{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,10,11]],"date-time":"2023-10-11T05:24:27Z","timestamp":1697001867659},"reference-count":26,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2004,3,2]],"date-time":"2004-03-02T00:00:00Z","timestamp":1078185600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Int J Dermatology"],"published-print":{"date-parts":[[2005,7]]},"abstract":"<jats:p>A 48\u2010year\u2010old woman presented with extensive facial ulceration of 1\u00a0year in duration. Based on the combination of the clinical appearance and a \u201cnondiagnostic\u201d biopsy taken elsewhere, the patient was started on oral prednisone at a dose of 40\u00a0mg\/day, with a working diagnosis of pyoderma gangrenosum. According to the patient, the ulceration worsened over the 2\u00a0months whilst on prednisone and pain control was a major issue, controlled for the most part with oral oxycodone. One month prior to our evaluation of the patient, she was started on dapsone at 50\u00a0mg\/day with no added benefit. Approximately 2\u00a0years prior to our evaluation, she developed raised, reddish, skin lesions on the abdomen and legs which recurred, but healed spontaneously each time after a few weeks.<\/jats:p><jats:p>Her past medical history was remarkable for the remote use of intravenous drugs (which she had stopped for the past 20\u00a0years) and the past and continued use of alcohol on a daily basis. She had recently been tested elsewhere and was found to be positive for hepatitis C, but not human immunodeficiency virus (HIV). Examination revealed several ulcerations of the face and forehead, with an \u201capple jelly\u201d coloration to the periphery and necrotic center. There was complete erosion of the nasal sidewall with apparent involvement of the septum (<jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"#f1\">Fig.\u00a01<\/jats:ext-link>). There were also scattered, smaller, nonulcerated, reddish to purplish lesions on the abdomen. Otolaryngologic examination revealed ulceration of the right ala and erosion of the nasal septum, but was otherwise unremarkable. No cervical or submental lymphadenopathy was noted.<\/jats:p><jats:p><jats:fig><jats:caption><jats:p>Facial ulceration at the time of initial evaluation<\/jats:p><\/jats:caption><jats:graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" position=\"anchor\" xlink:href=\"urn:x-wiley:00119059:media:IJD2035:IJD_2035_f1\"><jats:alt-text>image<\/jats:alt-text><\/jats:graphic><\/jats:fig><\/jats:p><jats:p>Routine laboratory tests showed the patient to be anemic, with a hemoglobin level of 11\u00a0g\/dL (normal, 12\u201315.5\u00a0g\/dL) and a white blood count of 13,300 (normal, 3500\u201310,500) The total bilirubin was normal at 1.0\u00a0mg\/dL, but several liver function tests were elevated by 2\u20134 times the upper limit of normal, including aspartate aminotransferase (AST), alanine aminotransferase (ALT), and \u03b3\u2010glutamyl transpeptidase (G\u2010GT). Hepatitis C tests were abnormal, including antibody to hepatitis B core antigen (anti\u2010HBc) and anti\u2010hepatitis C virus (anti\u2010HCV) antibodies, and hepatitis C\u2010RNA was positive. Anti\u2010hepatitis B surface antigen (anti\u2010HBsAg) and antibody were negative. Human immunodeficiency virus (HIV) was negative. Antinuclear antibodies, antineutrophil cytoplasmic antibodies (ANCA), serologic tests for syphilis, cryoglobulins and cryofibrinogens, renal function, and urinalysis were all either negative or normal. The chest X\u2010ray showed bilateral pulmonary nodules, confirmed by computed tomography (CT) scan of the chest. Serologic tests for several fungal organisms, including <jats:italic>Sporothrix<\/jats:italic> and <jats:italic>Cryptococcus<\/jats:italic>, were negative. Tuberculin skin testing was negative.<\/jats:p><jats:p>Two 4\u2010mm punch biopsies were taken from the right cheek and from the right lower lateral abdomen near the hip. Biopsy of facial tissue cultures for acid\u2010fast bacilli and fungal elements was negative. Bacterial culture revealed 1+<jats:italic>Staphylococcus aureus<\/jats:italic> and coagulase\u2010negative <jats:italic>Staphylococcus<\/jats:italic>. Polymerase chain reaction (PCR) testing for herpes and varicella viruses was negative.<\/jats:p><jats:p>Histologic examination of the skin biopsies revealed identical findings from both sites. There was parakeratosis, serum crust, and mild irregular epidermal acanthosis. Focal interfacial dermatitis was present and characterized by basal vacuolization and melanophages in the superficial dermis. Epidermotropism of atypical lymphocytes singly and in cell clusters was seen in the epidermis. In the papillary and reticular dermis, extending to the subcutaneous fat, there was a dense, diffuse, perivascular, interstitial, and periappendageal infiltrate composed of atypical lymphocytes, lymphocytes, histiocytes, plasma cells, and neutrophils. The atypical lymphocytes infiltrated the hair follicles with little associated spongiosis (pilotropism) (<jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"#f2\">Fig.\u00a02<\/jats:ext-link>). The atypical lymphocytes also surrounded and infiltrated the eccrine glands and blood vessels. Immunoperoxidase studies were performed on paraffin tissue and showed that the atypical lymphocytes were immunoreactive with CD3 (<jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"#f3\">Fig.\u00a03<\/jats:ext-link>) and \u03b2F1, but not with CD8, CD56, T\u2010cell\u2010restricted intracellular antigen\u20101 (TIA\u20101), or granzyme B. There was a reactive population of B lymphocytes immunoreactive with CD20. <jats:italic>In situ<\/jats:italic> hybridization for Epstein\u2013Barr virus (EBV), performed on paraffin sections, was negative. Lung biopsy of several nodules was accomplished via video\u2010assisted thoracoscopy and revealed a peripheral T\u2010cell lymphoma. Gene rearrangement studies of skin and lung nodules showed similar T\u2010cell clones and were positive for T\u2010cell rearrangement by PCR (TCR PCR, T \u03b3\u2010chain positive).<\/jats:p><jats:p><jats:fig><jats:caption><jats:p>Histopathology of a skin lesion. (a) Dense atypical lymphocytic infiltrate demonstrating pilotropism and epidermal involvement with little associated spongiosis (hematoxylin and eosin stain, \u00d7\u00a0200). (b) Higher power view better demonstrating the dense atypical lymphocytic infiltrate (hematoxylin and eosin stain, \u00d7\u00a0400)<\/jats:p><\/jats:caption><jats:graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" position=\"anchor\" xlink:href=\"urn:x-wiley:00119059:media:IJD2035:IJD_2035_f2\"><jats:alt-text>image<\/jats:alt-text><\/jats:graphic><\/jats:fig><\/jats:p><jats:p><jats:fig><jats:caption><jats:p>Strong CD3 immunoreactivity of the infiltrate (CD3 stain, \u00d7\u00a0200)<\/jats:p><\/jats:caption><jats:graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" position=\"anchor\" xlink:href=\"urn:x-wiley:00119059:media:IJD2035:IJD_2035_f3\"><jats:alt-text>image<\/jats:alt-text><\/jats:graphic><\/jats:fig><\/jats:p><jats:p>The final diagnosis was peripheral T\u2010cell lymphoma with pulmonary and skin involvement. There was no evidence for EBV infection.<\/jats:p><jats:p>The patient returned home to receive treatment with four cycles of hyper\u2010CVAD (cyclophosphamide, vincristine, adriamycin, and dexamethasone), followed by autologous stem cell transplantation. The facial erosions and ulcerations, as well as the systemic nodules, responded well to therapy, with only limited recurrence of the right lower lateral abdominal lesions, which were subsequently treated with radiation (<jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"#f4\">Fig.\u00a04<\/jats:ext-link>). The patient's clinical status 18\u00a0months after treatment is complete remission from lymphoma, but she has developed fulminant liver failure secondary to cirrhosis associated with hepatitis C.<\/jats:p><jats:p><jats:fig><jats:caption><jats:p>Facial lesions after completion of chemotherapy<\/jats:p><\/jats:caption><jats:graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" position=\"anchor\" xlink:href=\"urn:x-wiley:00119059:media:IJD2035:IJD_2035_f4\"><jats:alt-text>image<\/jats:alt-text><\/jats:graphic><\/jats:fig><\/jats:p>","DOI":"10.1111\/j.1365-4632.2004.02035.x","type":"journal-article","created":{"date-parts":[[2005,6,28]],"date-time":"2005-06-28T17:27:13Z","timestamp":1119979633000},"page":"579-583","update-policy":"http:\/\/dx.doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Destructive facial T\u2010cell lymphoma: the difficulty in diagnosis of pyoderma\u2010like processes"],"prefix":"10.1111","volume":"44","author":[{"given":"Lawrence E.","family":"Gibson","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bethany R.","family":"Hairston","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Robert J.","family":"Belt","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rokea","family":"El\u2010Azhary","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2004,4,16]]},"reference":[{"key":"e_1_2_2_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0025-6196(11)63593-3"},{"key":"e_1_2_2_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/0190-9622(91)70028-Z"},{"key":"e_1_2_2_4_2","doi-asserted-by":"publisher","DOI":"10.1001\/archderm.1968.01610180005001"},{"key":"e_1_2_2_5_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2230.1982.tb02470.x"},{"key":"e_1_2_2_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0190-9622(86)80310-3"},{"key":"e_1_2_2_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0190-9622(94)70224-1"},{"key":"e_1_2_2_8_2","first-page":"23","article-title":"Cutaneous Wegener's granulomatosis presenting as pyoderma gangrenosum\u2010like ulceration","volume":"5","author":"Daoud MS","year":"1995","journal-title":"Eur J Dermatol"},{"key":"e_1_2_2_9_2","doi-asserted-by":"publisher","DOI":"10.1001\/archderm.1994.01690070055008"},{"key":"e_1_2_2_10_2","doi-asserted-by":"publisher","DOI":"10.1002\/art.1780330829"},{"key":"e_1_2_2_11_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2133.1995.tb05010.x"},{"key":"e_1_2_2_12_2","first-page":"125","article-title":"Clinical spectrum associated with ANCA of defined antigen specificities in 98 selected patients","volume":"39","author":"Geffriaud\u2010Ricouard C","year":"1993","journal-title":"Clin Nephrol"},{"key":"e_1_2_2_13_2","doi-asserted-by":"publisher","DOI":"10.1002\/1529-0131(199809)41:9<1521::AID-ART2>3.0.CO;2-A"},{"key":"e_1_2_2_14_2","doi-asserted-by":"publisher","DOI":"10.1159\/000051679"},{"key":"e_1_2_2_15_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0889-857X(05)70237-4"},{"key":"e_1_2_2_16_2","doi-asserted-by":"publisher","DOI":"10.1006\/clim.2001.5200"},{"key":"e_1_2_2_17_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-4362.2003.01914.x"},{"key":"e_1_2_2_18_2","doi-asserted-by":"publisher","DOI":"10.1023\/A:1008418727472"},{"key":"e_1_2_2_19_2","first-page":"76","article-title":"Prognostic significance of T\u2010cell phenotype in aggressive non\u2010Hodgkin's lymphomas. 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