Image and content excerpted from the VisualDx clinical decision support system.
VisualDx images show variation in age, skin color, and disease stage. VisualDx has 52 images of Keratoderma, Palmoplantar.
Full text and additional images for Keratoderma, Palmoplantar are available in the following VisualDx packages:
Q82.8 – Other specified congenital malformations of skin
757.39 – Other specified congenital anomalies of skin
SynopsisPalmoplantar keratoderma (PPK) is thickening of the palms and/or soles that cannot be attributed to friction alone. Cases are either inherited or acquired. Heritable PPKs are identified by the presence of a family history and childhood onset; they may manifest in isolation, as the defining feature of a syndrome, or as a minor aspect of a syndrome (eg, congenital ichthyoses, Darier's disease).
Hereditary PPKs are approached and classified by the pattern of hyperkeratosis: diffuse, focal (often occurring over weight-bearing areas), or punctate.
Diffuse hereditary PPK:
- Vorner (epidermolytic) PPK and Unna-Thost (nonepidermolytic) PPK are the result of keratin mutations and show waxy or verrucous, white-yellow, symmetric hyperkeratosis.
- Mal de Meleda is a rare diffuse hereditary PPK associated with SLURP1 mutations and features stocking-glove distribution of hyperkeratosis with malodor and nail changes.
- Vohwinkel syndrome (mutilating PPK) has 2 variants: the classic form associated with deafness and mutations of the connexin gene GJB2 and the loricrin variant associated with loricrin mutations and ichthyosis. The PPK shows a diffuse honeycomb pattern. Additional features include starfish-shaped keratotic plaques on dorsal hands, feet, elbows, and knees as well as constricting digital bands termed "pseudo-ainhum," which may progress to autoamputation.
- Papillon-Lefèvre syndrome is associated with mutations in the gene that encodes cathepsin C and demonstrates diffuse PPK, periodontal disease with loss of teeth, and frequent cutaneous and systemic pyogenic infections.
- Other diffuse hereditary PPKs include Greither syndrome, Bart-Pumphrey syndrome (PPK with knuckle pads, leukonychia, and deafness), Huriez syndrome (PPK with scleroatrophy), Clouston syndrome (hidrotic ectodermal dysplasia), Olmsted syndrome (mutilating PPK with periorificial plaques), diffuse nonepidermolytic PPK with sensorineural deafness, and Naxos disease (diffuse nonepidermolytic PPK with woolly hair and cardiomyopathy).
- Isolated focal PPKs (striate PPKs) are due to autosomal dominant mutations in genes encoding desmosomal proteins. Lesions favor pressure points on feet and may present as linear plaques on hands.
- Howel-Evans syndrome is associated with mutations in the TOC gene, focal weight-bearing area plantar hyperkeratosis, milder palm involvement, and development of esophageal carcinoma.
- Richner-Hanhart syndrome is associated with mutations in the gene that encodes tyrosine aminotransferase. Accumulation of tyrosine leads to focal (or diffuse) hyperkeratotic plaques on the hands, feet, elbows, and knees, corneal inflammation/ulceration, and mental retardation in some cases. Diets low in phenylalanine and tyrosine may prevent complications.
- Focal PPK may also be seen in pachyonychia congenita type I and type II (syndromes with nail, skin, teeth, and eye anomalies) as well as Carvajal syndrome (striate focal epidermolytic PPK with woolly hair and dilated cardiomyopathy).
- Punctate PPKs are characterized by autosomal dominant inheritance and multiple firm 2–8 mm papules on the palms and soles. A pattern with lesions favoring palmar creases has been identified in patients of African descent.
- Focal acral hyperkeratosis and acrokeratoelastoidosis present as 2–4 mm papules (some umbilicated) at the marginal borders of hands and feet.
- Keratoderma climactericum – Seen in menopausal women, often associated with obesity or hypertension; pressure points on the soles of the feet are affected first.
- Infectious PPK – Associated with dermatophytosis, leprosy, HIV, syphilis, crusted scabies, and human papillomavirus infections.
- Chemical/drug-induced PPK – Associated with exposure to arsenic, halogenated aromatic chemicals such as dioxin, venlafaxine, verapamil, hydroxyurea, etodolac, quinacrine, proguanil, methyldopa, practolol, doxorubicin, bleomycin, imatinib, capecitabine, tegafur, lithium, gold, and mexiletine.
- Dermatosis-related PPK – May be associated with atopic and contact dermatitis, psoriasis, reactive arthritis (keratoderma blennorrhagicum), lichen planus, lichen nitidus, lupus erythematosus, and pityriasis rubra pilaris.
- PPK as a feature of systemic disease – Hypothyroidism, myxedema, diabetes mellitus, and chronic lymphedema.
- Malnutrition-associated PPK
- Aquagenic keratoderma – Most often affects palms in patients in the second decade of life. Symptoms develop within 5 minutes of immersion in water.
- Paraneoplastic PPK – Acrokeratosis paraneoplastica of Bazex is associated with squamous cell carcinoma of the upper GI tract, and "tripe palms" is associated with pulmonary or gastric malignancies. Other malignancies with associated paraneoplastic PPK include breast, bladder, and skin malignancies; myeloma; mycosis fungoides; and Sézary syndrome.
- Idiopathic PPK – a diagnosis of exclusion