Pulmonary involvement in juvenile eosinophilic fasciitis: A case report

Introduction: Eosinophilic fasciitis (EF) is a rare scleroderma-like disease, characterized by acute onset of symmetrical swelling, induration and thickness of the skin. Laboratory findings include peripheral eosinophilia, elevated inflammatory markers and increased gammaglobulemia. Although, the diagnosis is mainly clinically, a full thickness skin/fascia/muscle biopsy remains the gold standard for the definite diagnosis. Unlike systemic sclerosis, visceral involvement, such as pulmonary affection, is rare in EF. Although, few cases of systemic involvement in adult EF have been presented, we report a rare case of juvenile EF associated with pulmonary involvement. Case report: A 12-years old boy presented with symmetrical skin thickening of bilateral upper extremity, back, trunk, neck, face, and scalp of 2-weeks duration. The laboratory tests revealed marked peripheral eosinophilia 61.9% (normal 1–3%) and elevation of the acute phase reactants (erythrocyte sedimentation rate 40?mm/1st hour and C-reactive protein 15?mg/dL). Lung imaging study showed bilateral extensive pulmonary nodules. A full thickness skin/fascia/muscle biopsy revealed an inflammatory infiltration, fibrosis in the fascial and muscle tissues. These findings were concordant with EF. An initial treatment of intravenous (IV) methylprednisolone 30?mg/kg/day for 3 successive days was started followed by oral steroid (2?mg/kg/day) plus methotrexate (20?mg/week). Follow up revealed complete improvement in the skin thickening, pulmonary affection and systemic inflammation. Conclusion: To the best of our knowledge this is the first reported co-existence of pediatric eosinophilic fasciitis with pulmonary affection. Systemic involvement should be screened in EF cases, as it may have consequences in the management and outcome.     

A case of eosinophilic fasciitis without skin manifestations: a case report in a patient with lupus and literature review

Clinical Rheumatology - Eosinophilic fasciitis (EF) is a rare connective tissue disease that causes inflammation and fibrosis of the fascia, inducing pain and motor dysfunction. Characteristic skin...     

Tube thoracostomy-related necrotizing fasciitis: a case report

Spontaneous pneumothorax is a serious complication of pulmonary tuberculosis that requires immediate treatment. Necrotizing fasciitis is a serious, rapidly progressive infection of the subcutaneous tissue and fascia, most related to trauma or surgery. Here, we report a case of pulmonary tuberculosis with spontaneous pneumothorax. A standard procedure of tube thoracostomy was performed for lung re-expansion. Two days after the tube was removed, necrotizing fasciitis developed from the puncture site. Computed tomography of the chest showed focal thickness with gas formation and loss of the fat plane over the chest wall, which is compatible with the diagnosis of necrotizing fasciitis. Aggressive treatment was given, including emergency fasciectomy and adequate systemic antibiotic and antituberculous treatment. The necrotizing fasciitis was successfully treated. The patient was discharged and sent home with maintenance antituberculous therapy.     

Idiopathic chronic eosinophilic pneumonia: a case report

Idiopathic chronic eosinophilic pneumonia (ICEP) is a rare cause of chronic lung disease in children and adolescents. We described four-years old boy presenting with recurrent pneumonia and symptoms of bronchial asthma. Because of peripheral eosinophilia and bilateral pulmonary infiltrates patient investigated comprehensive and chronic eosinophilic pneumonia determined histopathologically. Other conditions causing eosinophilic pneumonia were ruled out. He showed a dramatic response to oral corticosteroid therapy. This report emphasizes that ICEP should be considered in pediatric age group on a cause for chronic hypoxemi or intractable symptoms of respiratory system.     

Propylthiouracil-associated eosinophilic pleural effusion: a case report

We describe an unusual case of a patient with eosinophilic pleural effusion (EPE) associated with long-term propylthiouracil (PTU) administration. A 43-year-old woman was admitted to our hospital after complaining of chest pain. She had had Graves' disease, which had been treated with PTU for 11 years. Right-sided pleural effusion was detected and the result of thoracentesis confirmed an EPE. The patient's detailed medical evaluation failed to reveal any other cause of EPE. PTU was terminated since it was thought to be the cause. Despite withdrawal of the medication, however, the pleural effusion persisted for 6 weeks, and steroid therapy was planned for 15 days in decreasing dosages. During the control visit 10 days after the initiation of steroid therapy, no pleural effusion was observed, and the steroid was discontinued. Rechallenge with PTU produced recurrent pleural effusion. Therapy with PTU was again terminated, and treatment with methimazole and a brief course of low-dose corticosteroids were begun. Chest radiography revealed disappearance of the effusion within 10 days and it did not recur during a 1-year follow-up. To our knowledge, there is only 1 other case in the English-language literature describing EPE caused by PTU. Our report is of particular importance because it describes the development of that disorder in the 11th year of PTU treatment. It also shows that steroid therapy can be effective in treating drug-induced EPE.     

Chronic progressive eosinophilic fasciitis: report of a 20-year failure to attain remission

A retrospective diagnosis of eosinophilic fasciitis was made in a patient with disabling contractural disease of 20 years' duration. Chronic moderate-dose corticosteroid therapy had failed to halt either clinical or histological progression of the disease, but rapid worsening of skin thickening and contracture followed withdrawal of prednisone. Muscle wasting was severe in spite of normal serum creatine kinase levels; urinary excretion of creatinine was consistently elevated.     

Eosinophilic fasciitis and eosinophilic colitis: a rare association

SIR, Although eosinophilic fasciitis (EF) was originally consideredto be a disease predominantly localized to the fascia, severalcase reports have subsequently reported other manifestations[1–7]. Aplastic anaemia, haemolytic anaemia, thrombocytopenia,lymphoproliferative disorders, thyroiditis, pulmonary fibrosis,Sjögren's syndrome, Raynaud's phenomenon, myositis, mediumvessel vasculitis, pericarditis, colitis and glomerulonephritisare some of the manifestations that have been reported in patientswith EF. To our knowledge, the association of eosinophilic colitis(EC) and EF has been reported only once before [6]. Here, wedescribe an additional patient with EF who developed EC. A 38-yr-old previously fit and healthy man initially presentedto the general physicians in December 2000 with a 2-month history     

Bronchial involvement in chronic eosinophilic pneumonia: a case report

Chronic eosinophilic pneumonia (CEP) is an idiopathic chronic condition characterized by alveolar filling with mixed inflammatory infiltrate consisting largely of eosinophils. On CT, it is usually observed as consolidation, often peripheral and patchy in distribution, with upper lobe dominance. Airway involvement in CEP is very rare. We report on a case of bronchial involvement in CEP.     

Chronic eosinophilic pneumonia: a case report and national survey

Few reports of chronic eosinophilic pneumonia (CEP) in the pediatric population can be found in the literature. Our patient, a 16-year-old male subject presenting with signs and symptoms of CEP, prompted a survey of pediatric pulmonary training centers in the United States to determine the prevalence of eosinophilic pneumonia. The survey showed a low prevalence of acute eosinophilic pneumonia and CEP in the pediatric population, with an overall male/female ratio of 1.6:1.     

Atypical eosinophilic fasciitis localized to the hands and feet: a report of four cases.

We describe 4 patients who had episodic localized swelling of an extremity, 2 of the hand and 2 of the foot, in combination with peripheral eosinophilia and a biopsy characteristic of eosinophilic fasciitis (EF). Surgical excision produced marked improvement in the patients' symptoms. Whether these patients, despite a characteristic biopsy, can be included in the spectrum of EF remains uncertain.     

美沙拉嗪致嗜酸粒细胞肺炎1例并文献复习

目的 探讨美沙拉嗪导致嗜酸粒细胞肺炎(eosinophilic pneumonia,EP)的临床特征、诊断、治疗以及预后.方法 回顾性分析北京积水潭医院收治的1例美沙拉嗪所致EP临床资料并进行相关文献复习.分别以“mesalazine(5-aminosalicylate)”和“eosinophilic pneumonia”或“Lung”为检索词通过Pubmed、Embase及Cochrane数据库进行检索,检索时间为1987年8月至2015年9月;以“美沙拉嗪(5氨基水杨酸)”和“嗜酸粒细胞肺炎”或“肺”为检索词通过“中国期刊网全文数据库(CNKI)”和万方数据库进行检索,检索时间为1994年1月至2015年9月.结果 本例为56岁女性,在未确诊炎症性肠病情况下自行服用“美沙拉嗪”1个月后出现干咳、发热,外周血白细胞(WBC) 25.54×109/L,嗜酸粒细胞(EOS) 5.57×109/L (21.8％).肺CT显示双侧浸润影;BALF中EOS比例33％;肺功能示DL CO为46％预计值.停药后症状及影像很快改善,但2个月后DL CO仍低.在以上检索文献中共报道美沙拉嗪所致EP 9例.包括本例共10例患者,男3例,女7例,年龄23～56岁,平均34.5岁.主要表现为干咳、发热;胸部CT显示双肺浸润影;外周血及BALF中EOS升高.肺功能显示限制性通气功能障碍及DLCO降低.停用美沙拉嗪后症状及影像完全恢复.结论 美沙拉嗪导致的EP罕见,症状及影像不特异.诊断依赖于用药史、外周血、BALF中EOS升高或活检肺组织有EOS浸润.用药过程中出现不能解释的呼吸系统表现应警惕美沙拉嗪导致EP的可能,应及时停药.本病预后良好.