Hailey-Hailey Disease

The patient is a 38 year-old cook with a 14 year history of a pruritic, occasionally painful rash on his torso.  He used triamcinalone cream in the past without relief.  No family history aof a similar process.

O/E.:  There are plaques in axillary folds, on the chest, back, and popliteal fossae.Some advancing borders appear vesicular on close observation.  There is mild post-inflammatory hyperpigmentation in healed sites.

Clinical Photos from L. Axilla:

Laboratory:  An incisional biopsy was taken from the crusted/vesicular edge.

Histopathology:  Courtesy Dr. Lynne Goldberg. Boston University Skip Path

Tentative Diagnosis:  Benign Familial Pemphigus (Hailye-sHaily)

Postscript:  After one month of doxycycline 100 mg b.i.d. and betamethasone 0.1% cream the patient was > 90% resolved.  He is pain and itch free.  He was asked to cut back on the cream to prn and will continue the doxycycline.  It will be tapered in a month.