We report the case of a 70yearold male patient with punctate palmoplantar keratodermic lesions for more than 40 years. Dec 29, 2016 palmoplantar keratoderma ppk is a group of skin conditions characterized by thickening of the skin on the palms of the hands and soles of the feet. Palmoplantar keratoderma ppk ichthyosis support group. Pdf we present the case of a 63yearold woman with a oneyear history of punctiform, hyperkeratotic lesions on the palms of both hands.
Citescore measures average citations received per document published. Inherited forms can occur with specific clinical and genetic findings 1. All articles are subjected to a rigorous process of revision in pairs, and careful editing for literary and scientific style. In rare forms of ppk, organs other than the skin may also be affected. Keratosis punctata palmoplantaris kppp is a rare genodermatosis with an autosomaldominant pattern of inheritance. Keratosis palmoplantaris associated with periodontopathy or papillon lefevre syndrome is a very rare genetic disorder with autosomal recessive mode of inheritance palmoplanntar is characterized by hyperkeratosis of the palms and.
Queratodermias queratodermia punctata jovenes lesiones puntiformes. Queratodermia palmoplantar genetic and rare diseases nih. Queratodermia palmoplantar epidermolitica eppk mim 144200 sinonimo. Palmoplantar keratoderma how is palmoplantar keratoderma. It is generally associated with mutations of the keratin 9 gene krt9, and rarely with the keratin 1 gene krt1.
The histopathological findings were consistent with type i hereditary punctate palmoplantar keratoderma ppkd buschkefischerbrauer keratoderma associated with. Punctate palmoplantar keratoderma is a form of palmoplantar keratoderma in which many tiny raindrop keratoses involve the palmoplantar surface, skin lesions which may involve the whole of the palmoplantar surface, or may be more restricted in their distribution 505. The various forms of ppk can be divided into hereditary forms with only skin problems, hereditary syndromes. Queratodermias palmoplantares hereditarias dermatologia. Palmoplantar means palms of the hands and soles of the feet, and keratoderma means thickened skin. Ceratodermia palmoplantar wikipedia, a enciclopedia livre. Dermis queratosis palmoplantar information on the diagnosis. Whereas psoriasis was the leading cause among the acquired conditions 17. She had also complained of diarrhoea, exertional breathlessness and cough, and weight loss. Diffuse hereditary palmoplantar keratodermas present in early childhood with redness of the palms and soles.
Diffuse hereditary palmoplantar keratodermas dermnet nz. Palmoplantar keratodermas are a diverse group of hereditary and acquired keratodermas in which there is hyperkeratosis of the skin of the palms and soles. So palmoplantar keratoderma is a disorder in which the skin of the palms and soles is abnormally thick. Only comments seeking to improve the quality and accuracy of. We report herein two cases with the diagnosis of unnathost disease, one of them have atypical histopathological findings. Two histopathological types of unnathost syndrome and their correlation with clinical features are.
There is a clear cutoff between affected and unaffected skin and the edge of the thickening is often red. Keratosis palmoplantarisperiodontopathiaonychogryposis syndrome palmoplantar hyperkeratosisperiodontopathiaonychogryposis syndrome palmoplantar keratodermaperiodontopathiaonychogryposis syndrome prevalence. Ppk can be either acquired during the lifetime more commonly or inherited. Sep 10, 2012 punctate palmoplantar keratoderma pppk is a rare entity with an estimated prevalence rate of about 1. A 61 year old woman was investigated for a 12 month history of progressive thickening of the skin of her palms and soles. Epidermolytic palmoplantar keratoderma vorner s keratoderma is an autosomal dominant disorder of keratinization characterized by diffuse, nontransgredient thickening of palms and soles, without associated ectodermal features. Palmoplantar keratoderma definition of palmoplantar. The absence of nail deformities and arachnodactyly in the present case eliminated the diagnosis of haim munk syndrome, an autosomalrecessive genodermatosis characterized by palmpolantar palmoplantar keratoderma and progressive earlyonset periodontitis hiperquerxtosis if you are a member of the aedv. All structured data from the file and property namespaces is available under the creative commons cc0 license. Clinical and histopathological study of palmoplantar keratoderma. Queratodermia punctata bravo n, rivera z, soto p, sunico n, ortiz w, ortega j, bellsmithe a, oliver m, perezalfonzo r. Punctate palmoplantar keratoderma pppk is a rare entity with an estimated prevalence rate of about 1. Palmoplantar keratoderma genetic and rare diseases.
The dermis was slightly compressed below the epidermis level by the keratotic plugs and relatively free of inflammatory infiltrate. Files are available under licenses specified on their description page. Queratodermias palmoplantares trastornos dermatologicos. It gives preference to those studies related to fungi and their pathogenic action on human beings and animals, but any scientific study on mycology will be considered. The palms and soles gradually become thicker and develop a yellowish, waxy appearance.
Hiperqueratosis palmoplantar periodontopat a onicogriposis specialised social services eurordis directory. We report the case of a 61yearold woman who presented with a long history of multiple symptomatic hyperkeratotic papules on the palms and soles. The histopathological findings were consistent with type i hereditary punctate palmoplantar keratoderma ppkd buschkefischerbrauer keratoderma associated with hodgkins lymphoma. Losartan belongs to the group of angiotensin ii receptor antagonists and is used for the treatment of hypertension and heart failure. 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. Pdf keratosis punctata palmoplantaris controlled with. Ppk can be either acquired during the lifetime more commonly. The various forms of ppk can be divided into hereditary forms with only skin problems, hereditary syndromes with ppk as an associated feature, and acquired forms. Queratodermia palmoplantar genetic and rare diseases.
Pdf epidermolytic palmoplantar keratoderma vorner type. Palmoplantar keratoderma ppk is a group of skin conditions characterized by thickening of the skin on the palms of the hands and soles of the feet. O presente caso referese a paciente com queratodermia palmoplantar difusa, nao transgressiva, iniciada na infancia, com diversos casos. Cardiomyopathy, palmoplantar keratoderma, woolly hair. Maximum number of cases were of hereditary variety of palmoplantar keratoderma unnathost syndrome 28. Jun 27, 2019 hiperqueratosis palmoplantar asociada a losartan actas dermosifiliograficas english edition however, in this case, the agent found was t. Palmoplantar keratoderma tripe palms associated with primary. An ebook reader can be a software application for use on a computer such as microsofts free reader application, or a booksized computer that is used solely as a reading device such as nuvomedias rocket ebook. Pdf as queratodermias palmoplantares familiares sao doencas pouco comuns.
Aquagenic syringeal acrokeratoderma asa is a rare form of palmoplantar keratoderma occurring after shortterm contact with water. A family of unnathost disease with one of them showing. There was no relevant past medical history or family history, but she had a 45 pack year history of cigarette smoking. Queratodermia palmoplantar patologia medicina clinica.
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