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Keratoderma Palmoplantaris Transgrediens

Presentation

A well-demarcated, thick, yellow hyperkeratosis is present over the palms and soles. An erythematous band is frequently present at the periphery of the keratosis. The surface is often uneven and verrucous. [emedicine.medscape.com]

The diagnosis was based on clinical presentation as he had a non-syndromic hereditary foul-smelling and yellowish palmoplantar keratoderma transgrediens. [pubmed.ncbi.nlm.nih.gov]

Different clinical presentations of the disease have been described; these depend on the ethnic background and geographic origins of the patients (Eckl et al. 2003). [nature.com]

" "Hereditary palmoplantar keratoderma," "Keratosis extremitatum progrediens," "Keratosis palmoplantaris diffusa circumscripta," "Tylosis," "Unna–Thost disease", and "Unna–Thost keratoderma"[4]) is inherited as an autosomal dominant condition and is present [en.wikipedia.org]

Perelman Department of Dermatology, New York University Abstract A 13-year-old boy and a 7-year-old boy, who are brothers, presented with a life-long history of erythema, hyperkeratosis, and desquamation of the hands and feet. [escholarship.org]

Treatment

Early treatment with acitretin in childhood may allow patients to have normal adult dentition. [emedicine.medscape.com]

[…] erythema gyratum repens; Greither disease; Greither's disease; Keratosis extremitatum hereditaria progrediens; Keratosis palmoplantaris transgrediens et progrediens; Transgrediens et progrediens palmoplantar keratoderma About the Disease Diagnosis & Treatment [rarediseases.info.nih.gov]

The following treatments soften the thickened skin and makes it less noticeable. [dermnetnz.org]

They found that erythema and hyperkeratosis of both hands were improved with both treatments. [hindawi.com]

Prognosis

A better understanding of the clinical characteristics and pathogenic gene characterization of NPPK will enhance the diagnosis of NPPK, identify related diseases, and inform on the precise therapy and prognosis. [hindawi.com]

Etiology

Pathogenesis The etiology of NPPK has been established. However, its pathogenesis has not been clearly elucidated. [hindawi.com]

Another possible differential diagnosis is symmetrical lividities of the soles, a rare dermatosis of unknown etiology, characterized by hyperhidrosis and symmetrical, bluish-red plaques on the soles of the feet, but its predilection is not limited to [jamanetwork.com]

Epidemiology

Rudan I, Campbell H, Rudan P: Genetic epidemiological studies of Eastern Adriatic Island isolates, Croatia: Objectives and strategies. Coll Antropol 1999;23:531–546. [karger.com]

Pathophysiology

Despite reports on mild T cell infiltration in the affected skin area, the pathophysiology of the skin redness and hyperkeratosis has not been characterized [24]. [hindawi.com]

Prevention

After the failure of acitretin, which had not prevented retractile and mutilating progression of the palmoplantar keratoderma, he had undergone surgery with graft excision of both palms. [pubmed.ncbi.nlm.nih.gov]

Elective extraction of involved teeth may prevent excess bone resorption. Appropriate antibiotic therapy may be required for periodontitis and recurrent cutaneous and systemic infections. [emedicine.medscape.com]

At the same time, local low-dose application of gentamicin exhibited a significant effect and prevented the potential ototoxicity and nephrotoxicity of aminoglycoside antibiotics [13]. [hindawi.com]

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