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Case Reports

Abstract

Galli Galli disease is a rare variant of the Dowling-Degos disease, an autosomal dominant pigmentary disorder appearing in 20-30s of age, characterized by erythematous macules and papules with reticular hyperpigmentation of the flexural sites, usually beginning in the groin and axillae, before spreading to other skin folds. Galli-Galli disease was first described in 1982 by Bardach, Gehbart, and Luger, when they observed hyperpigmented macules in two brothers. Since then, less than 70 cases have been described in literature, which may be under reported. Clinically, Dowling-Degos and Galli Galli are indistinguishable. Histopathologically, both entities exhibit hyperkeratosis, thinning of the suprapapillary epidermis, hyperpigmentation of the basal layer, and antler-like elongations of the rete ridges. Galli-galli disease additionally shows acantholysis to differentiate it from Dowling-Degos disease. Here we report a case of Galli Galli disease in a young woman in her twenties presented with a one-year history of a rough scaly papules and hyperpigmented macules appearing on her face, chest, abdomen, and thighs. The rash was associated with pruritis and worsened in the winter. The differential diagnosis was broad, but included Darier’s, Grover’s, Hailey-Hailey and possible co-existence with seborrheic dermatitis. Punch biopsy revealed epidermal hyperplasia with elongated rete ridges, basal hyperpigmentation and suprabasal acantholysis, consistent with Galli-Galli disease. The correct diagnosis is important as the patient may benefit form improvement in acantholysis with highly potent topical steroid application. We report this case to bring awareness and highlighting diagnostic features of this rare disease entity.