ABSTRACT
A policondrite recidivante é uma doença rara, multissistêmica, de etiologia desconhecida, provavelmenteautoimune, que acomete estruturas cartilaginosas e tecidos ricos em proteoglicanos. Orelha, nariz, articulações e árvore traqueobrônquica são os locais mais afetados durante os surtos inflamatórios, ocasionando destruição tecidual, deformidades permanentes e complicações respiratórias. Menos comumente, afeta olhos, coração e vasos sanguíneos.O diagnóstico é essencialmente clínico e o tratamento envolve o uso de corticoides e imunossupressores. Relatamos um caso de policondrite recidivante, em que a manifestação do segundo surto da doença ocorreu por meio de bloqueio atrioventricular com recuperação da condução cardíaca após tratamento com corticoide...
The relapsing polychondritis is a rare, multisystem disease, whose etiology is unknown. It is probably autoimmune and affects cartilaginous structures and proteoglycan-rich tissues. The ear, nose, joints and tracheobronchial tree are the most affected sites during inflammatory outbreaks causing tissue destruction,permanent deformities and respiratory complications. Less commonly, it affects the eyes, heart and blood vessels. The diagnosis is essentially clinical and treatment involves the use of steroids and immunosuppressive agents.We report a case of relapsing polychondritis where the second outbreak of the disease was manifested by na atrioventricular block with recovery of cardiac conduction after treatment with steroids...
Subject(s)
Humans , Female , Adult , Atrioventricular Block/complications , Autoimmune Diseases/diagnosis , Autoimmune Diseases/therapy , Polychondritis, Relapsing/complications , Polychondritis, Relapsing/etiology , Heart Atria/abnormalities , Adrenal Cortex Hormones/therapeutic use , Prednisone/administration & dosage , Heart Ventricles/abnormalitiesABSTRACT
Certas dermatoses, pertencentes ao grupo das síndromes paraneoplásicas mucocutâneas, podem ser o prenúncio de uma neoplasia previamente não conhecida. Tanto a síndrome de Sweet como a policondrite recidivante incluem-se neste grupo. A síndrome de Sweet e a PR são raramente encontradas em um mesmo paciente. A presença de policondrite recidivante e síndrome de Sweet em um mesmo paciente tem se revelado mais frequente em pacientes com neoplasias associadas, sobretudo hematológicas. Relata-se o caso de paciente do sexo masculino, 79 anos, com síndrome de Sweet e policondrite recidivante, em quem, subsequentemente, foi diagnosticada uma síndrome mielodisplásica.
The emergence of certain skin conditions belonging to the group of mucocutaneous paraneoplastic syndromes may indicate the future appearance of a previously unknown malignancy. Sweet's Syndrome and relapsing polychondritis are included in this group. Sweet's Syndrome and relapsing polychondritis are very rarely found together in the same patient. This dual occurrence is more commonly found in cancer patients with associated hematological malignancies. We report the case of a 79year-old male with Sweet's Syndrome and relapsing polychondritis, who was subsequently diagnosed with a myelodysplastic syndrome.
Subject(s)
Aged , Humans , Male , Myelodysplastic Syndromes/complications , Polychondritis, Relapsing/etiology , Polychondritis, Relapsing/pathology , Sweet Syndrome/etiology , Sweet Syndrome/pathology , Myelodysplastic Syndromes/pathology , RecurrenceABSTRACT
Relapsing Polychondritis [RP] is an uncommon inflammatory disorder of unknown cause characterized by an episodic and progressive course affecting predominantly the cartilage of the ears, nose and laryngotracheobronchial tree. It has seldom been described in children. We report on a 2-year old girl with a preliminary diagnosis of Juvenile Chronic Arthritis [JCA], when she was 1 year old. She was treated with Non Steroidal Anti-inflammatory Drugs [NSAIDs] and oral steroids. Six months later her polyarthritis relapsed and she had additional bilateral auricular chondritis; at this time RP was diagnosed due to the appearance of criteria of McAdam-Michet et al. RP should be considered in differential diagnosis of JCA. Steroids and systemic immunosuppressants can be used to treat the RP