Indian Journal of Dermatology
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CASE REPORT
Year : 2013  |  Volume : 58  |  Issue : 3  |  Page : 232-234

Encephalocraniocutaneous lipomatosis (Haberland syndrome): A case report and review of literature


1 Department of Pathology, NRI Medical College and General Hospital, Guntur District, Andhra Pradesh, India
2 Department of Pediatrics, NRI Medical College and General Hospital, Guntur District, Andhra Pradesh, India
3 Department of Radiology, NRI Medical College and General Hospital, Guntur District, Andhra Pradesh, India
4 Department of Neurology, NRI Medical College and General Hospital, Guntur District, Andhra Pradesh, India

Correspondence Address:
Kalyan Koti
Department of Pathology, NRI Medical College and General Hospital, Chinakakani, Mangalagiri Mandal, Guntur District, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.110835

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Encephalocraniocutaneous lipomatosis (ECCL) is a rare sporadic neurocutaneous syndrome characterized by presence of central nervous system, ocular and cutaneous anomalies. The exact pathogenesis is still not known. We present the third case from the Indian subcontinent, who is a five year old girl with history of right sided seizures. Dermatological examination showed alopecia on right side of the scalp and ipsilateral limbal dermoid and nodular skin tags over the upper eyelid. The computerized tomography scan of the brain revealed porencephalic cyst, cerebral calcifications and atrophy of right brain. The histopathology of the skin lesions showed lipomatous hamartoma and features of non scarring alopecia. The constellation of these findings and in adherence to the diagnostic criteria of ECCL proposed in 2009, we consider this report as a definite case of ECCL.


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