Weber Christian disease.
نویسندگان
چکیده
Weber Christian disease or idiopathic lobular panniculitis is a skin condition that features recurring inflammation in the fat layer of the skin. We report a case of a 17 year old boy with Weber Christian disease as its occurence is extremely rare with only sparse case reports available, who presented with eighteen-month history off fever with chills off and on, arthralgia predominantly of the knees, ankles, wrists and elbows without swelling or gross limitation of movement. There was also swelling around the eyes and puffiness of the face with lumpy swellings on the jaw, cheeks and chin and painful lumps on the anterior abdominal wall. On examination a febrile patient with pallor, pitting edema of the legs, periorbital edema and multiple non-matted firm to hard tender swellings of the face, jaw and chin and multiple firm tender lumps in the anterior abdominal wall was noted. Investigations revealed anemia, leucopenia, raised sedimentation rate and mild rise of liver enzymes. Biopsy of the abdominal nodule demonstrated a lobular panniculitis without vasculitis as seen in Weber Christian disease.
منابع مشابه
Systemic Weber-Christian disease with reversible bilateral ureteric obstruction.
It has been proposed that idiopathic retroperitoneal fibrosis may be a consequence of 'healed' retroperitoneal lesions of systemic Weber-Christian disease. However ureteric obstruction which is the hallmark of idiopathic retroperitoneal fibrosis, has not been described in systemic Weber-Christian disease. We report a patient with systemic Weber-Christian disease who, during a relapse, developed...
متن کاملSystemic Weber-Christian disease.
BACKGROUND Weber-Christian disease is a controversial entity that histologically presents as a lobular panniculitis. A systemic variant of this disorder, in which visceral fat is affected, has been reported. OBJECTIVE A 29-year-old woman with Weber-Christian disease developed a rapidly enlarging abdominal mass during hospitalization for a flare of her skin condition. The mass resolved spontan...
متن کاملIdiopathic lobular panniculitis (Weber Christian disease): a case report.
Weber Christian disease, an idiopathic relapsing febrile subcutaneous and visceral panniculitis is a rare disease in children. We report a case of Weber Christian disease for he first time from Kanti Children's Hospital.
متن کاملSystemic Weber-Christian disease
SYNOPSIS A patient suffering from Weber-Christian panniculitis was found at necropsy to have similar lesions in the visceral adipose tissue. When the lesions occur internally, diagnosis during life is complicated by the difficulty of examining visceral adipose tissue and the disease may often go unrecognized, but patients suffering from Weber-Christian panniculitis should be investigated bearin...
متن کاملOcular pathology associated with the Weber Christian syndrome.
Anterior and posterior uveitis has been described as an associated feature of the Weber Christian syndrome (Klien, I959), but only one report of a case has thus far appeared in the literature. The presence of uveitis in sections of an eye removed from a patient suffering from Weber Christian disease was described by Theobald (I953). Frayer, Wise, and Tsaltas (i968) described three cases of Webe...
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SUMMARY Histiocytosis X is associated with idiopathic Proliferation of histiocytes and infiltration of eosinophils in bone marrow Spleen, Liver and other Soft tissues. Clinically there are three distinct forms: 1 - Eosinophilic granuloma is the most benign and Localized form of the disease. 2- Letterer- Siwe is the most malignant, generalized and fatal form of the disease. 3- Hand- Schul...
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ورودعنوان ژورنال:
- The Journal of the Association of Physicians of India
دوره 51 شماره
صفحات -
تاریخ انتشار 2003