CASEATING CUTANEOUS GRANULOMAS IN A PATIENT WITH X-LINKED INFANTILE HYPOGAMMAGLOBULINEMIA

被引:29
作者
FLEMING, MG
GEWURZ, AT
PEARSON, RW
机构
[1] RUSH PRESBYTERIAN ST LUKES MED CTR,DEPT PATHOL,CHICAGO,IL 60612
[2] RUSH PRESBYTERIAN ST LUKES MED CTR,DEPT IMMUNOL MICROBIOL,CHICAGO,IL 60612
[3] RUSH PRESBYTERIAN ST LUKES MED CTR,DEPT PEDIAT,CHICAGO,IL 60612
关键词
D O I
10.1016/0190-9622(91)70097-L
中图分类号
R75 [皮肤病学与性病学];
学科分类号
100206 [皮肤病与性病学];
摘要
A 34-year-old man with X-linked infantile hypogammaglobulinemia, bronchiectasis, and chronic liver disease had a papular eruption on the trunk and upper extremities. A biopsy specimen revealed caseating granulomas, but special stains, cultures, and electron microscopy failed to revealed an infectious organism. Immunohistochemistry showed that the lymphocytes within the granulomas were almost exclusively of the CD8+ cytotoxic/suppressor T phenotype. Phenotypic analysis of the circulating lymphocytes showed normal numbers of CD4+ (helper/inducer) and CD8+ T cells, whereas B cells were undetectable. Other examples of noninfectious granulomatous disease have been reported in patients with primary hypogammaglobulinemia, but this is the first case of caseating cutaneous granulomatous disease to be reported in a patient with X-linked infantile hypogammaglobulinemia.
引用
收藏
页码:629 / 633
页数:5
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