Sneddon-Wilkinson Disease and Monoclonal Gammopathy of Undetermined Significance in the Elderly: Case Report

Case Rep Dermatol. 2019 Jul 10;11(2):209-214. doi: 10.1159/000487003. eCollection 2019 May-Aug.

Abstract

Sneddon-Wilkinson disease (SWD) or subcorneal pustular dermatosis is considered a rare pustular skin disease with chronic relapsing course. An association between SWD and other chronic conditions, such as IgA or IgG monoclonal gammopathy of undetermined significance (MGUS), IgA myeloma, pyoderma gangrenosum, thyroid gland disorders, and neoplastic diseases other than MGUS/myeloma, is known. We describe the case of a 92-year-old male patient with SWD and a concurrent IgG MGUS who had been treated with systemic betamethasone, topical mometasone furoate, and methylprednisolone aceponate, with a complete and durable resolution of symptoms and skin lesions without side effects. Systemic and topical steroids were very effective and well tolerated in our patient. This is the second case reported in the literature on the efficacy of a corticosteroid regimen in SWD in a fragile patient. This therapeutic approach (instead of dapsone therapy) has been used due to its relatively good safety profile.

Keywords: Corticosteroids; Monoclonal gammopathy of undetermined significance; Sneddon-Wilkinson disease; Subcorneal pustular dermatosis.

Publication types

  • Case Reports