Lutz M E, Daoud M S, McEvoy M T, Gibson L E
Department of Dermatology, Mayo Clinic, Rochester, Minnesota 55905, USA.
Cutis. 1998 Apr;61(4):203-8.
Subcorneal pustular dermatosis of Sneddon and Wilkinson (SPD) is a unique clinical and histologic entity. This disorder has been reported in association with monoclonal gammopathy and IgA pemphigus. We report ten additional cases of SPD and determine the frequency of monoclonal gammopathy and IgA pemphigus in patients with SPD and investigate the relationship of this entity with other dermatoses characterized histologically by a subcorneal pustule. The medical records of patients with SPD evaluated at the authors' institution from 1980 through 1995 were reviewed retrospectively. Medical records of twenty patients with pustular psoriasis were reviewed for comparison. Ten patients met the criteria for SPD. Their average age at diagnosis was 66 years. The characteristic flaccid pustules were often generalized and had a tendency to involve the flexural areas. Serum monoclonal gammopathy was present in four patients: three with IgA and one with IgG. On direct immunofluorescence examination, three patients had IgA deposits in the intercellular spaces. None of the patients exhibited both monoclonal gammopathy and IgA deposits. Repeated direct immunofluorescence studies were necessary to detect IgA pemphigus in one patient. SPD is a distinct clinical entity. The findings of IgA deposits intercellularly on immunofluorescence and monoclonal gammopathy may help to further identify this entity.
斯奈登和威尔金森角膜下脓疱性皮肤病（SPD）是一种独特的临床和组织学实体。该疾病已被报道与单克隆丙种球蛋白病和IgA天疱疮相关。我们报告另外10例SPD病例，并确定SPD患者中单克隆丙种球蛋白病和IgA天疱疮的发生率，同时研究该实体与其他以角膜下脓疱为组织学特征的皮肤病之间的关系。对1980年至1995年在作者所在机构接受评估的SPD患者的病历进行了回顾性研究。为作比较，对20例脓疱型银屑病患者的病历进行了回顾。10例患者符合SPD标准。他们诊断时的平均年龄为66岁。特征性的松弛脓疱常泛发，并有累及屈侧部位的倾向。4例患者存在血清单克隆丙种球蛋白病：3例为IgA，1例为IgG。直接免疫荧光检查显示，3例患者细胞间有IgA沉积。没有患者同时出现单克隆丙种球蛋白病和IgA沉积。有1例患者需要反复进行直接免疫荧光研究才能检测出IgA天疱疮。SPD是一种独特的临床实体。免疫荧光检查发现细胞间有IgA沉积以及单克隆丙种球蛋白病，可能有助于进一步明确该实体。
