先天性的濃甲症

　　先天性的濃甲症は常染色体優性遺伝病です。Ⅰ型は角質蛋白に関連しています。16および6aの変異に関連しており、Ⅱ型は角質蛋白に関連しています。17および6bの変異に関連しています。この病気は稀な甲肥厚性の遺伝性皮膚病です。

　　先天性的濃甲症の合併症は以下のようなものがあります：

　　1、甲溝炎

　　甲溝炎は爪の周囲の皮膚皺が炎症を起こす反応で、急性または慢性の膿性、圧痛性、痛性の甲周組織の腫れを示し、爪皺の膿瘍が原因です。

　　2、爪下膿瘍

　　炎症や他の要因によって壊死した組織は、爪下の膿瘍を形成することができます。

　　3、嵌甲

　　嵌甲は夏に多く発生し、趾間、足の縁、足底に米粒大の深い水疱が現れます。

　　4、指先や趾先の壊死

　　先天性的濃甲症で阻害される部位が異なるため、発生する臨床症状や徴候も異なります。急性の阻害と慢性の阻害で発生する症状も異なりますが、それらはすべて肢端の血流不足、壊疽や壊死を引き起こすことができます。

　　Type I congenital onychogryphosis is characterized by significantly thick nails (toes), hyperkeratosis of the palm and sole, excessive sweating, and hyperkeratotic skin lesions in the elbows, knees, and buttocks. Nail changes appear after birth, but are more common within a few months after birth. Mucosal leukoplakia is more common in the tongue and mouth, occasionally involving the larynx, causing hoarseness. In addition, calluses can be seen on the plantar part of the foot, and walking can cause blisters. Palm calluses are only seen in manual workers. Other types of clinical manifestations are similar to type I, but type II is accompanied by fetal teeth and multiple sebaceous cysts; type III is accompanied by corneal leukoplakia; type IV is onset later, and hyperpigmentation can be seen in the neck, waist, axilla, thigh, buttocks, and abdomen.

　　Currently effective methods for congenital onychogryphosis include health education, family investigation, genetic counseling, malformation and risk prediction, biochemical testing, amniotic fluid and villus sampling, genetic testing, and cytogenetic analysis.

　　congenital onychogryphosis patients have significantly thick nails (toes), hyperkeratosis of the palm and sole, and pathological examination shows pigment incontinence and amyloid deposition. This disease can be diagnosed based on clinical manifestations, skin lesion characteristics, and characteristic histopathological features.

　　congenital onychogryphosis patients should eat foods rich in vitamin C and carbonated foods, and avoid high-calcium foods and foods that affect calcium deposition. The patient's diet should be light and easy to digest, with more fruits and vegetables, a reasonable diet, and attention to adequate nutrition.

　　congenital onychogryphosis treatment, nail avulsion surgery for thick nails can only temporarily relieve symptoms. Removing the nail matrix is not beneficial to the efficacy, but nail matrix and nail bed scraping surgery is a simple and effective therapy. Keratolytic agents can be applied locally, such as lactic acid wash, salicylic acid, and urea preparations. Oral retinoic acid (isotretinoin) can clear keratotic papules and mucosal leukoplakia, but it is ineffective for掌跖角化. Delayed-onset congenital onychogryphosis patients can try activetin treatment.