KEGG   DISEASE: Palmoplantar keratoderma
Entry
H01673                      Disease                                
Name
Palmoplantar keratoderma
  Subgroup
Palmoplantar keratoderma, epidermolytic [DS:H00722]
Non-epidermolytic palmoplantar keratoderma [DS:H00723]
Striate palmoplantar keratoderma [DS:H00717]
Punctate palmoplantar keratoderma [DS:H01404]
Keratoderma, palmoplantar, with deafness [DS:H00716]
Description
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.
Category
Congenital malformation
Brite
Human diseases in ICD-11 classification [BR:br08403]
 14 Diseases of the skin
  Skin disorders involving specific cutaneous structures
   Disorders of the epidermis and epidermal appendages
    Disorders of epidermal keratinisation
     ED55  Palmoplantar keratodermas
      H01673  Palmoplantar keratoderma
Comment
Hereditary PPK are divided morphologically into four types; diffuse, focal, striate and punctate. PPK can be further subdivided histologically into epidermolytic and nonepidermolytic PPK.
Other DBs
ICD-11: ED55
MeSH: D007645
OMIM: 144200
Reference
  Authors
Schiller S, Seebode C, Hennies HC, Giehl K, Emmert S
  Title
Palmoplantar keratoderma (PPK): acquired and genetic causes of a not so rare disease.
  Journal
J Dtsch Dermatol Ges 12:781-8 (2014)
DOI:10.1111/ddg.12418
Reference
  Authors
Sakiyama T, Kubo A
  Title
Hereditary palmoplantar keratoderma "clinical and genetic differential diagnosis".
  Journal
J Dermatol 43:264-74 (2016)
DOI:10.1111/1346-8138.13219
Reference
PMID:8595410
  Authors
Shamsher MK, Navsaria HA, Stevens HP, Ratnavel RC, Purkis PE, Kelsell DP, McLean WH, Cook LJ, Griffiths WA, Gschmeissner S, et al.
  Title
Novel mutations in keratin 16 gene underly focal non-epidermolytic palmoplantar keratoderma (NEPPK) in two families.
  Journal
Hum Mol Genet 4:1875-81 (1995)
DOI:10.1093/hmg/4.10.1875
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