pemphigus vulgaris: pentingnya diagnosis dini, penatalaksanaan yang komprehensif dan adekuat (laporan kasus)
Pênfigo vulgar: a importância do diagnóstico precoce, manejo abrangente e adequado (relato de caso)
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Abstract
Pemphigus vulgaris is an autoimmune–mediated disease of skin and mucous membran leading to progressive blistering and chronic erosions. It often begins with blister formations which easily rupture. The characteristic feature is positive nikolsky sign which may or not be presented simultaneously. Establishment of early definite diagnosis is critical and requires correlation of clinical and histopathological findings. Because of this conditon is a potentially life-threatening, the risk of complications and mortality rate increases if initial management is non comprehensive and inadequate. Treatment is directed at supression of autoimmune process, typically administration of corticosteroids. This article report a case in a 51 years old woman who had painful chronic oral ulcer and poor general health condition. Prior to the visit to Oral Medicine clinic, patient was treated by her general practitioners for several months, without either established diagnosis nor comprehensive and adequate management, so that she had no clinical improvement. Clinical examination at the first visit in Oral Medicine clinic Cipto Mangunkusumo Hospital, revealed bula in the skin, conjunctivitis, easily bleed oral mucosae, widespread erosions and ulcerations of the lips, gingiva, tongue, and buccal mucosae. Biopsy of one of skin erosions demonstarting suprabasal intraepithelial acantholysis. Multi divisions in Hospital, such as Oral Medicine, Dermatology, Internal Medicine, Ophtalmology, ENT, were involved in treating this case. Patient received high dose methylprednisolone sistemically and prednisone topically for the lips. General remission achieved in several days. Thus, establishment of early definite diagnosis and adequate management are important in management of Pemphigus vulgaris.
DOI: 10.14693/jdi.v16i1.20
O pênfigo vulgar é uma doença de mediação autoimune da pele e das membranas mucosas que leva à formação progressiva de bolhas e erosões crônicas. Frequentemente, inicia-se com a formação de bolhas que se rompem facilmente. O achado característico é o sinal de Nikolsky positivo, que pode ou não estar presente de forma concomitante. O estabelecimento precoce de um diagnóstico definitivo é crucial e requer a correlação entre os achados clínicos e histopatológicos. Por ser uma condição potencialmente fatal, o risco de complicações e a taxa de mortalidade aumentam caso o manejo inicial seja incompleto e inadequado. O tratamento é direcionado à supressão do processo autoimune, tipicamente mediante a administração de corticosteroides. Este artigo relata o caso de uma mulher de 51 anos que apresentava úlceras orais crônicas dolorosas e comprometimento do estado geral de saúde. Antes do atendimento no ambulatório de Estomatologia, a paciente foi tratada por clínicos gerais durante vários meses, sem diagnóstico estabelecido ou manejo abrangente e adequado, de modo que não obteve melhora clínica. O exame clínico na primeira consulta no ambulatório de Estomatologia do Hospital Cipto Mangunkusumo revelou bolhas cutâneas, conjuntivite, mucosa oral com facilidade de sangramento, além de erosões e ulcerações disseminadas nos lábios, gengiva, língua e mucosa jugal. A biópsia de uma das erosões cutâneas demonstrou acantólise intraepitelial suprabasal. Múltiplas especialidades hospitalares, tais como Estomatologia, Dermatologia, Clínica Médica, Oftalmologia e Otorrinolaringologia, estiveram envolvidas no tratamento deste caso. A paciente recebeu metilprednisolona sistêmica em altas doses e prednisona tópica nos lábios. A remissão geral foi alcançada em poucos dias. Dessa forma, o estabelecimento precoce do diagnóstico definitivo e o manejo adequado são essenciais na condução do pênfigo vulgar.
DOI: 10.14693/jdi.v16i1.20
| Reference Key |
rezeki2012journalpemphigus
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|---|---|
| Authors | ;Sri Rezeki;Titiek Setyawati |
| Journal | arabian journal of chemistry |
| Year | 2012 |
| DOI |
10.14693/jdi.v16i1.20
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