetic studies performed showed a rearrangement of the EWSR1 locus, confirming the diagnosis of primary cutaneous Ewing sarcoma and suggesting the possibility of an alternate EWSR1 fusion transcript .Due to their rare nature and clinical similarity to other cutaneous tumors, primary cutaneous Ewing sarcoma should be considered when dealing with superficial small round cell tumors.

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Pleomorphic Fibroma in Association With Mutation In RB1 Gene

Amandeep Kaur, Julio A. Diaz-Perez, Antoinette B. Thomas, and Thomas L. Cibull

Department of Pathology, NorthShore University HealthSystem, Evanston, IL.

A 26 year-old female with germline retinoblastoma susceptibility (RB1) gene mutation and history of bilateral retinoblastomas and undifferentiated soft tissue sarcoma presented with a 8 mm polypoid lesion on the left shoulder. Histopathologic examination demonstrated a moderately cellular dermal spindle cell neoplasm with scattered large pleomorphic appearing cells. Rare mitosis was identified. The histopathologic features were felt compatible with a pleomorphic fibroma (PF). RB1 is a tumor suppressor gene located at 13q14 which plays a crucial role in cell cycle progression. RB1 mutations occur in almost all familial and sporadic forms of retinoblastoma, and is mutated at variable frequencies in a variety of other human cancers including small cell lung carcinoma, bladder carcinoma and soft tissue and bone sarcomas. Recurrent loss of RB1 on chromosome 13q is seen in PF as well as in spindle cell/ pleomorphic lipoma. It is important to be aware of PF and its association to RB1 loss so as not to over diagnose PF as dermal sarcoma in patients with germline RB1 mutation.

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Trichoblastic Carcinosarcoma

Parisa Mansoori, MD, Angelica M. Selim, MD, and Rami N. Al-Rohil, MBBS

Division of Dermatopathology, Department of Pathology, Duke University Medical Center, Durham, NC.

Trichoblastic carcinosarcoma is a biphasic malignant neoplasm comprised of malignant follicular germinative cells surrounded by malignant mesenchymal cells. A 79-year-old male presented with a fast growing nodule on his scalp. Excisional biopsy reveals a relatively circumscribed tumor with 2 distinct components. The first is composed of basaloid cells with peripheral palisading, and a reticular growth pattern. The second component is in the form of epithelioid-to-spindle cells with abundant cytoplasm, round nuclei with vesicular chromatin, and prominent nucleoli. Both components showed cytologic atypia, pleomorphism and mitotic activity (10 per 10 high-power-fields). Immunohistochemically, the basaloid component is immunoreactive with p63 and AE1/3, while the epithelioid/spindle cell component is immunoreactive with Vimentin. Both components are negative for SOX10, SMA, Desmin, Synaptophysin, Chromogranin, and Adipophilin. Overall the findings are interpreted as trichoblastic carcinosarcoma. One year post excision there is no evidence of recurrence or metastasis. In conclusion, trichoblastic carcinosarcoma is a rare malignant adnexal neoplasm that is not highly reported in the literature probably due to under recognition. Larger case series are warranted for prediction of clinical behavior.

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Isolated Primary Vaginal Chloroma Without Any Hematological Malignancy: A Rare Entity With Unique Presentation

Mitul B. Modi, MD,* Jigisha Chadhari, MD,† Vipulkumar Prajapati, MD,‡ and Irappa Madabhavi, MD, DM§

*PGY2, Resident Physician, Department of Pathology, Pennsylvania hospital of University of Pennsylvania health system, Philadelphia, PA, USA & Department of Pathology, Gujarat Cancer & Research Institute, Ahmedabad, Gujarat, India (Presenter, not sponsored); †Resident Physician, Department of Pathology, Baroda Medical College, Vadodara, Gujarat, India; ‡Assistant Professor, Department of Anatomy, B. J. Medical College, Ahmedabad, Gujarat, India; and §Fellow, Department of Medical Oncology, Gujarat Cancer & Research Institute, Ahmedabad, Gujarat, India.

Introduction: Chloroma (also known as myeloid sarcoma, granulocytic sarcoma) is a rare EM (extra medullary) tumour of immature myeloid cells. Isolated chloroma, defined by the absence of a history of leukemia, myelodysplastic syndrome (MDS) or myeloproliferative neoplasm and a negative bone morrow biopsy, has been described in limited case reports. We are reporting this rare case of chloroma in a female geriatric patient without initial presentation of acute myeloid leukemia (AML).

Case Presentation: A 38-year-old female (gravid 3, Para 3) with performance status of 1 had a 20-day complaint of irregular vaginal bleeding. Biopsy of the anterior vaginal fornix revealed small cell endocrine tumor. Pelvic examination showed polypoid mass, 4.5 × 4 × 2 cm, arising from lateral fornix of right side of vagina without any clinical history of weight loss. Biopsy showed chloroma and IHC stains were positive for LCA, MPO and c-kit, while negative for cytokeratin, synaptophysin, chromogranin, CD20, CD99 and CD79a. CT scan of thorax, abdomen and pelvis showed 5 × 5 cm mass lesion lateral to cervix, supra diaphragmatic lymphadenopathy, infra diaphragmatic lymphadenopathy, mild hepatomegaly, and splenomegaly. The patient has been on follow-up periodically after partial response of chemotherapy, and local radiotherapy.

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Mucinous Eccrine Nevus

Sara Moradi,* Vladimir Daoud,† Andrew Ricci, Jr,* and Torsten Ehrig*

Departments of *Pathology and †Surgery, Hartford Hospital, Hartford, CT.

Mucinous eccrine nevus (MEN) is a very rare entity with only 13 reported cases. We report a 23-year old male patient who presented with umbilical discharge/hyperhidrosis for a few months. A urachal remnant cyst or other cyst was suspected clinically; however, CT imaging did not reveal associated intraabdominal changes. Histologic evaluation of the excision specimen demonstrated an increased number of eccrine coils with hyperplastic epithelium situated within an abundant mucinous stroma. There was an area of overlying granulation tissue. The current case adds to the variation of clinical and histologic appearances of this entity. Whereas most cases of MEN occur on the extremities, occasional examples on the trunk, mostly back and buttocks, have been described. The most distinguishing feature of the current lesion is the prominent hyperplasia of the epithelium of the sweat coils, not unlike ductal hyperplasia of breast. Hyperplasia, to this extent, has been reported in only one previous case. The current case is one of a growing number occurring in adult age which raises the question of the etiology of this lesion.

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A Case of Systemic Lupus Erythematosus With Lupus Profundus in a Patient Previously Diagnosed With Kikuchi-Fujimoto Disease: A Cautionary Tale

Anh K. Pham,* Stephanie A. Castillo,† Dorothea Barton,*,† William Rigby,*,† Marshall Guill,*,† Roberta Lucas,*,† and Robert E. LeBlanc*,†

*Dartmouth-Hitchcock Medical Center, Lebanon, NH; and †Dartmouth Geisel School of Medicine, Hanover, NH.

A 25-year-old East Asian woman presented with a 3.5 cm brown, sclerotic, and lipoatrophic plaque on her right preauricular area. Of note, she had been diagnosed with Kikuchi-Fujimoto disease (KFD) as a teenager following excision of a painful left cervical lymph node. A biopsy of her preauricular plaque revealed interface and periadnexal lymphoplasmacytic inflammation with epidermal atrophy, basement membrane thickening, dermal mucin and subcutaneous hyaline lipomembranous fat necrosis. Following a diagnosis of lupus profundus, work-up revealed an ANA titer of 1:320, complex oral aphthae, and a history of photosensitivity and arthralgia which permitted the diagnosis of systemic lupus erythematosus (SLE). She responded well to hydroxychloroquine and intermittent intramuscular triamcinolone. Lupus profundus is an uncommon manifestation of SLE. Retrospectively, we believe that the initial manifestation of her disease was lupus lymphadenitis, which can be histologically indistinguishable from KFD. Therefore, all patients diagnosed with KFD should have serologic testing and be followed closely.

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Clear Cell Cutaneous Anaplastic Large Cell Lymphoma

Shira Ronen, MD,* Matan Rothschild, MD,† Jose Ollague, MD,‡ and Saul Suster, MD*

Departments of *Pathology and †Internal Medicine, Medical College of Wisconsin, WI; and ‡Department of Dermatology, Social Security Hospital, Guayaquil, Ecuador.

A 38-year-old man presented with a large ulcer with indurated borders and serosanguinous base measuring 9 cm. A biopsy of the lesion showed a dense mononuclear cell infiltrate replacing the dermis and focally infiltrating the epidermis. The infiltrate consisted of nests and sheets of large pleomorphic tumor cells with large atypical nuclei displaying nuclear irregularities, and clear cytoplasm, which imparted them with a clear cell appearance. Scattered “hallmark” cells with reniform or convoluted nuclei were also presented mixed with a few small lymphocytes. Immunohistochemical stains showed positivity of the tumor cells for CD3, CD30 and CD45RO, and negative staining for cytokeratin AE1/AE3, S-100 protein, ALK-1, CD20, p63, PAX5, CD15, and CD43. This case is remarkable for its striking clear cell appearance, which may lead to confusion for other tumors in the differential diagnosis. Awareness of this unusual morphologic appearance in anaplastic large cell lymphoma is of important for proper diagnosis.

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Hypergranulotic Dyscornification: A Mysterious Epithelial Reaction Pattern

Simon F. Roy, MD,* Christine J. Ko, MD,† Gilbert W. Moeckel, MD, PhD,‡ and Jennifer M. McNiff, MD†

*Department of Pathology, University of Montréal, Montréal, Canada; †Department of Dermatology, Yale University School of Medicine, New Haven, CT; and ‡Department of Pathology, Yale University School of Medicine, New Haven, CT.

Hypergranulotic dyscornification (HD) is a rare histologic reaction pattern that may be observed in solitary benign keratoses. The term ‘dyscornification’ was elected by Reichel in his original case series of 8 cases in 1999 to evoke how the corneocytes appear histologically to have a faulty maturation process, leading to their dull, eosinophilic anucleate appearance, typically in mounds above the dermal papillae. We retrospectively reviewed all cases recorded as displaying hypergranulotic dyscornification in our files between January 1st 1990 and September 1st 2018, and found 29 cases, including a challenging case of a cyst demonstrating HD. We demonstrate histologic, immunohistochemical and ultrastructural findings further distinguishing this process from epidermolytic hyperkeratosis and possibly indicating a faulty excretion of keratin. We trust that this detailed analysis of the features of HD will improve its recognition and spur thoughts about its etiology, cutaneous pathophysiology and clinical significance. Acknowledgements: ASDP for its mentorship award to Simon Roy.

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Basal Cell Carcinoma With Matrical Differentiation—A Rare Histologic Variant

Barbara Saenz, Christine S. Ahn, and Omar P. Sangüeza

Department of Dermatopathology, Wake Forest School of Medicine, Winston-Salem, NC.

Basal cell carcinoma (BCC) is the most common skin cancer in the United States. The histological variant with matrical differentiation is very rare, with less than 50 cases reported in the literatüre. We describe a 71-year-old man with a history of multiple non-melanoma skin cancers who presented to the dermatology surgery clinic with 2 new nodules on the back. On physical examination, there were 2 large nodules located on the upper back adjacent to a scar from a previous surgical procedure. Histopathologic examination revealed a proliferation of basaloid lobules with peripheral palisading and clefting with features of matrical differentiation, including shadow cells and bright red trichohyaline granules. Immunohistochemical stains were performed with β-catenin and EMA, which supported the diagnosis. This histologic variant of BCC differentiates toward the hair matrix, and can be confused with pilomatricomas if the matrical component predominates. There are some reports that suggest that this variant is associated with more aggressive behavior, with few cases associated with lymph node metastases.

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Erythema Migrans and Interface Changes: More Than a Fortuitous Association

Burak Tekin,* Yali Song,* Damian Dicostanzo,† and Bonnie A. Lee*

*Ackerman Academy of Dermatopathology, NY; and †Dermpath Diagnostics, Port Chester, NY.

The cutaneous manifestation of early Lyme disease, erythema migrans (EM), has classically been associated with a perivascular lymphocytic infiltrate accompanied by eosinophils in the center of the lesion and plasma cells at the periphery. Other findings such as spongiosis or interface change have also been reported. Herein, we present 13 patients with serologically confirmed (n = 8) or clinically suspicious/unequivocal (n = 5) presentations of early Lyme disease and EM, demonstrating less-reported histologic features. The major inflammatory pattern was variable, ranging from sparse superficial perivascular to dense superficial and deep perivascular, interstitial, and periadnexal. Vacuolar alteration represented the most consistent finding, with 12 cases (92%) showing at least focal interface changes accompanied by lymphocytes with a distorted, somewhat “squiggly” shape along the DEJ. Although generally considered a dermatosis with minimal epidermal changes, EM may manifest with focal interface changes, as anecdotally described in the literature and noted in nearly all cases of this series. This report aims to reiterate the wide histopathological spectrum of early Lyme disease, emphasizing vacuolar changes and “squiggly” lymphocytes.

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Atypical Adult Onset Still’s Disease: Atypical Persistent Patches and Plaques as Presenting Symptom of a Paraneoplastic Syndrome?

B. Joel Tjarks, MD, Stacy A. Klepeiss, MD, and Jaqueline M. Junkins-Hopkins, MD

Geisinger Medical Center, Danville, PA.

Background: Adult onset Still’s disease (AOSD) is an uncommon systemic inflammatory condition which often manifests with a transient urticarial eruption, characterized by a mild neutrophilic infiltrate (neutrophilic urticarial dermatosis). An association with underlying malignancy has recently been described. Rarely, patients have persistent lesions with epidermal changes.

Case: An 82-year-old female presented with a 4-month history of pruritic erythematous and scaly patches and plaques on the upper back, associated with intermittent fevers, weakness, fatigue, and 40 lb weight loss. Biopsy revealed individual and clustered necrotic keratinocytes within the epidermis overlying a dermal neutrophil-rich inflammatory infiltrate. A diagnosis of AOSD was made. Subsequent work-up revealed a markedly elevated serum ferritin of 13,208 ng/mL (ref: 13-150 ng/mL), neutrophil predominant leukocytosis, and negative ANA, further fulfilling criteria for AOSD. Imaging studies revealed bulky mediastinal lymphadenopathy, compatible with malignancy.

Conclusions: This case highlights the rare presentation of persistent lesions in AOSD, and acts as a reminder to clinicians and pathologists that this lesion may be the presenting sign of paraneoplastic syndrome.

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