Bloch-sulzberger syndrome (incontinentia Pigmenti): a case report.
Keywords:
Bloch-Sulzberger syndrome in continentia pigmenti, X-linked, Genetic diseases, pigmentation disorders.Abstract
Incontinentia pigmenti (IP) is a X-linked dominant neuro cutaneous syndrome with cutaneous, neurologic, ophthalmologic and dental manifestations mainly in female neonates. Starting from neonatal period, IP passes through stages of vesicular (90%), verrucous (70%), hyper pigmented (98%) and hypopigmented lesions. Authors report a case of IP in 8 months old female child who presented with vesiculo bullous skin eruption and verrucous lesions on hand and feet. The diagnosis was confirmed on histopathology. Variable pattern of lesions presenting over a period of time makes it difficult to diagnose. As disease is incurable, genetic counseling is of paramount importance.
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