Subcorneal pustular dermatosis

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Subcorneal pustular dermatosis

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Subcorneal pustular dermatosis

Sneddon-Wilkinson disease

Dermatology - TOTAL BODY: subcorneal pustular dermatosis   

Subcorneal pustular dermatosis is a chronic pustular dermatosis that frequently affects the flexures of the trunk and limbs but spares the face and mucosal surfaces.

The lesions typically have the appearance of "half-half" blisters, with half of each blister containing pus and half containing clear fluid.

 It is thought that this phenomenon represents an overlap with IgA pemphigus. An association with IgA monoclonal gammopathy and myeloma has been reported.

  • The condition remits spontaneously, often after many years.
  • Dapsone 50 to 150 mg po daily is usually effective therapy.

Phototherapy with psoralen with UVA (PUVA),broadband UVB, and narrowband UVB alone or in combination with dapsone and/or retinoids can be successful at controlling subcorneal pustular dermatosis.Long-term maintenance regimens may be needed.

Anecdotal case reports support the use of infliximab, tacalcitol ,mizoribine,ketoconazole, tetracycline, minocycline, benzylpenicillin, vitamin E,azithromycin,cyclosporine,colchicine, and adalimumab with mycophenolate mofetil.

Systemic and topical corticosteroids are generally ineffective but may provide some control. They have been used in combination with dapsone to treat associated conditions such as pyoderma gangrenosum and multiple myeloma. A good response to systemic corticosteroids is atypical and is suggestive of a diagnosis of pustular psoriasis.