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Mal meleda treatment

Sabine Duchatelet, Alain Hovnanian
Olmsted syndrome (OS) is a rare genodermatosis classically characterized by the combination of bilateral mutilating transgredient palmoplantar keratoderma (PPK) and periorificial keratotic plaques, but which shows considerable clinical heterogeneity. The disease starts usually at birth or in early childhood. About 73 cases have been reported worldwide. OS is observed in both sexes, although male cases are more frequent. The most suggestive symptoms associate PPK with pseudoainhum and periorificial keratotic plaques...
2015: Orphanet Journal of Rare Diseases
Amir Muzur
While the first traces of interest in skin diseases date back to the 14(th) century recipes from Glagolitic manuscripts, the scientific beginnings of dermatovenereology might be associated with the beginning of the 19(th) century, when in the Rijeka region a 'new disease', called after the village of Škrljevo, was studied by Giovanni Battista Cambieri. This, however, has not been the only disease named after a Croatian toponym; in 1826, Luca Stulli of Dubrovnik was the first to describe the 'mal de Meleda', nowadays recognized as a form of hereditary palmoplantar keratoderma...
2010: Acta Dermatovenerologica Croatica: ADC
A Marchac, C Blanchet-Bardon, M Revol, J-M Servant
BACKGROUND: Mal de Meleda (MDM, OMIM #248300) is a rare congenital palmoplantar keratosis. Deep fissures cause pain and limit extension of the metacarpo-phalangeal joints. We report the case of a patient operated on both hands with a 29-year interval between each hand. OBSERVATIONS: A 53-year-old patient with MDM demonstrated severe keratosis of the left hand. The same surgeon operated on the right hand. Both hands were operated using the same technique. Skin of the palm, the palmar side of the index, and the first phalangeal of third, fourth and fifth fingers were excised with a sharp rugine...
April 2009: Annales de Chirurgie Plastique et Esthétique
Sergei A Grando, Mark R Pittelkow, Karin U Schallreuter
The presence of an autocrine adrenergic and cholinergic intra/intercellular signal transduction network in the human epidermis contributes significantly to homeostatic and compensatory responses regulating vital functions in keratinocytes and melanocytes. The ligands produced control autocrine and paracrine loops to initiate responses through cognate receptors expressed within the same or adjacent cells. The epidermal adrenergic signal controls calcium homeostasis, cell growth, differentiation, motility, and pigmentation via the beta2 and alpha1 adrenoceptors...
September 2006: Journal of Investigative Dermatology
No abstract text is available yet for this article.
May 1950: Archives of Dermatology and Syphilology
Cagri Ergin, Seniz Ergin, Sevtap Arikan
Mal de Meleda is a rare autosomal recessive form of palmoplantar keratoderma characterized by hyperkeratosis of the palms and soles. The presence of yeast and dermatophytes was investigated in 29 mal de Meleda patients from Koprucay canyon, Turkey, a newer geographical focus, and was found in 62.0% and 20.7% of cases, respectively. Antifungal resistance of isolates was not detected.
2002: Scandinavian Journal of Infectious Diseases
A Bakija-Konsuo, A Basta-Juzbasic, I Rudan, M Situm, Magdalena Nardelli-Kovacic, Sonja Levanat, Judith Fischer, Daniel Hohl, Davorin Loncaric, Sven Seiwert, Harry Campbell
BACKGROUND: Mal de Meleda (keratoderma palmoplantaris transgrediens) is an autosomal recessive disorder, first described on the island of Mljet (Meleda), Croatia. The candidate region for the gene responsible for this disorder was found on the chromosome 8qter, and the responsible mutations have recently been identified in 12 Algerian and 7 Croatian families. OBJECTIVES: To fully characterize all 12 living cases originating from the original setting of the disease, the island of Mljet, in the light of new findings and using modern diagnostic technology...
2002: Dermatology: International Journal for Clinical and Investigative Dermatology
R Bergman, O Bitterman-Deutsch, M Fartasch, R Gershoni-Baruch, R Friedman-Birnbaum
Pseudoainhum is an infrequent complication in the autosomal-recessive keratodermas. We describe two related families in which the diagnosis of mal de Meleda keratoderma has been confirmed by mode of inheritance and ultrastructural findings. One family member, a 9-year-old girl, developed pseudoainhum which threatened the viability of her little fingers. This responded to treatment with etretinate. The treatment dilemma posed by keratoderma-induced pseudoainhum in children, i.e. the concern over the possible skeletal toxic effects of long-term etretinate treatment vs...
February 1993: British Journal of Dermatology
S H Jee, Y Y Lee, Y C Wu, Y C Lü, C C Pan
A family in Taiwan is reported to have Mal de Meleda with possibly autosomal recessive inheritance among 5 of its members. The glove- and sock-like erythrokeratosis and the hyperkeratotic plaques of the knees and elbows are the common features. Erythema of the nose, cheeks, and the perioral area in 4 affected members, actinic elastosis in 2 severely affected members, and partially impaired cell-mediated immunity in vitro in both affected and unaffected members are notable findings. Tigason (etretinate) treatment was beneficial in regard to hyperkeratinization, but not to erythema...
1985: Dermatologica
R Happle, P C van de Kerkhof, H Traupe
Hereditary disorders of keratinization may be a considerable handicap. Oral treatment with retinoids has been shown to be effective in many of these diseases. In the group of ichthyoses, the best results can be obtained in the various types of nonbullous congenital ichthyosis (erythrodermic autosomal recessive lamellar ichthyosis, nonerythrodermic autosomal recessive lamellar ichthyosis, autosomal dominant lamellar ichthyosis). It should be borne in mind, however, that retinoid therapy alone cannot lead to a complete response of these forms of ichthyosis and that this treatment cannot replace an appropriate topical treatment...
1987: Dermatologica
H Traupe, R Happle
Keratinization defects can be very severe and disfiguring diseases. The development of retinoids such as etretinate has provided us with an effective symptomatic form of oral therapy for these skin conditions. Based on our own experience, we briefly outline the therapeutic potential of etretinate in various keratinization defects (lamellar ichthyosis, Netherton syndrome, Sjögren-Larsson syndrome, mal de Meleda and juvenile pityriasis rubra pilaris). The toxicology of etretinate is reviewed with special regard to the treatment of children...
January 1985: European Journal of Pediatrics
V P Sybert, B A Dale, K A Holbrook
The hereditary palmar-plantar keratodermas are a heterogeneous group of disorders characterized by hyperkeratosis of the palms and soles and distinguishable by other clinical characteristics, associated abnormalities, and mode of inheritance. We report a family with a new autosomal dominant condition with clinical similarities to mal de Meleda and Greither's disease. Biochemical characteristics before and during treatment with oral isotretinoin were typical of hyperproliferative epithelium with expression of the 48-kd keratin...
January 1988: Journal of the American Academy of Dermatology
T Iio, S Shiraishi, K Sayama, Y Miki
A 38-year-old Japanese man was seen for severe palmoplantar keratoderma, extending to the dorsal aspects with red rims. He had had spontaneous amputations of the toes and surgical amputation of the right lower leg because of squamous cell carcinoma of the right sole. The clinical symptoms suggested those of mal de Meleda, except for the absence of consanguinity and of granular layers in the epidermis. The keratoderma improved with oral etretinate treatment.
January 1991: Journal of Dermatology
M L Reed, J Stanley, F Stengel, J L Shupack, D M Benjamin
Identical twins with mal de Meleda, a rare genodermatosis, displayed the characteristic "glove and sock" hyperkeratosis, hyperhidrosis, and malodor. Their parents, who are first cousins, are unaffected and originated from Calabria, Italy, which is not far from Meleda (Mljet). The disorder is probably transmitted as an autosomal recessive trait. Previous therapy with grenz rays, topical preparations of adrenocorticosteroids, lactic acid, retinoic acid, and bland emollients had been unsuccessful. One of the twins was treated with 13-cis retinoic acid taken by mouth for 16 weeks with dramatic improvement...
May 1979: Archives of Dermatology
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