Atypical cutaneous lymphoproliferative disorder in patients with HIV infection

Background: An inflammatory pruritic eruption which is characterized histologically by CD8+, atypical lymphocytes has been described in human immunodeficiency 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 between this entity and CTCL is unclear, a more descriptive term is 'atypical cutaneous lymphoproliferative disorder' (ACLD). The purpose of this study is to categorize the clinical, histologic, and immunophenotypic features of 16 HIV-infected patients with this skin disorder seen at the New York Department of Veterans Affairs Medical Center. Methods: A retrospective chart review was conducted on 16 HIV-infected patients with a histologic diagnosis of an atypical cutaneous lymphoproliferative infiltrate on skin biopsy. Skin biopsies were reviewed, and histologic features noted. Immunophenotyping was performed on 14 out of 16 samples; electron microscopy was performed on samples from five patients. Clinical manifestations, disease course, medication history, and response to treatment were noted. Results: The patients presented with a pruritic, persistent, generalized, papular eruption. Pigment changes, including hyperpigmentation and hypopigmentation were common. Histologically, lesional biopsies were characterized by a superficial and deep polymorphous infiltrate with atypical lymphocytes which were CD8+ predominant, Ki-1 negative, and occasionally CD7 antigen depleted. Sezary-like cells were present in the infiltrate in four out of five patients by electron microscopy. None of the patients has systemic manifestations of lymphoma; however, one of the 16 patients eventually developed frank CTCL. Conclusions: HIV-infected patients can present with a pruritic, widespread disorder, 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.