Facial swelling: an atypical presentation of cutaneous lymphoma

Lee, Woojin; Moon, Ik Jun; Won, Chong Hyun; Chang, Sung Eun; Choi, Jee Ho; Moon, Kee Chan; Lee, Mi Woo

doi:10.1111/ijd.13260

Cited by 11 publications

(8 citation statements)

References 21 publications

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“…2 However, occasionally, the earliest symptoms of some patients are characterized by facial or eyelid swelling and long-term fever, but no skin manifestations. 7,16,17 In addition, SPTCL can also involve multiple extracutaneous viscerals such as bone marrow, 4 breast, 5 mesenteric fat, 6 lymph node, 7 lung, 8,9 and muscle. 10 Common systemic manifestations, including B symptoms, hepatosplenomegaly, lymphadenopathy, hepatopathy, and increased LDH.…”

Section: Discussionmentioning

confidence: 99%

Chidamide induces long-term remission in rare subcutaneous panniculitis-like T-cell lymphoma: An unusual case report and literature review

Chai

et al. 2021

Int J Immunopathol Pharmacol

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Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare primary cutaneous lymphoma composed of CD8+ cytotoxic T-cell that is primarily localized in the subcutaneous tissue. No standard treatments are currently available for SPTCL due to its rarity. Chemotherapy, radiotherapy, immunosuppressive agents, and hematopoietic stem cell transplantation (HSCT) have been used frequently, however, the effects of these treatment approaches remain controversial. In this report, we present an unusual case of SPTCL in a 47-year-old woman whose initial symptoms were atypical. The patient was started on etoposide, vincristine, cyclophosphamide, doxorubicin, and prednisone (EPOCH) chemotherapy once diagnosed. After two cycles of chemotherapy, her clinical symptoms were not significantly improved. Subsequently, histone deacetylase (HDAC) inhibitor chidamide was added to the chemotherapy from the third cycle. She recovered gradually and achieved complete remission (CR) after four cycles of chemotherapy combined with chidamide, followed by chidamide monotherapy for maintenance. More than 1 year after the therapy, she remained in CR. Our case illustrates, for the first time, chidamide can be an effective agent to induce long-term remission for rare SPTCL.

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Section: Discussionmentioning

confidence: 99%

Chidamide induces long-term remission in rare subcutaneous panniculitis-like T-cell lymphoma: An unusual case report and literature review

Chai

et al. 2021

Int J Immunopathol Pharmacol

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“…In this series, we also found two patients with an unusual clinical presentation of facial swelling. Lee et al studied cases of cutaneous lymphoma presenting with facial swelling and found that extranodal natural killer (NK)/T‐cell lymphoma was the most common cause in primary cutaneous lymphoma, while SPTL, hydroa vacciniforme‐like lymphoma, and peripheral T‐cell lymphoma were also noted . Therefore, a high index of suspicion of cutaneous lymphoma including SPTL is critical, especially in patients with refractory facial swelling.…”

Section: Discussionmentioning

confidence: 99%

Clinicopathologic, immunophenotypic, and molecular analysis of subcutaneous panniculitis‐like T‐cell lymphoma: A retrospective study in a tertiary care center

2018

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Background Subcutaneous panniculitis‐like T‐cell lymphoma (SPTL) as strictly defined by World Health Organization‐European Organization for Research and Treatment of Cancer classification is a rare cytotoxic α/β T‐cell lymphoma, characterized by primary involvement of subcutaneous tissue mimicking panniculitis. Objectives To describe the clinicopathologic, immunophenotypic, and molecular features of SPTL. Methods A 10‐year retrospective study of 18 patients diagnosed with SPTL was thoroughly reviewed according to clinicopathology, immunophenotype, and T‐cell receptor (TCR) gene rearrangement. Results Of the 18 patients, 16 patients were definitely diagnosed with SPTL. The median age was 26 years (ranged 14‐53 years) with female predominance. Most patients presented with prolonged fever and subcutaneous nodules and/or plaques, usually located on lower extremities. 37.5% of patients had hemophagocytic syndrome. The main histopathology was lobular panniculitis with rimming of atypical lymphocytes highlighted by CD3+, CD8+, Beta‐F1+, granzyme B+, and Ki‐67 (50%‐90%). Monoclonal TCR gene rearrangement was found in 50% of patients and upper extremities involvement indicated a poor prognosis. Conclusion The correlation between clinicopathologic and immunophenotypic study is the most helpful method to give a precise diagnosis of SPTL. Rimming of CD8+ atypical lymphocytes highlighted by high Ki‐67 index is highly specific for the diagnosis of SPTL.

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“…Although the pathogenesis of facial swelling is unclear, Lee et al, (2016 p.1-6) suggested in his report of eight cases of cutaneous lymphomas that the angiocentric and pannicultis-like pattern was associated with dermal edema manifesting as facial swelling. They suggested that a suspicion of cutaneous lymphoma should be entertained in cases of recurrent and refractory facial swelling [9].…”

Section: Case Summarymentioning

confidence: 99%

Subcutaneous Panniculitic T-Cell Lymphoma in an Adolescent: A Case Report

2020

AHOR

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Rationale and Objectives: Subcutaneous panniculitic T-cell lymphoma (SPTCL) is a rare neoplasm accounting for less than 1% of pediatric Non-Hodgkin Lymphomas. It is most common in young adults with predominance of female cases at 0.5. Symptoms include multiple nodules involving the subcutaneous tissues of the extremities and trunk, neck and face. B symptoms such as fever, chills, night sweats and weight loss, have been reported. Rare extra-cutaneous manifestations include edema, involvement of the bone marrow, lymph nodes, liver, spleen, lungs and viscera. Laboratory abnormalities such as cytopenias and elevated lactate dehydrogenase have been recorded. Hemophagocytic syndrome (HPS) occurs in 33% of cases, which were correlated with fatal outcome. Case Report: We present a rare case of a 17 year-old male diagnosed with subcutaneous pannicultic T-cell lymphoma manifesting with prolonged fever, weight loss, and multiple subcutaneous nodules. He also presented with extra-cutaneous manifestations of facial edema, pleural effusion and ascites, lymph node enlargement, hepatosplenomegaly with jaundice and pancytopenia. Conclusion and Summary: Subcutaneous Panniculitic T-Cell Lymphoma may present with an unusual finding of facial edema, ascites and pleural effusion, lymph node enlargement, hepatosplenomegaly with jaundice and pancytopenia alongside skin manifestations of subcutaneous panniculitis. HPS may complicate the course, treatment decisions and outcome of the disease.

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Facial swelling: an atypical presentation of cutaneous lymphoma

Abstract: This collection of cases and literature review emphasizes the need for clinical suspicion of cutaneous lymphoma in the differential diagnosis of patients with refractory facial swelling.

Cited by 11 publications

References 21 publications

Chidamide induces long-term remission in rare subcutaneous panniculitis-like T-cell lymphoma: An unusual case report and literature review

Chidamide induces long-term remission in rare subcutaneous panniculitis-like T-cell lymphoma: An unusual case report and literature review

Clinicopathologic, immunophenotypic, and molecular analysis of subcutaneous panniculitis‐like T‐cell lymphoma: A retrospective study in a tertiary care center

Subcutaneous Panniculitic T-Cell Lymphoma in an Adolescent: A Case Report

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