Atypical cutaneous lymphoproliferative disorder in patients with HIV infection

Background An inflammatory pruritic eruption which is characterized histolo gically by CD8+, atypical lymphocytes has been described in human immunodef iciency virus (HIV)-infected patients. This disorder has been described as "pseudo-Sezary" or a "cutaneous T cell lymphoma (CTCL)-simulant", however, as these patients do not resemble CTCL clinically, and the relationship bet ween this entity and CTCL is unclear, a more descriptive term is "atypical cutaneous lymphoproliferative disorder" (ACLD). The purpose of this study i s to categorize the clinical, histologic, and immunophenotypic features of 16 HIV-infected patients with this skin disorder seen at the New York Depar tment of Veterans Affairs Medical Center. Methods A retrospective chart review was conducted on 16 HIV-infected patie nts with a histologic diagnosis of an atypical cutaneous lymphoproliferativ e infiltrate on skin biopsy. Skin biopsies were reviewed, and histologic fe atures noted. Immunophenotyping was performed on 14 out of 16 samples; elec tron microscopy was performed on samples from five patients. Clinical manif estations, disease course, medication history, and response to treatment we re noted. Results The patients presented with a pruritic, persistent, generalized, pa pular eruption. Pigment changes, including hyperpigmentation and hypopigmen tation were common. Histologically, lesional biopsies were characterized by a superficial and deep polymorphous infiltrate with atypical lymphocytes w hich were CD8+ predominant, Ki-1 negative, and occasionally CD7 antigen dep leted. Sezary-like cells were present in the infiltrate in four out of five patients by electron microscopy. None of the patients has systemic manifes tations of lymphoma; however, one of the 16 patients eventually developed f rank CTCL. Conclusions HIV-infected patients can present with a pruritic, widespread d isorder, often with pigment changes characterized by an atypical cutaneous lymphocytic infiltrate. This clinicopathologic disorder is a rare, reactive inflammatory condition which generally occurs in late stage HIV infection and rarely progresses to frank lymphoma.