A case of macrodystrophia lipomatosa of 5th digit of hand with literature review

Macrodystrophia lipomatosa (ML), a rare congenital disorder, is considered by some to be a localized form of Proteus syndrome. A 12year old girl presented with progressive enlargement of little finger of right hand since birth. Magnetic resonance imaging showed unencapsulated excessive fibroadipose tissue infiltrating interosseous muscles. Partial debulking of the lipomatous tissues was performed. Microscopic examination of the specimen revealed fat deposits, ligamentous, and neural tissue elements in normal structure. We report a case of atypical ML involving 5th digit with normal osseous elements.