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Year : 2022 | Volume
: 40
| Issue : 1 | Page : 56-57
Intravascular large B-cell lymphoma diagnosed by skin biopsy of a cellulitis-like skin lesion: Case report and review of literature
Mayuka Fuse1, Yoshimichi Kobayashi1, Miho Kabuto1, Masaki Iwasa2, Hitoshi Minamiguchi2, Noriki Fujimoto1
1 Department of Dermatology, Shiga University of Medical Science, Setatsukinowa, Otsu, Shiga, Japan
2 Division of Hematology, Shiga University of Medical Science, Setatsukinowa, Otsu, Shiga, Japan
Correspondence Address:
Dr. Noriki Fujimoto
Department of Dermatology, Shiga University of Medical Science, Setatsukinowa, Otsu, Shiga
Japan
Source of Support: None, Conflict of Interest: None
DOI: 10.4103/ds.ds_9_22
Dear Editor,
An 88-year-old Japanese woman visited her local physician for fever and erythema, pain, and swelling on the right lower leg in 2020. She was initially diagnosed with cellulitis and treated with antibiotics; however, no significant improvement was observed. One month later, she was referred to the Department of Hematology in our hospital with thrombocytopenia. She presented with solitary erythema and pain on the anterior surface of the right lower leg with indistinct borders [Figure 1]a. While there was a slight heat sensation and induration on the right leg, there was no inguinal lymphadenopathy. Laboratory examination showed elevated levels of white blood cells (9,800/μl; normal 3,700–8,600), C-reactive protein levels (6.07 mg/dl; 0.00–0.14), lactate dehydrogenase (1,532 U/l; 124–222), ferritin (747.0 ng/ml; 10-80), and soluble interleukin-2 receptor (3,540 U/ml; 121–613) and decreased level of platelets (83,000/μl; 158,000–348,000). No atypical cells were observed in the peripheral blood smear. A computed tomography scan revealed neither enlarged lymph nodes nor hepatosplenomegaly. A bone marrow examination showed a hemophagocytic image with no atypical cells. With a diagnosis of the hemophagocytic syndrome, subcutaneous panniculitis-like T-cell lymphoma was clinically suspected. A skin biopsy from the erythema of the right lower leg revealed intravascular infiltration and proliferation of large atypical mononuclear cells with prominent nucleoli within the vessels in the entire dermis and subcutaneous fat [Figure 1]b, [Figure 1]c, [Figure 1]d. These atypical mononuclear cells were not found outside the vessels. Immunohistochemical staining showed that intravascular cells were positive for CD20, MUM1, and C-myc and negative for CD3 and CD10, and the MIB1 labeling index was more than 90%. Based on these results, we diagnosed her with intravascular large B-cell lymphoma (IVLBCL) showing cellulitis. Because of advanced age and dementia, we considered that chemotherapy was not indicated in this case and decided to treat her only with best supportive care.
Figure 1: (a) Initial clinical presentation. Erythema and pain on the anterior surface of the right lower leg with indistinct borders. (b-d) Histopathological findings of intravascular infiltration and proliferation of large lymphocyte-like atypical cells with prominent nucleoli within the vessels in the entire dermis (hematoxylin and eosin [H and E], original magnification, ×40 [b], ×100 [c], and ×400 [d]).IVLBCL is a subtype of diffuse large B-cell lymphoma, causing lymphoma cell proliferation in the blood vessels. The basic pathology is multiple small infarcts of small blood vessels induced by the lumen-obstructing growth of tumor cells.[1] Although bone marrow biopsy has been considered the most useful test for diagnosis in the past, it has a very low positive rate for diagnosis due to the small total amount of tumor cells. In contrast, a random skin biopsy is useful in cases without skin findings.[2] Sixty percent of the cases in which random skin biopsies led to the diagnosis of intravascular lymphoma had no skin findings.[3] The incidence of cutaneous manifestations of IVLBCL was reportedly about 40%.[4] Skin manifestations of IVLBCL occur on all parts of the body and are particularly common on the lower legs and thighs. A variety of skin rash characteristics are observed, including nodules, papules, plaques, telangiectasia, and induration.[5] It is difficult to diagnose IVLBCL based on the clinical picture of the skin alone. Two months have passed from the onset of cellulitis-like lesion to the diagnosis of IVLBCL in our case.
To our knowledge, only three cases of IVLBCL with cellulitis-like cutaneous lesions, including our case, have been reported in the English literature [Table 1].[6],[7] The first case presented with painful erythema on the bilateral thighs, and the second case presented with painful erythema on the medial side of both thighs. Both patients were treated with chemotherapy resulting in remission. Our case presented with solitary cutaneous lesion with erythema, pain, and swelling on the right lower leg, which was diagnosed as cellulitis and treated with antibiotics. While previous cases presented multiple lesions on bilateral lower extremities, our case presented a solitary lesion. Although the exact mechanism how skin lesions are mediated by IVLBCL is not fully understood, it is said that if superficial vessels are occluded by IVLBCL cutaneous lesions present livedo racemosa; if vessels in the deep dermis or the septa of the subcutaneous fat are involved, cutaneous lesions present erythema nodosum or nodular vasculitis.[4] Multiple cutaneous lesions are usually observed in IVLBCL with skin manifestations including previous two cases like cellulitis.[5],[6],[7] In our case, solitary lesion might develop by chance with the above mechanism; however, we speculate that there is an another possibility that cellulitis might induce some adhesion molecules on the endothelium of blood vessels, which accumulated atypical cells of IVLBCL within blood vessels at the site.
Table 1: Review of the literature on intravascular large B-cell lymphoma with cellulitis-like skin lesionWe initially did not include IVLBCL and unexpectedly diagnosed our case with IVLBCL by skin biopsy. Although cellulitis-like IVLBCL is rare, we dermatologists should perform skin biopsy when we encounter refractory cases even though they present solitary lesion and look like common diseases such as cellulitis as our case.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
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