University of california at davis, school of medicine, sacramento, california. Palmoplantar keratoderma and spastic paraplegia also known as charcotmarietooth disease with palmoplantar keratoderma and nail dystrophy is an autosomal dominant or xlinked dominant condition that begins in early childhood with thick focal keratoderma over the soles and. Palmoplantar keratoderma and pleomorphic adenoma of. Palmoplantar keratoderma is also sometimes known as keratosis palmaris et plantaris. The 3 cases of mosaic cehk had histopathologic features of focal involvement in both the granular and spinous layers. The only slide with acral skin with continuous involvement was from a patient with a clinical diagnosis of vorners palmoplantar keratoderma. Histopathological examination of the skin biopsy taken from the patients sole. Two histopathological types of unnathost syndrome and their correlation with clinical features are. Pdf acquired palmoplantar keratoderma researchgate.
Palmoplantar keratoderma is a term used to define a marked thickening of the skin on the palms and soles, either as a focal entity, or diffuse. Palmar lesions are linear, whereas plantar lesions are focally distributed. Punctate palmoplantar keratoderma type i is a rare condition that affects the skin. Localized diffuse lesions with a red halo form on the palms and soles of infants. Palmoplantar keratoderma ppk constitutes a heterogeneous group of disorders characterized by thickening of the palms and the soles of individuals who are affected. Connexin 26 is a member of the connexin protein family. Original article palmoplantar psoriasis versus eczema. Case reports epidermolytic palmoplantar keratoderma.
Most palmoplantar keratodermas are not severe and are autosomal dominant. Clinical and histopathological study of palmoplantar. Pdf original article palmoplantar psoriasis versus. Palmoplantar keratodermas are a diverse group of hereditary and acquired keratodermas in which there is hyperkeratosis of the skin of the palms and soles. Epidermolytic palmoplantar keratoderma vorner type case report.
Pathology of keratodermas, cornification disorders. On the other hand, palmoplantar refers to the skin located on the soles of the feet as well as the palms of the hands. Palmoplantar keratoderma definition keratoderma is actually a term that literally means a marked skin thickening. Classification of keratodermas depends on whether or not it is inherited. Natural cure for palmoplantar keratoderma and alternative. Traditionally they have been classified as either hereditary or acquired and are distinguished from each other on the basis of mode of inheritance, presence of transgrediens defined as contiguous extension of hyperkeratosis beyond the palmar and. Eligibility statement for palmoplantar keratoderma and erythrokeratodermas 33701. Palmoplantar keratoderma is the name given to a group of conditions where there is abnormal thickening of the skin on the palms of the hands and soles of the feet.
Pdf palmoplantar keratodermas ppks are a diverse entity of disorders that are characterized by abnormal. Keratosis palmaris et plantaris of unna thost, showing either diffuse or localized, occasionally linear hyperkeratosis of the palms and soles fig. Histopathologically, eppk presents the characteristic features of epidermolytic hyperkeratosis and vacuolar cytolysis in the upper spinous and. Palmoplantar keratodermas ppk may be categorized as acquired or hereditary. The palmoplantar keratoderma ppk is a heterogeneous group of disorders. Maximum number of cases were of hereditary variety of palmoplantar keratoderma unnathost syndrome 28. Hereditary palmoplantar keratodermas ppk are a heterogeneous group of keratinizing. Palmoplantar refers to the skin on the soles of the feet and palms of the hands. Punctate porokeratotic keratoderma ali alikhan md 1, tracy burns bs 2, omid zargari md 3 dermatology online journal 16 1. Diffuse palmoplantar keratoderma focal keratoderma with or without nail involvement pachyonychia congenita phenotype focal keratoderma with pain and.
Histopathology of skin lesions in chronic arsenic toxicitygrading of. Occupationwise different types of manual workers such as laborers, farmers and. Palmoplantar keratoderma develops in the first or second decade of life. Introduction the term epidermolytic ichthyosis was agreed upon by the international ichthyosis consensus group in 2009 and replaces the older, confusing labels of bullous congenital ichthyosiform. So palmoplantar keratoderma is a disorder in which the sk in of the palms and soles is abnormally thick. Epidermolytic palmoplantar keratoderma of vornercase report. Palmoplantar keratoderma palmoplantar keratodermas are rare inherited disorders characterized by palmar and plantar hyperkeratosis. Hereditary palmoplantar keratodermas ppk are a heterogeneous group of. Palmoplantar keratoderma with deafness genetics home. Epidermolytic ichthyosis nord national organization for. Histopathology of affected skin reveals hyperkeratosis, acanthosis, and loosening of intercellular connections 17. Thostunna palmoplantar keratoderma can be made by histopathological detection of granular. Palmoplantar means palms of the hands and soles of the feet, and keratoderma means thickened skin.
Palmoplantar keratodermas ppks are a diverse entity of disorders that are characterized by abnormal thickening of the skin on the palms and soles. The next type of palmoplantar keratoderma that is relevant to podiatric practitioners is the focal palmoplantar keratoderma spectrum. Thostunna palmoplantar keratoderma is autosomal dominantly inherited. Our database lists the following as having palmoplantar keratoderma as a symptom of that condition.
Diagnosis of palmoplantar keratoderma ppk may involve a clinical exam, evaluating the medical and family history, histopathology viewing tissue from a skin biopsy under a microscope, and genetic testing if hereditary ppk is suspected. Diffuse ppk develops at birth or shortly thereafter and involves the entire. Thickening of the horny cell layer and epidermis is. The various forms of ppk can be divided into hereditary forms with only skin problems, hereditary syndromes. Cohen, in pediatric dermatology fourth edition, 20. It presents with thickening of the skin of the palms andor soles which may be diffuse involving most of the palms and soles or focal localised mainly to pressure areas. Palmoplantar keratoderma genetic and rare diseases. Palmoplantar psoriasis is its localized form that can exist alone in the absence of other typical psoriatic. This is an autosomal dominant condition and is present in childhood. Many cases could not be diagnosed by histopathology. It can occur in the first few months of life and almost always presents by age 3. Psoriasis is a common relapsing chronic inflammatory dermatosis that affects about 1.
Acquired palmoplantar keratoderma is more likely to present in adulthood compared with inherited keratodermas which usually present in childhood. Our patient, an 11yearold boy was undergoing treatment for progressive ppk by a dermatologist since the age of 1 year. Palmoplantar keratoderma and erythrokeratodermas inclusion criteria 33465 diagnosis of one of the following confirmed by consultant dermatologist. Early onset and positive family history suggest a genetic cause. Clinically there is a bilateral yellow thickening of the skin. Ppks has been based on history and clinical features combined with histopathology. Summary palmoplantar keratodermas comprise a diverse group of acquired and. It is a subtype of punctate palmoplantar keratoderma. In some cases, the presence of a positive family history is not observed. Clinical and histopathological study of palmoplantar keratoderma. Punctate palmoplantar keratoderma type i genetic and.
Palmoplantar keratoderma ppk is a common hereditary cutaneous disorder characterized by marked hyperkeratosis on the surface of palms and soles hennies et al. Keratoderma is a term that means marked thickening of the skin. Palmoplantar keratodermas dermatologic disorders msd. Connexin proteins form channels called gap junctions that permit the transport of nutrients, charged atoms. Transgrediens et progrediens palmoplantar keratoderma greithers. Histopathology, palmoplantar dermatitis, palmoplantar psoriasis. Palmoplantar keratodermas ppk comprise a heterogeneous group of keratinization disorders with. This chapter provides a practical overview of keratoderma, and is set out as below.
Palmoplantar keratodermas are a diverse group of dermatological disorders characterized by abnormal thickening of skin over palms. Whereas psoriasis was the leading cause among the acquired conditions 17. How to diagnose and manage hereditary palmoplantar. Palmoplantar keratoderma definition of palmoplantar. Palmoplantar keratoderma with deafness can be caused by mutations in the gjb2 or mtts1 genes the gjb2 gene provides instructions for making a protein called gap junction beta 2, more commonly known as connexin 26. Palmoplantar keratodermas comprise a diverse group of acquired and hereditary disorders marked by excessive thickening of the epidermis of palms and soles. Palmoplantar keratodermas foundation for ichthyosis. Keratinization disorder which presents at birth with generalized erythema, widespread bullae and desquamation resulting in denuded skin form of ichthyosis, an abnormality of epidermal maturation resulting in skin fragility epidermolytic hyperkeratosis is a descriptive term which refers to specific microscopic features i. It is related to the mutations of the krt6c and krt16 genes that. Epidermolytic palmoplantar keratoderma heppk hereditary epidermolytic palmoplantar keratoderma or vorners keratoderma is a rare genodermatosis first described by vorner in 1901. Familiar palmoplantar keratoderma, flaccid blisters, and. Hereditary focal palmoplantar keratoderma is a type of palmoplantar keratoderma that is caused by genetic abnormalities that may be inherited in an autosomal dominant or autosomal recessive manner in many cases, hereditary focal palmoplantar keratoderma begins during infancy or. Ppk has been classified into diffuse, focal, and punctate forms according to the pattern of hyperkeratosis on the palms and soles lucker et al.
Hematoxylineosin stained section of palmoplantar keratoderma tissue depicting variable degree of hypergranulosis and acanthosis. Palmoplantar keratoderma with knuckle pads and leukonychia and deafness. A study of the histopathology of palmoplantar psoriasis. Punctate palmoplantar keratodermas remedy publications llc. While hereditary forms of palmoplantar keratoderma ppk may represent the sole or dominant clinical feature. Punctatetype palmoplantar keratoderma is a type of palmoplantar keratoderma that is caused by genetic abnormalities that may be inherited in an autosomal dominant or autosomal recessive manner. A case of palmar psoriasis with well circumscribed. Palmoplantar keratodermas are a group of disorders characterized by thickening of the skin on the palms of the hands and soles of the feet of affected individuals.
Palmoplantar keratoderma ppk is a rare disorder in which dry, thick patches of skin develop on the soles of the hands and feet. Palmoplantar keratoderma, hyperkeratosis of paams and soles. Palmoplantar keratoderma and erythrokeratodermas version. A palmoplantar keratoderma excessive callus formation on palms and soles andor hair abnormalities may be present in some forms of the disorder. In addition to identifying thickened skin on the palms of the hands and soles of the feet, an exam is needed to check for involvement of other areas of the. The clinicalhistopathological evaluation enabled the diagnosis of vorner type epidermolytic hyperkeratosis. Both palmoplantar psoriasis and eczematous dermatitis of this skin area share. Major histopathologic clues for diagnosis article pdf available in iranian journal of pathology 94.
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