Incontinentia pigmenti (Bloch Sulzbelger syndrome)

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Incontinentia pigmenti (IP) is a rare hereditary disease that shows X-linked dominant inheritance and effects the skin, hair, teeth, and central nervous system. Initial symptoms are usually skin and cutaneous findings and cutaneous lesions are accompanied with other systems symptoms with different intensity in approximately 80% of the patients. One-year-old girl was admitted to our outpatient clinic because of dark marks and ulcers in her body. Dermatologic examination revealed hyperpigmented macules following the lines of Blaschko on the trunk and the extremities. There was hemorrhagic, crusted lesions and in some areas hyperkeratotic papules and verrucous plaques are detected on right arm, dorsum of hand and legs. The patient was diagnosed as IP because of typical symptoms. In our patient, systemic involvement was not detected. To review the diagnosis of IP is aimed in this study.

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Incontinentia pigmenti , the girl child

Cumhuriyet Medical Journal

496

Cumhuriyet Medical Journal