Multinucleate cell angiohistiocytoma with hypertrophic nerves

Abstract

Multinucleate cell angiohistiocytoma (MCAH) represents a rare benign skin lesion characterized by multiple papules that are usually found on the distal extremities or face of middle-aged women. We report on a 60-year-old male with a history of monoclonal gammopathy and severe rheumatoid arthritis who had several asymptomatic red-to-livid papules grouped on the right side of his trunk. The lesions had been present for a few years and were gradually enlarging. Biopsies from three lesions showed a spectrum of changes consisting of a proliferation of small venules together with thickened collagen bundles and increased numbers of interstitial cells including bizarre, multinucleated giant cells. Immunohistochemically, the multinucleated cells were positive for vimentin and lysozyme while the interstitial cells expressed CD68, factor XIIIa and lysozyme. Interestingly, strikingly enlarged dermal nerves were seen in all the three biopsies and two of the biopsies showed an accompanying infiltrate of lymphocytes and plasma cells.

Generalized multinucleate cell angiohistiocytoma: case report and literature review

“…CD68 expression in MCs was found to be variably positive in some cases, and negative in others, like ours. The multinucleated cells in MCAH have been suggested to be of fibroblastic origin, due to lack of expression of monocyte/macrophage markers . However, the occasional presence of pinocytotic activity of numerous lysosomes is suggestive of histiocytic origin.…”

“…18 In the immunohistochemical staining techniques, the endothelial cells stained positively for CD34, CD31, and factor VIII-related antigen. CD68 expression in MCs was found to be variably positive in some cases, 19 and negative in others, like ours. The multinucleated cells in MCAH have been suggested to be of fibroblastic origin, due to lack of expression of monocyte/macrophage markers.…”

“…However, the occasional presence of pinocytotic activity of numerous lysosomes is suggestive of histiocytic origin. Heterogeneity in CD68 expression has been attributed to different maturation or degenerative stages of the MCs …”

Everything you wanted to know about dermatofibroma but were afraid to ask

“…How did Böer‐Auer et al spark a neuron? Their recent report of multinucleate cell angiohistiocytoma with associated hyperneury (neural hyperplasia, nerve hypertrophy) rekindled two independent mental fires. First, does multinucleate cell angiohistiocytoma truly exist as an entity sui generis , or is it merely a fancy dermatofibroma?…”

Facial multinucleate cell angiohistiocytoma: long-term remission with 585 nm pulsed dye laser

“…Multinucleate cell angiohistiocytoma (MCA) is a rare, benign, but progressive skin condition, mostly affecting middle‐aged to elderly women, and was first described in 1985 by Smith and Wilson‐Jones . Its sites of predilection include the face, legs and dorsal hands.…”