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Deprecated: Implicit conversion from float 209.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 An+Bras+Dermatol 2014 ; 89 (3): 486-9 Nephropedia Template TP
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Incontinentia pigmenti or Bloch-Sulzberger syndrome: a rare X-linked genodermatosis* #MMPMID24937825
Marques GF; Tonello CS; Sousa JMP
An Bras Dermatol 2014[May]; 89 (3): 486-9 PMID24937825show ga
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.