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DEPARTMENTS: Teledermatology Viewpoint

Pruritic Erythroderma and Disseminated Ulcers

Stumpf, Matheo Augusto Morandi

Author Information
Journal of the Dermatology Nurses’ Association: 3/4 2022 - Volume 14 - Issue 2 - p 93-95
doi: 10.1097/JDN.0000000000000676
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Abstract

HISTORY

A 58-year-old man presented to the primary care clinic with a 5-year history of disseminated ulcers and erythroderma. The patient stated his condition began with itching and generalized redness of the skin; ulcers appeared 3 months after the cutaneous eruption. He denied drug use, comorbidities, exacerbating factors, and other systemic symptoms. The systemic examination was unremarkable. He used oral antihistamines previously without any improvement.

IMAGE QUALITY ASSESSMENT

Fully satisfactory.

TELEDERMATOLOGY IMAGING READER REPORT1

There are three images provided. Erythroderma can be noticed (Figure 1), as well as some ulcers on the face and upper limbs (Figures 2 and 3, respectively).

F1
FIGURE 1.:
Generalized erythroderma.
F2
FIGURE 2.:
Disseminated ulcers on the face.
F3
FIGURE 3.:
Disseminated ulcers on the upper right limb.

INTERPRETATION OF IMAGES

Erythroderma can be found in pharmacodermia, leprosy, psoriasis, and malignancies. However, a differential workup among cutaneous lymphoma, Sézary syndrome, mycosis fungoides, and leukemia cutis was done because of the presence of ulcers and a long history of 5 years of evolution.

RECOMMENDATION

Given the concerning appearance of the lesions, the next step in management was obtaining an appointment with dermatology for a punch skin biopsy and an immunohistochemical panel for malignancy workup. It is important to remember that punch skin biopsy should be performed in multiple sites to increase sensitivity. In this patient, three samples were collected: two made in the abdominal region with only erythroderma and one in the border of a left arm's ulcer.

The biopsy confirmed a primary cutaneous anaplastic large cell lymphoma (CALCL; a non-Hodgkin T-cell lymphoma). The immunohistochemical panel was positive for CD30 and CD3 markers and negative for anaplastic lymphoma kinase. A whole-body tomography was done to evaluate for metastasis and was negative. Blood tests such as blood count, renal function, human immunodeficiency virus, hepatitis, and syphilis antibodies were all normal. The patient received methotrexate up to 20 mg per week because no extracutaneous disease was observed and achieved clinical remission in 5 months.

RECOMMENDED FOLLOW-UP

Type of Visit

The patient maintained follow-up with both hematology and dermatology every 6 months to observe for systemic recurrence and cutaneous evolution, respectively.

CLINICAL PEARL

Primary cutaneous CD30 lymphoproliferative disorders are the second most common group of cutaneous T-cell lymphoma, accounting for 25% of all of them. This group includes primary CALCL and lymphomatoid papulosis. Because of the overlapping histologic and phenotypic features, clinical presentation and clinical course are used as decisive criteria to differentiate between these two entities (Willemze et al., 2019).

CALCL is an indolent lymphoma, with a 5-year survival rate of 96%, and more frequent in men with an overall peak in the sixth decade of life (Olivier et al., 2017). It presents as solitary, grouped, or, uncommonly, multifocal nodules and tumor; ulceration may be present or not. Lesions usually occur on the trunk, face, extremities, and buttocks and are usually asymptomatic. Cutaneous relapses are common, but extracutaneous dissemination occurs in only 10%–15% of patients (Oliveira et al., 2013; Shehan et al., 2004).

Histologically, these lesions show a diffuse infiltrate composed of large-sized T lymphocytes (CD4 and CD30) with characteristic morphology of anaplastic cells with round, oval, or irregular nuclei (Oliveira et al., 2013; Willemze et al., 2019). Immunohistochemical expression of the anaplastic lymphoma kinase gene is typically absent in primary CALCL but often present in primary systemic anaplastic large cell lymphoma (Oliveira et al., 2013; Shehan et al., 2004).

There are several clinical guidelines for cutaneous lymphoma management. The National Comprehensive Cancer Network and European Society for Medical Oncology recommend for staging a chest, abdomen, and pelvis computed tomography. Bone marrow examination is usually not indicated, and an excisional biopsy should be warranted in lymph node > 1.5 cm suggestive of infiltration (fixed, irregular, clustered). Blood tests include complete blood count, liver function, lactate dehydrogenase, and other comprehensive chemistries (Mehta-Shah et al., 2020; Willemze et al., 2018).

Treatment depends on clinical features. Radiation therapy or excision can be used in solitary or localized diseases. Systemic chemotherapy (generally doxorubicin-based combinations) is considered first-line when there is systemic/extracutaneous involvement. Methotrexate or systemic retinoid may be considered when only the skin is committed in multifocal segments. After remission, clinical follow-up is warranted as cutaneous recurrences are frequent (Baik et al., 2019; Shehan et al., 2004).

REFERENCES

Baik B. S., Lee W. S., Ji S. Y., Park K. S., Yang W. S., Kim S. Y. (2019). Treatment of primary cutaneous anaplastic large cell lymphoma. Archives of Craniofacial Surgery, 20(3), 207–211.
Mehta-Shah N., Horwitz S. M., Ansell S., Ai W. Z., Barnes J., Barta S. K., Clemens M. W., Dogan A., Fisher K., Goodman A. M., Goyal G., Guitart J., Halwani A., Haverkos B. M., Hoppe R. T., Jacobsen E., Jagadeesh D., Lunning M. A., Mehta A., Kim Y. H. (2020). NCCN guidelines insights: Primary cutaneous lymphomas, Version 2.2020. Journal of the National Comprehensive Cancer Network, 18(5), 522–536.
Oliveira L. S., Nobrega M. P., Monteiro M. G., Almeida W. L. (2013). Primary cutaneous anaplastic large-cell lymphoma—Case report. Anais Brasilerios de Dermatologia, 88(6, Suppl. 1), 132–135.
Olivier S., Dachelet C., Theate I., Tromme I., Baeck M. (2017). CD30+ cutaneous anaplastic large-cell lymphoma of the upper eyelid: A case report. Case Reports in Dermatology, 9(3), 206–210.
Shehan J. M., Kalaaji A. N., Markovic S. N., Ahmed I. (2004). Management of multifocal primary cutaneous CD30 anaplastic large cell lymphoma. Journal of the American Academy of Dermatology, 51(1), 103–110.
Willemze R., Cerroni L., Kempf W., Berti E., Facchetti F., Swerdlow S. H., Jaffe E. S. (2019). The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas. Blood, 133(16), 1703–1714.
Willemze R., Hodak E., Zinzani P. L., Specht L., Ladetto M.; ESMO Guidelines Committee (2018). Primary cutaneous lymphomas: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Annals of Oncology, 29(Suppl. 4), iv30–iv40.

1The standardized teledermatology reader report format is available for authors on the journal's Web site (www.jdnaonline.com) and on the submissions website online at http://journals.lww.com/jdnaonline/Documents/Teledermatology%20Column%20Template.pdf.

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