Résumé
Abstract: We report a case of granulomatous slack skin, a rare and indolent subtype of mycosis fungoides. It affects mainly men between the third and fourth decades. It is characterized by hardened and erithematous plaques that mainly affect flexural areas and become pedunculated after some years. Histological examination shows a dense infiltrate of small atypical lymphocytes involving the dermis (and sometimes the subcutaneous tissue) associated with histiocytic and multinucleated giant cells containing lymphocytes and elastic fibers (lymphophagocytosis and elastophagocytosis, respectively). Patients affected by this entity can develop secondary lymphomas. There are several but little effective therapeutic modalities described. Despite the indolent behavior of granulomatous slack skin, its early recognition and continuous monitoring by a dermatologist becomes essential for its management and prevention of an unfavorable outcome.
Sujets)
Humains , Mâle , Adulte , Tumeurs cutanées/diagnostic , Lymphome T cutané/diagnostic , Tumeurs cutanées/anatomopathologie , Tumeurs cutanées/traitement médicamenteux , Biopsie , Prednisone/usage thérapeutique , Immunohistochimie , Photographie (méthode) , Lymphome T cutané/anatomopathologie , Lymphome T cutané/traitement médicamenteux , Antinéoplasiques hormonaux/usage thérapeutiqueRésumé
A associação da dapsona, rifampicina e clofazimina tem se mostrado eficaz do tratamento da hanseníase multibacilar,entretanto a dapsona é responsável por inúmeros efeitos colaterais. Relata-se um caso de hepatoxocidade durante a poliquimioterapia, tratado com sucesso com a introdução de esquema alternativo com rifampicina,clofazimina e ofloxacino.
The combination of dapsone, rifampicin and clofazimine has proven quite effective in the treatment of multibacillary leprosy, however dapsone is responsible for numerous side effects. We report a case of hepatoxocidade during multidrug therapy, successfully treated with the introduction of alternative treatment with rifampicin,clofazimine and ofloxacin.
Sujets)
Humains , Mâle , Adulte , Dapsone/effets indésirables , Dapsone/usage thérapeutique , Lèpre multibacillaire/traitement médicamenteux , Clofazimine/usage thérapeutique , Lésions hépatiques dues aux substances , Ofloxacine/usage thérapeutique , Association de médicaments , Rifampicine/usage thérapeutiqueRésumé
A dapsona é uma medicação utilizada no tratamento da hanseníase e outras doenças cutâneas. Mesmo em doses habituais pode causar um quadro denominado síndrome da sulfona caracterizado por febre, hepatite,dermatite esfoliativa, linfadenomegalia, anemia hemolítica e atipia linfocitária. Trata-se de uma rara e grave reação de hipersensibilidade à sulfona com curso clínico imprevisível. Os autores relatam um caso de síndrome da sulfona, enfatizando seus principais achados clínicos,laboratoriais e histopatológicos.
Dapsone is a drug used to treat leprosy and other skin diseases. Even in normal doses can cause a condition called sulfona syndrome, characterized by fever, hepatiti sexfoliative dermatitis, generalised lymphadenopathy, hemolytic anemia and atypical lymphocytes in peripheral blood. Its a rare and severe hypersensitivity reaction to sulfone with an unpredictable clinical course. The authors report a case of sulfone syndrome, emphasizing its main clinical, laboratory and histopathological findings.