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S.M. Rajpara, C.M. King, Hailey–Hailey disease responsive to topical calcitriol, British Journal of Dermatology, Volume 152, Issue 4, 1 April 2005, Pages 816–817, https://doi.org/10.1111/j.1365-2133.2005.06489.x
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Conflicts of interest: none declared.
Sir, Hailey–Hailey disease (HHD) or familial benign chronic pemphigus is an autosomal dominant intraepidermal blistering disease which is characterized by flaccid vesicopustules, crusted erosions and expanding circinate plaques recurring in areas exposed to friction such as the axilla, groin, perineum and side of the neck.1 The treatment of HHD is often disappointing. We read with interest a case report of HHD in which 1α,24‐dihydroxyvitamin D3 (tacalcitol) was effective both clinically (in vivo) and in explant cultures (in vitro).2 We report a case of HHD which responded to topical calcitriol.
A 57‐year‐old man had had HHD since the age of 7 years. The groin and scrotal areas were involved, and were responsive to treatment until 18 months previously, when the disease extended to the axillae and the neck. Control was initially achieved with intermittent systemic flucloxacillin and a topical steroid and antibacterial combination (betamethasone valerate + fusidic acid, Fucibet® cream; Leo, Princes Risborough, U.K.), but this was lost following a fall in which he sustained soft tissue injuries to the ankles and lumbar spine and was hospitalized for a month. Examination showed inflammation, exudation and crusting involving the groin, axillae (Fig. 1a) and neck, which we attributed to prolonged bed rest and immobilization leading to increased skin friction in those areas. An incisional biopsy taken from the groin showed epidermal acanthosis with hyperkeratosis, parakeratosis and suprabasilar clefting, confirming the diagnosis of HHD. He was treated with a course of systemic flucloxacillin 500 mg four times daily for 7 days, and potent topical steroids with various combinations of antibacterials and antifungals for about 4 weeks. The response to treatment was poor. Calcitriol ointment 3 μg g−1 was gradually introduced as a replacement. The twice‐daily application was well tolerated and nonirritant. The crusting, exudation and weeping improved within days and there was some residual erythema and postinflammatory hyperpigmentation left after 4 weeks of treatment. His condition remained in virtual remission at follow up after 3 months (Fig. 1b) with the occasional application of calcitriol.
