Focal palmoplantar and gingival keratosis: A distinct palmoplantar ectodermal dysplasia with epidermolytic alterations but lack of mutations in known keratins

Kolde, G.; Hennies, Hans Christian; Bethke, Gudrun; Reichart, Peter A.

doi:10.1016/j.jaad.2004.07.029

Cited by 15 publications

(8 citation statements)

References 26 publications

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“…The patient's gingival lesion was normal in appearance, even though the focal palmoplantar and the gingival keratosis were leukoplakic in appearance. 3,4 These findings suggested that the clinico-pathological feature in the present case was associated with FPPK with epidermolytic degeneration.…”

Section: Focal Palmoplantar Keratoderma With Epidermolytic Degenerationsupporting

confidence: 59%

“…2 Focal palmoplantar and gingival keratosis is characterized clinically by focal palmoplantar hyperkeratosis with leukoplakic appearance on the labial surface of the attached gingival lesion, and histologically by focal epidermolytic PPK. 3,4 Here, we describe a case of FPPK without other typical cutaneous and extracutaneous features. The histopathological examination indicated focal epidermolytic PPK.…”

Section: Focal Palmoplantar Keratoderma With Epidermolytic Degenerationmentioning

confidence: 97%

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Focal palmoplantar keratoderma with epidermolytic degeneration

Oiso¹,

Kawara

Kawada

2009

Acad Dermatol Venereol

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Section: Focal Palmoplantar Keratoderma With Epidermolytic Degenerationsupporting

confidence: 59%

Section: Focal Palmoplantar Keratoderma With Epidermolytic Degenerationmentioning

confidence: 97%

Focal palmoplantar keratoderma with epidermolytic degeneration

Oiso¹,

Kawara

Kawada

2009

Acad Dermatol Venereol

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“…It is characterized by focal plantar hyperkeratosis in weight‐bearing areas, mild palmar friction‐related hyperkeratosis and gingival leukokeratosis, usually limited to the maxillary and mandibulary attached gingiva (Table ). Histology and electron microscopy show epidermolytic alterations both in the skin and mucosal keratinocytes . On an embryological perspective, focal PPK with gingival keratosis should be classified as ‘complex PPK’ rather than ‘syndromic PPK’.…”

Section: Syndromic Palmoplantar Keratodermasmentioning

confidence: 99%

“…Histology and electron microscopy show epidermolytic alterations both in the skin and mucosal keratinocytes. 36,37 On an embryological perspective, focal PPK with gingival keratosis should be classified as 'complex PPK' rather than 'syndromic PPK'. Its inclusion in the syndromic category of 'PPK with prominent mucosal involvement' is practical in essence and helps differential diagnosis.…”

Section: Palmoplantar Keratoderma With Prominent Mucosal Involvementmentioning

confidence: 99%

Hereditary palmoplantar keratodermas. Part II: syndromic palmoplantar keratodermas – Diagnostic algorithm and principles of therapy

Guerra

Castori²,

Didona

et al. 2018

Acad Dermatol Venereol

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Hereditary palmoplantar keratodermas (PPKs) comprise a large and heterogeneous group of disorders characterized by persistent thickening of the epidermis at palmar and plantar surfaces. Clinical and genetic features of isolated and complex PPKs have been reviewed in part I of this 2-part review. Here we focus on clinical and molecular classification of syndromic PPKs which are recognized by additional extracutaneous manifestations, in particular deafness, specific mucosal lesions, cardiomyopathy, inborn errors of metabolism, involvement of internal organs or disorders of sexual development. Other genetic diseases, which may show palmoplantar involvement, such as selected subtypes of hereditary epidermolysis bullosa, various hereditary ichthyoses and other keratinization disorders, several ectodermal dysplasias and some multisystem genetic disorders, are also briefly summarized. PPK diagnosis is based on inheritance pattern, age at onset, morphology, distribution and severity of hyperkeratosis, pattern of additional dermatological and systemic manifestations and laboratory findings. Molecular analysis is at present the gold standard to confirm the diagnosis in PPK forms due to mutations in known causative genes. No specific and curative therapy is currently available for PPKs which highly impair patients' quality of life. Topical treatments are symptomatic and offer only temporary relief. Among systemic treatments, retinoids improve disease symptoms in the majority of patients.

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“…Focal palmoplantar and gingival keratosis is characterized clinically by focal PPK with leukoplakic appearance on the labial surface of the attached gingival lesion, and histologically by focal epidermolytic PPK [16].…”

Section: Focal Typementioning

confidence: 99%

Hereditary Palmoplantar Keratosis

Kawakami¹

2013

Current Genetics in Dermatology

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Focal palmoplantar and gingival keratosis: A distinct palmoplantar ectodermal dysplasia with epidermolytic alterations but lack of mutations in known keratins

Cited by 15 publications

References 26 publications

Focal palmoplantar keratoderma with epidermolytic degeneration

Focal palmoplantar keratoderma with epidermolytic degeneration

Hereditary palmoplantar keratodermas. Part II: syndromic palmoplantar keratodermas – Diagnostic algorithm and principles of therapy

Hereditary Palmoplantar Keratosis

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