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Focal palmoplantar and gingival keratoderma

MedGen UID:
372097
•Concept ID:
C1835650
•
Disease or Syndrome
Synonyms: FOCAL PALMOPLANTAR AND GINGIVAL HYPERKERATOSIS SYNDROME; Focal palmoplantar and oral mucosa hyperkeratosis; Keratosis focal palmoplantar gingival; KERATOSIS, FOCAL PALMOPLANTAR AND GINGIVAL
SNOMED CT: Focal palmoplantar and gingival keratoderma (764963007)
 
Monarch Initiative: MONDO:0007860
OMIM®: 148730
Orphanet: ORPHA2200

Definition

A very rare form of focal palmoplantar keratoderma with characteristics of painful circumscribed hyperkeratotic lesions on weight-bearing areas of soles, moderate focal hyperkeratosis of palmar pressure-related areas and an asymptomatic leukokeratosis confined to labial and lingual attached gingiva. Additional occasional features may include hyperhidrosis, follicular keratosis and extended oral mucosa involvement. [from SNOMEDCT_US]

Clinical features

From HPO
Focal friction-related palmoplantar hyperkeratosis
MedGen UID:
322715
•Concept ID:
C1835654
•
Finding
Hyperkeratosis affecting the palm of the hand and the sole of the foot in areas exposed to friction.
See: Feature record | Search on this feature
Subungual hyperkeratosis
MedGen UID:
21379
•Concept ID:
C0038605
•
Finding
A thickening of the stratum corneum in the region beneath the nails.
See: Feature record | Search on this feature
Gingival hyperkeratosis
MedGen UID:
384034
•Concept ID:
C1857013
•
Disease or Syndrome
Hyperkeratosis of the gingiva.
See: Feature record | Search on this feature
Circumungual hyperkeratosis
MedGen UID:
870243
•Concept ID:
C4024681
•
Disease or Syndrome
A thickening of the stratum corneum, the outer layer of the skin, in the region surrounding the nails.
See: Feature record | Search on this feature
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Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVFocal palmoplantar and gingival keratoderma
Follow this link to review classifications for Focal palmoplantar and gingival keratoderma in Orphanet.

Recent clinical studies

Etiology

Prevention of bacterial endocarditis in localised juvenile periodontitis and Papillon-Lefevre syndrome patients.
Yusof ZA
Dent J Malays 1988 Nov;10(2):31-5. PMID: 2978721

Diagnosis

Focal palmoplantar and gingival hyperkeratosis: a case report.
Seehra J, Thakker N, Duxbury AJ, Sloan P, Ariyaratnam S
Dent Update 2007 Jun;34(5):290-2, 294. doi: 10.12968/denu.2007.34.5.290. PMID: 17624145

Therapy

Prevention of bacterial endocarditis in localised juvenile periodontitis and Papillon-Lefevre syndrome patients.
Yusof ZA
Dent J Malays 1988 Nov;10(2):31-5. PMID: 2978721

Clinical prediction guides

Focal palmoplantar and gingival keratosis: a distinct palmoplantar ectodermal dysplasia with epidermolytic alterations but lack of mutations in known keratins.
Kolde G, Hennies HC, Bethke G, Reichart PA
J Am Acad Dermatol 2005 Mar;52(3 Pt 1):403-9. doi: 10.1016/j.jaad.2004.07.029. PMID: 15761417
Focal palmoplantar and oral mucosa hyperkeratosis syndrome: a report concerning five members of a family.
Laskaris G, Vareltzidis A, Avgerinou G
Oral Surg Oral Med Oral Pathol 1980 Sep;50(3):250-3. doi: 10.1016/0030-4220(80)90379-5. PMID: 6447853

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