Angioimmunoblastic T-cell lymphoma presenting with dermatomyositis-like skin lesions: A case report and literature review

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Dear Editor, Angioimmunoblastic T-cell lymphoma (AITL) is a rare peripheral T-cell non-Hodgkin lymphoma with frequent B symptoms and generalized lymphadenopathy (LAP) and is associated with a poor prognosis. AITL has other features, including hepatosplenomegaly, hemolytic anemia, elevated LDH, and eosinophilia. Skin eruptions, including maculopapular, urticarial rash, and a rare case of erythema multiforme-like rash,[1] were observed in about 50% of the cases. In this report, we described the clinicopathologic findings of a case of AITL with dermatomyositis (DM)-like eruption. An elderly man presented with a 1-month history of asymptomatic rashes on the scalp and limbs. He denied fever, muscle weakness, or weight loss. Examination revealed widespread erythematous maculopapules with photo-accentuation on the extensor side of finger joints, shoulders and back, and heliotrope sign of the eyelids [Figure 1a-d]. No periungual telangiectasias were noted under dermoscopy [Supplementary Figure 1]. A generalized LAP with a large (1 cm × 3 cm), firm, asymptomatic mass was palpated in his left anterior neck. Dermatomyositis or lymphoma was considered clinically. Laboratory survey revealed elevated lactated dehydrogenase (LDH) 416 U/L (normal: 135–225 U/L), creatine kinase (CK) 42 U/L (normal: 39–308), anti-nuclear antibody (ANA) speckled pattern 1:80 (+), and anti-Mi-2 alpha autoantibody 1+. A skin biopsy from a knuckle lesion showed a moderately dense perivascular lymphocytic infiltrate with scattered atypical lymphocytes in the dermis without mucin deposition, angioinvasion, and interface change [Figure 1e-g]. The dermal infiltrate was positive for CD3, PD-1, BCL-6 and CXCL-13, and CD10 (focal positive) by immunohistochemistry study (IHC), and Epstein–-Barr virus (EBV)-encoded RNA (EBER) in situ hybridization was positive in scattered cells [Supplementary Figure 2]. A lymph node biopsy at a different hospital confirmed the diagnosis of AITL. Despite receiving chemotherapy, he died 8 months later.Figure 1: The cutaneous manifestations and histopathologic findings of skin biopsy from a knuckle. (a) erythematous maculopapules on knuckles, (b) erythematous and mild edematous change on upper and lower eyelids mimicking heliotrope rash, (c and d) widespread erythematous maculopapules on back and lower legs, (e) a dense superficial and dense inflammatory infiltrate in dermis (H and E, ×40), (f) close-up view showing a dense lymphocytic infiltration without interface change or epidermotropism (H and E, ×100), (g) scattered lymphocytes showing medium-sized hyperchromatic atypical nuclei with mildly irregular contours (arrows) in the reticular dermis (H and E, ×400).Supplementary Figure 1: No telangiectasias was noted under dermoscopy.Supplementary Figure 2: Immunohistochemistry study revealed positive for CD3, PD-1, BCL-6 and CXCL-13, and some CD10-positive lymphocytes in the infiltrate. Epstein–Barr virus-encoded RNA (EBER) in situ hybridization was focally positive (×200).DM-like rash has been reported in cases of peripheral T-cell lymphoma, subcutaneous panniculitis-like T-cell lymphoma, lupus erythematosus, chronic actinic dermatosis, paclitaxel-induced drug eruption, phototoxic/photoallergic dermatoses, multicentric reticulohistiocytosis, leishmaniasis,[2] and adult-onset still disease.[3] DM-like eruption has been reported in five cases of AITL [Supplementary Table 1].[2,4-7]Supplementary Table 1: Summary of case reports with dermatomyositis-like angioimmunoblastic T-cell lymphomaHistologically, cutaneous AITL is characterized by infiltration of small-to medium-sized lymphocytes, EBV-positive B-cell immunoblasts, follicular dendritic cells, and follicular helper T (Tfh) cells.[8] Markers for Tfh cells include PD-1, BCL-6, CD10, and CXCL-13 (more specific); at least two markers are required to make a diagnosis of AITL. Ancillary studies include EBER in situ hybridization and T-cell receptor gene rearrangement analysis. Of the five previously reported cases of AILT with DM-like eruption, three showed positive Tfh markers and EBER staining in the dermal infiltrates.[5-7] In our case, the IHC profile of the dermal infiltrate was consistent with skin involvement of AITL. Although our patient had two major criteria according to Sontheimer’s criteria for cutaneous DM [Supplementary Table 2], the skin biopsy did not show typical features of DM, including interface change and dermal mucin deposits. Consequently, the diagnosis of cutaneous DM was not established. In the reported cases of AITL with DM-like eruption, elevated CK and aldolase were noted in only one case,[5] and none had data on the myositis autoantibody panel. Fever and generalized LAP were observed in four cases, which may facilitate differentiating from DM.Supplementary Table 2: Sontheimer criteria for Cutaneous Dermatomyositis (Curr Opin Rheumatol. 1999 Nov;11(6):475-82)A study of ANAs in patients with malignancies revealed a higher rate of positive ANAs in malignancies, including 31.8% in patients with lymphomas (ANA titers 1:80–1:320), compared to 6.4% in healthy individuals. Only about a quarter of ANA-positive cancer patients had rheumatologic diseases. The finding of a low ANA titer without other evidence of a breakdown in self-tolerance in our patient suggests a low probability of a rheumatologic presentation. Similarly, a low titer of anti-MI-2, a myositis-specific antibody, is typically considered insignificant. While these findings might pose diagnostic challenges, we consider them unrelated to the patient’s primary diagnosis of AITL. The mechanism of DM-like eruption in AITL remains to be fully elucidated, but might involve cytokines overproduction, particularly interleukin-6 (IL-6). Within the tumor microenvironment of AITL, IL-6 is actively produced by Tfh cells, dendritic cells, and macrophages, promoting plasma cell proliferation. Elevated levels were consistently detected in AITL, correlating directly with disease activity. In DM, the level of IL-6 in skin lesions is positively correlated with the severity of pruritus and overall disease activity. This shared dysregulation of the proinflammatory IL-6 pathway in both AITL and DM offers some insight into the potential immunopathogenesis of the DM-like rash in AITL. In summary, we reported the 6th case of AITL with skin involvement manifesting DM-like eruption clinically, and it is important to include AITL in the differential diagnosis of DM-like eruptions. A complete physical examination with skin biopsy and lymph node biopsy is crucial to establish a correct diagnosis. Ethical approval The study was conducted in accordance with the Declaration of Helsinki and was approved by the Institutional Review Board of National Cheng Kung University Hospital (IRB number: B-EC-114-016) on 2025/06/02. The patient consent was waived by the IRB. Data availability statement The datasets generated during and/or analyzed during the current study are publicly available. Financial support and sponsorship Nil. Conflicts of interest Prof. Julia Yu-Yun Lee , an editorial board member at Dermatologica Sinica, had no role in the peer review process of or decision to publish this article. All authors declared no conflicts of interest in writing this paper.

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