Cutis marmorata teleangiectatica congenitaの1例

CUTIS MARMORATA TELEANGIECTATICA CONGENITA

I．緒言

　Cutis marmorata teleangiectatica conge-nita （以下C．m．t．c．と略す）は1922年Lohuizen^1）により稀有な先天性皮膚異常に対し初めて命名されたものである。

　以来症例を重ねるうち今日までに本症はその特異な臨床ならびに病理組織学的様相から疾患としての独立性が確認されている。本邦では1963年水野^2）の報告が最初と思われる。すなわち彼は初め先天性陥凹性網状紅斑として報告したが，後にその病像が本症に全く一致することから先駆者にならつて診断名をC．m．t．c．と訂正した。以後今日までに本邦における本症の症例報告は数える程しかなく^3）−8），原著としての報告は横関，北川^7）および浅越^8）の2論文をみるにすぎない。そしてまた浅越の記載によれば内外の症例をあわせても30例を数えるにすぎない。

A 16-year-old girl was noticed to have cutis marmorata over the entire body surface, purpura on the left part of the face and on the scalp, and erosion on both the patellar regions and around the ankles at hir birth. The two latter lesions disappeared soon.

At her first visit, cutis marmorata with telangiectases over the entire body surface except the neck, axillae, genitals, palms and soles and angiokeratoma-like eruptions around the patellar regions were noted. Some parts of the skin were atrophic and dilated veins could be seen. On both the legs there were many scars due to repeated ulcers by trauma. No subjective symptoms and no enanthemas were noted. Laboratory tests including electroencephalography were within normal limits.

Histologic specimen revealed small patchy areas of proliferated and dilated capillaries and in-creased endothelial cells were scattered throughout the dermis and subcutis, surrounded by an infiltrate of histiocytic cells and maldevelopment of the connective tissue. In the deep layer of the cutis dilated veins with a thick wall were noted.

The patient's parents are cousins. Her sisters and brothers did not have the same disorder.

Although the tendency to improvement by aging is well recognized among dermatologists, there have been no reported cases of complete cure and it is a matter of course that the disease has remained in this adult patient.