Multiple Clustered Dermatofibromas Associated With Pulmonary Arterial Hypertension.

BACKGROUND: Dermatofibromas (DFs) are common, benign, fibrohistiocytic tumors of the skin. Clinically, if they present in a rapid succession, they are termed multiple eruptive DFs. When they arise in a localized distribution, they are termed multiple clustered dermatofibromas (MCDFs). These DF variants are rare, and the subtype of multiple eruptive DFs has been associated with autoimmune diseases, hypertriglyceridemia, pregnancy, HIV, and primary pulmonary hypertension. Conversely, there is a paucity of published clinical associations with MCDF.

METHODS: A 23-year-old white woman with pulmonary arterial hypertension presented with a 7-year history of multiple, asymptomatic, firm, brown, and violaceous papules clustered on the right hip.

RESULTS: Histopathologic examination of the biopsies demonstrated acanthotic epidermis, proliferation of cytologically bland fibrohistiocytic spindle cells in a fascicular pattern in the dermis, and collagen trapping on the periphery. Immunohistochemistry was focally positive for factor XIIIa and negative for CD34, thus supporting the clinical diagnosis of MCDF.

CONCLUSIONS: A case of MCDF associated with pulmonary arterial hypertension is presented. To date, there have been greater than 25 reported cases of MCDF, but only one other publication has described an associated systemic comorbidity.