Linear IgA dermatosis in association with angioimmunoblastic T‐cell lymphoma infiltrating the skin: A case report with literature review

Colmant, Caroline; Camboni, Alessandra; Dekeuleneer, Valérie; Dachelet, Claire; Baeck, Marie

doi:10.1111/cup.13576

Cited by 7 publications

(9 citation statements)

References 18 publications

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“…Histopathology revealed a dense dermal and hypodermal infiltrate of atypical lymphocytes with perivascular and interstitial involvement, as well as sub‐epidermal blisters with a heavy perivascular infiltrate of atypical T‐lymphocytes and neutrophils. As in our patient, DIF showed a strong IgA linear deposition pattern along the dermal‐epidermal junction, consistent with a paraneoplastic LAD process along with evidence of concurrent AITL 3 . Additional case reports of LAD in the setting of AITL are summarized in Table 1 4,15 .…”

Section: Discussionsupporting

confidence: 76%

“…Although there is a paucity of reported blistering diseases associated with AITL, the clinical and histopathologic features seen in our patient best represent LAD. A recent case report described patients with AITL presenting with painless vesicular eruptions on an erythematous base 3 . Histopathology revealed a dense dermal and hypodermal infiltrate of atypical lymphocytes with perivascular and interstitial involvement, as well as sub‐epidermal blisters with a heavy perivascular infiltrate of atypical T‐lymphocytes and neutrophils.…”

Section: Discussionmentioning

confidence: 99%

“…A recent case report described patients with AITL presenting with painless vesicular eruptions on an erythematous base. 3 Histopathology revealed a dense dermal and hypodermal infiltrate of atypical lymphocytes with perivascular and interstitial involvement, as well as sub-epidermal blisters with a heavy perivascular infiltrate of atypical T-lymphocytes and neutrophils. As in our patient, DIF showed a strong IgA linear deposition pattern along the dermal-epidermal junction, consistent with a paraneoplastic LAD process along with evidence of concurrent AITL.…”

Section: Conflict Of Interestmentioning

confidence: 96%

“…As in our patient, DIF showed a strong IgA linear deposition pattern along the dermal-epidermal junction, consistent with a paraneoplastic LAD process along with evidence of concurrent AITL. 3 Additional case reports of LAD in the setting of AITL are summarized in Table 1. 4,15 Although uncommonly seen, this case highlights the importance of considering LAD in the differential diagnosis for patients with a bullous eruption in the setting of AITL.…”

Section: Conflict Of Interestmentioning

confidence: 99%

“…Angioimmunoblastic T‐cell lymphoma (AITL) is a form of follicular helper T‐cell (FHT) lymphoma with variable cutaneous manifestations ranging from frank neoplastic T‐cell infiltration of the skin to one of an inflammatory dermatosis reflective of autoimmune or paraneoplastic phenomena 1 . Autoimmune vesiculobullous disorders are uncommon in this disease and mainly occur as paraneoplastic pemphigus 2‐4 . We present a case of linear IgA bullous dermatosis (LAD) in the setting of AITL.…”

Section: Introductionmentioning

confidence: 99%

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Linear IgA bullous dermatosis in the setting of angioimmunoblastic T‐cell lymphoma

Andriano

Tannenbaum

et al. 2022

J Cutan Pathol

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We report an 80‐year‐old male developing linear IgA bullous dermatosis (LAD) in the setting of angioimmunoblastic T‐cell lymphoma (AITL). This phenomenon is rare, as only three cases have been described in the literature. The pathophysiologic process can be attributed to dysregulation in somatic hypermutation and the expression of chemokine receptor 5 in AITL, contributing to increased IgA. Immunoglobulin production resulting from clonal plasma cell expansion may be because of the B‐cell promotional effect by neoplastic follicular helper T‐cells. Beyond providing a pathophysiologic platform for AITL‐associated LAD, we also briefly summarized prior cases. This report demonstrates the importance of considering LAD in the differential diagnosis for patients with a bullous eruption in the setting of AITL.

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Section: Discussionsupporting

confidence: 76%

Section: Discussionmentioning

confidence: 99%

Section: Conflict Of Interestmentioning

confidence: 96%

Section: Conflict Of Interestmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Linear IgA bullous dermatosis in the setting of angioimmunoblastic T‐cell lymphoma

Andriano

Tannenbaum

et al. 2022

J Cutan Pathol

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S2k guidelines on diagnosis and treatment of linear IgA dermatosis initiated by the European Academy of Dermatology and Venereology

Caux,

Patsatsi,

Karakioulaki

et al. 2024

Acad Dermatol Venereol

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IntroductionLinear IgA dermatosis (LAD) is a rare subepidermal autoimmune bullous disease (AIBD) defined by predominant or exclusive immune deposits of immunoglobulin A at the basement membrane zone of skin or mucous membranes. This disorder is a rare, clinically and immunologically heterogeneous disease occurring both in children and in adults. The aim of this project is to present the main clinical features of LAD, to propose a diagnostic algorithm and provide management guidelines based primarily on experts' opinion because of the lack of large methodologically sound clinical studies.MethodsThese guidelines were initiated by the European Academy of Dermatology and Venereology (EADV) Task Force Autoimmune Bullous Diseases (AIBD). To achieve a broad consensus for these S2k consensus‐based guidelines, a total of 29 experts from different countries, both European and non‐European, including dermatologists, paediatric dermatologists and paediatricians were invited. All members of the guidelines committee agreed to develop consensus‐based (S2k) guidelines. Prior to a first virtual consensus meeting, each of the invited authors elaborated a section of the present guidelines focusing on a selected topic, based on the relevant literature. All drafts were circulated among members of the writing group, and recommendations were discussed and voted during two hybrid consensus meetings.ResultsThe guidelines summarizes evidence‐based and expert opinion‐based recommendations (S2 level) on the diagnosis and treatment of LAD.ConclusionThese guidelines will support dermatologists to improve their knowledge on the diagnosis and management of LAD.

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Idiopathic linear IgA bullous dermatosis treated with prednisone

et al. 2021

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We present a case of a 43-year-old man with a medical history of paroxysmal atrial fibrillation that presented with acute onset generalised vesiculobullous rash of 1-week duration. The rash was initially noticed on his groin and then spread to his hands, feet and mucosal surfaces. Laboratory tests were unremarkable, including an extensive infection aetiology work-up. Punch biopsies were obtained of a fresh lesion and were stained with H&E and sent for direct immunofluorescence. Light microscopy and immunofluorescence study demonstrated a subepidermal blister with predominant neutrophilic infiltrates and a linear band of IgA at the dermoepidermal junction, respectively. The patient was diagnosed with linear IgA bullous dermatosis and was subsequently treated with 0.5 mg/kg of prednisone daily following previous case reports. At 1-week follow-up as an outpatient, the bullae became crusted, and the rash was nearly completely regressed.

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Linear IgA dermatosis in association with angioimmunoblastic T‐cell lymphoma infiltrating the skin: A case report with literature review

Cited by 7 publications

References 18 publications

Linear IgA bullous dermatosis in the setting of angioimmunoblastic T‐cell lymphoma

Linear IgA bullous dermatosis in the setting of angioimmunoblastic T‐cell lymphoma

S2k guidelines on diagnosis and treatment of linear IgA dermatosis initiated by the European Academy of Dermatology and Venereology

Idiopathic linear IgA bullous dermatosis treated with prednisone

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