• | X-linked dominant; therefore usually lethal in males |
• | stage I – vesicular (birth to 1-2 weeks) |
• | stage II – verrucous (2 to 6 weeks) |
• | stage III – hyperpigmented (3-6 months) |
• | stage IV – hypopigmented (second to third decade) |
• | hair – scarring alopecia (30%) |
• | teeth – anodontia, peg teeth (66%) – look for in mom |
• | ddx: epidermolysis bullosa, impetigo, HSV, epidermolytic hyperkeratosis, congenital syphilis |
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