Palmoplantar Keratodermas

Genetic palmoplantar keratoderma

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Defined by a more or less permanent thickening of the stratum corneum (histological hyperkeratosis) of the palms and soles.
 
Classification
  • Clinical-morphological characteristics
    • Diffuse - entire palmar or plantar surface
    • Focal - most often localized to pressure points. Divided in:
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      • Areata/nummular type (generally on the soles)
      • Striate type (generally on the palms)
      • Can evolve into diffuse form
    • Punctate - characterized by the presence of numerous keratotic papules measuring 1 to 10mm.
      • Center is often depressed and marked by a black dot which, once removed, leaves a small crater.
      • With age, the lesions take on a callous appearance
      • Typical onset in adolescence
      • Types
        • Punctate PPK type I, Buschke-Fisher-Brauer
        • Punctate PPK type II, porokeratosis punctata palmaris and plantaris
        • Punctate PPK type III, acrokeratoelastoidosis
        • Focal acral hyperkeratosis
        • Hereditary papulotranslucent acrokeratoderma
  • Extent of the lesions
    • Transgradient - extend beyond Wallace lines to dorsal suface of the hands and feet
    • Progradiens - lesions exist at a distance, for example on the elbows or knees
  • Association with extra-cutaneous manifestations
    • Isolated/non-syndromic
    • Complex - Have predominant palmoplantar PPK, but additional skin findings without systemic findings that would classify as syndromic
      • Loricrin keratoderma
      • Olmsted syndrome
      • Striate PPK with wooly hair
      • PPK and congenital alopecia 1 (PPKCA1), Stefanovic type
      • PPK and congenital alopecia 2 (PPKCA2), Wallis type
      • Hyperkeratosis-hiperpigmentation syndrome
      • PPK with pigmentation defects and skin carcinoma
    • Syndromic
  • Inheritance pattern
    • AD, AR, XL, mitochondrial
    • Hereditary doesn’t mean congenital
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Tratamento geral
  • Sintomático
  • Monitorizar impacto na marcha e sistema musculoesquelético
  • Tratar infeções fúngicas frequentes
  • Prevenção de estrituras (tratamento queratolítico, cirurgia)
  • Podologistas, pedras pommes
  • Queratolíticos tópicos
    • Ureia, ácido salicílico, ácido láctico
  • Retinoides orais
    • Etretinato, acitretina
  • Calcipotriol
  • Injeção de toxina botulínica para tratar hiperhidrose
  • inibidores mTOR (sirolimus oral ou tópico) na paquioníquia congénita
  • Injeção plantar de siRNA inibe K6a anormal e reduz calosidades na paquioníquia congénita
 
 

Acquired palmoplantar keratoderma

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Thickening of the skin in the palms and soles that is not hereditary, it is ogten attributed to an underlying disease.
 
DDx principal
  • Keratoderma induced by mechanical factors
    • Professions: diggers, blacksmiths, agricultural workers
    • Housework
  • Drug induced keratoderma
    • Psoriasiform acrokeratotic conditions induced by beta-blocker medications
    • Bleomycin, hydroxyurea, lithium, quinacrine, tegfur, venlafaxine, verapamil
    • Chemotherapy: associated with acral erythema and pain due to toxic effect
    • Bleomycin associated with pigmentation and edema
    • Pseudodermatomyositis of the hands (hydroxyurea)
    • Folliculitis and acne (lithium)
  • Acquired aquagenic palmoplantar keratoderma
    • Abnormality appears when the hands are immersed in water for 3 to 5 minutes (”hand in bucket sign”)
    • Etiology
      • Idiopathic
      • Associated with mutations in CFTR gene (cystic fibrosis)
      • Associated with various diseases (atopy, hyperhidrosis, use of COX-2 non-steroidal anti-inflammatory drugs)
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