Ramot Yuval, Czarnowicki Tali, Maly Alex, Navon-Elkan Paulina, Zlotogorski Abraham
Departments of Dermatology, The Center for Genetic Diseases of the Skin and Hair, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
Pediatr Dermatol. 2011 Sep-Oct;28(5):538-41. doi: 10.1111/j.1525-1470.2010.01163.x. Epub 2010 Jun 9.
Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature syndrome is a recently described chronic inflammatory syndrome consisting of widespread annular violaceous skin lesions and multisystemic inflammatory manifestations. We report a 12½-year-old boy with a young-age onset of recurrent fevers, annular violaceous plaques, alopecia areata, lipodystrophy, low weight and height, deformed fingers, wide-spaced nipples, chronic anemia, and elevated acute phase reactants. An abdominal punch biopsy demonstrated dense perivascular and interstitial infiltrates in the dermis, composed mainly of mononuclear cells. This syndrome may represent a new autosomal recessive auto-inflammatory genodermatosis. Increased awareness may lead to the discovery of more cases, and clarify its pathogenesis.
伴有脂肪营养不良和体温升高综合征的慢性非典型嗜中性皮肤病是一种最近描述的慢性炎症综合征，由广泛的环形紫罗兰色皮肤病变和多系统炎症表现组成。我们报告一名12岁半的男孩，发病年龄较小，有反复发热、环形紫罗兰色斑块、斑秃、脂肪营养不良、体重和身高偏低、手指畸形、乳头间距宽、慢性贫血以及急性期反应物升高。腹部穿刺活检显示真皮层有密集的血管周围和间质浸润，主要由单核细胞组成。该综合征可能代表一种新的常染色体隐性遗传性自身炎症性皮肤病。提高认识可能会发现更多病例，并阐明其发病机制。
