Favarato Maria Helena Sampaio, Miranda Sofia Silveira de Castro, Caleiro Maria Teresa Correia, Assad Ana Paula Luppino, Halpern Ilana, Fuller Ricardo
Internal Medicine Department, University of São Paulo, São Paulo(SP),Brazil.
An Bras Dermatol. 2013 Jul-Aug;88(4):635-8. doi: 10.1590/abd1806-4841.20131948.
Cutaneous mucinosis is a group of conditions involving an accumulation of mucin or glycosaminoglycan in the skin and its annexes. It is described in some connective tissue diseases but never in association with mixed connective tissue disease. This report concerns two cases of cutaneous mucinosis in patients with mixed connective tissue disease in remission; one patient presented the papular form, and the other reticular erythematous mucinosis. These are the first cases of mucinosis described in mixed connective tissue disease. Both cases had skin lesions with no other clinical or laboratorial manifestations, with clinical response to azathioprine in one, and to an association of chloroquine and prednisone in the other.
皮肤黏蛋白沉积症是一组涉及皮肤及其附属器中黏蛋白或糖胺聚糖积聚的病症。在一些结缔组织疾病中有相关描述，但从未与混合性结缔组织病相关联。本报告涉及两例处于缓解期的混合性结缔组织病患者发生皮肤黏蛋白沉积症的病例；一例患者表现为丘疹型，另一例为网状红斑性黏蛋白沉积症。这些是混合性结缔组织病中描述的首例黏蛋白沉积症病例。两例患者均有皮肤病变，无其他临床或实验室表现，其中一例对硫唑嘌呤有临床反应，另一例对氯喹和泼尼松联合用药有临床反应。
