Launay F, Albès B, Bayle P, Carrière M, Lamant L, Bazex J
Service de dermatologie, CHU Purpan, Toulouse, France.
Rev Med Interne. 2004 Feb;25(2):154-9. doi: 10.1016/j.revmed.2003.10.014.
We report four cases of subcorneal pustular dermatosis or Sneddon-Wilkinson disease. Clinical and histological lesions and immunofluorescence data were presented. This disease is classified among neutrophilic dermatitis.
All of four patients presented with clinical and histological lesions compatible with the diagnosis of Sneddon-Wilkinson disease. Indeed, direct and indirect immuno-testing were negative. We noted an association with a benign IgA monoclonal gammapathy in one case and with a seronegative polyarthritis in one other case. Three patients correctly responded to dapsone. One of them after transient improvement was resistant to dapsone and then dramatically responded to etretinate.
Subcorneal pustular dermatosis is a chronic disease, rarely described in literature. It's a pustular eruption, involving the trunck, axillae and inguinal holds. It's often associated with monoclonal gammapathy, particulary IgA. Its nosological situation is still contested, especially with IgA pemphigus sharing with it the association with IgA monoclonal gammapathy and the same efficacy of dapsone. We discuss relationships between both diseases.
我们报告了4例角层下脓疱性皮肤病或斯内登-威尔金森病。展示了临床和组织学病变以及免疫荧光数据。该疾病归类于嗜中性皮病。
所有4例患者均呈现出与斯内登-威尔金森病诊断相符的临床和组织学病变。确实，直接和间接免疫检测均为阴性。我们注意到1例患者与良性IgA单克隆丙种球蛋白病相关，另1例患者与血清阴性多关节炎相关。3例患者对氨苯砜反应良好。其中1例患者在短暂改善后对氨苯砜耐药，随后对阿维A酯反应显著。
角层下脓疱性皮肤病是一种慢性病，文献中鲜有描述。它是一种脓疱性皮疹，累及躯干、腋窝和腹股沟部位。它常与单克隆丙种球蛋白病相关，尤其是IgA。其疾病分类情况仍存在争议，特别是与IgA天疱疮，二者均与IgA单克隆丙种球蛋白病相关且对氨苯砜有相同疗效。我们讨论了这两种疾病之间的关系。
