Incontinentia pigmenti Stage 1 is not simply vesiculo-bullous but vesiculo-pustular
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Date
2024
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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.”. © 2023 Wiley Periodicals LLC.
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Keywords
Blister , Eosinophils , Humans , Incontinentia Pigmenti , Infant, Newborn , Male , Rare Diseases , Skin , acne , acrodermatitis continua , Article , bullous skin disease , candidiasis , central nervous system , congenital syphilis , disease exacerbation , eosinophil , epidermolysis bullosa , eye , gene , gene mutation , genodermatosis , herpes simplex , histopathology , human , IKBKG gene , impetigo , incontinentia pigmenti , inflammation , Langerhans cell histiocytosis , nail , newborn , pustule , scabies , skin , skin defect , staging , Staphylococcus infection , tooth , vesiculopustule , X chromosome linked disorder , blister , incontinentia pigmenti , male , pathology , rare disease