García-Patos V, Bartralot R, Fonollosa V, Arnal C, Boronat M, Gelpí C, Rodríguez J L, Castells A
Department of Dermatology, Hospital Universitari Vall d'Hebron, Barcelona, Spain.
Br J Dermatol. 1996 Oct;135(4):613-6.
We report a boy with overlap manifestations of systemic sclerosis and dermatomyositis (sclerodermatomyositis) whose disease showed a changing clinical pattern, and who had mechanic's hands, which are a cutaneous marker of myositis. Serological studies revealed antinuclear antibodies with a homogeneous nucleolar pattern. The anti-PM-Scl antibody was demonstrated by immunoblotting. HLA typing was positive for HLA-DR3/4. After a follow-up period of 11 years, no progression to severe systemic involvement was detected, and aggressive treatment was not administered. The recognition of subsets of patients with homogeneous clinical features and serological markers should permit the recognition of separate conditions among overlap syndromes. This would have prognostic and therapeutic implications.
我们报告了一名患有系统性硬化症和皮肌炎重叠表现（硬皮病性皮肌炎）的男孩，其病情呈现出不断变化的临床模式，并且患有机械工手，这是肌炎的一种皮肤标志物。血清学研究显示抗核抗体呈均匀核仁型。通过免疫印迹法证实了抗PM - Scl抗体。HLA分型显示HLA - DR3/4呈阳性。经过11年的随访期，未发现进展为严重的全身受累情况，也未进行积极治疗。识别具有相同临床特征和血清学标志物的患者亚组应有助于在重叠综合征中识别出不同的疾病状况。这将具有预后和治疗意义。
