Palmoplantar keratodermas encompass a broad range of inherited disorders. They may be categorized based on degree of skin involvement (eg, localized to the palms and soles, expanding to involve other cutaneous sites and adnexal structures) and on whether they are part of a recognizable syndrome that affects other organs, such as the eyes and ears. Secondary infections are common. Examples include the following:
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Howel-Evans syndrome: This autosomal dominant Autosomal Dominant Genetic disorders determined by a single gene (Mendelian disorders) are easiest to analyze and the most well understood. If expression of a trait requires only one copy of a gene (one allele)... read more form has extracutaneous manifestations, with onset between ages 5 years and 15 years. Esophageal cancer Esophageal Cancer The most common malignant tumor in the proximal two thirds of the esophagus is squamous cell carcinoma; adenocarcinoma is the most common in the distal one third. Symptoms are progressive dysphagia... read more
may develop at a young age. -
Unna-Thost disease and Vorner disease: These are autosomal dominant forms.
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Papillon-Lefèvre syndrome: This autosomal recessive Autosomal Recessive Genetic disorders determined by a single gene (Mendelian disorders) are easiest to analyze and the most well understood. If expression of a trait requires only one copy of a gene (one allele)... read more form causes manifestations before age 6 months. Severe periodontitis Periodontitis Periodontitis is a chronic inflammatory oral disease that progressively destroys the tooth-supporting apparatus. It usually manifests as a worsening of gingivitis and then, if untreated, with... read more
can result in loss of teeth. -
Vohwinkel syndrome: In this autosomal dominant form, patients may also develop digital autoamputation and high-frequency hearing loss.
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Diffuse nonepidermolytic palmoplantar keratoderma: This autosomal dominant form develops in infancy and causes well-demarcated, symmetric keratoderma involving the entirety of the palms and soles.
Treatment of Palmoplantar Keratodermas
Symptomatic measures can include emollients Moisturizing agents Topical dermatologic treatments are grouped according to their therapeutic functions and include Cleansing agents Moisturizing agents (emollients, skin hydrators, and softeners) Drying agents... read more , keratolytics Keratolytics Topical dermatologic treatments are grouped according to their therapeutic functions and include Cleansing agents Moisturizing agents (emollients, skin hydrators, and softeners) Drying agents... read more , and physical scale removal. Secondary infections require treatment with antimicrobials. Oral retinoids are sometimes used.
