ABSTRACT
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.
Subject(s)
Humans , Male , Adult , Skin Neoplasms/diagnosis , Lymphoma, T-Cell, Cutaneous/diagnosis , Skin Neoplasms/pathology , Skin Neoplasms/drug therapy , Biopsy , Prednisone/therapeutic use , Immunohistochemistry , Photography , Lymphoma, T-Cell, Cutaneous/pathology , Lymphoma, T-Cell, Cutaneous/drug therapy , Antineoplastic Agents, Hormonal/therapeutic useABSTRACT
Pseudoxanthoma elasticum is a rare inherited multisystem disorder that is characterized by a pathological mineralization of the elastic connective tissue, which involves predominantly the skin, eyes and cardiovascular system. Its cause lies on mutations in the ABCC6 gene, which lead to reduction or absence of the transmembrane transport ADP dependent protein (MRP6), causing an accumulation of extracellular material and subsequent deposition of calcium and other minerals in the elastic tissue. The authors report two cases of pseudoxanthoma elasticum, emphasizing its major clinical features and the importance of early diagnosis of the disorder, aiming for adequate therapeutic management of associated complications.
Subject(s)
Female , Humans , Middle Aged , Pseudoxanthoma Elasticum/pathology , Early Diagnosis , Fluorescein Angiography , Retina/pathology , Skin/pathologyABSTRACT
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.