Case Description
A 32-year-old man with a history of hidradenitis suppurativa Hurley III, receiving antibiotic and surgical treatments. The patient sought medical advice for a new nodule in perianal region which had grown during the past 2 months. He gave a history of regular unprotected receptive anal intercourse.
Physical examination revealed an ulcerated, firm, violaceous nodule adjacent to anus [Figure 1]. He also presented with sinus tracks in the inguinal region and perineum due to hidradenitis and a diffuse maculopapular eruption. No adenopathies were detected.
Figure 1.
Ulcerated, firm, violaceous nodule adjacent to anus
Different microbiologic tests and biopsy of the nodule were performed. Results for HIV and syphilis (treponemal and nontreponemal) were positive. CSF examination was normal. Our patient received penicillin and antiretroviral therapy.
Biopsy revealed a diffuse infiltrate of medium-large size atypical cells in the dermis and subcutis, with sparing epidermis. Cells showed eccentric nucleus with clumped chromatin, prominent nucleolus, abundant and basophilic cytoplasm, and frequent mitoses [Figure 2]. Immunohistochemistry was positive for CD45, CD38, CD138, and Epstein–Barr Virus (EBV). Cells were negative for CD20, CD3, CD5, CD30, bcl-6, and Pax5.
Figure 2.
Diffuse infiltrate of medium-large size atypical lymphoid cells in dermis and subcutis, sparing epidermis (hematoxylin-eosin, original magnification, ×200)
What is your Diagnosis?
Diagnosis
Perianal plasmablastic lymphoma in hidradenitis suppurativa area.
Discussion
Plasmablastic lymphoma is a rare entity associated with immunosuppression, especially with HIV. It is also related with EBV and Human Herpesvirus 8 infection. Tumor cells are positive for CD45, CD38, and CD138, while CD20, CD3, CD5, CD30, bcl-6, and Pax5 are usually negative.[1]
Oral cavity is the most frequent location, followed by lymph nodes, gastrointestinal tract. Tumors affecting only the skin are less common.[2] They usually present over the legs as erythematous-violaceous nodules with a tendency to ulcerate.
Positron emission tomography-computed tomography and bone marrow biopsy showed no signs of progression. He was treated with bortezomib, cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) and radiotherapy, with satisfactory response.
Some studies have proven association between hidradenitis suppurativa and lymphoma.[3,4] Chronic inflammation in perianal area due to hidradenitis might explain this location.
Prognosis of this lymphoma is poor. Accepted therapies include CHOP, dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin, lenalidomide and bortezomib.[5]
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
References
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