Familial Benign Pemphigus (Hailey-Hailey Disease) Treatment & Management

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

Medical Care

Familial benign pemphigus waxes and wanes in intensity. Soothing compresses (aluminum acetate 1:40 dilution) followed by intermittent use of mild corticosteroid preparations (class V or class VI corticosteroids) and topical antibiotics (clindamycin or erythromycin) result in transient improvement. More widespread flares of familial benign pemphigus may require systemic antibiotics to suppress protease activation and acantholysis. Erythromycin and tetracycline are favored. Bacterial culture and sensitivity can help guide appropriate therapy.

In patients with refractory cases of familial benign pemphigus (Hailey-Hailey disease), dapsone, systemic corticosteroids, methotrexate (MTX), retinoids (isotretinoin or acitretin), [9] and etretinate have been tried and have been reported to be of value in some anecdotal reports.

Most patients with familial benign pemphigus at the author's institution respond well to anti-infective therapy and short courses of corticosteroids, and other immunosuppressive agents have only rarely been helpful in the author's experience. Topical tacrolimus ointment has been a valuable addition to the treatment regimen and has been able to control familial benign pemphigus well, even without the adjunctive use of topical corticosteroids.

Most experts believe that the judicious use of topical corticosteroids should be first-line therapy and that topical calcineurin inhibitors can be a second-line option or used when significant amounts of topical corticosteroids are needed in sensitive body-fold areas. [10] The safety of long-term topical calcineurin inhibitor use for this purpose has not been established and close monitoring of patients is advised.

Topical tacrolimus ointment has been found to be helpful in familial benign pemphigus, [11] and photodynamic therapy with 5-aminolevulinic acid has been used for recalcitrant cases. [12]

Reports [13, 14, 15] indicate that low-dose botulinum toxin type A injection may be of benefit for familial benign pemphigus. Control of hyperhidrosis, which aggravates familial benign pemphigus (Hailey-Hailey disease), may be the mechanism for this off-label, novel approach. Remissions of up to at least 12 months have been achieved using only botulinum A toxin injection. Only a few isolated reports have been published, [13, 14] and further work is needed to explore the benefits of this off-label procedure.

Oral glycopyrrolate has also been used to treat familial benign pemphigus and is thought to work by controlling sweating. This systemic anticholinergic agent has a short half-life and is thought to have fewer central nervous system–related anticholinergic adverse effects when compared with other systemic anticholinergic agents. [16]

Isolated reports of oral acitretin [17] therapy or intramuscular alefacept [18] leading to improvement in familial benign pemphigus warrant further study. A recent report suggets that topical cadexomer iodine powder may be of value. [19] Also, a recent case report documented the success of narrow-band ultraviolet B phototherapy in a patient who was unimproved on various topical treatments, oral prednisone and etretinate. [20] A single case report of remission induced by multiple treatments of long-pulsed alexandrite laser brings additional promise of potential long-term control, though many more studies are needed. [21]

Topical tacrolimus and 50% zinc oxide paste may be of value. [22]

Oral tacrolimus along with intralesional botulinum toxin type A (100 U diluted with 5 mL of bacteriostatic saline per cm2) has also been of benefit in a 2015 report. [23]

Next:

Surgical Care

Dermabrasion, carbon dioxide laser ablation, and pulsed dye laser therapy have been tried in the treatment of familial benign pemphigus, with variable success. [24, 25, 26] Photodynamic therapy does not seem to be of value. [27]

Previous
Next:

Diet

To help minimize friction, it is recommended that patients with familial benign pemphigus maintain their weight at appropriate levels.

Previous
Next:

Activity

Instruct patients with familial benign pemphigus (Hailey-Hailey disease) to select cool and comfortable clothing that reduces heat, moisture, and friction. Patients should avoid fabrics or clothing styles that rub or irritate affected areas. Washing new shirts may soften the collars. In some cases, pain may limit physical activities.

Previous
Next:

Prevention

Maintaining a healthy weight and keeping the body folds cool and dry as much as possible help prevent flares of familial benign pemphigus.

Previous
Next:

Long-Term Monitoring

Almost all patients with familial benign pemphigus can be treated successfully on an outpatient basis, but many patients may need to be excused temporarily from manual labor so that affected body folds can heal promptly under treatment.

Familial benign pemphigus patients require regular evaluation to be sure that secondary infection is brought under control and that the adverse effects of topical corticosteroids (eg, cutaneous atrophy) are avoided.

Individuals who receive intermittent courses of systemic corticosteroids should be evaluated for possible decrease in bone density and should be instructed regarding diet and therapy that may maintain bone density.

One report describes squamous cell carcinoma arising in the setting of familial benign pemphigus. Biopsy specimens should be taken from any suspicious infiltrated areas.

Previous