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.
Friedler
S
S
Ronald O. Perelman Department of Dermatology, New York University, Department of Veterans Affairs Medical Center, New York, USA.
Parisi
M T
MT
Waldo
E
E
Wieczorek
R
R
Sidhu
G
G
Rico
M J
MJ
D000163:Acquired Immunodeficiency Syndrome / Q000150:complications / Q000821:virology; D000328:Adult; D000368:Aged; D015703:Antigens, CD / Q000032:analysis; D003937:Diagnosis, Differential; D005500:Follow-Up Studies; D015658:HIV Infections / Q000150:complications*; D006801:Humans; D007150:Immunohistochemistry; D008232:Lymphoproliferative Disorders / Q000150:complications / Q000378:metabolism / Q000473:pathology*; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D012867:Skin / Q000737:chemistry / Q000473:pathology / Q000648:ultrastructure; D012871:Skin Diseases / Q000150:complications / Q000378:metabolism / Q000473:pathology*; D013601:T-Lymphocytes / Q000737:chemistry / Q000473:pathology / Q000648:ultrastructure
D015703:Antigens, CD