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Inherited acrokeratoelastoidosis is classified as a form of inherited punctate palmoplantar keratoderma (PPKP), specifically Type 3 PPKP, according to the classification of palmoplantar keratodermas (PPK). [10] Chromosome 2 appears to be the most likely locus responsible for inherited acrokeratoelastoidosis. [11]
Palmoplantar keratodermas are a heterogeneous group of skin disorders characterized by abnormal thickening (scleroderma) of the stratum corneum of the palms and soles. Autosomal recessive, dominant, X-linked, and acquired forms have all been described in medical literature. [1]: 505 [2]: 211 [3]
Papillon–Lefèvre syndrome (PLS), also known as palmoplantar keratoderma with periodontitis, [1] [2] is an autosomal recessive [3] genetic disorder caused by a deficiency in cathepsin C. [ 4 ] [ 5 ]
Quality of life can possibly can be decreased, therefore getting treatment is recommended. [6] Too much dry skin can be painful for some and cause discomfort. [ 8 ] There is limited data on the life expectancy of an affected person, but this disease alone does not reduce a person's lifespan.
Naxos disease [1] (also known as "diffuse non-epidermolytic palmoplantar keratoderma with woolly hair and cardiomyopathy" [1] or "diffuse palmoplantar keratoderma with woolly hair and arrhythmogenic right ventricular cardiomyopathy", first described on the island of Naxos by Dr. Nikos Protonotarios [1]) is a cutaneous condition characterized by a palmoplantar keratoderma. [1]
Most of the signs of Haim–Munk syndrome begin to manifest during the first 2–4 years of life. [5] Commons signs at this stage are thickening and scaling of the skin of the palms, soles (palmoplantar keratoderma) and elbows, and shedding of the primary dentition caused by recurrent episodes of dental caries and periodontitis.
Simple keratodermas Diffuse palmoplantar keratodermas. Diffuse epidermolytic palmoplantar keratoderma; Diffuse nonepidermolytic palmoplantar keratoderma; mal de Meleda; Focal palmoplantar keratoderma. Striate palmoplantar keratoderma; Punctate palmoplantar keratoderma. Keratosis punctata palmaris et plantaris; Spiny keratoderma; Focal acral ...
Usually, the basic forms of treatment of both deafness and palmoplantar keratoderma are combined, that is, used at the same time, to treat PKD, these include: Deafness: Surgery, hearing aids, cochlear implant [4] Palmoplantar keratoderma: Oilments, emolients, keratolytic agents, topical retinoids [5]