KEGG   DISEASE: 掌蹠角化症
エントリ  
H01673                                                             
名称    
掌蹠角化症
  下位グループ
表皮剥離性掌蹠角化症 [DS:H00722]
非表皮融解性掌蹠角化症 [DS:H00723]
線状掌蹠角化症 [DS:H00717]
点状掌蹠角化症 [DS:H01404]
難聴を伴う掌蹠角化症 [DS:H00716]
概要    
Palmoplantar keratodermas (PPK) comprise a heterogeneous group of keratinization disorders with hyperkeratotic thickening of palms and soles. Sporadic or acquired forms of PPKs and hereditary forms exist. The causes of acquired PPK vary, and include exposure to certain chemicals, side effects of certain drugs, and metabolic disorders. There is as yet no cure for hereditary PPK. In patients with acquired PPK, the cause should be treated or eliminated, if possible. In both instances, optimized treatment can lead to a significant improvement in symptoms. Topical therapy with urea-based ointments improves the skin's absorption of moisture and has keratolytic effects. Topical vitamin D therapy is another option.
カテゴリ  
先天奇形
階層分類  
ICD-11 による疾患分類 [BR:jp08403]
 14 皮膚の疾患
  特定の皮膚構造が関与する皮膚疾患
   表皮及び表皮付属器の疾患
    表皮の角化症
     ED55  掌蹠角化症
      H01673  掌蹠角化症
治療薬   
トコフェロール・ビタミンA [DR:D04805]
尿素 [DR:D00023]
タカルシトール水和物 [DR:D01472]
マキサカルシトール [DR:D01098]
エトレチナート [DR:D00316]
コメント  
Hereditary PPK are divided morphologically into four types; diffuse, focal, striate and punctate. PPK can be further subdivided histologically into epidermolytic and nonepidermolytic PPK.
リンク   
ICD-11: ED55
MeSH: D007645
OMIM: 144200
文献    
  著者
Schiller S, Seebode C, Hennies HC, Giehl K, Emmert S
  タイトル
Palmoplantar keratoderma (PPK): acquired and genetic causes of a not so rare disease.
  雑誌
J Dtsch Dermatol Ges 12:781-8 (2014)
DOI:10.1111/ddg.12418
文献    
  著者
Sakiyama T, Kubo A
  タイトル
Hereditary palmoplantar keratoderma "clinical and genetic differential diagnosis".
  雑誌
J Dermatol 43:264-74 (2016)
DOI:10.1111/1346-8138.13219
文献    
PMID:8595410
  著者
Shamsher MK, Navsaria HA, Stevens HP, Ratnavel RC, Purkis PE, Kelsell DP, McLean WH, Cook LJ, Griffiths WA, Gschmeissner S, et al.
  タイトル
Novel mutations in keratin 16 gene underly focal non-epidermolytic palmoplantar keratoderma (NEPPK) in two families.
  雑誌
Hum Mol Genet 4:1875-81 (1995)
DOI:10.1093/hmg/4.10.1875
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