Treatment of Hailey-Hailey disease with tacrolimus ointment and clobetasol propionate foam

Journal of Drugs in Dermatology, March-April, 2004 by Saleem A. Umar, Pradip Bhattacharjee, Robert T. Brodell

Abstract

Hailey-Hailey disease, or familial benign chronic pemphigus, is a chronic disease without a known cure. Current therapeutic strategies attempt to suppress Hailey-Hailey outbreaks and allow the patient to live comfortably with this condition. We have found that applying topical tacrolimus 0.1% ointment (Protopic[R]) twice a day to affected areas is an excellent way to control Hailey-Hailey disease. In addition to effectively controlling Hailey-Hailey outbreaks, tacrolimus is a relatively safe and noninvasive mode of treatment, without significant side effects. We recommend intermittent therapy with clobetasol propionate 0.05% foam (Olux Foam[R]) for patients who break through suppressive therapy with tacrolimus a few times per year. In patients with frequent outbreaks of Hailey-Hailey disease despite suppressive therapy with tacrolimus, we recommend alternating the tacrolimus with clobetasol propionate 0.05% foam every 6 weeks.

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Introduction

Although there is no definitive cure for Hailey-Hailey disease, many types of treatments have been employed to control disease outbreaks. Antibiotics, super-potent topical corticosteroids, systemic corticosteroids, immunosuppressants (13), dermabrasion (21,22). C[O.sub.2] laser therapy (23-26), and surgical exciasion (27,29) are a few recommended treatment modalities which have been proposed in the literature. We report two cases of recalcitrant Hailey-Hailey disease which were controlled using tacrolimus 0.1% ointment (Protopic[R], Fujisawa Healthcare. Inc., Deerfield. IL. USA) and clobetasol propionate 0.05% foam (Olux Foam[R]. Conneties Corporation. Palo Alto. CA. USA). In both cases. many forms of treatment had been utilized previously, but produced inconsistent results. This sequential regimen for treating Hailey-Hailey disease is not only effective in maintaining stable control over lesional outbreaks, but it is relatively safe from unwanted side effects.

Case 1

A 46-year-old African American male presented for evaluation of waxing and waning rashes in his axillae and groin over a period of 10 years. A positive fungal culture growing Trichophyton rubrum had been found at one point in the distant past. The condition tended to be worse in the summer season. Treatment with a variety of topical antifungal agents led to inconsistent responses. Clotrimazole-betamethasone dipropionate cream (Lotrisone[R]) was found to work better than other topical agents.

Physical examination revealed moist erythema, scaling, and hyperpigmentation with some honey-colored crusting in his axillae (Figure 1) as well as in the deep folds of his groin.

A 4 mm punch biopsy was performed, and histologic examination revealed a hyperplastic epidermis with foci of acantholytic cells (Figure 2). There were prominent suprabasalar clefts, edema within the papillary dermis and a superficial chronic perivascular inflammatory infiltrate. PAS stain was negative for fungal organisms. The presence of this histologic pattern in a clinical setting, that included chronic involvement of intertriginous areas with sparing of mucous membranes, led to the diagnosis of Hailey-Hailey disease (benign familial chronic pemphigus).

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The patient's condition was noted to wax and wane with inconsistent improvement utilizing a variety of topical steroid agents. Eventually we found that alternating between tacrolimus 0.1% ointment (Protopic[R]) and clobetasol propionate 0.05% foam (Olux Foam[R]) for a period of days to weeks for breakthrough flares led to the best control (Figure 3). The tacrolimus ointment was applied 2 times per day. The clobetasol propionate 0.05% foam was applied sparingly to active lesions 2-3 times per day to bring flares under control, and then control was maintained with monotherapy using only tacrolimus ointment.

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Case 2

A 50-year-old Caucasian female presented for evaluation of a rash in the folds of her groin area. This rash had been present since the age of 18 years, with the diagnosis of Hailey-Hailey disease confirmed by skin biopsy which showed broad areas of acantholysis and underlying chronic inflammation. The patient improved consistently with oral prednisone, but side effects including weight gain, purpura, fragile skin, and acne limited its use.

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Physical examination revealed erythematous papulo-vesicular patches bilaterally in the folds of her groin measuring 3-4 cm in diameter (Figure 4).

The patient's condition was controlled with a variety of topical steroids for several years. Clobetasol propionate 0.05% foam (Olux Foam[R]) worked most effectively of all the steroids utilized (Figure 5). To decrease the risk of topical steroid side effects, we used tacrolimus 0.1% ointment (Protopic[R]) two times per day for six week periods alternating with the clobetasol propionate foam for six week periods. During the latter cycle, clobetasol propionate foam was applied as little as two times per week to maintain control.

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