Erythema multiforme-like eruption in a case of angioimmunoblastic T-cell lymphoma: A case report and review of literature
Year: 2024
Type: article
Abstract: Dear Editor, Angioimmunoblastic T-cell lymphoma (AITL), a variant of peripheral T-cell lymphoma (PTCL), usually involves the skin in approximately 50% of patients.[1] Its diverse cutaneous manifestations may pose a challenge in diagnosis, particularly when it precedes the diagnosis of AITL.[1] Herein, we report a case with an initial erythema multiforme (EM)-like eruption, which was subsequently confirmed as AITL via lymph node biopsy. A 67-year-old man with hypertension presented to our emergency room with a 2-week history of progressive epigastric pain, accompanied by intermittent fever up to 38.7ºC, general malaise, dark urine color, and jaundice. Skin eruption with widespread asymptomatic targetoid erythematous-to-violaceous papules and plaques were observed on the face, trunk, and four extremities [Figure 1a-c] for 4 days. Palms and soles were both involved, but no mucosal lesions were found. Blood tests revealed pancytopenia (white blood cell count 3.88 × 103/μL, hemoglobin 11.0 g/dL, and platelet count 40 × 103/μL), elevated liver function (GOT/GPT 269/175 IU/L), elevated total/direct bilirubin level (10.28/8.57 mg/dL), and increased lactate dehydrogenase level (1972.2 IU/L). A clinical impression of EM was made. Herpes simplex virus (HSV) ½ Immunoglobulin (Ig) M, IgG, and Mycoplasma pneumoniae IgM were negative. Skin biopsy revealed interface dermatitis with basal vacuolar degeneration and scattered apoptotic keratinocytes at the dermoepidermal junction [Figure 1d and e]. Atypical lymphocytes were found in the epidermis [Figure 1f] and around the blood vessels in the upper dermis [Figure 1g], which were positive for CD3 [Figure 1h] and programmed cell death (PD)-1 [Figure 1i] and negative for CD10. Initial treatment with intravenous methylprednisolone (40 mg every 8 h) was started, but the skin lesions did not fade. Abdominal computed tomography was done due to persistent jaundice and revealed hepatosplenomegaly and numerous enlarged lymph nodes in the cardiophrenic, paraaortic, and paracaval areas. Further positron emission tomography revealed increased uptake in the spleen, bone marrow and lymph nodes of bilateral neck, right supraclavicular, mediastinal, bilateral axillary, abdominal and bilateral inguinal regions. Inguinal lymph node biopsy showed nearly complete effacement of the lymph node architecture by proliferation of atypical lymphoid cells with prominent hyperplastic vasculature. Immunohistochemistry stain demonstrated that the atypical lymphocytes were positive for CD3, CD4, PD-1, and CXCL13. Bone marrow biopsy also revealed T-cell lymphoma involvement. A diagnosis of AITL was confirmed 3 weeks after admission. Unfortunately, his AITL rapidly progressed before he started chemotherapy, and the patient expired shortly 9 days after diagnosis.Figure 1: Widespread asymptomatic erythematous-to-violaceous targetoid papules and plaques on the (a) face and (b) trunk. (c) Purpuric plaques with atypical target lesions were visible at the close-up view. (d) The skin biopsy revealed interface dermatitis, dense perivascular lymphocytic infiltrates, and hemorrhage in the upper dermis (H and E, ×40). (e) There is basal vacuolar degeneration and scattered apoptotic keratinocytes (H and E, ×200). (f and g) Atypical lymphocytes were found (f) in the epidermis and (g) around the vessels in the dermis (black arrow) (H and E, ×400). (h and i) On immunohistochemical staining, the lymphoid cells were positive for (h) CD3 and (i) programmed cell death-1 (×200).AITL is an aggressive subtype of PTCL deriving from a monoclonal proliferation of the follicular helper T-cells, involving 1%–2% of non-Hodgkin lymphomas and 15%–20% of PTCL.[1] It mainly affects the elderly, with the median age of onset being 65 years.[1] AITL usually presents with a constellation of symptoms, including generalized lymphadenopathy, hepatosplenomegaly, and B-symptoms, such as fever, night sweats, and weight loss.[1] It is also often accompanied by autoimmune phenomena, including hemolytic anemia and polyclonal hypergammaglobulinemia.[1] Cutaneous manifestations are very frequent in AITL, which may precede the systemic symptoms.[1] Nonspecific morbilliform rash mimicking viral exanthem or drug eruption is most commonly reported, while the presentations of pruritus, erythema, erythroderma, nodules, urticarial eruption, petechiae, purpura, and papulovesicles have been described.[1-6] These various cutaneous presentations are summarized in Supplementary Table 1. Skin involvement of AITL also shows nonspecific histopathological features. The most common finding is a superficial perivascular lymphocytic infiltrate without atypia, which is nonspecific and may be difficult to differentiate from other inflammatory conditions.[7] In our case, the presence of atypical lymphocytes in the skin biopsy suggests that the EM-like eruption may be directly caused by AITL.Supplementary Table 1: Summary of previous literature and the present case on the cutaneous manifestations of angioimmunoblastic T-cell lymphomaEM, a self-limiting inflammatory skin disease, is characterized by its “target lesions.”[8] In most cases, EM is precipitated by HSV or Mycoplasma pneumoniae infection.[8] However, it may also be associated with malignancy, particularly in persistent or refractory EM.[8] EM has been reported to precede the diagnosis of cholangiocarcinoma, renal cell cancer, and metastatic breast cancer.[8] To the best of our knowledge, EM-like eruption as the initial presenting symptom of AITL is extremely rare. Only one other case was reported by Hattori et al.[5] describing a Japanese woman with EM major like rash accompanied by fever, hepatosplenomegaly, and numerous lymphadenopathies, who was subsequently diagnosed as AITL. Chemotherapy was initiated immediately, and the patient showed a favorable response. In conclusion, AITL is usually diagnosed at an advanced stage given its diverse clinical presentations and may pose a challenge in diagnosis. A clinical suspicion for AITL should be considered in cases of EM associated with constitutional abnormalities and lymphadenopathy, especially if atypical lymphocytes are detected in the skin biopsy. Early identification and proper management of AITL is important since delayed diagnosis may carry a poor prognosis. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent form. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed. Data availability statement Data sharing not applicable to this article as no datasets were generated or analyzed during the current study. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.
Source: Dermatologica Sinica
Language: English
Subfield: Dermatology
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $70
OpenAlex ID: W4404792578