Mycosis fungoides with spongiosis: a case report

Abstract

Background: Mycosis fungoides (MF) is the most common form of cutaneous T-cell lymphoma (CTCL). CTCL are an uncommon, heterogeneous group of non-Hodgkin lymphomas (NHLs) of T- and B-cell origin where the skin is the primary organ of involvement. It is characterized by malignant CD4⁺ T-cells infiltrating the skin and other organs, leading to progressive skin and systemic involvement. Histopathologically, MF is characterized by atypical lymphocytes demonstrating epidermotropism without spongiosis. Spongiosis is the histological hallmark of intercellular epidermal edema, viewed as clear spaces within the epidermis, and is very common in benign inflammatory dermatoses. Very few studies have reported MF in sub-Saharan Africa (SSA). We are reporting a case of MF with a rare presentation of spongiosis treated successfully with a low dose total skin electron beam therapy (TSEBT) followed by maintenance therapy of low dose Methotrexate (MT) at the Ocean Road Cancer Institute (ORCI) in Tanzania. This is the first case of MF to be managed with low-dose TSEBT in Tanzania. The authors wish to create awareness of the disease among physicians and pathologists and expand on the data paucity in SSA.

Case description: We are reporting a case of a 31-year-old male of African origin who self-referred to our oncology center with a 4-year history of skin rashes throughout the body, which was unresponsive to topical steroid treatment. The biopsy was taken, and the patient was diagnosed with MF CD 3 positive with spongiosis. The patient was treated with radiotherapy, whereby he received low dose total skin electron beam therapy (TSEBT) 12 Gy in 3 fractions at a daily dose of 4 Gy, followed by maintenance therapy of low dose Methotrexate and attained an excellent therapeutic response.

Conclusion: Spongiosis is an infrequent presentation of MF. Low-dose TSEBT provides reliable and rapid reduction of disease burden in patients with MF, which could be administered safely multiple times during a patient's disease with an acceptable toxicity profile. Lack of tendency to perform skin biopsies and cost constraints in assessing multiple immunophenotypic markers lead to missing the diagnosis. Diagnosis and treatment of MF in resource-limited countries is challenging.

Keywords: Case report; Chemotherapy; Mycosis fungoides; Ocean Road Cancer Institute; Total skin electron beam radiotherapy.

Fig. 1

Photomicrographs of Hematoxylin and Eosin-stained sections showing a skin biopsy: A and B hyperkeratotic skin with extensive band lymphocytic inflammatory infiltrate in the papillary and reticular dermis. C and D Acanthosis and prominent spongiosis, Note the epidermotropism of the hyperchromatic lymphocytes with cerebriform nuclei, as well as occasional Pautrier microabscesses. Blood investigations: Complete blood count: WBC 8.67 109/L; 8.6 g/dL; HCT 26.4%; MCV 58.6 fL; NEU 6.39 10 3/μL; PLT 656.0 103/μL, Liver function tests: ALT 47.50 IU/L; AST 21.8000 IU/L and Renal function test: Serum creatine 84.170 µmol/L

Fig. 2

Showing the normal chest x-ray

Fig. 3

Showing the initial presentation of the patient’s upper trunk

Fig. 4

Showing the initial presentation of the patient’s lower trunk

Fig. 5

Showing the progress of the patient’s upper trunk (2 weeks post TSEBT)

Fig. 6

Showing the progress of the patient’s upper trunk (1-month post-TSEBT)

Fig. 7

Showing recurrent lesion (6 months post TSEBT)

Fig. 8

After radiotherapy

Fig. 9

A timeline of significant events