• Indian J Dermatol Venereol Leprol · May 2011

    Case Reports

    Congenital generalized lipodystrophy of Berardinelli-Seip type: a rare case.

    • Sujay Khandpur, Amrendra Kumar, and Rajesh Khadgawat.
    • Department of Dermatology and Venereology, All India Institute of Medical Sciences, New Delhi, India. sujay_khandpur@yahoo.com
    • Indian J Dermatol Venereol Leprol. 2011 May 1; 77 (3): 402.

    AbstractCongenital generalized lipodystrophy of Berardinelli-Seip type is a rare autosomal recessive disorder characterized by nearly complete absence of adipose tissue and a consequent generalized muscular appearance, which is recognized easily at birth. The condition is associated with various dermatological and systemic manifestations. We report a case of this form of lipodystrophy. The patient had several cutaneous manifestations, including severe acanthosis nigricans, generalized hyperpigmentation, curly scalp hair, prominent subcutaneous veins, and enlarged clitoris. She also had associated celiac disease.

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