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1. 常染色体劣性先天性魚鱗癬autosomal recessive congenital ichthyosis [show details]
… Examination of the hair frequently reveals trichorrhexis invaginata, which is visible using light microscopy as a bamboo-like configuration in which the distal hair shaft invaginates… abnormal hair, and increased risk of atopy. Scale can assume a characteristic pattern called ichthyosis linearis circumflexa,…
2. 寄生虫妄想：疫学、臨床症状、評価、診断delusional infestation epidemiology clinical presentation assessment and diagnosis [show details]
… symptoms such as apathy and amotivation. Some experts, but not all, consider the coexistence of phobias and hypochondriasis with delusions of infestation as secondary delusional infestation . The differential…
3. ゴーシェ病：初期評価、モニタリング、および臨床経過gaucher disease initial assessment monitoring and clinical course [show details]
…bruising, petechiae, pallor, and increased pigmentation. If examining a neonate, specifically look for ichthyosis. Eye examination for pingueculae (small, soft, brownish patches of tissue on the sclerae, just … Annual monitoring of protein electrophoresis and serum free light chains are recommended in adults since there is an elevated risk of myeloma.… with GD are subject to the same psychosocial problems as any child with a chronic disease (anger, fear, insecurity, isolation) . Visceromegaly, growth abnormalities, and delayed puberty may affect their …
4. 先天性魚鱗癬の概要と分類overview and classification of the inherited ichthyoses [show details]
… increased skin markings or hyperlinearity Keratosis pilaris (keratotic elevations around the hair follicles), most prominent on the upper arms and legs, is present in many patients. There is a marked seasonal … hair abnormalities (eg, Netherton syndrome), or neurologic involvement (eg, Sjogren-Larsson syndrome, Refsum disease). The diagnosis of ichthyosis is based upon the…
5. 内臓悪性腫瘍の皮膚症状cutaneous manifestations of internal malignancy [show details]
… Ichthyosis is a generalized scaling of the skin that can be either congenital or acquired. Acquired ichthyosis can be associated with inflammatory,… appearance of fine hair growth, which is predominantly localized to the head and neck but may spread to the torso, arms, and legs . A strong association of this unusual hair growth has been… fragments of monoclonal light chains with or without heavy chains are deposited in the kidney and other tissues,…

「魚鱗癬」

　　[★]

英: ichthyosis
関: 角化症

概念

角質層の形成と剥脱機構に異常が生じ、結果として角質層の異常堆積が起こり、皮膚表面が魚のウロコ状を呈する疾患群

魚鱗癬

遺伝性の魚鱗癬

内臓病変を随伴する魚鱗癬（魚鱗癬症候群）

後天性魚鱗癬

遺伝性の魚鱗癬 (NDE.229)

		尋常性魚鱗癬	伴性遺伝性魚鱗癬	水疱型先天性魚鱗癬様紅皮症	非水疱型先天性魚鱗癬様紅皮症，葉状魚鱗癬	道化師様魚鱗癬
頻度		しばしば	ときに	まれ	まれ	きわめてまれ
遺伝形式		常染色体優性	X染色体劣性	常染色体優性	常染色体劣性	常染色体劣性
発症時期		乳幼児	生下時～出生直後	生下時～出生直後	生下時	生下時
皮膚症状	部位	四肢，体幹（背部＞腹部）間擦部伸側＞屈側	腹部＞背部間擦部伸側＝屈側	全身	全身	全身
皮膚症状	形態	細かい鱗屑	黒褐色，大きな鱗屑	角質増殖が強い	潮紅，細かい鱗屑～暗褐色，大きな鱗屑（葉状魚鱗癬）	きわめて厚い角質，ひびわれ，眼瞼外反
病理組織		角質の肥厚と顆粒層の菲薄化	角質の肥厚，顆粒層はほぼ正常	顆粒変性	わないものがある）	著明な角質増殖
病因		フィラグリン産生低下	ステロイドスルファターゼの欠損	ケラチン遺伝子（K1/K10）の異常	一部はトランスグルタミナーゼの欠損	不明