A 26-year-old woman developed progressive pruritic erythema and patches that became persistent and worsened over the years. Recurrent Staphylococcus aureus infections appeared due to disruption of the skin barrier, and antibiotics were administered. Physical examination revealed indurated erythema and scaly patches with diffuse lichenification, plaques, and tumors affecting 40% of her body surface (Figure 1). Some skin lesions had an infiltrating appearance, with irregular borders and atrophy in a “bathing trunk” distribution with variation in size and shape. An extensive workup was performed, with active infection ruled out. The blood immunophenotype showed 632 total lymphocytes with 25% CD4+/CD26-. Skin biopsy (Figure 2) immunohistochemistry showed CD3+, CD4+, CD5+, CD2-, CD7-, CD8-, and CD30-. A positron emission tomography/computed tomography scan found hypermetabolism of regional axillary and inguinal lymph nodes without extra nodal involvement. The axillary node biopsy found a mature T-cell phenotype (CD3+, CD4+, CD5+, CD7-, CD8-, and CD30-).
Physical skin examination
Lateral and frontal view of disseminated erythema and scaly plaques with an infiltrating appearance, atrophy, and variation in size and shape (A, B). Scaly plaque with irregular borders (C).
Lateral and frontal view of disseminated erythema and scaly plaques with an infiltrating appearance, atrophy, and variation in size and shape (A, B). Scaly plaque with irregular borders (C).
Skin biopsy
Photomicrograph of skin excision showing mild spongiotic and psoriasiform pattern with hematoxylin and eosin stain, 2x (A). Epidermotropism by atypical lymphocytes of cerebriform nuclei and Pautrier-type microabscesses with hematoxylin and eosin stain, 40x (B).
Photomicrograph of skin excision showing mild spongiotic and psoriasiform pattern with hematoxylin and eosin stain, 2x (A). Epidermotropism by atypical lymphocytes of cerebriform nuclei and Pautrier-type microabscesses with hematoxylin and eosin stain, 40x (B).
What is the diagnosis?
Primary cutaneous anaplastic large cell lymphoma (PC-ALCL)
Sézary syndrome
Mycosis fungoides
Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS)
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Disclosure Statement
The authors indicated no relevant conflicts of interest.
