Case Presentation
A 31-year-old African male teacher presented to the Ocean Road Cancer Institute (ORCI) with a persistent and bothersome skin condition. This had been troubling him for the past four years. His primary concern was an itchy rash initially appearing as small, raised areas on his trunk. Over time, these spots multiplied and spread to other parts of his body, including his limbs, face, and buttocks. As the condition progressed, the rash became more severe, leading to ulceration.
Despite seeking medical attention between 2017 and 2020 and receiving various topical treatments, including steroids and moisturizing creams. However, the patient experienced no improvement. He reported no previous history of a similar illness and had no underlying chronic health issues. He had not been exposed to occupational chemicals, drugs, radiation, or infections before the onset of the rash. None of his family members had a history of a similar condition or a background of atopy. Additionally, the patient did not consume alcohol or use tobacco products. He did mention occasional bouts of diarrhoea but denied experiencing evening fevers, night sweats, weight loss, or joint pain.
Examination
Upon examination, the patient appeared generally well, with normal vital signs and an Eastern Cooperative Oncology Group (ECOG) performance status of 1. The skin examination revealed extensive hyperpigmented erosive plaques, nodules, and tumors covering more than 95% of his body, including non-sun-exposed areas. Notably, there were ulcers on the left knee and elbow. Despite the severe skin involvement, there were no significant abnormalities in peripheral lymph nodes or the spleen. Systemic and neurological examinations showed normal cognitive function, intact cranial nerves, and no signs of meningeal irritation. The cardiovascular, respiratory, and abdominal systems appeared normal.
Diagnosis
A skin biopsy confirmed the diagnosis of Mycosis Fungoides (MF), a type of cutaneous T-cell lymphoma, with notable spongiosis. Immunohistochemistry testing demonstrated CD3 positivity, while CD4, CD5, and CD8 assessments were not conducted. Further investigations, such as urinalysis and microbiology, were not performed. HIV-1 serology testing was negative, and imaging studies, including chest X-rays and abdominal ultrasounds, revealed no abnormalities.
Following a comprehensive assessment, the patient received a score of 6 points based on the International Society for Cutaneous Lymphomas (ISCL) diagnostic algorithm. The evaluation included criteria such as persistent and progressive patches or thin plaques, non-sun-exposed location, size or shape variation, superficial lymphoid infiltrate, lymphocytic atypia, and CD3+ positivity. The final diagnosis was Mycosis Fungoides (MF), categorized as stage 3 (T3N0M0BX) by the ISCL and the Cutaneous Lymphoma Task Force of the European Organization of Research and Treatment of Cancer (EORTC).
Management: Mycosis Fungoides
Upon admission, the initial treatment plan involved a 30-day course of oral Prednisolone (10 mg b.i.d.) and a 10-day regimen of oral Ampiclox capsules (500 mg q.i.d). Subsequently, the patient underwent low-dose Total Skin Electron Beam Therapy (TSEBT) in October 2021, receiving 12 Gy in 3 fractions at a daily dose of 4 Gy. This therapy utilized 3D conformal radiotherapy (3DCRT) with a linear accelerator (LINAC), exposing the entire skin to radiation in an upright, half-naked position. The treatment involved a 12 MeV beam, employing three anterior and posterior fields with gantry angles of 1080 and 720 for the upper and lower halves, respectively.
Remarkably, after three TSEBT sessions, the patient experienced significant improvement, with lesions, itching, and pain subsiding. Following the completion of TSEBT, the patient transitioned to maintenance oral methotrexate (50 mg weekly with a 1-week rest every 4 weeks), achieving a complete response (CR) within three months.
However, five months later, a recurrence was observed in the patient’s left leg, prompting localized TSEBT (12 Gy in four fractions), resulting in another complete response. Two years post-initial treatment, the patient remains in remission and continues maintenance of oral methotrexate (50 mg weekly). The patient did not encounter any acute or late effects associated with radiotherapy.
Discussion: Mycosis Fungoides
This is a case of advanced Mycosis Fungoides (MF) with a distinctive feature of spongiosis, which exhibited a complete and enduring response following low-dose Total Skin Electron Beam Therapy (TSEBT).
Diagnosing MF poses a considerable challenge, particularly in resource-limited settings, due to atypical clinical presentations resembling other dermatoses. This patient’s lesions initially mimicked psoriasis, atopic dermatitis, or chronic eczema, highlighting the diagnostic complexity. The International Society for Cutaneous Lymphomas (ISCL) proposed a diagnostic algorithm that integrates various characteristics for a comprehensive diagnosis, including clinical, histopathologic, immunopathologic, and molecular biological aspects.
Treatment options for MF range from skin-directed therapies in the early stages to systemic therapies in the advanced stages. In this case, low-dose TSEBT proved effective, with advantages including shorter treatment duration, reduced toxicities, cost-effectiveness, improved patient compliance, and the ability to use the therapy more frequently over a patient’s lifetime.
Despite being generally considered incurable, most MF patients have indolent forms with long-term survival rates. Early-stage patients typically receive skin-directed therapies, while advanced-stage cases need systemic therapy. However, in resource-limited settings, the therapeutic options are limited to steroids, chemotherapeutic agents, radiotherapy, and heliotherapy. Low-dose TSEBT stands out with the highest overall response rate among skin-directed therapies.
