全身性肥胖細胞症の1例

A CASE OF SYSTEMIC MASTOCYTOSIS

I．緒言

　従来色素性蕁麻疹は皮膚のみの変化と考える人が多く，皮膚以外の組織変化については，注意されることが少なかつた。リンパ節において肥胖細胞の増殖を証した報告はあつたが（Gray^1），1938），本症が全身性変化を示すと考えられるまでには至らなかつた。しかし1949年，Ellis^2）は肝，脾，胸腺，骨髄，腎，膵およびリンパ節に肥胖細胞の著明な増殖を認めた剖検例を報告して，本症が全身性疾患であることを指摘した。以後全身性変化を伴なう症例が相次いで報告され，現在では従来の色素性蕁麻疹と，全身性変化を伴なうものとを一括して，肥胖細胞の増殖を来たす疾患という意味で，肥胖細胞症Mastocytosisなる名称が広く用いられている。本邦でもすでに3例の全身性肥胖細胞症が西脇ら^3），西脇^4）および福代ら^5）によつて報告されている。

　特異な皮疹を示し，骨髄および末梢血に肥胖細胞の増殖を認めた1例を経験したので報告する。

A case of systemic mastocytosis in a 17-year-old girl was reported. Nothing contributive was found in her past and family history.

History of present illness : Small reddish papules appeared on the abdomen about the age of three, which covered the entire body surface within half a year. No bulla formation was noted and there were no subjective sensations such as itching.

Present status : On hospitalization, many reddish-brown, soft, slightly elevated, small papules covered the entire body surface, except for the scalp, palms and soles, closely and in retiform arrangement. Urtication by stroking was markedly positive on the papules. No swelling of the lymph nodes, liver, and spleen could be proved.

Laboratory findings : Hematologic studies on the peripheral blood showed normal results, except for slightly increased mast cells. The results of chemical studies on the blood scrum were within normal limits. No pathologic findings were found in roentgenograms of the bones. A marked increase (15.6%) of mast cells was revealed in the bone marrow.

Considerable increases in free histamine in the urine (447.2 μg/day) and in the skin lesions (484.2 μg/day) were proved, but there were no symptoms and signs due to the excessive histamine release.

Histopathologic specimen of the lesion showed a dense mast cell infiltrate in the upper and mid dermis.

This is the reported fourth case in Japan.