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Revista Cubana de Pediatría

Print version ISSN 0034-7531On-line version ISSN 1561-3119

Abstract

GARCIA GARCIA, Alina et al. Clinical review of 28 cases of pigmentary incontinence. Rev Cubana Pediatr [online]. 2010, vol.82, n.3, pp.20-27. ISSN 0034-7531.

INTRODUCTION. The pigmentary incontinence is an uncommon genodermatosis usually present in female sex characterized by alterations in derivatives of superficial ectoderm and of neuroectoderm. The aim of present research was to summarize the clinical features of this entity. METHODS. Twenty eight medical records from children seen in the clinical genetics consultation of "William Soler" Children Hospital (Ciudad de La Habana) diagnosed with pigmentary incontinence. From the medical records we got the data on onset and course of this disease, as well as the information offered by inter-consultations of some specialties including Dermatology, Stomatology and Neurology. When it was possible a clinical re-assessment of involved was carried out. RESULTS. Only 1 of the 28 patients was of male sex. Among the first lesions detected were the presence of vesicles in 13 cases (43,3%), erythema in 2 cases and epidermis shedding in 2 cases. There were hyperchromic maculae in 27 children (96,6%), hypochromic in only one and verrucous type in three. Lesions were distributed in lower extremities in 22 cases (73,3%) in upper extremities and thorax in 19 cases (63,3%) and in 21 patients (70%) were bilateral. Regarding the skin annexes there was alopecia in 3 cases (10%), hypodontia in 8 cases (26,6%) and nails dystrophy in 3 cases (10%), as well as blue sclera in 6 cases (20%) and strabismus in 5 cases (16,6%). As an expression of damage in the central nervous system there was mental retardation in 12 cases (40%) and convulsions in 6 cases (20%). CONCLUSIONS. The pigmentary incontinence is a heterogeneous entity from the clinical point of view but it is possible its recognition due to skin alterations to go through foreseeable stages. It is necessary to take into account that it could be masked by the skin color or by the moment of physical examination of patient.

Keywords : Pigmentary incontinence; ectodermal dysplasia; genodermatosis.

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