Progressive symmetrical erythrokeratoderma on the face: A rare condition and successful treatment with calcipotriol.

EKV: erythrokeratoderma variabilis PSEK: progressive symmetrical erythrokeratoderma INTRODUCTION Erythrokeratodermas are rare genodermatoses that are characterized by well-defined erythematous, hyperkeratotic plaques. They are categorized into 2 main types: progressive symmetrical erythrokeratoderma (PSEK) and erythrokeratoderma variabilis (EKV). Because their histologic findings are very similar, they have to be differentiated clinically. PSEK, or Gottron syndrome, was first described by Darier in 1911 but takes its name from an article published in 1922 by Gottron. It is characterized by symmetrical, nonmigratory, well-defined, erythematous, hyperkeratotic plaques or reddish orange to brownish color lesions with marked erythema at their periphery. Lesions commonly occur on the knees, elbows, hands, and feet and may rarely involve the face. The trunk is generally spared. PSEK usually appears in infancy or childhood and shows an equal sex incidence. Also, as the name suggests, symmetry in PSEK lesions is more remarkable than that in EKV lesions. PSEK shows a heterogeneous phenotypic variability. Although it shows mainly autosomal dominant inheritance, autosomal recessive and sporadic cases are also reported. The bioactive form of vitamin D3 (calcipotriol) is found to modulate epidermal proliferation and differentiation. Efficacy, tolerability, and safety of calcipotriol ointment in disorders of keratinization have been shown. In this report, we describe a rare case of PSEK and successful treatment with calcipotriol.