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Dermatology

Palmoplantar Keratodermas

Built from Dermatology, 5th Edition

What’s inside

10 sections · 135 slides

  1. 01

    Overview

    • What this topic covers

    1 slide

  2. 02

    Introduction and Classification

    What PPK is, how it is grouped, and how it is worked up

    • Palmoplantar keratoderma: definition
    • Inherited versus acquired PPK
    • Three patterns of involvement
    • Diffuse palmoplantar keratoderma
    • Focal palmoplantar keratoderma
    • Punctate palmoplantar keratoderma
    • Diffuse palmar keratoderma, close view
    • Punctate keratosis, coalescing lesions
    • Extra descriptive terms
    • Digital constriction bands (pseudoainhum)
    • Genodermatoses associated with digital constriction bands (pseudoainhum)
    • Clinical work-up of a patient with PPK
    • Primary palmoplantar keratodermas (PPKs)
    • Primary palmoplantar keratodermas (PPKs) (continued)
    • Primary palmoplantar keratodermas (PPKs) (continued)
    • Primary palmoplantar keratodermas (PPKs) (continued)
    • Primary palmoplantar keratodermas (PPKs) (continued)
    • Primary palmoplantar keratodermas (PPKs) (continued)
    • Primary palmoplantar keratodermas (PPKs) (continued)
    • Primary palmoplantar keratodermas (PPKs) (continued)
    • Primary palmoplantar keratodermas (PPKs) (continued)
    • Primary palmoplantar keratodermas (PPKs) (continued)
    • Primary palmoplantar keratodermas (PPKs) (continued)
    • Primary palmoplantar keratodermas (PPKs) (continued)
    • Primary palmoplantar keratodermas (PPKs) (continued)
    • PPK as part of another genetic skin disease
    • Other genodermatoses that feature palmoplantar keratoderma (PPK)
    • Other genodermatoses that feature palmoplantar keratoderma (PPK) (continued)
    • Other genodermatoses that feature palmoplantar keratoderma (PPK) (continued)
    • Skin biopsy: what it can show
    • Histologic patterns in selected hereditary palmoplantar keratodermas (PPKs)
    • Histologic patterns in selected hereditary palmoplantar keratodermas (PPKs) (continued)
    • Histologic patterns in selected hereditary palmoplantar keratodermas (PPKs) (continued)
    • The gap junction and connexins
    • Connexin channels and the gap junction plaque
    • Human connexin disorders
    • Human connexin disorders (continued)
    • Human connexin disorders (continued)
    • Genetic mutations behind hereditary PPKs
    • Genetic mutations underlying hereditary palmoplantar keratodermas

    40 slides

  3. 03

    Diffuse PPK Without Associated Features

    Isolated, non-syndromic forms - epidermolytic and non-epidermolytic

    • Diffuse epidermolytic PPK (Vorner-Unna-Thost): overview
    • EPPK: how the mutation causes disease
    • EPPK: clinical course
    • Diffuse epidermolytic PPK (Vorner-Unna-Thost)
    • Diffuse epidermolytic PPK, sole involvement
    • EPPK: what the biopsy shows
    • Histologic features of palmoplantar keratoderma
    • EPPK: diagnosis and management
    • Diffuse non-epidermolytic PPK (NEPPK): overview
    • NEPPK subtypes: genes and mechanism
    • Mal de Meleda
    • Mal de Meleda
    • NEPPK: biopsy and treatment
    • Keratolytic winter erythema and CTSB-related PPK
    • Keratolytic winter erythema

    15 slides

  4. 04

    Diffuse PPK With Ichthyosis

    Loricrin keratoderma and KLICK syndrome

    • Loricrin keratoderma
    • Loricrin keratoderma vs Vohwinkel syndrome
    • KLICK syndrome
    • KLICK syndrome

    4 slides

  5. 05

    Diffuse PPK With Associated Features

    Syndromic diffuse PPK - hearing loss, cancer risk, hair and nail change, cardiomyopathy

    • Vohwinkel syndrome: genetics and skin findings
    • Mutilating palmoplantar keratoderma in Vohwinkel syndrome
    • Vohwinkel syndrome: hearing and treatment
    • Bart-Pumphrey syndrome
    • Bart-Pumphrey syndrome
    • Mitochondrial PPK with hearing impairment
    • Huriez syndrome
    • Huriez syndrome
    • PPK with sex reversal and squamous cell carcinoma
    • Clouston syndrome
    • Clouston syndrome
    • Odonto-onycho-dermal dysplasia (Schopf-Schulz-Passarge)
    • Olmsted syndrome
    • Olmsted syndrome: treatment insight
    • Papillon-Lefevre and Haim-Munk syndromes
    • Naxos disease
    • The PPK - woolly hair - cardiomyopathy triad

    17 slides

  6. 06

    Focal PPK Without Associated Features

    Striate/focal PPK - a desmosome and keratin disorder

    • Striate (focal) palmoplantar keratoderma
    • Striate and areata focal palmoplantar keratoderma
    • Striate PPK: related conditions and treatment

    3 slides

  7. 07

    Focal PPK With Associated Features

    Pachyonychia congenita, Richner-Hanhart, Howel-Evans, and Carvajal syndromes

    • Pachyonychia congenita: overview
    • >90%
    • Pachyonychia congenita: nail and skin findings
    • Pachyonychia congenita
    • Pachyonychia congenita, nail and sole detail
    • Pachyonychia congenita: other features and treatment
    • Richner-Hanhart syndrome (tyrosinemia type II)
    • Richner-Hanhart syndrome (tyrosinemia II)
    • Richner-Hanhart syndrome, corneal findings
    • Richner-Hanhart syndrome: treatment
    • Howel-Evans syndrome
    • Howel-Evans syndrome
    • Carvajal syndrome
    • Carvajal syndrome

    14 slides

  8. 08

    Punctate PPK Without Associated Features

    Buschke-Fischer-Brauer type, spiny keratoderma, and marginal papular keratoderma

    • Punctate PPK, Buschke-Fischer-Brauer type
    • Punctate PPK: course and work-up
    • Punctate keratoses of the palmar creases
    • Punctate keratoses of the palmar creases
    • Conditions causing palmoplantar pits
    • Differential diagnosis of palmoplantar pits
    • Spiny keratoderma
    • Spiny palmoplantar keratoderma
    • Marginal papular keratoderma
    • Marginal papular keratoderma
    • Marginal papular keratoderma, close view
    • Focal acral hyperkeratosis: histology

    12 slides

  9. 09

    Punctate PPK With Associated Features

    Cole disease and PLACK syndrome

    • Cole disease
    • PLACK syndrome

    2 slides

  10. 10

    Acquired Keratodermas

    PPK that develops later in life - drugs, cancer, hormones, and idiopathic forms

    • Keratoderma climactericum
    • Keratoderma climactericum
    • PPK and internal cancer
    • Arsenical keratoses
    • Other acquired causes
    • Aquagenic palmoplantar keratoderma
    • Aquagenic wrinkling of the palms
    • Aquagenic keratoderma, pebbly change
    • Aquagenic PPK: treatment
    • Circumscribed palmar or plantar hypokeratosis
    • Circumscribed palmar hypokeratosis
    • Circumscribed hypokeratosis: treatment
    • Diffuse PPK in a related ichthyosis syndrome
    • Diffuse palmar keratoderma in an infant with KID syndrome
    • A related erosive skin fragility disorder
    • Palmar keratoderma in ectodermal dysplasia-skin fragility syndrome
    • How to approach a PPK patient
    • Key takeaways
    • References
    • References (continued)
    • References (continued)
    • References (continued)
    • References (continued)
    • References (continued)
    • References (continued)
    • References (continued)
    • Dermatology, 5th Edition (2-Volume Set)

    27 slides