Extranodal NK/T-Cell Lymphoma Nasal Type Mimicking Pyoderma Gangrenosum

Delgado‐Jiménez, Yolanda; Pérez‐Gala, S.; Nam-Cha, Syonghyun; Jones‐Caballero, María; Fraga, Javier; García‐Diez, Amaro; Fernández‐Herrera, Jesús

doi:10.2340/00015555-019

Cited by 6 publications

(7 citation statements)

References 10 publications

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“…At the time of diagnosis, they are usually localized. The skin lesions may present as a generalized erythematous maculopapular rash or as multiple subcutaneous nodules that may be ulcerated [10]. Our patient had generalized cutaneous plaques, painful nodules, and ulcers with hemorrhagic secretions.…”

Section: Discussionmentioning

confidence: 75%

“…In the majority of cases, clinical manifestations present as single or multiple nodules or tumours that persist and progress over time. PC-ENKTL with clinical onset as ulcer-necrotic lesions, with bulla formation or purulent secretion was considered as similar to ulcerative lesions of pyoderma gangrenosum, in particular can be hard to differentiate from pyoderma gangrenosum [8][9][10]. In contrast to other types of ENKTL, PC-ENKTL is characterized by a highly aggressive clinical course with a high occurrence rate and advanced stage [11].…”

Section: Introductionmentioning

confidence: 99%

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Primary Cutaneous Extranodal NK/T-Cell Lymphoma, Nasal Type, with Extensive Skin Lesions: A Case Report with Immune Cell Shift

Xie¹,

Shi²,

Kong³

2020

Preprint

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Background: Primary cutaneous extranodal natural killer (NK)/T-cell lymphoma, nasal type (PC-ENKTL), is a rare entity of malignancies of NK cells or cytotoxic T cells, characterized by an association with Epstein-Barr virus (EBV) infection. Despite its aggressive behavior, PC-ENKTL is mostly found as a localized disease. Data regarding PC-ENKTL with generalized skin lesions have only rarely been characterized in clinical studies so far. Case presentation: We present a case of PC-ENKTL, nasal type, in a 38-year–old female with a history of disseminated cutaneous plaques, ulcers and painful nodules that originated in her right leg skin as a small raised papule and expanded quickly over the entire body, ranging in diameter from 1 cm to 6 cm. The patient did not show any involvement of other sites except skin. Histologic, immunophenotypic, genetic and clinical features consistent with the diagnosis of primary cutaneous NK/T-cell lymphoma, nasal type. The status of immune cells was analyzed using a panel of monoclonal antibodies and revealed negative stain for CD163, CD68, programmed cell death (PD-1)/programmed cell death l ligand 1(PD-L1), and FoxP3. Chemotherapy followed by radiotherapy was planned but the patient was experienced disease progression and died at 3 months as a result of lymphoma.Conclusions: In contrast to NK/T-cell lymphoma with localized disease, PC-ENKTL with generalized skin lesions tend to be more aggressive, with short survival and extremely poor response to therapy. We propose that the immune cell shift may be related to the severity of the patient.

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Section: Discussionmentioning

confidence: 75%

Section: Introductionmentioning

confidence: 99%

Primary Cutaneous Extranodal NK/T-Cell Lymphoma, Nasal Type, with Extensive Skin Lesions: A Case Report with Immune Cell Shift

Xie¹,

Shi²,

Kong³

2020

Preprint

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“…Firstly, to the best of our knowledge and after a thorough review of the literature, this is the first reported case of CD30+ PCLCL presenting as PG like lesion in a patient with ulcerative colitis. Yet, PG-like lesions have been previously reported to represent NK/T cell lymphoma, 23 B cell lymphoma 25 and mycosis fungoides. 24 Secondarily, the coexistence of CD30+ PCLCL and MF is remarkable too.…”

Section: Discussionmentioning

confidence: 99%

“…Biopsies may demonstrate edema, mixed inflammatory infiltrate, predominantly neutrophilic infiltrate, lymphocytic vasculitis, follicular-based pustule, or necrosis and hemorrhage. In spite of the heterogeneous histological picture of PG, histopathological examination of the ulcer can help to differentiate PG from some of its mimickers, including Wegener granulomatosis, polyarteritis nodosa, sporotrichosis, antiphospholipid syndrome 22 and variable types of lymphoma including extranodal NK/T-Cell lymphoma nasal type, 23 mycosis fungoides 24 and primary cutaneous B cell lymphoma. 25 In our patient the diagnosis of PG was very tempting due to the history of UC, but the histological results revealed the right diagnosis.…”

Section: Histology From the Border Of The Pg Like Lesion On The Rightmentioning

confidence: 99%

Mycosis fungoides and CD30+ cutaneous T-cell lymphoma simulating pyoderma gangrenosum in a patient with ulcerative colitis

Salameh

Barzilai²,

Baum³

et al. 2009

J Dermatol Case Rep

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Our case presents the rare coexistence of primary mycosis fungoides and primary CD30+ cutaneous large cell lymphoma, rather than the CD30+ cutaneous large cell lymphoma developing from mycosis fungoides. This case also presents the development of a pyoderma gangrenosum-like lesion of CD30+ cutaneous large cell lymphoma in a patient with ulcerative colitis. An observation that emphasizes the need for a high index of suspicion in cases diagnosed as pyoderma gangrenosum based solely on clinical appearance.

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“…Skin is another commonly involved site, presenting with various clinical manifestations, such as subcutaneous nodules, papules, erythema and ulceration 3 . Bulla formation is an extremely rare presentation and may be related to extensive fibrinoid degeneration of vessel walls caused by lymphoma cell infiltration 4 . Systemic symptoms such as fever and night sweating are also common, and around 3% of patients develop haemophagocytic lymphohistiocytosis 5 …”

Section: Figurementioning

confidence: 99%

Extranodal NK/T cell lymphoma, nasal type, mimicking a bullous pyoderma gangrenosum

Liu

2021

Aust J Dermatology

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Extranodal NK/T-Cell Lymphoma Nasal Type Mimicking Pyoderma Gangrenosum

Cited by 6 publications

References 10 publications

Primary Cutaneous Extranodal NK/T-Cell Lymphoma, Nasal Type, with Extensive Skin Lesions: A Case Report with Immune Cell Shift

Primary Cutaneous Extranodal NK/T-Cell Lymphoma, Nasal Type, with Extensive Skin Lesions: A Case Report with Immune Cell Shift

Mycosis fungoides and CD30+ cutaneous T-cell lymphoma simulating pyoderma gangrenosum in a patient with ulcerative colitis

Extranodal NK/T cell lymphoma, nasal type, mimicking a bullous pyoderma gangrenosum

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