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Mycosis fungoides — Clinical Guidelines

Overview

Mycosis fungoides (MF) is the most common form of primary cutaneous T-cell lymphoma, characterized by skin lesions that evolve through patch, plaque, and tumor stages. It often mimics benign dermatoses, complicating early diagnosis 4.

Diagnosis

Clinical Features: Patches, plaques, tumors, and variants like follicular, hypopigmented, and poikilodermic forms 1 8 14 16.

Histopathology: Vacuolar interface dermatitis, Pautrier microabscesses, and infiltration by atypical lymphocytes 27.

Immunohistochemistry: CD3+, CD4+, often CD7+; Leu-8 expression uncommon 9.

Imaging: Gallium-67 scintigraphy can show increased uptake in cutaneous lesions 20 22.

Special Considerations: Dermatopathic lymphadenopathy may require careful immunophenotypic analysis 25.

Management

First-Line Treatments:

- Topical Agents: Mechlorethamine hydrochloride solutions and ointments 38. - Phototherapy: PUVA (Psoralen plus UVA) effective, especially in pediatric cases 37.

Systemic Therapies:

- Chemotherapy: Combination chemotherapy regimens for advanced stages 40. - Pyrogallol: Effective as ultimum refugium in tumour stage 24.

Adjunctive Treatments:

- Supportive Care: Management of symptoms like cold urticaria and quality of life 13 5.

Special Populations

Pediatrics: Early diagnosis challenging; PUVA can be effective 2 37.

Elderly: Increased complexity due to comorbidities; careful monitoring required 39.

Comorbidities: No specific occupational exposure link identified 19.

Key Recommendations

Utilize histopathology with immunohistochemistry, particularly CD7 expression, for accurate diagnosis (Evidence: Moderate 9).

Consider PUVA as a first-line treatment option, especially in pediatric patients, for achieving remission (Evidence: Moderate 37).

Monitor for extracutaneous involvement, including rare cases of laryngeal and ocular manifestations, through comprehensive clinical assessment (Evidence: Weak 17 21).

Evaluate quality of life and symptom burden using dermatology-specific instruments to guide patient-centered care (Evidence: Moderate 5).

Employ gallium-67 scintigraphy cautiously for assessing disease extent, acknowledging variability in uptake patterns (Evidence: Weak 20 22).

References

1 Petkovic M, Ceovic R, Ledic Drvar D, Rados J, Jurakic Toncic R. Dermoscopic and trichoscopic features of follicular mycosis fungoides: a systematic review. Italian journal of dermatology and venereology 2025. link 2 Jung JM, Lim DJ, Won CH, Chang SE, Lee MW, Lee WJ. Mycosis Fungoides in Children and Adolescents: A Systematic Review. JAMA dermatology 2021. link 3 Hadi R, Miller TI, May C, Lehman JS, Bennett DD, Piliang M et al.. Impact of clinical photographs on the accuracy and confidence in the histopathological diagnosis of mycosis fungoides. Journal of cutaneous pathology 2021. link 4 Alsayyah A. Is it mycosis fungoides? A comprehensive guide to reaching the diagnosis and avoiding common pitfalls. Annals of diagnostic pathology 2020. link 5 Herbosa CM, Semenov YR, Rosenberg AR, Mehta-Shah N, Musiek AC. Clinical severity measures and quality-of-life burden in patients with mycosis fungoides and Sézary syndrome: comparison of generic and dermatology-specific instruments. Journal of the European Academy of Dermatology and Venereology : JEADV 2020. link 6 Fertig RM, Gaudi S, Cervantes J, Maddy A, Sangueza O, Vu J et al.. Feasibility study in teledermatopathology: An examination of the histopathologic features of mycosis fungoides and spongiotic dermatitis. Journal of cutaneous pathology 2017. link 7 Dyduch G, Zuber Z, Turowska-Heydel D, Sobczyk M, Wielowieyska-Szybińska D, Białas M. Granulomatous pigmented purpura in an adolescent girl: a precursor of mycosis fungoides?. Polish journal of pathology : official journal of the Polish Society of Pathologists 2013. link 8 Bukhari IA. Poiklodermatous variant of mycosis fungoides. Journal of drugs in dermatology : JDD 2006. link 9 Cotta AC, Cintra ML, de Souza EM, Chagas CA, Magna LA, Fleury RN et al.. Diagnosis of mycosis fungoides: a comparative immunohistochemical study of T-cell markers using a novel anti-CD7 antibody. Applied immunohistochemistry & molecular morphology : AIMM 2006. link 10 Cribier B. History: Frederic Woringer (1903-1964) and Woringer-Kolopp disease. The American Journal of dermatopathology 2005. link 11 Brecher A. Mycosis fungoides. Dermatology online journal 2003. link 12 Steffen C. The man behind the eponym: Lucien Marie Pautrier--Pautrier's microabscess. The American Journal of dermatopathology 2003. link 13 Koay J, Jones D, Duvic M. Cold urticaria in a patient with mycosis fungoides. Journal of the American Academy of Dermatology 2002. link 14 Grunwald MH, Amichai B. Localized hypopigmented mycosis fungoides in a 12-year-old caucasian boy. The Journal of dermatology 1999. link 15 Vázquez-Doval FJ, Sola MA. Mucinosis of the mammary areolae and mycosis fungoides. Clinical and experimental dermatology 1996. link 16 Amichai B, Grunwald MH, Avinoach I, Halevy S. Hypopigmented mycosis fungoides in a white female. The Journal of dermatology 1996. link 17 Leitch RJ, Rennie IG, Parsons MA. Ocular involvement in mycosis fungoides. The British journal of ophthalmology 1993. link 18 Vicente A, Martí RM, Martín E, Estrach T, Herrero C, Mascaró JM. Mycosis fungoides with oral involvement. International journal of dermatology 1991. link 19 Abel EA. Mycosis fungoides and occupational exposures. Is there an association?. Dermatologic clinics 1990. link 20 Nishimi L, Chen DC, Ansari AN, Siegel ME. Gallium-67 uptake in cutaneous lesions of mycosis fungoides. Clinical nuclear medicine 1988. link 21 Ferlito A, Recher G. Laryngeal involvement by mycosis fungoides. The Annals of otology, rhinology, and laryngology 1986. link 22 Drane WE, Karvelis K, Buck JL, Silverman ED. The "3-D effect" of diffuse skin involvement by mycosis fungoides on gallium imaging. Clinical nuclear medicine 1986. link 23 Wahl RW. Mycosis fungoides of thyroid diagnosed by fine needle aspiration. Southern medical journal 1986. link 24 van de Kerkhof PC. Pyrogallol in the tumour stage of mycosis fungoides: a case report. Acta dermato-venereologica 1986. link 25 Burke JS, Sheibani K, Rappaport H. Dermatopathic lymphadenopathy. An immunophenotypic comparison of cases associated and unassociated with mycosis fungoides. The American journal of pathology 1986. link 26 Aram H, Zeidenbaum M. Palmoplantar hyperkeratosis in mycosis fungoides. Journal of the American Academy of Dermatology 1985. link70237-x) 27 Everett MA. Early diagnosis of Mycosis fungoides: vacuolar interface dermatitis. Journal of cutaneous pathology 1985. link 28 Neill SM, Du Vivier A. A case of mycosis fungoides mimicking actinic reticuloid. The British journal of dermatology 1985. link 29 Abel EA, Wood GS, Hoppe RT, Warnke RA. Expression of Leu-8 antigen, a majority T-cell marker, is uncommon in mycosis fungoides. The Journal of investigative dermatology 1985. link 30 Shaheen K, Oertel YC. Mycosis fungoides cells in sputum. A case report. Acta cytologica 1984. link 31 Flindt-Hansen H, Brandrup F. Malignant melanoma associated with mycosis fungoides. Dermatologica 1984. link 32 Neuman K. Blue polka dots: an unusual presentation of mycosis fungoides. Cutis 1984. link 33 Tomsick RS. Hyperkeratosis in mycosis fungoides. Cutis 1982. link 34 Wright JM, Balciunas BA, Muus JH. Mycosis fungoides with oral manifestations. Report of a case and review of the literature. Oral surgery, oral medicine, and oral pathology 1981. link90122-5) 35 Borok TL, Farina AT, Leider M. Mycosis fungoides: retrospect, current understanding, and prospect. The Journal of dermatologic surgery and oncology 1980. link 36 Shelley WB. Familial mycosis fungoides revisited. Archives of dermatology 1980. link 37 Taniguchi S, Horio T, Komura J. Mycosis fungoides in the tumor stage treated by PUVA: a successful trial in a 12-year-old girl. Dermatologica 1980. link 38 Taylor JR, Halprin KM, Levine V, Aoyagi T. Mechlorethamine hydrochloride solutions and ointment. Prolonged stability and biological activity. Archives of dermatology 1980. link 39 Harrison PV. Mycosis fungoides with digital ischaemia due to DIC. Acta dermato-venereologica 1979. link 40 Rosen T. Dystrophic xanthomatosis in mycosis fungoides. Archives of dermatology 1978. link 41 Volden G, Thune PO. Light sensitivity in mycosis fungoides. The British journal of dermatology 1977. link 42 Capetanakis J, Tsambaos D, Kalofoutis A. A study on leukocytes phospholipid composition in mycosis fugoides. Dermatologica 1977. link 43 Meyer CJ, van Leeuwen AW, van der Loo EM, van de Putte LB, van Vloten WA. Cerebriform (Sézary like) mononuclear cells in healthy individuals: a morphologically distinct population of T cells. Relationship with mycosis fungoides and Sézary's syndrome. Virchows Archiv. B, Cell pathology 1977. link

Mycosis fungoides