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Incontinentia pigmenti Stage 1 is not simply vesiculo-bullous but vesiculo-pustular
Authors:Tubanur Çetinarslan MD  Regina Fölster-Holst MD  PhD  Dirk Van Gysel MD  PhD  Matthias Buchner MD  Rudolf Happle MD  PhD
Affiliation:1. Department of Dermatology, Manisa Celal Bayar University, Manisa, Turkey;2. Department of Dermatology, Venereology and Allergology, Universitätsklinikum Schleswig-Holstein, Campus Kiel, Kiel, Germany;3. Department of Pediatrics, OLV Hospital Aalst, Aalst, Belgium;4. Department of Dermatology, Medical Center – University of Freiburg, Freiburg, Germany
Abstract:
Incontinentia pigmenti (IP) is a rare X-linked dominant, male-lethal disorder characterized by pathognomic skin lesions. As described in the literature the typical cutaneous changes follow the pattern of Blaschko's lines and develop in four stages that usually start at birth. Stage 1 is called vesicular, bullous or inflammatory. The vesicles are rapidly filled with eosinophils and thus turn into pustules. Thus, the term “pustular” is relevant to the first phase of IP, and the stage can be considered as “vesiculopustular/inflammatory” to be more precise than “vesicular” or “bullous.”
Keywords:incontinentia pigmenti  inflammatory  newborn  pustule  Stage 1  vesiculopustular
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