Real-Life Barriers to Diagnosis of Early Mycosis Fungoides: An International Expert Panel Discussion 

Abstract

Mycosis fungoides (MF) is a rare, primary cutaneous T-cell lymphoma that is challenging to diagnose due to its heterogeneous clinical presentation and complex histology. The subtlety of the initial clinical appearance of MF can result in diagnostic delays and hesitancy to refer suspected cases to specialist clinics. An unmet need remains for greater awareness and education. Therefore, an international expert panel of dermatologists, oncologists, hematologists, and dermatopathologists convened to discuss and identify barriers to early and accurate MF diagnosis that could guide clinicians toward making a correct diagnosis. Confirmation of MF requires accurate assessment of symptoms and clinical signs, and subsequent correlation with dermatopathological findings. This review summarizes the expert panel's guidance, based on the literature and reallife experience, for dermatologists to help include MF in their list of differential diagnoses, along with simple clinical and histopathologic checklists that may help clinicians to suspect and identify potential MF lesions and reduce diagnostic delays.

Cutaneous T‐cell lymphoma—Focus on some problems, and some solutions

“…If the biopsies performed so far have not confirmed the lymphoma, and the suspicion persists clinically over time, I suggest repeating this approach in the following month/s. Moreover, topical corticosteroids should be discontinued 2 weeks before biopsy 9 …”

“…Moreover, topical corticosteroids should be discontinued 2 weeks before biopsy. 9 Finally, in patients in whom the diagnosis of cutaneous lymphoma is already well established, a new skin biopsy may have the value of supporting the choice for a particular treatment. In fact, the histology may show a progression to another stage of the disease (large cell transformation, change of phenotype, other), with impact on the prognosis, 15 or may show another (additional) diagnosis such as a drug reaction, or another inflammatory skin disease, or an infection, and therefore allowing a specific therapeutic approach.…”

“…In conclusion, the definitive diagnosis and a correct classification of a cutaneous lymphoma is only possible if done by experts 9,10 who know how to integrate all these characteristics. Ideally, experts from different disciplines discuss the situation of the patient in a dedicated board with the participation of dermatology, pathology, oncology, hematology, radiology, nuclear medicine, radiotherapy.…”

Mechanism of action of chlormethine gel in mycosis fungoides

“…2,6,7 Early-stage MF (~70% of patients) presents as patches and/or plaques on the skin (often in sun-protected areas), usually without extracutaneous involvement. 3,8,9 Progression to more advanced stages involves the development of cutaneous tumours or generalized erythroderma, sometimes with lymph node, blood and visceral organ involvement. 10,11 Although patients with stage IA MF generally have a normal life expectancy, 12 the prognosis for advanced disease is poor, with one study reporting a median survival of approximately 5 years and a 5-year survival rate of just 52%.…”

“…MF typically affects older adults and has a chronic indolent, relapsing‐and‐remitting clinical course, progressing slowly from early‐stage disease (stages IA–IIA) to more advanced stages (IIB–IVB) over a prolonged period, even decades 2,6,7 . Early‐stage MF (~70% of patients) presents as patches and/or plaques on the skin (often in sun‐protected areas), usually without extracutaneous involvement 3,8,9 . Progression to more advanced stages involves the development of cutaneous tumours or generalized erythroderma, sometimes with lymph node, blood and visceral organ involvement 10,11 .…”