Incontinentia pigmenti is a rare X-linked genodermatosis that affects mainly female neonates. Skin manifestations are the most common and occur in four quite distinct phases. A female infant presented vesiculobullous lesions on trunk and limbs, and a verrucous lesion on the right palm. Biopsy revealed eosinophil exocytosis and pigment incontinence, confirming the clinical hypothesis. Although uncommon, incontinentia pigmenti should be taken into consideration as a possible differential diagnosis when vesiculobullous and verrucous lesions are present in childhood. Keywords: genetics; incontinentia pigmenti; X chromosome Resumo: A incontinência pigmentar é uma genodermatose rara, ligada ao X, que afeta, principalmente, neonatos do sexo feminino. As manifestações cutâneas são as mais prevalentes, e ocorrem em quatro fases bem distintas. Lactente feminina com lesões vesicobolhosas em tronco e membros e lesão verrucosa em palma. À biopsia, observou-se exocitose eosinofílica e derrame pigmentar, compatível com a suspeita clíni-ca. Apesar de incomum, a incontinência pigmentar deve ser lembrada entre os diagnósticos diferenciais, de lesões vesicobolhosas e verrucosas da infância. Palavras-chave: Cromossomo X; Genética; Incontinência pigmentar

Simultaneous occurrence of granuloma annulare and necrobiosis lipoidica is quite rare. There are seven reported cases in the literature, but only one presenting ulcerated necrobiosis lipoidica. We report a 39-year-old male with histopathologically confirmed granuloma annulare and ulcerated necrobiosis lipoidica, without diabetes mellitus.