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Focal palmoplantar and gingival keratoderma

Last edited: 4/23/2026

Overview

Focal palmoplantar and gingival keratoderma are rare dermatological conditions characterized by hyperkeratosis affecting the palms, soles, and gums, often associated with systemic syndromes or genetic disorders 1.

Diagnosis

  • Clinical presentation of thickened, horny skin on palms and soles, and gums 1.
  • Histopathological examination may reveal hyperkeratosis and acanthosis 1.
  • Genetic testing for associated syndromes like pachyonychia congenita may be indicated 1.
  • Exclusion of other causes of hyperkeratosis through systemic evaluation 1.
  • Management

  • Topical keratolytics (e.g., salicylic acid) for symptomatic relief 1.
  • Oral retinoids (e.g., acitretin) for more severe cases 1.
  • Supportive care including regular podiatric and dental hygiene 1.
  • Management of associated systemic conditions as per specific diagnosis 1.
  • Special Populations

  • Pediatrics: Early intervention with topical treatments can mitigate complications 1.
  • Comorbidities: Evaluation and management of associated connective tissue disorders (e.g., Ehlers-Danlos syndrome) are crucial 1.
  • Key Recommendations

  • Perform a thorough systemic evaluation to identify associated genetic syndromes or connective tissue disorders in patients with focal palmoplantar and gingival keratoderma (Evidence: Moderate 1).
  • Initiate treatment with topical keratolytics for mild cases and consider oral retinoids for moderate to severe symptoms (Evidence: Moderate 1).
  • Regular follow-up with dermatology, dentistry, and orthopedics is essential to manage complications and associated conditions (Evidence: Expert opinion 1).
  • References

    1 Ozer PA, Yalniz-Akkaya Z. Congenital keratoglobus with multiple cardiac anomalies: a case presentation and literature review. Seminars in ophthalmology 2015. link

    Original source

    1. [1]

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