Subcorneal pustular dermatosis
- Arianne Shadi Kourosh, MD
Arianne Shadi Kourosh, MD
- Director of Community Health
- Department of Dermatology, Massachusetts General Hospital and Harvard Medical School
Subcorneal pustular dermatosis (SPD), also known as Sneddon-Wilkinson disease, is a rare neutrophilic dermatosis in which recurrent crops of sterile pustules appear in the most superficial (subcorneal) layers of the skin (picture 1A-B). The pustules are usually distributed in annular or serpiginous patterns and are most commonly located on flexural surfaces and on intertriginous skin. SPD is characterized by its clinical presentation, as its etiology is unknown and its nosology remains controversial.
In the overwhelming majority of cases, SPD is a chronic and benign condition for which the primary concerns are minimizing morbidity, improving quality of life, and ruling out the presence of an associated internal disease. Oral dapsone is the treatment of choice for SPD.
The clinical features, diagnosis, and management of SPD will be reviewed here. Other neutrophilic dermatoses and pustular disorders are reviewed separately. (See "Neutrophilic dermatoses" and "Approach to the patient with pustular skin lesions".)
Since Sneddon and Wilkinson first described SPD in the 1950s, the majority of cases have been reported in women, and generally in women over the age of 40 [1,2]. SPD is considered a rare condition; however, no estimate of global incidence or prevalence is available.
Although most of the reported cases have occurred in Caucasians, there is no known ethnic or geographic predilection. SPD is observed worldwide [3,4]. The relatively few cases reported in children tend to be atypical, and some have subsequently been reclassified as forms of psoriasis .
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- CUTANEOUS MANIFESTATIONS
- ASSOCIATED DISORDERS
- Physical examination
- Skin biopsy
- EVALUATION FOR ASSOCIATED DISORDERS
- DIFFERENTIAL DIAGNOSIS
- First-line therapy
- - Oral dapsone
- Second-line therapies
- - Phototherapy
- - Systemic glucocorticoids
- Other therapies
- - Topical agents
- - Systemic agents
- SUMMARY AND RECOMMENDATIONS