Atypical multicentric reticulohistiocytosis with paraproteinemia

Abstract

Two women had multiple subcutaneous nodules that showed features of multicentric reticulohistiocytosis (MR). Neither had joint symptoms. Both had a raised erythrocyte sedimentation rate, an immunoglobulin G paraproteinemia, and raised levels of nonhepatic serum alkaline phosphatase. The skin lesions have been followed up, using light and electron microscopy, immunoperoxidase, and histochemical methods. The material in the giant cells stained positively for gamma heavy chain determinants: the light chain type in each case was that of the paraprotein. An attempt to reproduce the skin lesions in one patient by intradermal injection of her paraprotein failed.

Multicentric Reticulohistiocytosis-Mimicking Dermatomyositis

“…A cephalocaudal distribution of the nodules has been described [2] and sometimes a diffuse, irritable erythema or severe pruritus precedes the appearance of the more characteristic nodules [4,5,6,7]. Other atypical findings in MRH are hypochromic plaques [8], telangiectasias [9, 10], deep nodules [11,12,13,14], erythroderma[15] and neurofibroma-like nodules [16]. Palpebral xanthelasma-like lesions were observed in 17% of cases in a review including 96 patients [3].…”