title: Incontinentia pigmenti or Bloch-Sulzberger syndrome: a rare X-linked genodermatosis.
abstract: Incontinentia pigmenti is a rare X-linked genodermatosis that affects mainly female neonates. The first manifestation occurs in the early neonatal period and progresses through four stages: vesicular, verruciform, hyperpigmented and hypopigmented. Clinical features also manifest themselves through changes in the teeth, eyes, hair, central nervous system, bone structures, skeletal musculature and immune system. The authors report the case of a patient with cutaneous lesions and histological findings that are compatible with the vesicular stage, emphasizing the importance of early diagnosis and appropriate therapeutic management.
authors.affiliation: Instituto Lauro de Souza Lima, Bauru, SP, Brazil.
authors.forename: Gabriela Franco
authors.initials: GF
authors.lastname: Marques
authors.affiliation: Instituto Lauro de Souza Lima, Bauru, SP, Brazil.
authors.forename: Claudio Sampieri
authors.initials: CS
authors.lastname: Tonello
authors.affiliation: Instituto Lauro de Souza Lima, Bauru, SP, Brazil.
authors.forename: Juliana Martins Prazeres
authors.initials: JM
authors.lastname: Sousa
mesh_terms: D005260:Female; D040181:Genetic Diseases, X-Linked / Q000473:pathology*; D006801:Humans; D007184:Incontinentia Pigmenti / Q000473:pathology*; D007223:Infant; D035583:Rare Diseases / Q000235:genetics / Q000473:pathology*; D012872:Skin Diseases, Vesiculobullous / Q000235:genetics / Q000473:pathology