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Subcorneal pustular dermatosis
Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports
Courtesy of Frederick A. Pereira, MD
Subcorneal pustular dermatosis is a rare, chronic, neutrophilic dermatosis of unknown etiology, in which flaccid pustules and vesicopustules arise in crops on truncal and flexural skin. The condition can occur at any age, more commonly in females. It usually follows a relapsing and remitting course, but generally the patient remains systemically well.
The condition may be difficult to differentiate from other vesiculopustular dermatoses. Some cases may be a variant of pustular psoriasis, whereas others appear to overlap with the subcorneal pustular variant of immunoglobulin A (IgA) pemphigus. In ‘classical’ subcorneal pustular dermatosis, immunofluorescence studies are negative; however, there are patients with identical clinical and histologic features that have intercellular IgA deposition in the upper epidermis targeting desmosomal components, most commonly desmocollins.
Subcorneal pustular dermatosis is associated with monoclonal immunoglobulin A (IgA) paraproteinemia and myeloma, which may occur many years after presentation. Other reported associations are pyoderma gangrenosum, inflammatory bowel disease, rheumatoid arthritis, infections (in particular Mycoplasma pneumoniae ), and drugs.
Management Strategy
Dapsone is the treatment of choice (25–200 mg daily) and normally results in resolution of the rash within 4 weeks; the drug usually needs to be continued long term because relapse is common on withdrawal of therapy. After control is gained, the dose should be tapered to the lowest dose required to maintain remission. Other sulfones ( sulfapyridine , salazosulfapyridine ) have also been found to be beneficial in isolated reports.
In a proportion of cases the response to dapsone is poor; retinoids (acitretin) can be used at an initial dose of 0.5–1.0 mg/kg daily and then reducing to the lowest dose that will maintain control. Alternatively, for those unable to tolerate dapsone in the dose required, retinoids have been added to dapsone; this has enabled lower doses of each to be used. There are a few case reports detailing good response to phototherapy – psoralen and UVA (PUVA), narrowband UVB, or broadband UVB – in combination with dapsone or retinoids.
Both topical and systemic corticosteroids have been reported in isolated cases to provide some degree of control. Unacceptably high doses of systemic corticosteroids may be required, but combination with other treatments may allow control with lower doses.
A number of dapsone -resistant cases have been treated with novel therapies such as anti-tumor necrosis factor-alpha (TNF-α) inhibitors, but experience with these newer agents is limited.
Subcorneal pustular dermatosis tends to run a chronic course. Maintenance of a continuing beneficial response may be difficult, as may be inferred from the extensive range of treatment options described.
Specific Investigations
Subcorneal pustular dermatosis: a review of 30 years of progress
Watts PJ, Khachemoune A. Am J Clin Dermatol 2016: 653–71.
The authors review the available literature from the past 30 years. They argue that no clinical or histological feature truly distinguishes subcorneal pustular dermatosis (SPD) from the annular variant of pustular psoriasis. They suggest that a reappraisal of SPD is required and it should be reclassified as a generalized form of pustular psoriasis.
Subcorneal pustular dermatosis: a clinical study of ten patients
Lutz ME, Daoud MS, McEvoy MT, et al. Cutis 1998; 61: 203–8.
Four of the seven patients in whom immunoglobulins were checked had paraproteinemia: three of IgA and one of IgG type; none of these patients had IgA deposits on direct immunofluorescence.
Subcorneal pustular dermatosis and episcleritis associated with poorly controlled ulcerative colitis
Wargo JJ, Adams M, Trevino J. BMJ Case Rep 2017 Jan 30; 2017: bcr2016218123.
A case of subcorneal pustular dermatosis associated with poorly controlled ulcerative colitis. The patient was started on dapsone; resolution of symptoms was reported with control of his ulcerative colitis.
Sneddon–Wilkinson disease in association with rheumatoid arthritis
Butt A, Burge SM. Br J Dermatol 1995; 132: 313–5.
Useful overview of all reported cases associated with either seronegative or seropositive arthritis.
Subcorneal pustular dermatosis triggered by Mycoplasma pneumonia : a rare clinical association
Bohelay G, Duong TA, Ortonne N, et al. J Eur Acad Dermatol Venereol 2015; 29: 1022–5.
A case of subcorneal pustular dermatosis in association with Mycoplasma pneumoniae pneumonitis. Literature review identified seven cases associated with M. pneumoniae.
Subcorneal pustular dermatosis associated with Coccidioides immitis
Iyengar S, Chambers CJ, Chang S, et al. Dermatol Online J 2015; 21: 13030/qt35r6z6bx.
Subcorneal pustular dermatosis associated with Coccidioides immitis . Symptoms resolved promptly following initiation of systemic antifungal therapy.
Sneddon-Wilkinson disease induced by sorafenib in a patient with advanced hepatocellular carcinoma
Tajiri K, Nakajima T, Kawai K, et al. Intern Med 2015; 54: 597–600.
A case of subcorneal pustular dermatosis that developed after sorafenib administration. It improved after cessation of sorafenib and worsened by its readministration.
Subcorneal pustular dermatosis
Sneddon IB, Wilkinson DS. Br J Dermatol 1956; 68: 385–93.
Three of six patients responded to dapsone 50–100 mg daily.
Sneddon–Wilkinson disease in association with rheumatoid arthritis
Butt A, Burge SM. Br J Dermatol 1995; 132: 313–5.
A further five patients who responded to dapsone.
Subcorneal pustular dermatosis and IgA gammopathy
Burrows D, Bingham EA. Br J Dermatol 1984; 111: 91–3.
The addition of etretinate enabled control in a patient intolerant of higher-dose dapsone. Acitretin starting at 25 mg daily could be substituted.
Sneddon–Wilkinson disease. Four case reports
Launay F, Albes B, Bayle P, et al. Rev Med Interne 2004; 25: 154–9.
Three of four patients responded to dapsone.
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