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DERMATOLOGY DIGEST

A derm photo quiz

LT Kenneth J. Meehan, PA-C, MPAS,
DEPARTMENT EDITOR

Two ulcerative, necrotic lesions—and a history of HIV infection

Shylashree Chikkamuniyappa, MD; Jason Loos, MD

Dr. Chikkamuniyappa and Dr. Loos are with the Department of Pathology, University of Texas Health Science Center at San Antonio. The authors have indicated no relationships to disclose relating to the content of this article. Lieutenant Meehan practices dermatology at Tripler Army Medical Center, Honolulu, Hawaii.

A 35-year-old HIV-infected man presented to the dermatology clinic with two ulcerative and necrotic skin lesions on the middle back that he had noticed 4 days earlier (see Figure 1). The left lesion measured 434 cm and the right measured 2 x 2 cm. No other skin lesions were present, and the patient was otherwise asymptomatic.

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The physical examination and medical and surgical histories were unremarkable. The patient's current CD4 count was 15 cells/mm³; the viral load was 390,000 copies/mL. A punch biopsy of a lesion was obtained. The differential diagnosis included numerous opportunistic infections, various dermatoses, drug reactions, and cutaneous lymphomas.

What is the diagnosis?

Dermatophytosis
Drug reaction
Anaplastic large cell lymphoma
Eosinophilic folliculitis

Discussion

The diagnosis is primary cutaneous anaplastic large cell lymphoma (ALCL), T-cell type. ALCL is a large, confusing, and heterogeneous group of lesions that are rarely found in HIV-infected patients. The patient's immunosuppressed state likely contributed.

The diagnosis is made using immunohistochemical staining. The defining feature is a proliferation of predominantly large lymphoid cells with strong expression of the cytokine receptor CD30 and a characteristic growth pattern. The cells have been found in the lymph nodes, skin, lungs, bones, liver, and nasal cavity.

Punch biopsy in this patient revealed a diffuse infiltrate in the dermis that extended to the subcutis (see Figure 2). The neoplastic cells are large and atypical and have abundant cytoplasm and pleomorphic, horseshoe-shaped nuclei. The neoplastic cell nucleus is eccentric and kidney shaped and has prominent eosinophilic nucleoli.

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A battery of immunostains found CD3 (pan T-cell marker), CD 45RO (T-cell marker), CD30 (lymphocyte activation marker), and TIA-1 (cytotoxic granule-associated proteins). Anaplastic lymphoma kinase (ALK-1), epithelial membrane antigen (EMA), CD20 (pan B-cell marker), cytokeratin, S-100 (a neural protein), and CD56 (natural killer-cell marker) were not found.

There is an important distinction between primary systemic ALCL, which is typically seen in children and young adults, and cutaneous ALCL, which is more typically seen in adults and the elderly. ALK, EMA, and translocation are typically present in primary systemic ALCL, which usually requires systemic chemotherapy and is associated with a worse prognosis than cutaneous ALCL. Cutaneous ALCL has not shown translocation and is typically ALK and EMA negative.

Conservative treatment usually includes complete excision and localized radiotherapy; treatment for this patient's cutaneous ALCL consisted of localized radiotherapy. He was carefully examined, and his disease was staged with a chest radiograph, CT of the abdomen, and bone marrow biopsy.