Abstract
Lymphomatoid papulosis (Lyp) is a rare, chronic skin condition falling in the category of primary cutaneous CD30+ lymphoproliferative disorders. It clinically presents with relapsing and remitting papules, plaques, and nodules that may heal with atrophic scaring. A recently identified variant of LyP, known as LyP Type E, is angioinvasive on histology and clinically characterized by papules that can rapidly ulcerate and form into eschar-like lesions. Immunohistochemistry will show small to medium sized infiltrates of CD30+, CD8+, CD4+ lymphocytes invading the dermal and subcutaneous vessels with resulting erythrocyte extravasation and occasional fibrin deposition in the walls of nearby vessels.
We present a case of a 41-year-old African American female with a 4-year history of a relapsing and remitting hyperpigmented rash to her extremities without pruritis, burning, or pain. A recent hyperpigmented patch on her right hip developed into a plaque with subsequent erosion. Biopsy revealed a CD4+/CD30+/TIA-1+/Granzyme+ cutaneous atypical lymphocytic infiltrate characterized by moderate cytologic atypia with lymphocyte enlargement and scattered mitotic figures. In additionl the infiltrate demonstrated angiocentric and angiodestructive features in association with a necrotizing panniculitis-like T-cell infiltrate. Although mycosis fungoides with CD30+ transformation cannot be completely ruled out, the pattern was most compatible with LyP type E, given CD30 positivity as well as angiodestructive features. Although there have been previous reports of LyP type E, this case was unique in that it presented with necrotizing panniculitis and cytotoxic marker expression.