Asymptomatic Macules and An Ulcerated Shoulder in an Elderly Woman

Antonio Giovanni Richetta, MD, PhD; Simona Giancristoforo, MD; Carlo Mattozzi, MD; Sara D'Epiro, MD; Stefano Calvieri, MD


December 09, 2016

Another condition to consider in the differential diagnosis of primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma is a cytotoxic variant of lymphomatoid papulosis (a cutaneous CD30+ lymphoproliferative disorder).[5] Investigators proposed the term lymphomatoid papulosis type D for this lymphomatoid papulosis variant that has histopathologic features which may not only be difficult to distinguish from primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma, but may also impact the clinical management and prognosis.[5]

The histologic appearance of primary cutaneous aggressive epidermotropic CD81 T-cell lymphoma lesions varies. Epidermotropism is seen in all stages of disease, with tumor cells predominately confined to the epidermis, most extensively involving the basal layer.[1,6] This pronounced epidermotropism may follow a linear distribution or a pagetoid pattern throughout the epidermis. In all cases of aggressive epidermotropic CD8 T-cell lymphoma, the epidermis itself may be acanthotic or atrophic, with necrotic keratinocytes, ulceration, variable spongiosis, and occasional blister formation.[2,7]

Single necrotic keratinocytes may be seen at the dermoepidermal junction or, in some cases, the basal necrotic keratinocytes may be confluent at the basal layer, giving rise to a dermoepidermal cleft. Invasion and destruction of adnexal skin structures, including pilosebaceous units, eccrine ducts, and hair follicles, is frequently seen, as well as perivascular distribution of neoplastic cells with or without signs of angioinvasion.[1,2,7] Nerves are generally spared whereas intercellular edema and necrosis are commonly present in the central part of lesions. The actual tumor cells are medium-to-large pleomorphic T cells or blastic cells, and they are accompanied by a variable number of reactive macrophages and dendritic cells, rare eosinophils, and a few plasma cells.[1,7] The immunophenotype of tumor cells is characterized by βF1+, CD3+, CD81, CD4-, granzyme B+, perforin+, TIA-1+, CD45RA+, and CD45RO-.[1,6,7,10] CD56 expression is variable, as is that of CD2, CD7, and CD5.[2,7,8,9,11]

In this clinical case, the patient had pathologic findings of intraepidermal infiltrates of atypical lymphoid cells. Moreover, the immunohistologic phenotype of these cells led to the diagnosis of cutaneous lymphoma. Interestingly, the patient's primary disease manifestation was rapidly spreading maculopapular eruptions on her leg and arms. Extracutaneous dissemination is common, with metastasis to unusual sites (eg, lung, testes, CNS, oral cavity); however, lymph nodes are often spared. Because of the aggressive clinical course of primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma, patients should be evaluated with a thorough physical examination, peripheral blood evaluation (complete blood cell count, liver function tests, lactate dehydrogenase, flow cytometry), and staging scans.[4]


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