No Abstract available.

Pachydermodactyly is a kind of benign fibromatosis in which asymptomatic symmetrical soft tissue swellings occur on the proximal phalanges and the proximal interphalangeal joints of the hands. Although young women can also be affected, the young men are affected most commonly. There is no bony or articular abnormality on radiographic study. Histological examination of skin demonstrated epidermal hyperplasia, hyperkeratosis, acanthosis and thickened reticular dermis by deposition of collagen. It is important to recognize this disease identity without misdiagnosis for other rheumatologic diseases to avoid inappropriate and possibly toxic treatments. We report a case of pachydermodactyly and discuss the differential diagnosis.
Female ; Male ; Humans ; Diagnosis, Differential

Primary adrenal insufficiency is one of the rare clinical manifestations of antiphospholipid antibody syndrome. The pathogenesis is mainly ascribed to the hemorrhagic infarction secondary to thrombosis. We describe a 19-year-old man who developed symptoms of adrenal insufficiency and then was diagnosed of primary antiphospholipid antibody syndrome, which is the first reported case in adult patients in Korea. The possibility of adrenal failure should be considered in the management of antiphospholipid antibody syndrome and also, when primary adrenal insufficiency is accompanied by no definite etiology, the antiphospholipid antibody syndrome should be considered as a possible etiology.
Adult ; Male ; Female ; Humans

Gastrointestinal manifestations are common in systemic lupus erythematosus (SLE). Intestinal pseudo-obstruction (IpO) is a rare, poorly understood and recently recognized gastrointestinal manifestation of SLE. We report a 26-year-old female with SLE for 3 years. 10 months ago, IpO was diagnosed first, and it was responded well to high-dose steroid therapy. After then, oral prednisolone and azathioprine were administered, but the patient had been lost to follow up until recurrence of IpO. She was admitted with diffuse abdominal pain, distension, frequent vomiting, and intermittent dysuria due to recurrent IpO accompanied with bilateral ureterohydronephrosis. Despite of high dose steroid therapy, her symptom and imaging findings were not improved. The patient was treated with pulses of cyclophosphamide, and then the patient's symptoms and signs were gradually subsided. Three weeks following cyclophosphamide therapy, she was able to eat without vomiting and following abdominal CT showed nearly complete resolution of diffuse intestinal distension and bilateral ureterohydronephrosis. High level of awareness of IpO in SLE and appropriate medical treatment is needed to prevent unnecessary surgical treatment. And if this complication is refractory to corticosteroid, active immunosuppressive therapy, such as cyclophosphamide, should be considered.
Female ; Humans

Panniculitis is an inflammation within adipose tissue and most commonly affects the subcutaneous fat. Frequently, the panniculitis is associated with certain drugs and systemic diseases, such as various rheumatologic diseases, idiopathic Weber-Christian disease, infection and malignancy. Panniculitis in dermatomyositis (DM) is most commonly an incidental histopathologic finding that less commonly manifests a clinical component. We report the case of a woman with DM who presented with panniculitis as a clinical finding. A 51-year-old woman was admitted to our hospital with diffuse nodular, indurated, painful erythematous plaques on buttocks, back and chest. 18-months ago, she had been diagnosed DM at our hospital. Her medication on admission was azathioprine. High-dose glucocorticoid was prescribed after the diagnosis of lobular panniculitis confirmed by skin biopsy. After then, the patient was getting better and discharged with the medication tapered.
Female ; Humans ; Biopsy

Adult-onset Still's disease (AOSD) is a systemic inflammatory disorder with the characteristics of high spiking fever, evanescent salmon-colored skin rash, arthralgia or arthritis, lymphadenopathy, hepato-splenomegaly, sore throat, leukocytosis, negative autoantibody and hyperferritinemia. There are reports that rheumatic diseases such as dermatomyositis, polymyositis, hypertrophic osteoarthropathy, and polymyalgia rheumatica are associated with neoplasms. And small cell lung cancer, thymoma, lymphoma, leukemia, and breast cancer are known to be more associated with paraneoplastic syndromes mimicking rheumatic diseases. We experienced a case with bronchioloalveolar carcinoma who developed clinical manifestations of AOSD. Although there are several reports that AOSD is associated with paraneoplastic syndrome, to our knowledge, this is the first case of bronchioloalveolar carcinoma mimicking AOSD reported in the world.
Adult ; Male ; Female ; Humans ; Breast Neoplasms ; Lung Neoplasms

Behcet's disease is chronic and systemic inflammatory vasculitis, characterized by immunologically involving in variable size of arteries and veins. Clinically, principal manifestations are recurrent oral ulcer, genital ulcer, skin and eye lesions. Compared to other connective tissue disease, cancer is not accompanied commonly in Behcet's disease. But, immunological confusion such as T cell depletion or B cell hyperplasia, or long-term of immunosuppressive treatment lead to occurrence of malignancy. Recently, we experienced a case of maxillary mass, induced to abrupt headache in Behcet's disease, confirmed diffuse large B cell lymphoma by biopsy, and treated by rituximab-CHOP chemotherapy. Thus we report these with literature review.
Biopsy

Kikuchi-Fujimoto's disease (KFD), or histiocytic necrotizing lymphadenitis, is a rare benign and self-limiting disease. KFD are confused with systemic autoimmune disease as they present with localized lymphadenopathy, fever, fatigue, arthritis, leukopenia. Furthermore as KFD can occur associated with other autoimmune disease, we need to diagnose carefully. Here, we describe a case of 27-year-old female patient, diagnosed as KFD, who subsequently developed adult onset Still's disesase (AOSD). As far as we know, this is the first case of KFD with AOSD in Korea.
Adult ; Male ; Female ; Humans

Panniculitis is an inflammation of subcutaneous fat tissue, and found in variety of clinical setting, like erythema nodosum, lupus or other connective tissue diseases. Panniculitis, as a clinical feature of dermatomyositis, is rare and should be included in one of the sign to be differentiated. We present a 77-year-old female dermatomyositis patient who showed a clinically and histologically evident lobular panniculitis, and successfully treated with oral prednisolone.
Female ; Humans ; Erythema Nodosum

Wells' syndrome is an inflammatory dermatosis with associated aberrant eosinophil responses caused by unknown factors. Its histology is characterized by erythematous plaques with "flame figures" in the dermis, which is potentially diagnostic but not pathognomic. Cases of Wells' syndrome in patients with Churg-Strauss syndrome (CSS), which is characterized by antineutrophil cytoplasmic antibody-related necrotizing vasculitis, marked peripheral eosinophilia, and eosinophil tissue infiltrates, have rarely been reported, and the pathogenic association between these two diseases remains undetermined. Differences of clinical and histopathologic features of these two diseases suggest that they are distinct disease entities, even though, in part, they share pathogenic mechanisms. Here we present a new case with Wells' syndrome in a patient with CSS, treated with systemic steroid.