Hermanns-Lê T, Arrese J E, Piérard G E
Service de Dermatopathologie, Université de Liège.
Ann Dermatol Venereol. 1998 Feb;125(2):124-6.
Cutaneous manifestations of myelodysplastic syndromes are rare and polymorphous. They can be the direct expression of the hematological disease or represent signs of a vasculitis or a neutrophilic syndrome. Myelodysplastic syndromes progress sometimes toward an acute leukemia.
A 53-year-old woman suffering from myelodysplastic syndrome presented for several months a cutaneous vasculitis without any histological specificity. In time, such presentation was complicated by the simultaneous occurrence of a cutaneous Langerhans cell histiocytosis and an acute monoblastic leukemia type LMA 4. The disease was rapidly fatal.
The complication of a myelodysplastic syndrome by concurrent Langerhans cell histiocytosis and acute monoblastic leukemia suggests a pathogenic relationship between these two latter disorders.
骨髓增生异常综合征的皮肤表现罕见且多样。它们可以是血液系统疾病的直接表现，也可以代表血管炎或嗜中性粒细胞综合征的体征。骨髓增生异常综合征有时会进展为急性白血病。
一名53岁患有骨髓增生异常综合征的女性，出现皮肤血管炎数月，无任何组织学特异性。随着时间推移，这种表现并发了皮肤朗格汉斯细胞组织细胞增多症和LMA 4型急性单核细胞白血病，病情迅速致命。
骨髓增生异常综合征并发朗格汉斯细胞组织细胞增多症和急性单核细胞白血病提示后两种疾病之间存在致病关系。
