Subcorneal pustular dermatosis

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Disease Overview

Subcorneal pustular dermatosis (SPD) is a rare skin disease in which pus-filled pimples or blisters (pustules) form under the top (subcorneal) layer of the skin.[14911][14912][14971][14972][14973] It is most common in middle-aged adults (particularly women) but can develop in children.[14912][14973] Pustules usually appear over a few hours and grow together to form round or wavy patterns.[14912][14971] They most often form in areas where the skin may touch or rub together, such as the groin area, underarms, inside the elbows, and behind the knees.[14912][14971] The pustules may be mildly itchy or painful, but despite being pus filled, are not infected.[14912] The diagnosis of SPD is made based on the appearance of the pustules and the  results of a skin biopsy (histologic findings).[14912][14971] The cause of SPD is not known.[14912] There is currently no evidence it is inherited (no familial cases have been reported) and it is not contagious.[14911][14912] SPD may be associated with other diseases or health problems including several autoimmune diseases, blood (hematologic) diseases, infections, and cancers. Rarely, it has been associated with taking certain medications (drug-induced SPD).[14912] SPD may be treated with is oral dapsone, which often improves symptoms within one month. However, the pustules may return when treatment is stopped.[14912][14972] Other therapies have been tried with mixed results.[14912] While SPD may cause discomfort and cosmetic concerns, it typically does not affect overall health.[14971]


Synonyms

  • Sneddon-Wilkinson disease
  • Pustulosis subcornealis
  • Subcorneal pustular dermatitis
  • Sneddon Wilkinson disease

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National Organization for Rare Disorders