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Benign familial chronic pemphigus

Synonym:

Hailey-Hailey disease

Benign familial chronic pemphigus is an autosomal dominant disorder characterized by recurrent vesicles, bullae and erosions typically involving the neck, axillae and groin.

Histologically related to keratosis follicularis (Darier's disease) patients have been described with both conditions simultaneously.

Hailey-Hailey disease is frequently worse in warm weather.

Treatment:

  • Topical potent glucocorticoids, such as clobetasol 0.05% cream bid, are frequently all that is needed to control this problem.
  • Patients should be advised to avoid heat.
  • Oral etretinate can produce improvement but, as treatment must be continued, it should be started with reluctance.
  • As with Darier's disease, the lesions of Hailey-Hailey disease are prone to overgrowth with bacteria. Treatment with appropriate broad spectrum antibiotics is very helpful in controlling the problem.
  • Case reports show benefit from Dapsone, cyclosporine, lasabrasion and methotrexate
Benign familial chronic pemphigus


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