Hailey-Hailey Disease

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Overview

Hailey-Hailey disease (HHD), also called benign familial pemphigus, is an autosomal-dominant disorder characterized by a defect in keratinocyte adhesion. HHD most frequently involves intertriginous areas, where exacerbations are triggered by heat, sweating, friction, and/or secondary infections. Lesions also affect the chest and back, but these lesions tend to be easier to control. Hence, the therapeutic strategy is to minimize and/or control all of these provocative factors.

First Steps

1. The following method of care for intertriginous areas is helpful:
   Apply an astringent, either aluminum chloride 6% in a roll-on preparation twice daily or for more severe cases, aluminum chloride 20% solution once daily. These agents are in alcohol vehicles and will burn if there is extensive fissuring of the lesions.
2. Immediately after application of astringent, apply clindamycin 1% solution. Mupirocin is in an ointment base and is less acceptable in the intertriginous areas.
3. After both astringent and topical antibiotic applications, apply an absorbent powder.
4. Most acute flares are associated with secondary infection with S. aureus. If secondary infection is apparent and/or if cultures reveal the presence of pathogenic bacteria, administer oral antibiotics, based on the results of bacterial sensitivities. cephalexin 500 mg 3 times daily, doxycycline 100 mg twice daily, TMP/SMZ DS twice daily, and clindamycin 300 mg twice daily are all options. The duration of treatment should be at least 7-10 days, and often must be longer.
5. Nystatin ointment may be applied to control candidal overgrowth, which may be a contributing factor.
6. Apply a superpotent topical steroid in a cream or foam base twice daily.

Alternative Steps

1. Tacrolimus ointment 0.1% may be used instead of topical steroids twice daily. This may be safely used for long periods in the groin and axilla, with intermittent pulses of potent topical steroids for breakthroughs. Burning may occur, and may be ameliorated by a brief course of potent topical steroids prior to instituting the tacrolimus.
2. Calcipotriene ointment 0.005% applied twice daily can be used on plaques on the trunk. Irritation may limit use in intertriginous areas.

Subsequent Steps

1. Overgrowth with Candida albicans may complicate aggressive antibiotic treatment. Fluconazole 100-200mg daily may be required to control this complication.
2. Relapses with recurrent colonization/infection with S. aureus may be prevented by including 5-10 days of rifampin 600 mg/day with the antibiotic regimens outlined above.
3. Botulinum toxin (BT) (dose for BTXA 50 units/axilla and 50-100 units/groin) will markedly reduce intertriginous sweating. This can lead to a prolonged remission (from a few months up to a year or more) of HHD. BTX is as effective as laser procedures for the short term (first year) and can be used repeatedly to maintain control.
4. Laser ablation with erbium: YAG or short pulsed CO2 laser can produce prolonged remissions (up to years) of refractory areas of HHD. This therapy is particularly useful for lesions in the axillae, buttocks cleft, and groin. Surgical excision and possibly photodynamic therapy can produce similar results.
5. For very severe cases, systemic corticosteroids (1 mg/kg/day prednisone) can lead to improvement. Cyclosporine A 5mg/kg/day is an alternative. These are not long-term management strategies, but they are useful to treat severe flares.
6. Systemic retinoids, methotrexate, and dapsone, will at times be useful in refractory disease, especially if generalized. Their use has largely been replaced by the use of BTX and cyclosporine.

Pitfalls

1. Prophylactic systemic antibiotics should be avoided to minimize the emergence of resistant strains.
2. Resident microflora also can develop resistance to topical antibiotics. Hence, if long-term topical antibiotics are employed, clindamycin resistance may occur.
3. Although HHD is classified with the disorders of cornification, such as Darier's disease and the ichthyoses, it is not helped and even may be exacerbated by systemic retinoids.
4. Cyclosporine A and systemic steroids can have significant side effects when used for periods longer than several months. These prolonged courses should be avoided in HHD, as alternative agents are available.
5. Dapsone can produce acute hemolysis in patients who are G6PD deficient; therefore, blood serum for enzyme activity must be obtained prior to initiating therapy.
6. Do not use cornstarch powder. Cornstarch is a nutritious substrate for microbes; hence, cornstarch powder has no place in modern topical therapy.

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