Bordignon Matteo, Zattra Edoardo, Montesco Maria Cristina, Alaibac Mauro
Unit of Dermatology, University of Padua, Padua, Italy.
Am J Clin Dermatol. 2008;9(1):51-5. doi: 10.2165/00128071-200809010-00006.
Subcorneal pustular dermatosis (SPD) [Sneddon-Wilkinson disease] is a benign and uncommon disorder characterized by a chronic, relapsing vesiculopustular eruption of unknown etiology. We present a case of SPD in a young Black woman in whom ELISA was performed to test for desmoglein 1 and 3 antigens (the first reported case of evaluation for these antigens in a patient with SPD). The test revealed the absence of both antibodies. The patient was successfully treated with topical corticosteroids and narrow-band UVB phototherapy. In this report, we review both the pathophysiology of SPD, which has yet to be clarified, and its treatment. Data obtained from our case report add further support to the hypothesis that a non-antibody-mediated mechanism is operative in SPD. The treatment of choice for SPD is dapsone. However, the combination of corticosteroids and UVB phototherapy should be considered a valid therapeutic option in patients who are not appropriate candidates for dapsone therapy.
角层下脓疱性皮肤病（SPD）[斯内登 - 威尔金森病]是一种良性且罕见的疾病，其特征为病因不明的慢性复发性水疱脓疱性皮疹。我们报告一例年轻黑人女性的SPD病例，对其进行了酶联免疫吸附测定（ELISA）以检测桥粒芯糖蛋白1和3抗原（这是第一例对SPD患者进行这些抗原评估的报道病例）。检测显示两种抗体均缺失。该患者通过外用糖皮质激素和窄谱中波紫外线（UVB）光疗成功治愈。在本报告中，我们回顾了尚未明确的SPD的病理生理学及其治疗方法。从我们的病例报告中获得的数据进一步支持了非抗体介导机制在SPD中起作用的假说。SPD的首选治疗药物是氨苯砜。然而，对于不适合氨苯砜治疗的患者，糖皮质激素和UVB光疗的联合应用应被视为一种有效的治疗选择。
