Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020473 (hyperlipidemia)
 15,891 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 60-year-old man presented to the Emergency Department (ED) with large, painful, indurated plaques on the right thigh, left abdomen, left chest, and right chest, which began without any preceding trauma on the right thigh 3 weeks prior to presentation in the ED. He was initially treated with cefazolin 1 g three times daily as home infusions. When the lesions continued to progress, he was admitted to the hospital and placed on amoxicillin/clavulanate and vancomycin. He had a single episode of fever of 102 degrees F, but his white blood cell count and differential remained normal. An initial biopsy showed a dermal inflammatory infiltrate composed primarily of neutrophils and eosinophils with rare flame figures in the dermis. There was minimal fat seen in this biopsy. A differential diagnosis of Wells or Sweet's syndrome was entertained, and he was placed on 60 mg/day prednisone with no resolution of his symptoms. The patient's past medical history included hypertension, hyperlipidemia, peripheral neuropathy, and hiatal hernia. His family history was significant for emphysema in both parents and coronary artery disease in his father. Both of his parents smoked cigarettes. His grandfather, who was a coal miner, also had emphysema. Whilst on antibiotics and prednisone, the plaques on the patient's right thigh, right abdomen, and left chest expanded and ulcerated, draining an oily liquid (Figs 1 and 2). An incisional biopsy was obtained from his thigh. Histopathology showed a septal and lobular panniculitis with fat necrosis, neutrophils, and histiocytes (Fig. 3). Special stains for organisms were negative. Tissue sent for bacterial and fungal culture had no growth. Amylase and lipase levels were normal. Rheumatoid factor, antinuclear antibody (ANA), antineutrophil cytoplasmic antibody (ANCA), cryoglobulins, and antiphospholipid antibodies were all normal. The alpha1-antitrypsin level was low at 25 mg/dL (ref. 75-135). The alpha1-antitrypsin phenotype was PiZZ. The patient had a normal glucose-6-phosphate dehydrogenase level and was placed on dapsone 200 mg/day. The inflammation resolved and, over the course of several months, the involved areas healed with scarring. The patient denied any pulmonary complaints but, during his hospitalization, was found incidentally to have an oxygen saturation of 88% on room air. He was sent for evaluation by a pulmonologist, and pulmonary function tests revealed a mixed restrictive and obstructive pattern with a forced expiratory volume in 1 to forced vital capacity (FEV(1)/FVC) ratio of 63% of predicted. He had never smoked. He was placed on supplemental oxygen but, as his pulmonary disease has been stable, he has not been treated with intravenous antitrypsin inhibitor.
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PMID:alpha1-Antitrypsin deficiency presenting with panniculitis and incidental discovery of chronic obstructive pulmonary disease. 1791 Jul 20

A 58-year-old man with a history of hyperlipidemia and hypertension presented to the dermatology clinic with a 3-month history of a sudden onset, progressively worsening pruritic eruption involving the torso and extremities. Prior treatment included azithromycin and oral and intramuscular steroids, without improvement. Laboratory results demonstrated a serum eosinophil count of 7x10(3)/uL (normal 0-4). A 4-mm punch biopsy of the plaque on the patient's left thigh revealed a diffuse dermatitis with innumerable eosinophils with formation of "flame figures." Histologically, these findings are consistent with a diagnosis of Wells syndrome (WS). A work up for possible underlying malignancy found that the patient had underlying clear cell renal carcinoma. The eruption largely resolved following right laparoscopic nephrectomy with negative surgical margins, thus confirming the diagnosis of paraneoplastic WS. However, 2 years later the patient developed metastasis to his liver, lungs, and ribs. The patient's cancer has continued to progress despite treatment with high-dose interleukin-2, oral sunitinib, afinitor. avastin, azacytidine, and currently axitinib. Our case is the first to describe eosinophilic cellulitis arising in a patient with underlying renal cell carcinoma.
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PMID:Recurrent paraneoplastic wells syndrome in a patient with metastatic renal cell cancer. 2494 51