Souissi Amel, Fenniche Samy, Benmously Rym, Marrak Hayet, Khayat Olfa, Debbiche Achraf, Ben Ayed Mohamed, Mokhtar I
Service de Dermatologie, Hôpital Habib Thameur, Tunis, Tunisie.
Tunis Med. 2007 Nov;85(11):985-7.
Sneddon-Wilkinson disease (SW) is chronic and benign disease belonging to the heterogeneous spectrum of neutrophilic diseases. It is characterised by flask, superficial, aseptic pustules, mainly involving the trunk, axilla and inguinal folds, favourably responsive to sulfones.
We report an original case with an atypical clinical presentation and course.
A 25-year old woman presented with an erythematous pustular eruption that had appeared one week before. Similar flare-ups were reported for 3 years. No drug intake history nor recent infection were reported. Physical examination showed superficial pustules mainly located on the face and limbs, lying on an erythematous skin and sometimes grouped in large plaques with a serpiginous disposition having a characteristic aspect of hypopyon pustule. A specimen taken from the pustules did not show bacterial or fungal infection. Histological examination of a biopsy specimen showed subcorneal pustules with a dermal peri-capillary neutrophilic infiltrate. Direct immuno-fluorescence was negative. No associated diseases were found. The patient was resistant to a 1 month treatment with dapsone (100 mg/day). Improvement was obtained with acitretin (25 mg/day) within 3 months.
Our case has some specificities: cephalic involvement, resistance to dapsone and favourable outcome to a low dose of acitretin.
斯尼登 - 威尔金森病（SW）是一种慢性良性疾病，属于嗜中性粒细胞疾病的异质性范畴。其特征为烧瓶状、浅表性、无菌性脓疱，主要累及躯干、腋窝和腹股沟褶皱处，对砜类药物反应良好。
我们报告一例具有非典型临床表现和病程的原始病例。
一名25岁女性出现一周前出现的红斑脓疱疹。据报告类似的皮疹发作已持续3年。未报告用药史及近期感染情况。体格检查显示浅表脓疱主要位于面部和四肢，位于红斑皮肤上，有时聚集成大的斑块，呈匐行状分布，具有脓性脓疱的特征性外观。从脓疱中采集的标本未显示细菌或真菌感染。活检标本的组织学检查显示角质层下脓疱，真皮毛细血管周围有嗜中性粒细胞浸润。直接免疫荧光检查为阴性。未发现相关疾病。患者对氨苯砜（100毫克/天）治疗1个月无反应。服用阿维A（25毫克/天）3个月内病情改善。
我们的病例有一些特殊性：头部受累、对氨苯砜耐药以及对低剂量阿维A反应良好。
