Bullous CD4+ CD8+ adult T‐cell leukemia/lymphoma, a rare diagnostically challenging cutaneous variant

Kamath, Preetha; Abrahams, Jennifer; Cho‐Vega, Jeong Hee

doi:10.1111/cup.13337

Cited by 4 publications

(4 citation statements)

References 12 publications

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“…Histopathologically, they show dermal perivascular and interstitial lymphoid infiltrates with absent or sparse reactive background inflammatory cells, commonly with epidermal infiltration in the form of lymphocytic epidermotropism and Pautrier‐like microabscesses 1 . Different TCLs can involve the skin with overlapping clinical, histomorphologic and immunohistochemical features 1,3‐6 . Histopathologic differential diagnosis of ATLL includes MF/SS, as well as other T‐cell lymphomas (TCL), for example peripheral TCL‐not otherwise specified (PTCL‐NOS), T‐cell prolymphocytic leukemia (TCPLL), and angioimmunoblastic T‐cell lymphoma (AITCL) that involve the skin 1,2,7‐11 …”

Section: Badr Abdullgaffar Suad Abdulrahmanmentioning

confidence: 99%

“…Acute variant has leukocytosis, peripheral blood involvement with flower cells, skin rash with massive lymphadenopathy, hepatosplenomegaly, hypercalcemia, elevated LDH and constitutional symptoms with a rapidly progressive course. The skin lesions vary from patches, plaques, papules, nodules, bullous lesions, erythroderma to purpura with overlapping inflammatory, infectious and neoplastic differential diagnoses 1‐5 . Histopathologically, they show dermal perivascular and interstitial lymphoid infiltrates with absent or sparse reactive background inflammatory cells, commonly with epidermal infiltration in the form of lymphocytic epidermotropism and Pautrier‐like microabscesses 1 .…”

Section: Badr Abdullgaffar Suad Abdulrahmanmentioning

confidence: 99%

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Adult T‐cell leukemia/lymphoma clinically confused with viral/drug skin eruptions and pathologically misinterpreted as mycosis fungoides/Sézary syndrome

AbdullGaffar

Abdulrahman

2020

J Cutan Pathol

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Adult T‐cell leukemia/lymphoma (ATLL) is a lymphoproliferative disorder of mature CD4‐positive T‐cell lymphoid cells associated with retrovirus human T‐lymphotropic virus type‐1 (HTLV‐1) with a wide clinical and pathologic spectrum. We report a case of a 53‐year‐old African man who presented with fever and skin eruptions on the trunk composed of non‐itchy erythematous reticulated macules and papules initially suspected for viral exanthem or drug rash. Skin punch biopsy showed a dermal T‐cell lymphoid infiltrate with epidermotropism. The patient developed generalized lymphadenopathy and his peripheral blood showed lymphocytosis with atypical lymphocytes with convoluted nuclei. Our initial diagnosis was mycosis fungoides with Sézary syndrome. However, some clinical and histopathologic features were unusual. The acute onset, lack of previous skin lesions, the histomorphologic features of the dermal, nodal and peripheral blood lymphocytes and the geographic origin of the patient raised the suspicion of other T‐cell lymphomas, particularly ATLL. This was confirmed by a positive anti‐HTLV‐1 serology. Our final diagnosis was acute variant ATLL. Different T‐cell lymphomas can involve the skin with overlapping clinical, histomorphologic and immunohistochemical features. Some clinical and pathologic features should alarm dermatologists and pathologists to the possibility of ATLL particularly in patients from HTLV‐1 endemic geographic areas.

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Section: Badr Abdullgaffar Suad Abdulrahmanmentioning

confidence: 99%

Section: Badr Abdullgaffar Suad Abdulrahmanmentioning

confidence: 99%

Adult T‐cell leukemia/lymphoma clinically confused with viral/drug skin eruptions and pathologically misinterpreted as mycosis fungoides/Sézary syndrome

AbdullGaffar

Abdulrahman

2020

J Cutan Pathol

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“…Immunohistochemically, the atypical cells were positive for CD3, CD4, CD5, CD25, and CCR4, and Vesiculobullous ATLL consists of two types: one with intraepidermal vesicles and another with subepidermal blisters. [2][3][4] In the present case, we performed immunofluorescence analysis of the biopsy specimen to determine the depth of the blister, indicating intralamina lucida blister (Figure 1e-g). Reportedly, the cases with intraepidermal blisters (pompholyx type) showed better prognosis, 2,4 whereas those with subepidermal blisters like the present case had a worse prognosis.…”

mentioning

confidence: 91%

“…Reportedly, the cases with intraepidermal blisters (pompholyx type) showed better prognosis, 2,4 whereas those with subepidermal blisters like the present case had a worse prognosis. 3 In mycosis fungoides, the vesiculobullous subtype has been thought to be associated with poor prognosis. 5 The pathomechanisms of blister formation in the lesions of cutaneous T-cell lymphoma remain unclear.…”

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confidence: 99%