Atypical cutaneous features in adult onset Still's disease.

Adult onset Still's disease is a rare but potentially serious disease. We present five cases of adult-onset Still's disease seen by us over a period of one year. The patients were all females and 28-39 years of age. Symptoms had been present for 2-6 weeks in three patients. The other two had been on a few years' follow-up for rheumatoid arthritis before the onset of rashes and fever. The patients had persistent erythematous maculopapular eruptions on face, body and extremities, with moderate to severe pruritus and/or a burning sensation that decreased their quality of life. The typical evanescent rash was not observed. High ferritin values were detected in all the patients and total serum IgE was increased in two. All the patients were started on oral prednisolone (0.5-1.0 mg/kg/day), and methotrexate (10-15 mg/week) had to be added in three patients. One patient was started on tocilizumab due to recalcitrant disease and one was lost to follow-up. Further investigation and classification of the various atypical cutaneous findings in adult-onset Still's disease is necessary.

Aggressive angiomyxoma of the vulva in a patient with systemic lupus erythematosus.

Aggressive angiomyxoma is a rare, slow-growing mesenchymal neoplasm with a tendency to recur. It mainly involves the pelvis, vulva, perineum, vagina, and urinary bladder in adult women of reproductive age group. We describe a 26-year-old female with large swellings of both labia majora which was histologically diagnosed as aggressive angiomyxoma. She also had systemic lupus erythematosus. The swelling was surgically removed and she had no recurrence at 1-year follow-up. Although it is a rare tumor, it must be considered as a differential diagnosis for any mass in the perineum or soft tissue of the pelvis. Long-term follow-up is necessary for early diagnosis of local recurrence.

Lest we forget Hansen's disease (leprosy): an unusual presentation with an acute onset of inflammatory polyarthritis and the rheumatology experience.

Several forms of arthritis and rheumatism can sometimes complicate leprosy. However, its presentation as an acute onset arthritis is unusual. We report two adult male naïve patients who presented to our rheumatology outpatient clinic with acute onset inflammatory polyarthritis, skin rash and mild sensory neurodeficit. Borderline lepromatous leprosy (in type I lepra reaction) was diagnosed. We also refer to 19 case records of Hansen arthritis in the clinic database (1998-2007) from approximately 35,000 patients and a community study to highlight the missed diagnosis of Hansen's disease and its unusual association with rheumatoid arthritis. In countries like India where leprosy is endemic, this disease also merits attention in rheumatology clinics.

Síndrome da sulfona e reaçäo reversa / Sulfone syndrome and reverse reaction

Um homem de 68 anos de idade, cor preta, interna-se por hanseníase multibacilar, nao tratada, apresentando infiltraçao cutânea difusa e nódulos raros de Eritema nodoso hansênico. Na época, (1982) foi tratado com sulfona e talidomida. Um mês e meio após o início do tratamento surge erupçao cutânea eritematosa difusa, acompanhada de febre alta, adenomegalia generalizada e hepatoesplenomegalia. O hemograma demonstrou leucocitose intensa com linfocitose e atipias de linfócitos. Evoluiu com icterícia, desidrataçao, confusao mental, falecendo no 4( dia após instalaçao da erupçao referida. Na necrópsia o substrato do exantema generalizado é um infiltrado linfocitário pleiomórfico em pele, mucosas e vísceras. Este quadro foi interpretado como a "Síndrome da Sulfona", manifestaçao de hipersensibilidade à esta droga e que em muitos aspectos mimetiza ou induz o aparecimento de uma monucleose infecciosa. Há algumas publicaçoes sobre esta síndrome, algumas relatando casos fatais, más nao há descriçao de achados necroscópicos. Ao lado do infiltrado linfocitário atípico observou-se também reaçao granulomatosa tuberculóide generalizada englobando infiltrado de padrao virchoviano com baciloscopia rica. Os autores acreditam que este quadro representaria uma reaçao reversa induzida pela "Síndrome da Sulfona".