We present a case of
Sneddon-Wilkinson disease in a 52-year-old female at her first presentation to dermatology. Outlined in the case are various investigations undertaken at this initial presentation, including rheumatologic and
hematologic malignancy markers, which identified
immunoglobulin A gammopathy. The systemic and topical
therapies used to treat the patient's condition are described, as well as her response to these treatments. In this discussion, we explain the epidemiology, pathophysiology, and clinical presentation of
Sneddon-Wilkinson disease. Various medical conditions having known association with
Sneddon-Wilkinson disease are discussed, including
immunoglobulin A or
immunoglobulin G monoclonal gammopathies and
lymphoproliferative disorders. A comprehensive differential diagnosis for
Sneddon-Wilkinson disease is provided, including
immunoglobulin A pemphigus,
acute generalized exanthematous pustulosis and pustular
psoriasis, among others. We describe the systemic and topical
therapy options for the treatment of
Sneddon-Wilkinson disease, of which first line treatment is systemic
dapsone. This patient serves as an excellent case of
Sneddon-Wilkinson disease with unexpected
immunoglobulin A gammopathy.