The lipodystrophy syndromes are a heterogeneous group of syndromes characterized by selective loss of fat from various parts of the body. 1 They are classified into 2 major types: familial and acquired. The main subtypes of familial lipodystrophies are congenital generalized lipodystrophy, an autosomal recessive disorder characterized by near complete lack of metabolically active adipose tissue from birth, and familial partial lipodystrophy, Dunnigan type, an autosomal dominant disorder characterized by loss of subcutaneous fat from the extremities at puberty and excess fat accumulation in the face and neck. Patients with acquired generalized lipodystrophy have generalized loss of subcutaneous fat, but those with acquired partial lipodystrophy have fat loss limited to the face, trunk, and upper extremities. 2 (Table 1) Acquired partial lipodystrophy is a rare condition with approximately 250 described patients of various ethnic origins. 3 This variety (like acquired generalized lipodystrophy) occurs approximately 3 times more often in women, begins during childhood, and has underlying autoimmunity. 2 Case Report A 12-year-old boy presented with a history of progressive loss of fat for the last 2 years. He was a product of a non-consanguineous marriage, born at term with a birth weight of 3 kg. His development was normal and the child was asymptomatic until 10 years of age when progressive thinning of face was first noted. There was no history of fever, loss of appetite, polyuria, polydypsia, or chronic diarrhea. He had been evaluated for tuberculosis and malabsorption syndromes. There was no history of similar cases in the family. Clinical examination was unremarkable except for a marked symmetrical atrophy of fat over buccal region and temples (Images 1 and 2). His weight, height, triceps skinfold, and subscapular skinfold thickness were 35 kg, 141cm, 3.37 mm, 4.37 mm, respectively. There was no hepatomegaly. Laboratory evaluation revealed normal levels of hemoglobin, serum electrolytes, blood urea, fasting and postprandial blood sugar, liver, and the thyroid function tests (Table 2). First phase insulin response (FPIR) in intravenous glucose tolerance test: (1'+3') was 109 mU/mL with 6.93 value for K; fasting glucose/insulin ration (Go/Io) was 13.70; homeostatic model assessment (HOMA) was 1.80. The lipid profile was also normal with serum total cholesterol, high-density lipoprotein cholesterol, low-density lipoprotein cholesterol, and total triglyceride levels of 149 mg/dL, 41mg/dL, 18 mg/dL, and 90 mg/dL, respectively. The Mantoux test was negative, and urinalysis revealed no abnormality. Autoantibodies were negative. Plasma leptin level was 5 mg/mL for BMI: 20.29 kg/m². Plasma complement 3 (C 3) level was reduced to 21 mg/dL. On skin biopsy, there was a granular accumulation of C 3 in the basement membrane (Image 3). A diagnosis of partial lipodystrophy was considered because of gradual onset loss of subcutaneous fat from the face, neck, trunk, and upper extremities occurring during childhood (essential crit...
