I. Overview Infantile abdominal distal lipodystrophy is a restrictive skin and subcutaneous fat atrophy that occurs in the abdomen and groin of infants, with an unknown etiology. There is no family history. The age of onset is not limited to infants, but there are cases of onset after 2 years of age, therefore, Lee et al. suggested that it should be renamed as juvenile abdominal distal lipodystrophy. The etiology of this disease is unknown. Clinical manifestations The abdomen is initially a light purple-blue patch, gradually becoming dark red with clear boundaries, atrophic depression on the surface, surrounded by a red halo, and eccentrically enlarged. It may spread to most of the abdomen or groin, and even extend to the chest and back. However, the face, neck and upper and lower extremities are often not involved. It is more common in infants or within 9 years of age, with the majority of cases occurring at 2 years of age. The course of the disease is slow and tends to stop after 9 years of age. Lack of systemic symptoms and self-conscious symptoms. Histopathology: atrophy and thinning of epidermis, reduction of dermal collagen fibers, no degeneration, loss of subcutaneous fat, mild inflammatory reaction. V. Diagnosis Based on clinical manifestations, lesion characteristics, and histopathological characteristics, the diagnosis can be made. Differential diagnosis Based on the clinical features, age range, pathological tissue atrophy changes, it can be distinguished from patchy atrophy, progressive idiopathic atrophy and Gowers’ total atrophy. VII. Treatment No special treatment. Symptomatic treatment. Prognosis The course of the disease is slow, with a tendency to stop development after 9 years of age.