黒色表皮腫を伴つたPartial Lipodystrophy症例

A CASE OF PARTIAL LIPODYSTROPHY WITH ACANTHOSIS NIGRICANS

　Partial lipodystrophyは，1885年Mitchell^1）によつて初めて記載されたが，次いでBarraquer^2），Shaw^3）などにより本症の臨床像が詳述された．1911年Simons^4）は，本症を上半身贏痩および下半身肥大を特徴とする一種のTrophoncuroseと考え，Lipodystrophia progrcssivaの病名を冠した．以後欧米においては，小児期に発症することが多いこと，内分泌異常および腎障害を伴なうこと，知能障害および精神々経障害などを伴なうことなどから，小児科，内科，精神科領域での報告が多く，Progrcssive lipodystrophy，lipodystrophia pro-gressiva, Barraquer-Simons'　discase，Barraquer'sdisease，lipodystrophia facialis，cephalothoracicob-rachial form of lipodystrophy，progressive cepha-lothoracic lipodys trophy，partial lipodystrophyなど種々の名称により，200例余の報告をみたが^5），本邦においては20例にみたず^6），かなり稀な疾患と思われる．

　著者らは，下顎部と躯幹が贏痩し，頬部と四肢が正常あるいは軽度に肥満した症例を経験したので，若干の文献的考察を加えて報告する．

Woman, aged 29, who had no same disease in the relatives by blood, began to walk in 1 year and 9months of age and had infantile beriberi and chronic diarrhea. Menarchi accured in 12years of age. She noticed the emaciation of mandibule and trunk since 3 or 4years.

The patient is 149.5cm tall, weighs 40 kg and measures 76 cm around the chest. The liver is pal-pable 2 fingerbreadths. No motor dysfunction and sensory abnormality are noted.

Absence of subcutaneous fat is noted symmetrically on the horizontal line of the mouth angle, mandi-bule, neck, breast, abdomen, back and lumbar region.

Histologic examination reveals absence of subcutaneous fat tissue, but no inflammation and no change of adnexies. The lesion of axille shows a picture of acanthosis nigricans.

A classification of dystrophia subcutanea with reference to clinical findings is presented. Pathogenesis of the disease is considered to be the disorder of the nervous system, especially, that of the vicinity of diencep-halon.