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A 45-year-old construction worker is referred for treatment of painful foot “calluses.” The patient has suffered with this condition for 20 years and states that lately it has appreciably worsened and no longer responds to over-the-counter salicylic acid preparations and moisturizers. When asked about his family history, he reports that his brother and father struggle with the same condition. The patient currently takes hydrochlorothiazide for control of hypertension but otherwise is in good health. A physical examination of his soles reveals hyperkeratosis, scaling, and superficial fissures. Mild scaling and xerosis of his palms are also noted.
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Palmoplantar keratoderma (PPK) manifests as either generalized or localized areas of hyperkeratosis. The soles may be more severely affected than the palms due to increased pressure and confinement. The hereditary variants of PPK are traditionally divided into 4 subtypes: diffuse, focal, striate, and punctate.1
Keratodermas can be inherited either by autosomal dominant or recessive patterns.2 Examples of the former include Unna-Thost, Vohwinkel syndrome, and Huriez syndrome. Autosomal recessive variants include Papillon-Lefevre syndrome and Nagashima PPK. Mal de Meleda may be inherited in either manner. These conditions are associated with underlying systemic abnormalities such as hearing loss, eye disease, and dental anomalies.2 Molecular genetic analysis has provided further guidance as to classification.3 Acquired PPK may be idiopathic or associated with inflammatory skin conditions such as psoriasis and pityriasis rubra pilaris, and underlying malignancy.2
Palmoplantar keratoderma can result in painful fissuring that limits mobility. Topical treatment of PPK consists of emollients and keratolytics. More severe cases may improve with oral retinoids.4 Targeting specific genetic defects shows promise in the management of hereditary variants.5
Lawrence Schiffman, DO, is the founder and director of Miami Skin Dr, in Doral, Florida. Stephen Schleicher, MD, is director of the DermDox Dermatology Centers, associate professor of medicine at Geisinger Commonwealth Medical College, and clinical instructor of dermatology at Arcadia University and Kings College.
References
1. Sakiyama T, Kubo A. Hereditary palmoplantar keratoderma “clinical and genetic differential diagnosis”. J Dermatol. 2016;43(3):264-274. doi:10.1111/1346-8138.13219
2. Stanway A. Palmoplantar keratoderma. DermNet. 2005. Accessed August 15, 2023. https://dermnetnz.org/topics/palmoplantar-keratoderma
3. Kamaleswaran S, Ousager LB, Bach RO, Bygum A. [Hereditary palmoplantar keratoderma – a focus on clinical and molecular genetic aspects]. Ugeskr Laeger. 2014;176(7A):V05130280.
4. Bodemer C, Steijlen P, Mazereeuw-Hautier J, O’Toole EA. Treatment of hereditary palmoplantar keratoderma: a review by analysis of the literature. Br J Dermatol. 2021;184(3):393-400. doi:10.1111/bjd.19144
5. Lee G, Lowe PM. Treatment of keratin 16 palmoplantar keratoderma with topical erlotinib. JAMA Dermatol. 2022;158(2):216-217. doi: 0.1001/jamadermatol.2021.5302.
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