Dados do Trabalho

Título

PIGMENTARY INCONTINENCE (OR BLOCH-SULZBERGER SYNDROME): A CASE REPORT IN A FEMALE INFANT WITH EPILEPSY

Apresentação do caso

Patient L.V.M.B., female, 3 months old, born and resident in Frutal-MG. She was admitted to the Pediatric Emergency Room of the Hospital de Clínicas da UFTM, referred from the Hospital in the city of origin, due to an unprecedented convulsive crisis 1 day ago, which was characterized by spastic movements in the topography of the hemiface on the right (right eye and traction of the labial commissure) and which was preceded by hyporexia and irritability, according to the mother's report on admission.
The patient underwent physical examination of all segments, but changes were only observed in the dermatological examination. According to the mother's report, the child had skin changes since birth, but initially it was a mild condition composed of small hyperchromic papules and vesicles located on the upper limbs. However, there was a progressive worsening of the lesions, mainly on the 7th day after birth, with the appearance of bubbles and grouped vesicles with an erythematous base and a yellowish center, in the upper and lower limbs, face and scalp, predominantly in the left hemibody (see images 1- 4). At that moment, the patient was admitted to the Hospital of the city of origin with suspicion of Impetigo, having been treated with antibiotic therapy and after 4 days, the blisters ruptured spontaneously and the patient was discharged with antibiotic therapy at home. However, the mother reports that the patient showed a worsening in the number and extent of the lesions and they progressed to the stage presented at admission. Pathological examination then revealed spongiotic dermatitis with eosinophilic exocytosis and melanophages in the superficial dermis, which is characteristic of Incontinence Pigmenti. Histopathological findings can be seen on image 12 (hematoxylin-eosin stain, 40X magnification) and on images 13 and 14 (hematoxylin-eosin stain, 100X magnification).

Discussão

The reported case brings to light the discussion about Pigmentary Incontinence (or Bloch-Sulzberger Syndrome) which is an X-linked dominant genodermatosis. diagnosis. Therefore, the objective of this case was to show that the general pediatrician or general practitioner are usually the first professionals to come across this patient. Therefore, these professionals need to know the IP to include it among the differential diagnoses of vesicobullous lesions in childhood and differential diagnoses of epilepsy.

Comentários finais

multisystem involvement, the management is multidisciplinary

Referências (se houver)

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Declaração de conflito de interesses de TODOS os autores

Anna Rita Barcelos Martin, the author responsible for submitting the manuscript entitled (PIGMENTARY INCONTINENCE (OR BLOCH-SULZBERGER SYNDROME): A CASE REPORT IN A FEMALE INFANT WITH EPILEPSY) and all the co-authors presented here, declare that “WE DO NOT HAVE”, CONFLICT OF INTEREST