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

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.

Subcorneal pustular dermatosis (Sneddon-Wilkinson disease): a case report

“…The literature describes isolated cases of successful use of drugs such as colchicine, cyclosporine, in liximab, etanercept, tumor necrosis factor alpha blockers, maxacalcitol, mizoribine, ketoconazole, tetracycline, minocycline, benzylpenicillin, vitamin E, azithromycin [11], doxycycline [8,12], adalimumab [13]. The ef icacy of systemic and topical glucocorticosteroids in elderly patients has also been reported [14,15].…”

Distinguishing annular pustular psoriasis from subcorneal pustular dermatosis—A diagnostic dilemma in a 10‐year‐old boy

“…Finally, there is a well‐established association between SPD and monoclonal gammopathy, mainly IgA 10,11 . Therefore, if SPD is in the differential diagnosis, it is important to exclude the diagnosis of IgA pemphigus and multiple myeloma at disease presentation and throughout the disease course 12…”

“…8,9 Finally, there is a well-established association between SPD and monoclonal gammopathy, mainly IgA. 10,11 Therefore, if SPD is in the differential diagnosis, it is important to exclude the diagnosis of IgA pemphigus and multiple myeloma at disease presentation and throughout the disease course. 12 The following case report illustrates the challenge of distinguishing SPD and APP and underscores the hypothesis that the two disorders may be related.…”