[Incontinentia pigmenti (Block-Sulzberger syndrome) associated with congenital retinal fold--a long-term 18-year observation].
Report on a case of incontinentia pigmenti presenting with the main characteristics features: patchy hyperpigmentation of the skin, alopecia and dental anomalies. In addition to the rare hyperpigmentation of the lower eyelids, hemiatrophy of the right face and horizontal nystagmus were found. Eye involvement consisted of a congenital retinal fold with retinal vessels draen into and bordered by pigment clumps.--When the patient with 14 total retinal detachment associated with a totally liquefied vitreous occurred and surgical reattachment failed. As a rarity in this case, the full picture of retrolental fibroplasia was not present; it was possible to follow developments over a period of 18 years.
D000505:Alopecia / Q000150:complications; D005150:Facial Hemiatrophy / Q000150:complications / Q000473:pathology; D005260:Female; D006801:Humans; D007223:Infant; D008137:Longitudinal Studies; D009759:Nystagmus, Pathologic / Q000150:complications; D010859:Pigmentation Disorders / Q000150:complications* / Q000473:pathology; D012160:Retina / Q000002:abnormalities* / Q000473:pathology; D013577:Syndrome
Incontinentia pigmenti (Bloch-Sulzberger Syndrom) mit Ablatio falciformis congenita--eine Langzeitbeobachtung über 18 Jahre.