Subcorneal pustular dermatosis (Sneddon-Wilkinson disease)—therapeutic problems

Abstract

A patient with subcorneal pustular dermatosis with a fatal outcome is presented. Treatment with corticosteroids, vitamin E, dapsone, sulphapyridine and levamisole was ineffective. Only systemic treatment with retinoic acid and a new aromatic retinoic acid derivative (Ro 10-9359) produced a satisfactory clinical response, but a complete remission was not obtained.

Infliximab (Anti–Tumor Necrosis Factor α Antibody)

“…Despite this therapy, the patient experienced progressive pustular eruptions accompanied by increased cellular and serological markers of inflammation ( Figure 3). The reported association of SPD with pyoderma gangrenosum, 8 rheumatoid arthritis, 1,9 and inflammatory bowel diseases 8,10 led to a therapeutic trial with infliximab (Remicade; Centocor BV, Leiden, the Netherlands), a chimeric anti-tumor necrosis factor ␣ (TNF-␣) antibody proven highly effective in the treatment of inflammatory bowel diseases and rheumatoid arthritis. [11][12][13][14] Prior to treatment with infliximab, workup to exclude evidence of occult infection included chest radiography, urinalysis, and abdominal ultrasound.…”

“…The other therapeutic options are occasionally effective. [1][2][3][4][5][6][7] In our patient, SPD was finally successfully controlled after several relapses by a maintenance therapy consisting of acitretin and methylprednisone. However, the disease was eventually unresponsive to this treatment.…”

Successful Treatment of Subcorneal Pustular Dermatosis (Sneddon-Wilkinson Disease) by Acitretin: Report of a Case

“…When dapsone is ineffective or poorly tolerated, as in our patient, other treatments have been found to be irregularly efficient: vitamin E [2], cyclins [3], colchicine [4, 5, 6], methotrexate [7], PUVA or narrow-band (TL-01) UVB phototherapy [3, 5, 6, 7, 8, 9]. Systemic corticosteroids are less effective [2, 7, 8, 9, 10]than in Sweet’s disease.…”

“…Systemic corticosteroids are less effective [2, 7, 8, 9, 10]than in Sweet’s disease. Topical retinoic acid and corticosteroids are useful only for localized lesions.…”

“…Associated diseases were rheumatoid arthritis in 2 cases [2, 7]and benign monoclonal gammopathy in 5 cases [4, 5, 6, 10, 11]. Most cases were treated with etretinate [2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12]. The initial doses were 0.5 mg/kg body weight/day or less in 2 cases, and 1 mg/kg body weight/day or more in 9 cases.…”