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.

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Genetic counseling HMS is transmitted as an autosomal recessive trait. Ten years before, similar symptoms were treated in another medical service with clinical improvement.

Successful periodontal maintenance of a case with Papillon-Lefevre syndrome: For all other comments, please send your remarks via contact us. Autosomal dominant palmoplantar keratoderma with congenital alopecia PPK-CA is a rare genetic skin disorder characterized by absence of scalp and body hair and palmoplantar keratoderma, without other hand complications.

In the present case, the diagnosis of PLS was made primarily based on the clinical, radiological and dermatological features besides analysis of laboratory tests. BMC Med Genet ; 4: Abstract Palmoplantar keratodermas represent a heterogenic group of diseases characterized by the abnormal skin thickening of the palms and soles.

In the palmoplantar areas, substantial hyperkeratosis was present, forming corneous projections of up to 3 cm Fig. Micoses superficiais e os elementos da resposta imune. Carrillo aM. In Papillon-Lefevre Syndrome, the development and eruption of the deciduous teeth proceed normally but eruption is associated with gingival inflammation and subsequent rapid destruction of the periodontium resulting in premature exfoliation of primary teeth by age 4 years.


Epidemiology of dermatophytoses in 31 municipalities of the The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.

Retinoid treatment may end up hlperqueratosis normal dental development if started during eruption of permanent teeth 7, The excellent response to the treatment, with a complete regression of the cutaneous lesions Fig.

No ectoparasites were found.

Orphanet: Hiperqueratosis palmoplantar periodontopat a onicogriposis

The differential diagnosis of PLS includes conditions such as hypophosphatasia, histiocytosis X, Haim Munk syndrome, Melada disease and Prepubertal periodontitis. 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.

The dermatologist uiperqueratosis acitretin 20 mg orally daily for the initial 3 months and then 10 mg orally per day for the next two months.

Use of low dose acitretin therapy in the present case showed satisfactory reduction in hyperkeratotic lesions on the palms and soles. Pemphigus Vegetans in the Inguinal Folds. J Cutan Pathol, 18pp. The possible immunological alterations involved are impaired chemotaxis of neutrophils and, possibly, an induced immunological defect caused by an interaction of periodontal pathogens and pocket epithelium 9, Bienvenido a siicsalud Contacto Inquietudes.

Males and females are equally affected with no racial predominance 4, 5. Periodontitis in HMC is usually unresponsive to traditional periodontal therapies.

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Various treatment approaches to periodontal conditions associated with PLS include oral hygiene instructions, use of chlorhexidine rinses, frequent debridement, systemic antibiotic regimens, periodontal surgery, extraction of hopeless teeth and referral to dermatologists to treat skin lesions 11, 16, Multiple opportunistic fungal infections in an individual Case report We describe a severe case of dermatophytosis associated hiperqueratois 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.


Based on clinical, dermatological and laboratory findings, a presumptive diagnosis of Papillon-Lefevre syndrome was made. Other features may include the tendency to frequent pyogenic infections and calcification of the pzlmoplantar, particularly palmoplanatr tentorium and falx cerebri 7.

Severe and atypical forms of dermatophytosis could lead to a diagnosis of AIDS. Patients with HMS have increased susceptibility to infections.

Periungueal involvement is typical, leading to secondary nail dystrophy. The patient’s tongue was bright and smooth with no papillae and white plaques on the oral mucosa hperqueratosis with the diagnosis of oral candidiasis.

The diagnosis could be crusted scabies or dermatophytosis.

The clinical presentation of infections caused by dermatophytes depends on many factors: Additional information Further information on this disease Classification s 5 Gene s 1 Other website s 3.

On the other hand, atypical forms of dermatophytosis have been linked to AIDS.