(DH) is a rare, blistering skin disease described by Duhring in 1884.1 DH is strongly associated with the HLA-DQ2 phenotype, a gateway in which dietary gluten reaches inflammatory cells and stimulates an autoimmune process. The etiopathogenesis involves IgA anti-endomysial antibodies directed against tissue transglutaminase (TG); the presumed skin autoantigen is epidermal TG. IgA/TG immune complexes form locally within the papillary dermis leading to neutrophil chemotaxis and degranulation (which forms neutrophilic abscesses), proteolytic cleavage disrupting the lamina lucida, and blister subepidermal blister formation.
2Current standard-of-care for DH is oral dapsone and a gluten-free diet. We describe a teenage patient in whom resolution of lesions was achieved with adjuvant use of topical dapsone 5% gel (aczone), the first case in the literature.A 14-year-old male had been suffering recurrent eruptions of blisters on his chest and arms for 16 months. He had been diagnosed with direct immunofluorescent proven DH based on granular IgA deposition in the upper papillary dermis. Though distinguishing LABD (linear IgA bullous dermatosis) from DH is often clinically impossible, the finding of IgA in a granular pattern at the dermoepidermal junction with accentuation in the dermal papillae was specific for DH in our patient. Upon an exacerbated eruption of blisters on the chest and shoulders, the patient presented to our clinic.On physical examination he presented with multiple, welldefined, pink keratotic papules, plaques and diffuse, hypopigmented macules and patches on the chest and shoulders, equally distributed on the left and right side. Primary lesions were counted before treatment and numbered 33 on the left side and 34 on the right side of the chest, respectively.The patient did not maintain a gluten-free diet and was receiving daily oral dapsone (25 mg). Twice daily application of topical dapsone (Aczone gel, 5%) was initiated on the right side of the patient's chest and Aquaphor ointment to the left. Two physicians were blinded to which side received the medication. The patient was asked to follow up in four weeks.Over the following three days the patient's skin lesions did not improve on either side of the chest. The oral dosage of dapsone was modified to 25 mg and 50 mg on alternating days. The patient continued to apply the topical products unilaterally as originally prescribed. On a follow-up visit four weeks later, the blinded physicians observed improvement of the lesions on the right side of his chest in comparison to the left. Physical exam demonstrated two remaining ulcerated vesicles on the right chest and five on the left without signs of erosion. It was also noted that relative to the left side, the skin on the right side was significantly smoother. The patient was then allowed to expand usage of topical dapsone to all affected areas and had similar improvements.There have been no reports on treating dermatitis herpetiformis using topical dapsone and the current standard-ofcare remains oral dap...