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医源性皮质醇减少后发生的泛发性脓疱型银屑病(冯·祖姆布施型)

Generalized pustular psoriasis (von Zumbusch) following iatrogenic hypocortisolism.

作者信息

Augey Frédéric, Dissard Christine, Normand Isabelle, Daumont Michèle

机构信息

Department of Dermatology, Centre hospitalier Lucien Hussel 38209 Vienne Cedex, France.

出版信息

Eur J Dermatol. 2004 Nov-Dec;14(6):415-7.

PMID:15564207
Abstract

Generalized pustular psoriasis can be triggered by hypocalcemia, pregnancy, stress and drugs but frequently has no obvious cause. We report a case which was only cured after treatment of iatrogenic adrenal axis suppression. A 41 year old woman had been suffering for nine months from a generalized pustular psoriasis which had occurred after a three week topical corticosteroid therapy of plaque psoriasis with 90 g of betamethasone dipropionate + 2% salicylic acid. Successive systemic treatments failed but topical corticosteroids brought relief to the patient. Cortisol level was found to be very low. Further investigations showed iatrogenic adrenal axis suppression. Hydrocortisone supplementation brought spectacular improvement and complete healing in a few months.We suggest that our patient was extremely sensitive to corticosteroids because the first pustules appeared after a conventional topical treatment. Adrenal axis suppression has never been involved in the aggravation of inflammatory dermatoses except in two cases of severe atopic dermatitis. Endogen corticosteroids inhibit proinflammatory cytokines by a feed-back mechanism and might have a great importance in the immune regulation loop. Cortisol level measurement should be considered in corticodependent inflammatory dermatoses.

摘要

泛发性脓疱型银屑病可由低钙血症、妊娠、压力和药物引发,但通常无明显病因。我们报告一例经医源性肾上腺轴抑制治疗后才得以治愈的病例。一名41岁女性患泛发性脓疱型银屑病9个月,该病在使用90克二丙酸倍他米松+2%水杨酸对斑块状银屑病进行为期三周的局部皮质类固醇治疗后出现。连续的全身治疗均失败,但局部皮质类固醇使患者症状缓解。发现皮质醇水平极低。进一步检查显示医源性肾上腺轴抑制。补充氢化可的松带来显著改善,并在几个月内实现完全治愈。我们认为我们的患者对皮质类固醇极度敏感,因为首个脓疱在常规局部治疗后出现。除两例严重特应性皮炎外,肾上腺轴抑制从未参与炎症性皮肤病的加重过程。内源性皮质类固醇通过反馈机制抑制促炎细胞因子,可能在免疫调节环路中具有重要意义。对于皮质类固醇依赖的炎症性皮肤病,应考虑测量皮质醇水平。

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