Rongioletti Franco, Cozzani Emanuele, Parodi Aurora
Section of Dermatology, DISEM, University of Genoa, Italy.
J Cutan Pathol. 2010 Oct;37(10):1084-7. doi: 10.1111/j.1600-0560.2009.01386.x. Epub 2009 Jul 22.
Scleromyxedema is the generalized and sclerodermoid form of lichen myxedematosus. Its typical histological features include a diffuse deposition of mucin in the papillary and mid reticular dermis, an increased of collagen deposition, and a proliferation of irregularly arranged fibroblasts. We describe a 76-year-old man presenting with scleromyxedema associated with IgGlambda monoclonal gammopathy whose biopsy showed histological features of an interstitial granulomatous-like process consistent with interstitial granuloma annulare. The significance of these unusual granulomatous findings in the setting of scleromyxedema are unknown and have been described only once in the literature. This observation expands the spectrum of scleromyxedema and highlights the difficulty in diagnosing this disabling condition.
硬化性黏液水肿是黏液水肿性苔藓的全身性硬皮病样表现形式。其典型的组织学特征包括黏蛋白在乳头层和网状真皮中层的弥漫性沉积、胶原沉积增加以及不规则排列的成纤维细胞增殖。我们描述了一名76岁男性,患有与IgGλ单克隆丙种球蛋白病相关的硬化性黏液水肿,其活检显示具有与环状肉芽肿性间质炎一致的间质肉芽肿样病变的组织学特征。这些在硬化性黏液水肿背景下不寻常的肉芽肿性发现的意义尚不清楚,且仅在文献中被描述过一次。这一观察结果拓宽了硬化性黏液水肿的范围,并突出了诊断这种致残性疾病的困难。