Saint-Jean M, Gagey-Caron V, Jossic F, Barbarot S, Hamidou M, Stalder J-F
Service de dermatologie, CHU Hôtel-Dieu, 1 place A.-Ricordeau, Nantes cedex 01, France.
Ann Dermatol Venereol. 2011 May;138(5):399-404. doi: 10.1016/j.annder.2010.11.015. Epub 2010 Dec 30.
Amicrobial pustulosis of the skin folds represents a new entity within the spectrum of neutrophilic dermatoses. This disease is characterized by acute onset of pustular lesions in the skin folds, association with an autoimmune disorder, and improvement under systemic corticosteroids.
A 24-year-old woman had been presenting pustular dermatosis for several months involving the inguinal folds and the scalp. The pustules coalesced to form oozing and crusting plaques. Eczematous lesions were located on the trunk. She also presented macrocytic anemia related to autoimmune erythroblastopenia. Bacteriological culture was negative. Antinuclear antibodies were found with anti-SSA specificity. Histopathological examination of a skin biopsy specimen showed pustules in the epidermis together with an inflammatory dermal infiltrate. Cutaneous direct immunofluorescence testing was negative. The patient responded to systemic corticosteroids.
Thirty-six cases of amicrobial pustulosis of the skin folds have been reported in the literature. All but two previously reported patients were females with an autoimmune disorder (chiefly systemic lupus erythematosus). The clinical picture is characterized by aseptic pustular lesions of the major and minor skin folds of the scalp and the anogenital area associated with eczematous lesions. Diagnostic criteria have recently been proposed. This disease responds to systemic corticosteroids. We report a new case of amicrobial pustulosis of the skin folds associated with autoimmune erythroblastopenia, which to the best of our knowledge has been described only once in the literature.
皮肤褶皱处无菌性脓疱病是嗜中性皮病谱系中的一种新疾病。该疾病的特征为皮肤褶皱处脓疱性皮损急性起病，与自身免疫性疾病相关，且在全身应用糖皮质激素治疗后病情改善。
一名24岁女性出现脓疱性皮肤病数月，累及腹股沟褶皱和头皮。脓疱融合形成渗出性结痂斑块。湿疹样皮损位于躯干。她还出现与自身免疫性成红细胞减少相关的大细胞性贫血。细菌培养阴性。发现抗核抗体具有抗SSA特异性。皮肤活检标本的组织病理学检查显示表皮有脓疱，真皮有炎性浸润。皮肤直接免疫荧光检测阴性。患者对全身应用糖皮质激素治疗有反应。
文献中已报道36例皮肤褶皱处无菌性脓疱病。除2例先前报道的患者外，其余均为患有自身免疫性疾病（主要为系统性红斑狼疮）的女性。临床表现为头皮及肛门生殖器区域大小皮肤褶皱处的无菌性脓疱性皮损，伴有湿疹样皮损。最近已提出诊断标准。该疾病对全身应用糖皮质激素治疗有反应。我们报告1例与自身免疫性成红细胞减少相关的皮肤褶皱处无菌性脓疱病新病例，据我们所知，该病例在文献中仅被描述过一次。
