Hailey-Hailey Disease 2
A 54-year-old woman presented with a 26-year history of intermittent, malodorous, pruritic skin lesions. Several of her relatives have experienced similar rashes.
Past treatments have included oral prednisone, topical steroids, chronic oral doxycycline, and short courses of amoxicillin, cephalexin and cefuroxime, all of which temporarily improved the condition.
Physical examination revealed erythematous eroded scaly papules and plaques in the inguinal and inframammary regions. The remaining skin examination was unremarkable. What’s your diagnosis?
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A skin biopsy confirmed the diagnosis of Hailey-Hailey disease, otherwise known as “benign familial pemphigus.” This condition is inherited in an autosomal dominant fashion, and most patients (70%) have an identifiable family history.
A majority of cases arise between the second and fourth decades of life, with recurrent vesicular or crusted erythematous papules and plaques. Lesions have a predilection for intertriginous and rubbed areas, particularly the groin, axillae, neck, perineum and inframammary region. Mucous membranes are typically spared.
Factors that exacerbate or trigger eruptions include sweating, heat, friction, infection and UV radiation. Lesions arise within 24 hours of minor physical trauma.
Many patients complain of associated pruritus, while others suffer from pain or malodor of the affected areas. The course of the disease is characterized by frequent remission and recurrence, and many individuals experience relapse at the onset of summer.
Suspicion is raised by the presence of characteristic lesions and a positive family history. A skin biopsy can confirm the diagnosis by demonstrating acantholysis of keratinocytes above the basal layer in a “dilapidated brick wall” pattern.
Hailey-Hailey disease must be differentiated from chronic fungal or bacterial infections, including candidal intertrigo. Pemphigus vulgaris has a similar appearance but typically features more diffuse involvement. Grover’s disease and Darier’s disease are other acantholytic disorders that can produce similar lesions histologically, although these conditions typically have different distributions and morphologies. Contact dermatitis should also be considered in the differential diagnosis.
The overall prognosis of Hailey-Hailey disease is good. Many patients experience improvement in their condition as they age, although some patients may worsen with time.1
Medical therapy, both topical and systemic, provides temporary suppression of the disease but no cure. Although the role of infection in the pathogenesis of Hailey-Hailey disease remains controversial, many patients improve with antibiotic treatment. Antibiotics (topical or systemic) and antiseptics also reduce malodor associated with the lesions.
First-line therapies include topical corticosteroids or antibiotics and, if needed, systemic corticosteroid tapers or oral antibiotics (e.g., penicillins, tetracycline, or minocycline).2
Some patients can be controlled with long-term, low-dose oral antibiotics. Anecdotally, recalcitrant Hailey-Hailey may improve with oral dapsone, methotrexate, retinoids or cyclosporine. Patients with limited disease have been treated with surgical excision and closure or grafting, but recurrences may arise in the periphery of treated areas.
Treatment with carbon dioxide laser ablation and dermabrasion has also been reported.3,4 Surgical measures may alleviate the condition by reducing sweating in the treated skin, although the mechanism by which improvement occurs is not entirely known.
Case reports have shown that chemodenervation of the sweat glands with botulinum toxin type A has improved the course of Hailey-Hailey disease.5
Our patient was able to control her disease with intermittent topical triamcinolone cream. She was instructed to avoid sweating or activities that would cause friction of the affected areas. Oral antibiotics ameliorated occasional flares.
Tricia J. Brown, MD, practices dermatology at Methodist Willowbrook Hospital in Houston, Texas. Raymond L. Cornelison Jr., MD, is professor and chairman of the department of dermatology at the University of Oklahoma Health Sciences Center in Oklahoma City.
2. Galimberti RLet al. Int J Dermatol. 1988. 27:495.