Subcorneal Pustular Dermatosis Treatment & Management

Updated: Feb 11, 2019
  • Author: Lidija Kandolf Sekulovic, MD, PhD; Chief Editor: Dirk M Elston, MD  more...
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Treatment

Approach Considerations

Dapsone

Dapsone is the treatment of choice. The response is slower than that seen with dermatitis herpetiformis, with resolution usually occurring in about 4 weeks. Once disease control has been established, the dose should be tapered to the lowest dose needed to maintain control. Sulfapyridine and sulfamethoxypyridazine may also be used, but only a few isolated reports support their effectiveness.

Acitretin

Acitretin (and formally etretinate) has been used to successfully treat subcorneal pustular dermatosis (SPD) and should be considered as an alternative or additional treatment for those who are intolerant of, or unresponsive to, dapsone. Once disease control has been established, the dose should be tapered to the lowest dose needed to maintain control. Isotretinoin at 0.5 mg/kg/d appears to be ineffective.

Phototherapy

Phototherapy with psoralen with UVA (PUVA), [45] broadband UVB, and narrowband UVB alone or in combination with dapsone and/or retinoids can be successful at controlling subcorneal pustular dermatosis. [46] Long-term maintenance regimens may be needed. [47]

Additional therapies

Anecdotal case reports support the use of infliximab, tacalcitol, [48] maxacalcitol, [49] mizoribine, [50] ketoconazole, [51] tetracycline, minocycline, benzylpenicillin, vitamin E, [52] azithromycin, [53] cyclosporine, [54, 55] colchicine, pentoxifylline, [56] intravenous immunoglobulins, [57] and adalimumab with mycophenolate mofetil. [58] Antimyeloma treatment should be considered in cases of subcorneal pustular dermatosis associated with IgA monoclonal gammopathy of undetermined significance, refractory to other therapies. [59]

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.

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Long-Term Monitoring

Long-term follow-up is recommended. Periodic evaluations with serum protein electrophoresis and direct immunofluorescence should be performed every few years.

Paraproteinemia, myeloma, intraepidermal IgA staining, and pustular psoriasis may develop several years after the initial presentation of subcorneal pustular dermatosis (SPD). Identifying these conditions, as well as other associated diagnoses, can improve the understanding of the etiology and pathogenesis of subcorneal pustular dermatosis, clarify its relationship with IgA pemphigus and pustular psoriasis, and help define its nosologic classification.

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