Kikuchi-Fujimoto's disease (KFD) or histiocytic necrotising lymphadenitis is a benign and self-limited disease, of unknown aetiology, which affects mainly young women. It presents with localised lymphadenopathy, predominantly in the cervical region, accompanied by fever and leukopenia in up to 50% of the cases. KFD has been rarely described in association with systemic lupus erythematosus (SLE), and its diagnosis can precede, postdate or coincide with the diagnosis of SLE. We present a patient with the diagnosis of SLE characterised by arthritis, leukopenia, malar rash, photosensitivity and positive ANA, besides cervical lymphadenopathy whose biopsy was compatible with KFD, which improved after using prednisone. Although the presence of lymphadenopathy is not uncommon in SLE patients, particularly in the phases of disease activity, the concomitance with KFD has rarely been reported in the literature. Its recognition is necessary because one can avoid laborious investigation for infectious and lymphoproliferative diseases.
Antiphospholipid (aPL) antibodies classically have been associated with thrombotic phenomena and abortion in patients with autoimmune diseases. The objective of the present work was to evaluate the frequency of such antibodies in patients infected with HIV and study its association with the presence of clinical manifestations of antiphospholipid syndrome (APS). Using a transversal study, a population of patients diagnosed with HIV, identified through an enzyme-linked immunosorbent assay (ELISA) test and confirmed by Western blotting, aged above 17 years old, was investigated. Through a standard questionnaire, the presence of APS manifestations was investigated, as well as the frequency of rheumatic manifestations. Antibodies against beta2 glycoprotein I (anti-beta2 GPI) and anticardiolipin (aCL) IgA, IgG, and IgM were investigated by the ELISA method using commercial kits (QUANTA Lite, INOVA Diagnostics). Ninety patients were studied, 47 (52.2%) male and 43 (47.8%) female. Clinical manifestations of APS were detected in 12 patients (13.3%) of the studied population, whereas arthralgia was the most common rheumatic manifestation (38.9%). Of the 90 patients, 40 (44.4%) were reactive for at least one type of aPL antibody (aCL and/or anti-beta2 GPI). The frequency of aCL was 17.8%, from which 15 (16.7%) had aCL IgG, 3 (3.3%) IgM, and 1 (1.1%) IgA. The frequency of the anti-beta2 GPI antibody was 33.3%, from which 29 (32.2%) were positive for isotype IgA, 4 (4.4%) isotype IgM, and 1 (1.1%) isotype IgG. No association was observed between immunoreactivity for aPL antibodies in general or each isotype in particular and the presence of APS manifestation. In the present study, it was possible to observe a relatively high frequency of aPL antibodies, particularly for isotype IgA anti-beta2 GPI in HIV. However, there was no association to APS manifestations, suggesting that such antibodies had no etiopathogenic role in these complications in patients with such retroviral infection.
RESUMOO hiperparatireoidismo primário (HPP) é uma doença metabólica que resulta da secreção anormal do hormônio da paratireóide (PTH), sendo uma das causas mais comuns de hipercalcemia na população geral. Apresentamos o caso de uma paciente de 47 anos, com diagnóstico prévio de lúpus eritematoso sistêmico (LES) há dois anos, que apresentou manifestações clínicas (dor óssea difusa, labilidade emocional, tumoração em ramo mandibular esquerdo) e laboratoriais (cálcio séri-co= 13,5mg/dL, fosfato= 1,8mg/dL, fosfatase alcalina= 3.028U/L, PTH intacto= 1472pg/dL) que culminaram com o diagnóstico de HPP, complementado pelo estudo anátomo-patológico, que evidenciou adenoma de paratireóide. Após o tratamento com calcitonina nasal 400UI/dia, pamidronato 90mg IV/semana e, por fim, a paratireoidectomia subtotal, a paciente apresentou melhora do quadro clínico e normalização dos exames laboratoriais. A presente descrição se constitui no segundo caso relatado na literatura, com tal associação de doenças. Embora os mecanismos etiopatogênicos do HPP em LES não estejam ainda definidos, essa associação deve ser sempre lembrada, uma vez que a estratégia terapêutica tende a mudar completamente. Primary hyperparathyroidism (PHP) is a metabolic illness that results from autonomous secretion of parathyroid hormone and is one of the most common causes of hypercalcemia. We present the case of a 47-yearold female with a previous diagnosis of systemic lupus erythematosus (SLE) in whom clinical (diffuse bone pain, emotional lability, jaw tumor) and laboratory features (calcium= 13.5mg/dL, phosphate= 1.8mg/dL, alkaline phosphatase= 3028U/L, PTH intact= 1472pg/dL) prompted the diagnosis of PHP secondary to parathyroid adenoma as demonstrated by the anatomopathology. After treatment with calcitonin spray 400UI per day, IV pamidronate 90mg/week, and subtotal parathyroidectomy, the patient status improved with normal laboratory tests. This is the second report to describe the coexistence of these two disorders in a single patient. Although the pathophysiology of the association of PHP and SLE is not known, the recognition of this association has a practical implication since the therapeutical strategy is completely different.
Dapsone is a drug primarily used for the treatment of Hansen's disease, and it has also been employed in cases of bullous lupus erythematosus and some types of cutaneous vasculitis. Recently, its efficacy in the treatment of cutaneous lesions in dermatomyositis has been observed. We present two cases of dermatomyositis, amyopathic form, which were refractory to habitual treatment, but had an excellent response to dapsone therapy. 1,2 For half a century, dapsone has been used for its anti-inflammatory properties, particularly aimed at leukocytes, for the treatment of Hansen's Disease and autoimmune diseases, such as bullous lupus erythematosus and relapsing polychondritis. Literature has shown some reports of DM with good response of its cutaneous manifestations to the use of dapsone. We hereby present two patients with refractory amyopathic dermatomyositis, who responded to treatment with dapsone. Case 1Fifty five-year-old white male patient, who presented, four years ago, erythematous pruritic cutaneous eruptions, with the finding of photosensitivity in the face and upper trunk, shawl Sign and V-Sign on physical examination, besides heliotrope and Gottron's Sign. Muscle strength was preserved (degree: 5+/5+). Histopathological study of the skin revealed an epidermis with discrete acanthosis, edematous dermis with mononuclear infiltrate surrounding vessels and annexes, besides decrease of pillous follicles. Antinuclear factor (ANF) was negative, and Creatinophosphokinase (CPK) values were within normal range. No muscle biopsies or electroneuromyography (ENMG) were carried out. The case was diagnosed as amyopathic dermatomyositis, and treatment with chloroquine diphosphate at 250 mg/day was begun, without satisfactory response. Dapsone at 50 mg/day was then introduced and after two months the dose was built up to 100 mg/day, with a significant improvement. Later on, patient had the onset of an insidious ventilatory-dependent thoracic pain associated with weight loss, with the finding, on tomographic investigation, of a pulmonary nodule, the histopatho-
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