RESUMO
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.
Assuntos
Edema/patologia , Eritema/patologia , Exantema/patologia , Pele/patologia , Doença de Still de Início Tardio/patologia , Adulto , Feminino , HumanosAssuntos
Anticorpos Anticitoplasma de Neutrófilos/imunologia , Crioglobulinas/imunologia , Toxidermias/imunologia , Propiltiouracila/efeitos adversos , Vasculite/induzido quimicamente , Vasculite/imunologia , Adulto , Antitireóideos/efeitos adversos , Toxidermias/diagnóstico , Toxidermias/etiologia , Feminino , Humanos , Pele/patologia , Vasculite/diagnósticoRESUMO
BACKGROUND: Pemphigus vulgaris was almost fatal before the advent of glucocorticoids. Unfortunately, the high doses and prolonged administration of glucocorticoids, which often needed to control the disease, result in numerous adverse effects many of which are serious. AIMS: To evaluate the patients with pemphigus vulgaris on treatment in respect of osteoporosis and to compare the frequency of osteoporosis in these patients with the healthy ones. METHODS: The study consisted of 40 patients with pemphigus vulgaris and 34 healthy controls. Bone mineral density measurements were obtained by dual- energy X-ray absorptiometry. Blood serum, bone parameters, and biochemical hormonal measurements were examined in both groups. RESULTS: When the bone mineral density values of patients with pemphigus vulgaris were compared with those of the control group, there was no significant difference between hip bone mineral density values, while lumbar region T and Z scores were found significantly low in the patient group (p = 0.034 and p = 0.006, respectively). Osteoporosis, osteopenia, and normal dual-energy X-ray absorptiometry rates in the patient group were found to be 32.5%, 32.5%, and 35%, respectively. These rates were found to be 18%, 23%, and 59% in control group, respectively. There were more fractures in the patient group and the difference was statistically significant (p = 0.004). CONCLUSION: An increase in osteoporosis frequency and secondary fracture to osteoporosis in the patients with pemphigus vulgaris was detected.