Subcorneal Pustular Dermatosis: A Review of 30 Years of Progress

Abstract

Subcorneal pustular dermatosis (SPD), also known as Sneddon-Wilkinson disease, is a rare, benign yet relapsing pustular dermatosis. Its incidence and prevalence have not been well studied. It characteristically presents as hypopyon pustules on the trunk and intertriginous areas of the body. SPD is similar to two other disease entities. Both SPD-type immunoglobulin (Ig)-A pemphigus and annular pustular psoriasis clinically and histologically present similarly to SPD. Immunologic studies separate SPD-type IgA pemphigus from SPD and pustular psoriasis. However, there is still an unclear designation as to whether SPD is its own entity distinct from pustular psoriasis, as the once thought characteristic histologic picture of psoriasis does not hold true for pustular psoriasis. SPD has been reported to occur in association with several neoplastic, immunologic, and inflammatory conditions. Dapsone remains the first-line treatment for SPD, although dapsone-resistant cases have been increasingly reported. Other therapies have been used singly or as adjunctive therapy with success, such as corticosteroids, immunosuppressive agents, tumor necrosis factor inhibitors, and ultraviolet light therapy. This article provides a review of the last 30 years of available literature, with a focus on successful treatment options and a suggestion for reappraisal of the classification of SPD.

Pentoxyfilline as a treatment for subcorneal pustular dermatosis

“…Dapsone as a first‐line treatment for SPD is often effective in achieving complete remission. Refractory cases have been successfully treated with etretinate, acitretin, PUVA, narrow‐band UVB, infliximab, and corticosteroids (Watts & Khachemoune, ). In this case, the patient failed to respond to acitretin and topical corticosteroids and could not tolerate dapsone.…”

Improvement of recalcitrant Sneddon–Wilkinson disease with apremilast

“…The condition may be difficult to differentiate from other vesiculopustular dermatoses like such as pustular psoriasis. 1 A 65-year-old woman suffered a 15 year history of recalcitrant Sneddon-Wilkinson dermatosis. She no other relevant medical history.…”

Hypopyon pustules of Sneddon‐Wilkenson disease

“…Histologic examination typically reveals subcorneal neutrophilic blisters and superficial perivascular infiltrates. Direct immunofluorescence studies are routinely negative . The disease occurs most commonly in females usually after the 4th decade of life, although rare cases in children have been reported .…”

“…Other reported associations include pyoderma gangrenosum, multiple myeloma, cryoglobulinemia, leukemias, lymphomas, inflammatory bowel disease, thyroid disorders, multiple sclerosis, and connective tissue disease. There have also been a few case reports of suspected drug‐ and infection‐induced SPD …”

“…For patients unresponsive to dapsone, other treatment options include phototherapy, topical and systemic corticosteroids, acitretin, and TNF‐α inhibitors like infliximab. Relapses following discontinuation of treatment are common, and maintenance therapy should be considered …”