Ichthyosis congenita gravisの1剖検例

AN AUTOPSY CASE OF ICHTHYOSIS CONGENITA GRAVIS

　先天性魚鱗癬は，Richter^1）により1792年に初めてSingularis epidermidis deformitasと記載され，その後Riecke（1900）^2）は本症をIch-thyosis congenita（I．c．と略）と称している。このうち，重症でしかも予後絶対不良とされているものはきわめてまれであり，その名称も諸家によりI．c．gravis（I．c．gr．と略），Harlequin fetusあるいはKeratosis diffusa fetalisなどと称せられている。かかる症例の剖検例報告は，本邦では20例にも達しない。われわれは最近その1剖検を得，かつ1957年にCurt Stern^3）の報告したI．c．gr．例の剖検所見をも参照し得たので，本邦の文献上に認められたI．c．の剖検所見を要約し，若干の文献的考察を加えることとした。

A case of this disease in a premature female of 35 weeks in foetal life, was reported.

Since her birth, the skin had been generally erythematous, markedly hyperkeratotic and had many fissures. She had also ectropion, flat ala nasi, compressed ear lobes. Her mouth was left open.

She died on the 4th day after birth. Autopsy revealed generalized stasis, residual extra-medullary hematopoiesis, and thickend mucosal membrane of the mouth and vagina. No parti-cular finding in the endocrine organs.

Histologic specimen of the skin showed the characteristics of this disease-marked hyper-keratosis, acanthosis, and thinning of the granular layer.

No consanguinity nor the same disease could not be found in her family.

A review on the reported 15 cases in Japan was performed.