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Palmoplantar Keratoderma (Keratosis Palmaris et Plantaris)

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Group of mostly hereditary disorders characterized by thickening of the palms and soles as a result of excessive keratin formation leading to hypertrophy of the stratum corneum (hyperkeratosis).

Also Known As:

Keratosis Palmaris et Plantaris; Keratosis, Palmoplantar; Meleda Disease; Keratoderma, Palmoplantar; Disease, Meleda; Keratodermas, Palmoplantar; Keratoses, Palmoplantar; Palmoplantar Keratodermas; Palmoplantar Keratoses; Palmoplantar Keratosis; Hyperkeratosis Palmaris et Plantaris

Networked: 281 relevant articles (9 outcomes, 8 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Darier Disease (Darier's Disease)
2.	Erythema
3.	Psoriasis (Pustulosis Palmaris et Plantaris)
4.	Malformed Nails (Pachyonychia)
5.	Hyperhidrosis

Experts

1.	McGrath, John A: 6 articles (06/2008 - 02/2002)
2.	Richard, Gabriele: 6 articles (06/2008 - 09/2002)
3.	McLean, W H Irwin: 5 articles (01/2015 - 02/2002)
4.	McGrath, J A: 5 articles (06/2014 - 03/2001)
5.	Christiano, Angela M: 5 articles (10/2009 - 02/2002)
6.	Sprecher, Eli: 5 articles (06/2008 - 09/2002)
7.	Itin, Peter: 4 articles (01/2012 - 09/2002)
8.	van Geel, M: 4 articles (01/2009 - 01/2005)
9.	Bergman, Reuven: 4 articles (06/2008 - 05/2005)
10.	Kelsell, D P: 4 articles (04/2001 - 01/2000)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Palmoplantar Keratoderma:

1.	Etretinate FDA Link 02/01/1993 - "Etretinate therapy produced a significant improvement in the palmoplantar keratoderma, but it was of no apparent value in treating nail keratoderma." 01/01/2015 - "Punctate palmoplantar keratoderma type 1: a novel AAGAB mutation and efficacy of etretinate." 09/01/2015 - "Low-dose etretinate shows promise in management of punctate palmoplantar keratoderma type 1: Case report and review of the published work." 01/01/1984 - "Three patients, a mother and her two sons, with mutilating palmoplantar keratoderma (Vohwinkel's syndrome) were successfully treated with oral etretinate (Tigason). " 01/01/1984 - "Mutilating palmoplantar keratoderma successfully treated with etretinate."
2.	Botulinum Toxins (Botulinum Toxin)IBA 02/01/2005 - "Botulinum toxin is helpful in aquagenic palmoplantar keratoderma." 05/01/2009 - "Besides palmar and plantar hyperhidrosis, botulinum toxin has demonstrated efficacy in different conditions associated with hyperhidrosis, such as dyshidrosis, multiple eccrine hidrocystomas, hidradenitis suppurativa, Frey syndrome, but also in different conditions worsened by hyperhidrosis such as Hailey-Hailey disease, Darier disease, inversed psoriasis, aquagenic palmoplantar keratoderma, pachyonychia congenital. "
3.	Mal de MeledaIBA 11/01/2014 - "Palmoplantar keratoderma of the Gamborg-Nielsen type is caused by mutations in the SLURP1 gene and represents a variant of Mal de Meleda." 06/01/2014 - "Mutations in SLURP1 cause mal de Meleda, a rare palmoplantar keratoderma (PPK). " 01/01/2013 - "Mal de Meleda (MDM) is a rare, autosomal recessive form of palmoplantar keratoderma. " 07/01/2011 - "Mal de Meleda is a rare transgressive palmoplantar keratoderma with an estimated prevalence of 1 in 100,000 individuals. " 01/01/2010 - "This, however, has not been the only disease named after a Croatian toponym; in 1826, Luca Stulli of Dubrovnik was the first to describe the 'mal de Meleda', nowadays recognized as a form of hereditary palmoplantar keratoderma. "
4.	PseudoainhumIBA 05/01/2015 - "A 49-year-old male presented with diffuse and striate palmoplantar keratoderma, thickened nails, knuckle pads, and pseudoainhum. " 11/01/2013 - "An examination revealed palmoplantar keratoderma of the palms and soles of the feet with no pseudoainhum. " 08/01/2009 - "We report a 32-year-old Japanese woman with mild palmoplantar keratoderma (PPK) with severe sensorineural hearing loss, knuckle pads and pseudoainhum of her toes. " 08/01/2009 - "Novel mutation p.Gly59Arg in GJB6 encoding connexin 30 underlies palmoplantar keratoderma with pseudoainhum, knuckle pads and hearing loss." 05/01/1997 - "We report a 32-year-old man with an unusual combination of congenital ichthyosis, sclerosing palmoplantar keratoderma with pseudoainhum, and bizarre striate hyperkeratosis in the fixtures, but no systemic involvement. "
5.	EmollientsIBA 01/01/2015 - "Symptomatic management of the palmoplantar keratoderma includes regular application of emollients, specialist footwear and early treatment of fissures and super-added infection, particularly tinea pedis. " 01/01/2007 - "We have described a 4-year-old girl who is treated with bland emollients and topical keratolytics such as urea and surprisingly observed marked improvement in skin hyperkeratosis and palmoplantar keratoderma. "
6.	Urea (Carbamide)FDA LinkGeneric 01/01/2007 - "We have described a 4-year-old girl who is treated with bland emollients and topical keratolytics such as urea and surprisingly observed marked improvement in skin hyperkeratosis and palmoplantar keratoderma. "
7.	Erythromycin (Erycette)FDA LinkGeneric 01/01/2004 - "Homozygous palmoplantar keratoderma type bothnia improved by erythromycin: a case report."
8.	alitretinoin FDA Link 08/01/2014 - "Alitretinoin: a new treatment option for hereditary punctate palmoplantar keratoderma (Brauer-Buschke-Fischer syndrome)."
9.	Burnett Schwartz Berberian syndromeIBA 05/01/1995 - "No therapeutic effect was detected in palmoplantar keratoderma or keratosis pilaris. " 03/01/2012 - "While there is significant phenotypic overlap among syndromes of the Ras/MAPK pathway, individuals with CS are more likely than individuals with CFC syndrome to present with cutaneous papillomas, palmoplantar keratoderma and full eyebrows, and are less likely to present with ulerythema ophryogenes, keratosis pilaris or multiple naevi. " 05/01/1993 - "Our two patients had many of the cardinal features of the disorder: a peculiar craniofacial appearance with sparse curly hair, low-set posteriorly rotated ears, moderate to severe mental retardation, heart defects, and cutaneous abnormalities such as ichthyosis, widespread keratosis pilaris, keratosis pilaris atrophicans faciei, and palmoplantar keratoderma." 05/01/1995 - "The 67 patients included in the study were at least 12 years of age and had the following diseases: ichthyosis vulgaris (n = 9), X-linked ichthyosis (n = 8), congenital ichthyosis (n = 10), hereditary palmoplantar keratoderma (n = 20), keratosis pilaris (n = 9), and Darier's disease (n = 11). " 03/01/2008 - "Keratosis follicularis spinulosa decalvans (KFSD) is a rare condition characterized by diffuse keratosis pilaris with a scarring alopecia of the scalp and associated photophobia, facial erythema, and palmoplantar keratoderma. "
10.	Type I Keratins (Type I Keratin)IBA 10/01/1993 - "Identification of the genetic locus for keratosis palmaris et plantaris on chromosome 17 near the RARA and keratin type I genes." 10/01/1994 - "The palmoplantar keratoderma variant investigated in this study is thus genetically different from epidermolytic palmoplantar keratoderma, which recently has been shown to result from mutations in the gene for the type I keratin 9." 06/01/1995 - "We have now investigated a family with the striated form of palmoplantar keratoderma (type Brünauer-Fuhs-Siemens) for linkage to either the type II keratin gene cluster on chromosome 12q or the type I keratin gene cluster on chromosome 17q. "

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 05/01/2004 - "A 49-year-old man electively chose to undergo a trial of intravenous chemotherapy with 5-fluorouracil (5-FU) for his inherited punctate palmoplantar keratoderma (PPK). "
2.	Skin Transplantation (Skin Grafting) 03/01/2008 - "We present two cases of Olmsted syndrome with severe palmoplantar keratoderma treated with excision and skin grafting, along with long-term clinical results 11 years (patient 1) and 6 years (patient 2) following the initial surgery." 03/01/2008 - "Palmoplantar keratoderma and skin grafting: postsurgical long-term follow-up of two cases with Olmsted syndrome." 02/01/1990 - "The palmoplantar keratoderma failed to improve with etretinate, but considerable benefit resulted from full-thickness excision of the skin of both palms, followed by skin grafting. "
3.	Gas Lasers 10/01/2011 - "Palmoplantar keratoderma: treatment with CO2 laser case report and review of the literature."
4.	Sutures (Suture) 01/01/2012 - "Herein we reported a case of pycnodysostosis, showing short stature with widening of the sutures, unfused anterior and posterior fontanelles, crowding of teeth with dental caries and typical radiological features associated with ichthyosis vulgaris and palmoplantar keratoderma."
5.	Radiotherapy 02/01/2015 - "These lesions were ameliorated with the treatment of the underlying mycosis fungoides and the palmoplantar keratoderma resolved promptly with radiotherapy. "