Lagrange S, Chosidow O, Piette J C, Wechsler B, Godeau P, Frances C
Services de Médecine Interne, Groupe Hospitalier Pitié-Salpêtrière,Paris, France.
Lupus. 1997;6(6):514-20. doi: 10.1177/096120339700600607.
Amicrobial pustulosis of the folds is a new entity which was initially described in association with systemic lupus erythematosus (SLE). We report four cases of this rare pustulosis occurring in the course of various auto-immune disorders. Dermatological features were similar in all patients. The primary lesions were small pustules which coalesced and formed erosive areas underlined with pustular lesions. They were localized in the folds. Three of the four patients had also isolated pustules on the scalp. Spongiform pustules were histologically present. A primary bacterial or fungal infection was ruled out by cultures on appropriate media. Serum calcium level was normal. Associated auto-immune diseases were: systemic lupus erythematosus, incomplete systemic lupus erythematosus, 'idiopathic' thrombocytopenic purpura and myasthenia gravis without thymoma (one of each). The relationship between this peculiar form of pustulosis and autoimmunity remains to be elucidated. The pustulosis did not respond to antibiotics but disappeared with systemic or topical corticosteroids.
褶皱部无菌性脓疱病是一种新的疾病实体，最初被描述为与系统性红斑狼疮（SLE）相关。我们报告了4例发生于各种自身免疫性疾病过程中的这种罕见脓疱病。所有患者的皮肤特征相似。原发性损害为小脓疱，脓疱融合并形成有脓疱性损害的糜烂区。它们局限于褶皱部。4例患者中有3例头皮上也有孤立的脓疱。组织学上可见海绵状脓疱。通过在合适培养基上培养排除了原发性细菌或真菌感染。血清钙水平正常。相关的自身免疫性疾病有：系统性红斑狼疮、不完全性系统性红斑狼疮、“特发性”血小板减少性紫癜和无胸腺瘤的重症肌无力（各1例）。这种特殊形式的脓疱病与自身免疫之间的关系仍有待阐明。脓疱病对抗生素无反应，但使用全身性或局部皮质类固醇后消失。
