Localized chronic fibrosing vasculitis of the skin: an inflammatory reaction that occurs in settings other than erythema elevatum diutinum and granuloma faciale.

Abstract

Erythema elevatum diutinum (EED) and granuloma faciale (GF) are chronic, localized forms of cutaneous leukocytoclastic vasculitis that result in patterned (storiform or concentric) fibrosis. EED often occurs in systemically ill patients as bilaterally symmetrical plaques, papules, or nodules, often over the dorsa of joints. GF occurs as one or a few plaques on the face. Eosinophils and plasma cells are prominent in GF, whereas neutrophils are plentiful in EED. Rarely, extrafacial lesions accompany facial ones in GF, and there are a few reports of upper respiratory tract masses with GF-like histologic features. We report on eight patients with solitary cutaneous lesions with histologic features similar to those of EED or GF, but whose clinical picture was not that of either disease. One, whose histology resembled GF, had a large multinodular dermal and subcutaneous mass that persisted despite attempted resection. Unusual histologic findings in other cases included storiform fibrosis with dense infiltrates of plasma cells, branching nerve fascicles admixed with EED-like changes, and EED-like areas adjacent to zones mimicking a sclerotic fibroma. Chronic fibrosing venulitis can be seen outside the stereotypic settings of GF and EED and is an inflammatory reaction pattern that does not signify a specific diagnosis. Because of transitions between EED or GF-like areas and those of patterned sclerosis with plasma cell-rich infiltrates, we believe that some "inflammatory pseudotumors" of the skin, and perhaps of other sites could be the result of localized vasculitis.