| H01673 | |
| H番号 | H01673 |
| 名称 | 掌蹠角化症 |
| 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 |
| ネットワーク | - |
| 病因遺伝子 | - |
| 病原体 | - |
| 環境要因 | - |
| 発癌物質 | - |
| 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 ICD-10: L85.1 MeSH: D007645 OMIM: 144200 |
| 文献 | PMID:25176457 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 PMID:26945534 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 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 |