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副甲状腺機能低下は血清無機燐の上昇と同時に血清カルシウムの低下を来し,その疾患としては,特発性副甲状腺機能低下症,仮性副甲状腺機能低下症(Albright症候群),続発性副甲状腺機能低下症(最も多く甲状腺疾患における甲状腺摘出後の発症1,2))があげられている.この内,特発性副甲状腺機能低下症(以下IdH)は,本態不明な比較的まれな疾患であるとされており,下記のごとく規定される3a).1)低Ca血症の原因となるクル病及び骨軟化症がレ線上証明されないこと,2)慢性テタニーの存在,3)腎機能障害,脂肪性下痢症,慢性下痢症,Alkalosisの存在しないこと,4)副甲状腺ホルモン注射により尿中燐の著明な排泄増加をきたすこと.
我々は最近,数年来癲癇として治療されていた患者で,昨年発作の頻発した頃より,脱毛と共に,顔面を除くほとんど全身の瘙痒を伴う紅斑,びらん,落屑性の著明な皮膚症状を随伴した本症の1症例に遭遇した.本邦におけるIdHの皮膚科的詳細な記載例は,ほとんどみられず,また,本症例のごとき著明な皮膚症状を示したIdHは海外文献にも稀有である.本例は吉野ら5)が既に内科的報告を行つているが,以下その症例につき若干の文献を参照して皮膚科的症状,病因論の研究などを主体として述べてみたい.
A 51-year-old man, who had had malaria in the past and had cataract, coxitis deformans and epilepsy was treated as epilepsy for about 7 years. Last spring, when the patient suffered from frequent attacks, he developed pruriginous, exfoliative dermatitis-like lesion over the entire body, except the face, and depilation of scalp and axillar hairs and roughness of the nails.
Idiopathic parathyroid hypofunction was confirmed by the laboratory examinations.
With the administration of vitamine D2 and gluconic acid calcium, the seizures disappeared on the next day and skin condition improved rapidly, leaving slight degree of pigmentation. Scalp and axillar hairs regenerated.
Whereas dryness and roughness of the skin, depilation and change of the nail are the usual skin manifestations of the disease, the case developed such remarkable skin lesion is the first one in Japan. Only 3 cases of the disease are collected in the literature.
Current concept upon the etiology of the disease is obscure, however, recent investigation favours the view of organ specific autoimmun disease.
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