To the Editor:
Lymphomatoid papulosis (LyP) type D is a newly proposed LyP subtype.1,2 The diagnosis of this entity can be challenging to the dermatopathologists because it is histologically indistinguishable from primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma and requires accurate clinicopathologic correlation.3 Most LyP type D cases are reported in young patients (median age 29 years) and express CD30.1,4 Here, we report a rare case of LyP type D affecting an elderly man where the tumor cells are negative for CD30.
A 73-year-old white man, with unremarkable clinical history, presented with two 0.5-cm itchy papules in the abdomen and right leg, which was noticed a month ago and became ulcerated (clinical pictures never taken). He has a history of similar papules in different parts of his body since 5 years ago that either resolved spontaneously (within ∼3 weeks) or with topical betamethasone. Previous skin lesions were not biopsied. Skin examination showed 5 erythematous macules (spontaneously healing lesions) and 2 healed hyperpigmented scars (biopsy sites). Same topical treatment was recommended if needed and he has been followed for 6 months with no new lesions.
The rest of physical examination and laboratory findings (immunoglobulins, β2-microglobulin, T4, complete blood cell count, electrolytes, liver function test, HIV1/2, human T-lymphotropic virus-1, PRP, cytomegalovirus, Hepatits B virus, Hepatits C virus) were unremarkable. Flow cytometry of the peripheral blood failed to detect aberrant T cells. Clinical staging showed no evidence of systemic lymphoma.
Two skin biopsies (abdomen and right leg) showed similar histological features. The epidermis was ulcerated with striking epidermotropic lymphoid infiltrate. The lymphoid cells were atypical and medium to large cells (large cells representing >25% of all cells) (Figs. 1A, B) admixed with numerous eosinophils (Fig. 1C), neutrophils, and histiocytes. Although these shave biopsies included up to superficial reticular dermis, angiocentriticy, vascular damage, or granulomas were not identified. Immunophenotyping revealed that the tumor cells were positive for CD3 (Fig. 1D), CD2 (Fig. 1E), CD8 (Fig. 1F), TCR βF1 (Fig. 1G), CD45R0 (Fig. 1H), and TIA-1 (Fig. 1I) and negative for CD4 (Fig. 1J), CD30 (Fig. 1K), CD56, CD7, and CD20. There are numerous CD4+ cells in the dermis. These cells may represent a combination of normal reactive CD4+ T cells and dermal histiocytes/dermal dendritic cells expressing CD4. However, we cannot exclude the possibility of CD4+ LyP cells also. In the context of the epidermotropic component was clearly CD8+ and CD4−, we favor that the neoplasm has a CD8+ phenotype rather than CD8+/CD4+ phenotype.
Combining the morphologic and clinical findings (recurrent, self-healing eruption of papules with a waxing and waning course), a diagnosis of CD30− LyP type D was made. However, the histological and immunophenotypic findings raised 2 main following differential diagnoses: primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma and CD8+ mycosis fungoides with large cell transformation. The former is characterized by a rapid onset of patches/papules/tumors in elderly adults, frequently exhibiting necrosis and ulcers. The clinical course is aggressive with a median survival of 32 months and rapid extracutaneous dissemination.3 Histologically, however, this lymphoma is nearly identical to our case including extensive necrosis, massive epidermotropism, positivity for CD8 and cytotoxic markers, and negativity for CD30.3 In mycosis fungoides with large cell transformation, the clinical course and striking epidermotropism seen in our case would be unusual.5
This case demonstrates the importance of considering LyP type D in the differential diagnosis even in elderly adults with massively epidermotropic CD8+ cytotoxic T cells and CD30 negativity. An accurate clinicopathologic correlation is crucial to avoid overdiagnosis that might lead to overtreatment for this low-grade cutaneous lymphoma.
1. Saggini A, Gulia A, Argenyi Z, et al.. A variant of lymphomatoid papulosis simulating primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma. Description of 9 cases. Am J Surg Pathol. 2010;34:1168–1175.
2. Willemze R, Jaffe ES, Burg G, et al.. WHO-EORTC classification for cutaneous lymphomas. Blood. 2005;105:3768–3785.
3. Gormley RH, Hess SD, Anand D, et al.. Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma. J Am Acad Dermatol. 2010;62:300–307.
4. Cardoso J, Duhra P, Thway Y, et al.. Lymphomatoid papulosis type D: a newly described variant easily confused with cutaneous aggressive CD8-positive cytotoxic T-cell lymphoma. Am J Dermatopathol. 2012;34:762–765.
5. Edinger JT, Clark BZ, Pucevich BE, et al.. CD30 expression and proliferative fraction in nontransformed mycosis fungoides. Am J Surg Pathol. 2009;33:1860–1868.