El losartán pertenece al grupo de los inhibidores de los receptores de angiotensina II, fármacos muy utilizados en el momento actual para el tratamiento de la. Queratodermia palmoplantar epidermolítica asociada a pelo lanoso, syndrome de Ehlers Danlos y miocardiopatía dilatada. Gaceta Dermatológica Ecuatoriana. Dermatol. peru. ; 13 (2): – HIPERQUERATOSIS PALMOPLANTAR Y ENFERMEDAD PERIODONTAL: SÍNDROME DE PAPILLON – LEFÉVRE – A.

Author: Najar JoJojora
Country: Gambia
Language: English (Spanish)
Genre: Personal Growth
Published (Last): 14 September 2007
Pages: 424
PDF File Size: 7.99 Mb
ePub File Size: 9.31 Mb
ISBN: 386-8-85491-718-5
Downloads: 72383
Price: Free* [*Free Regsitration Required]
Uploader: Virg

A scalp biopsy revealed follicular fungal invasion and Majocchi’s granuloma.

Go to the members area of the website of the AEDV, https: The clinical presentation, differential diagnosis, therapeutic and periodontal management of an 8-year old male child diagnosed with this syndrome is discussed.

It is also expressed at high levels in various immune cells including polymorphonuclear leukocytes, macrophages and their precursors 12, Epidemiology of dermatophytoses in 31 municipalities of the Genetic counseling HMS is transmitted as an autosomal recessive trait. A peculiar deformity of the fingers tapered, pointed phalangeal ends and a claw-like volar curve is typical. In the initial phase, the dental treatment involved a professional prophylaxis, oral hygiene instructions and use of 0.

Management of the skin manifestations requires topical emollients, keratolytics including salicylic acid and urea and oral retinoids acitretin, etretinate, and isotretinoin.

HIV patients usually have superficial mycoses in many stages of the disease. The left mandibular first permanent molar appeared almost entirely out of its socket without any bone support giving the plamoplantar a “floating-in-air” appearance Fig.

Dermatophyte infections cause specific humoral and cellular immune response, and the protective response against dermatophytes is mainly mediated by hypersensitivity reaction of the delayed type.


Its prevalence is estimated to be one to four per million individuals in the general population with a carrier rate hiperquerarosis two to four per 3. There are palmoplantarr multiple congenital variants, with mutated genes and chromosomal localization recently discovered. Severe and atypical forms of dermatophytosis could lead to a diagnosis of AIDS. Only comments seeking to improve the quality and accuracy of information on hiperqueartosis Orphanet website are accepted.

Due to the severity of the presentation we requested an anti-HIV serology, which was positive. There was loss of gingival stippling with bleeding occurring on probing the involved gums.


For all other comments, please send your remarks via contact us. Onychogryposis, arachnodactyly, acroosteolysis and pes planus are additional features that help to distinguish HMS from other forms of palmoplantar hyperkeratosis.

The patient’s elbows and knees were not affected. Continuing navigation will be considered as acceptance of this use. Body and facial keratosis pilaris are additional features which appear in the following years. Med Mycol, hiperquwratosispp. Papillon-Lefevre syndrome treated with acitretin. The majority of reported cases are descendants of a few consanguineous families from a religious isolate in Cochin, India.

Subscribe to our Newsletter. The diagnosis of hypophosphatasia could be excluded in the present case owing to normal values hiperquerratosis alkaline phosphatase obtained in laboratory tests.

J Cutan Pathol, 18pp. On the other hand, atypical forms of dermatophytosis have been linked to AIDS. Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted.

Hum Genet ; The diagnosis could be crusted scabies or dermatophytosis. The patient’s tongue was bright and smooth with no papillae and white plaques on the oral mucosa consistent with the diagnosis of oral candidiasis.


The documents contained in this web site are presented for information purposes only.

The excellent response to the treatment, with a complete regression of the cutaneous lesions Fig. The lesions may be atypical, extensive and severe. Si continua navegando, consideramos que acepta su uso. We describe a severe case of dermatophytosis associated with exfoliative erythroderma, substantial palmoplantar keratoderma, onychodystrophy affecting all nails, diffuse non-scarring alopecia and tissue fungal invasion by Trichophyton tonsuranswhich led us to the diagnosis of AIDS.

J Med Genet Jan; Diagnostic methods Diagnosis is clinical but can be confirmed by detection of the disease-causing mutation. Show more Show less.

There was a problem providing the content you requested

If you are a member of the AEDV: Management and treatment Management of the skin manifestations requires topical emollients, keratolytics including salicylic acid and urea and oral retinoids acitretin, etretinate, and isotretinoin. In addition to the marked palmoplantar keratosis, patients have scaly erythematous and circumscribed patches on the elbows, knees, forearms, shins and dorsum of the hands.

Here we present an update of these diseases known as congenital palmoplantar keratodermas with and without associated manifestations, together with the acquired forms.