Familial Benign Pemphigus (Hailey-Hailey Disease) Clinical Presentation

Updated: Aug 20, 2020
  • Author: Thomas N Helm, MD; Chief Editor: Dirk M Elston, MD  more...
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Presentation

History

A family history of benign familial pemphigus usually is present. Commonly, patients may not have symptoms until ages 30-49 years. Delayed diagnosis of familial benign pemphigus also is common, especially if the patient's lesions respond to topical corticosteroids, antibiotics, or antifungals.

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Physical Examination

With familial benign pemphigus, vesicles and erythematous plaques with overlying crusts typically occur in the genital area, as well as the chest, neck, and axillary areas, as shown in the images below.

Right axilla with erosive erythematous plaques. Right axilla with erosive erythematous plaques.
Lumbar back with erythematous plaques with impetig Lumbar back with erythematous plaques with impetiginized crust.
Left axilla with tender erythematous plaques. Left axilla with tender erythematous plaques.
Eroded and crusted plaques in right groin at base Eroded and crusted plaques in right groin at base of penis.
Erythema of scrotum and erosive plaques in left gr Erythema of scrotum and erosive plaques in left groin (simulated intertrigo).
Central groin with yellow crust over cleaned plaqu Central groin with yellow crust over cleaned plaques.

Burning and itching accompany the eruption, and a malodorous drainage occurs in some cases as a result of secondary infection. Symptoms related to staphylococcal and candidal overgrowth are common in familial benign pemphigus. Multiple asymptomatic longitudinal white bands on the fingernails also have been described. Involvement of mucosa is rare. The characteristic clinical appearance of familial benign pemphigus, as well as biopsy findings, readily confirms the diagnosis.

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Complications

Cellulitis, abscess formation, scarring, and depression concerning the chronic and refractory nature of the skin condition are possible complications of familial benign pemphigus.

Systemic corticosteroid therapy may result in the adverse effects of steroids, such as osteoporosis, cataracts, striae, ulcers, and others.

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