{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,22]],"date-time":"2026-07-22T23:11:17Z","timestamp":1784761877005,"version":"3.55.0"},"reference-count":15,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2007,2,6]],"date-time":"2007-02-06T00:00:00Z","timestamp":1170720000000},"content-version":"vor","delay-in-days":220,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Skinmed"],"published-print":{"date-parts":[[2006,7]]},"abstract":"<jats:p>A 17\u2010year\u2010old girl previously in good health presented with a 2\u2010month history of recurrent, high\u2010grade fever; general fatigue; anorexia; a 10\u2010kg weight loss; and multiple, painful, reddish skin lesions on the lower abdomen. Some lesions were ulcerated, with an oily yellowish brown discharge. A systemic review was unremarkable other than bleeding from the nose. Her medical and family histories were unremarkable. On examination, the patient was pale, jaundiced, and febrile (temperature of 39\u00b0C). She had enlarged lymph nodes in the axillary and inguinal areas. There was moderate hepatosplenomegaly. Local skin examination revealed multiple erythematous, tender, and firm subcutaneous nodules of variable size (1\u20132 cm) on the lower abdomen. Some nodules were ulcerated, with oily yellowish brown discharge and overlying ecchymosis (<jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"#f1\">Figures\u20031<\/jats:ext-link> and <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"#f2\">2<\/jats:ext-link>). Mucous membranes were free of lesions. Laboratory investigations showed pancytopenia, an elevated erythrocyte sedimentation rate (&gt;80 mm\/h), normal renal function tests, abnormal hepatic function tests (alanine aminotransferase 172 U\/L, aspartate aminotransferase 229 U\/L, alkaline phosphatase 72S U\/L, and total bilirubin 100 \u03bcmol\/L [normal range 0\u201318 \u03bcmol\/L]), conjugated bilirubin 45 \u03bcmol\/L (normal range 0\u20135 \u03bcmol\/L), and high triglycerides 855 mg\/dL (normal range 20\u2013200 mg\/dL). Prolonged prothrombin time, 26 seconds (normal range 13\u201316 seconds); prolonged activated partial thromboplastin time, 61 seconds (normal range 26\u201338 seconds); positive disseminated intravascular coagulation studies evidenced by low fibrinogen, 74 mg\/dL (normal range 160\u2013350 mg\/dL); and positive fibrinogen degradation products were also noted. Throat, midstream urine, and blood culture results were negative. Serologic tests for syphilis, HIV, and hepatitis B and C viruses were negative. Epstein\u2010Barr virus and cytomegalovirus serologic values revealed evidence of past infection. Tuberculin and Coombs tests were negative. The \u03b1<jats:sub>1<\/jats:sub>\u2010antitrypsin level was normal. Antinuclear and anti\u2010smith antibodies, rheumatoid factor, and cryoglobulins were negative. CT showed enlarged lymph nodes in the axillary and inguinal areas, bilateral small pleural effusion, moderate hepatosplenomegaly, severe fatty infiltration of the liver, and thickening of lower abdominal subcutaneous tissue. A liver biopsy showed steatohepatitis. Bone marrow aspirate and trephine were normal. A deep punch biopsy of a nodule from the right lower abdomen revealed lobular panniculitis with atypical lymphocytes and large macrophages with cytophagocytosis (\u201cbeanbag\u201d cells) (<jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"#f3\">Figures\u20133<\/jats:ext-link> and <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"#f4\">4<\/jats:ext-link>). Immunohistochemistry showed that these atypical cells were positive for CD3, CD8, granzyme B, and perform, and negative for CD56. T\u2010cell gene rearrangement studies on skin lesions revealed a monoclonal T\u2010cell receptor (\u03b3\u2010chain) gene rearrangement, supporting the diagnosis of subcutaneous panniculitis\u2010like T\u2010cell lymphoma. On presentation, the initial treatment included 6 U of fresh frozen plasma, 2 U of packed red blood cells, and 2 g IV fibrinogen for 3 consecutive days. The patient was started on prednisolone 60 mg orally once daily and cyclosporine A 5 mg\/kg\/d orally in two divided doses. The fever and other systemic symptoms and skin lesions resolved within 2 weeks after the treatment. The prednisolone dose was tapered gradually, and a maintenance dose of cyclosporine A was continued. The patient's condition remained in remission at 12\u2010month follow\u2010up; there was no evidence of clinical relapse.<\/jats:p><jats:p><jats:fig><jats:caption><jats:p> <jats:italic>Multiple, erythematous, firm, ulcerated subcutaneous nodules on the lower abdomen<\/jats:italic> <\/jats:p><\/jats:caption><jats:graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" position=\"anchor\" xlink:href=\"urn:x-wiley:15409740:media:SKM4689:SKM_4689_f1\"><jats:alt-text>image<\/jats:alt-text><\/jats:graphic><\/jats:fig><\/jats:p><jats:p><jats:fig><jats:caption><jats:p> <jats:italic>Close\u2010up view of the skin nodules on the right side of the abdomen<\/jats:italic> <\/jats:p><\/jats:caption><jats:graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" position=\"anchor\" xlink:href=\"urn:x-wiley:15409740:media:SKM4689:SKM_4689_f2\"><jats:alt-text>image<\/jats:alt-text><\/jats:graphic><\/jats:fig><\/jats:p><jats:p><jats:fig><jats:caption><jats:p> <jats:italic>Deep punch biopsy of a nodule from the right lower abdomen revealing lobular panniculitis with lymphocytic infiltration<\/jats:italic> <\/jats:p><\/jats:caption><jats:graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" position=\"anchor\" xlink:href=\"urn:x-wiley:15409740:media:SKM4689:SKM_4689_f3\"><jats:alt-text>image<\/jats:alt-text><\/jats:graphic><\/jats:fig><\/jats:p><jats:p><jats:fig><jats:caption><jats:p> <jats:italic>Lobular panniculitis with characteristic \u201cbeanbag\u201d cells and atypical lymphocytes<\/jats:italic> <\/jats:p><\/jats:caption><jats:graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" position=\"anchor\" xlink:href=\"urn:x-wiley:15409740:media:SKM4689:SKM_4689_f4\"><jats:alt-text>image<\/jats:alt-text><\/jats:graphic><\/jats:fig><\/jats:p>","DOI":"10.1111\/j.1540-9740.2006.04689.x","type":"journal-article","created":{"date-parts":[[2007,2,7]],"date-time":"2007-02-07T00:53:03Z","timestamp":1170809583000},"page":"195-197","source":"Crossref","is-referenced-by-count":17,"title":["Subcutaneous Panniculitis\u2010Like T\u2010Cell Lymphoma With Hemophagocytic Syndrome Successfully Treated With Cyclosporin A"],"prefix":"10.1111","volume":"5","author":[{"given":"Abdullateef A.","family":"Al Zolibani","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Saleh","family":"Al Fawzan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ahmad A.","family":"Al Robaee","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Muhammad 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