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Treatment with cyclosporin A in dermatology includes an ever increasing spectrum of diseases. Further indications include the use (along with corticoids) in the group of bullous diseases. A 57 year-old patient who was never sick except for a mild ethylic hepatopathy developed a major eruption of bullae (on a non-inflamed or inflamed backgroud) on the lower half of the trunk, beneath the knees, on the thighs and rarely on the forearms. The condition was complicated by the formation of extensive necrotic foci at the sites where the surface of the bullae was damaged and by the general alteration of the patient. The assumed diagnosis was bullous pemphigoid. The histological examination revealed the diagnosis more accvirately as pemphigus vulgaris. The treatment used was cyclosporin A, 5 mg/kg/day. Already on the fifth day dramatic improvement of the local finding was recorded. Laboratory monitoring revealed however a steady increase of creatinine levels. Therefore cyclosporm A was gradually discontinued for six weeks and the local finding was cured. Revision of the histological examination and of the final diagnosis were pemphigoid buUosus. After more than one year the patient has no signs of dermatosis and laboratory parameters are within normal limits.

MeSH
bulózní pemfigoid diagnóza etiologie farmakoterapie MeSH
cyklosporin aplikace a dávkování terapeutické užití MeSH
lidé středního věku MeSH
lidé MeSH
Check Tag
lidé středního věku MeSH
lidé MeSH
mužské pohlaví MeSH
Publikační typ
kazuistiky MeSH

Článek

Protrahované infekcie u pacienta s morbus Milroy v kombinácii s morbus Recklinghausen
[Protracted infection in a patient with Milroy's disease combined with Recklinghausen's disease]

Zelenková, Hana
Autor Zelenková, Hana

Československá dermatologie. 1999 ; Roč. 74 (č. 4) : s. 176-179.

Medvik
Zdroj

Milroy's disease, familial congenital lymphoedema (Meige, 1889, Nonne, 1891, Milroy, 1892) trophoedema, is a rare strictly autosomal dominant disease with varying penetration and expresivity. It is characterized by congenital lymphoedema or oedema developing soon after birth. The lymphoedema spreads slowly, sometimes in attacks, and affects mainly the lower extremities, rarely the upper ones or other sites. Later the changes imitate elephantiasis. The described case concerns twin boys born in 1963 where in 1964 Milroy's disease was diagnosed. In the investigated patient the left lower extremity was affected. The monstrously developing changes of the leg were gradually treated by the method of „shave therapy" in 1983 - 1996 six operations. Since 1990 development of malum perforans ped.l.sin., operated (by application and shift of a flap) three times. From 1987 -1998 repeatedly erysipelas (42x) and development of drug allergy. In 1986 for the first time doubts were expressed as regards the diagnosis of Milroy's disease on account of numerous clinical symptoms suggesting Recklinghausen's disease. In the brother bizarre changes in the region of the neck and right ear were found treated by surgery in 1997. Since 1987 also Recklinghausen's disease is suspected. The patient is at present a complete invalid, he does not agree with the proposed amputation.