TITLE:
Atypical Cutaneous Lymphoproliferative Disorder: A Fatal Mimic of Cutaneous T-Cell Lymphoma in a Patient with HIV Infection
AUTHORS:
Veronica Nguyen, Russell Dorer, David M. Aboulafia
KEYWORDS:
Atypical Cutaneous Lymphoproliferative Disorder; Mimic Fatal; Cutaneous T-Cell Lymphoma; HIV Infection
JOURNAL NAME:
World Journal of AIDS,
Vol.3 No.1,
March
28,
2013
ABSTRACT:
Atypical cutaneous lymphoproliferative
disorder (ACLD) is a rare condition that has been associated with HIV infection.
Patients with ACLD present with diffuse, erythematous and pruritic skin lesions
accompanied by generalized lymphadenopathy. The clinical characteristics of
ACLD overlap most notably with several other conditions including Mycosis
Fungoides/Sézary Syndrome (MF/SS), a cutaneous lymphoma of T-cell lineage.
Unlike Mycosis Fungoides, the noxious infiltrates of ACLD are not monoclonal
but polyclonal and consist of cytotoxic CD8+ T-cells instead of CD4+ T-cells or
B-cells. Highly active antiretroviral therapy (HAART) has been reported to
improve ACLD. We describe the case of a Caucasian man with longstanding HIV
infection who presented with severe erythroderma. Skin and lymph node biopsies
showed polyclonal CD8+ T-cell infiltrates. Gene rearrangement studies did not
reveal an obvious clonal disorder. Hallmark peripheral blood findings
consisting of a severe depletion of CD4+ T-lymphocytes and markedly elevated
CD8+ cells provided an important diagnostic clue. Despite the purported
benefits of HAART in ameliorating this disorder, erythroderma and extreme
pruritus improved only after the patient began taking mycophenolate mofetil and
hydroxyurea. Unfortunately, he succumbed to complications of
methicillin-resistant Staphylococcus aureus septicemia. We alert readers to
this rare HIV-associated condition which may mimic other benign and malignant
skin conditions and briefly discuss diagnostic and therapeutic options.