Clinical Challenge: Hypopigmented Macules on the Trunk and Extremities - Dermatology Advisor

Clinical Challenge: Hypopigmented Macules on the Trunk and Extremities

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A 50-year-old black man comes to the clinic seeking treatment for a 20-plus year history of a skin condition manifested as hypopigmented macules distributed on his trunk and extremities. Personal and family history are negative for atopic dermatitis, asthma, and seasonal allergies. He experiences minimal pruritus. Past treatment with topical triamcinolone acetonide and antifungal creams resulted in minimal improvement.

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Hypopigmented mycosis fungoides (HMF) is an uncommon variant of primary cutaneous T-cell lymphoma and was first reported in 1973.1 The condition is characterized by the appearance of hypopigmented or dyschromic patches. Compared with the classic variant, the prevalence is higher...

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Hypopigmented mycosis fungoides (HMF) is an uncommon variant of primary cutaneous T-cell lymphoma and was first reported in 1973.1 The condition is characterized by the appearance of hypopigmented or dyschromic patches. Compared with the classic variant, the prevalence is higher in younger patients and has been reported in both children and adolescents.2 The condition is most commonly diagnosed in darker-skinned individuals and Asians.3

Histopathology of HMF is similar to that of classic mycosis fungoides, and both often require repeated biopsies over the course of the disease to confirm the diagnosis. Immunohistochemical analysis results are also similar for both types, although some cases of HMF have a predominant CD8+ cell epidermotropism.4

HMF does not tend to progress, and the disease has a favorable prognosis.5 For this reason, aggressive therapy is generally avoided, especially in younger patients. Photochemotherapy achieves lasting remission in the majority of patients6; narrow-band ultraviolet B therapy is also efficacious and is preferred in children.7,8 Excimer laser therapy has been reported to clear lesions and may prove to be a viable option when disease is of limited distribution.9 Topical clobetasol and nitrogen mustard, standard therapies for patch stage MF, may prove of value as well.10

References

  1. Ryan EA, Sanderson KV, Barták P, Samman PD. Can mycosis fungoides begin in the epidermis? A hypothesis.  Br J Dermatol. 1973;88:419-429.
  2. Neuhaus IM, Ramos-Caro FA, Hassanein AM. Hypopigmented mycosis fungoides in childhood and adolescence.  Pediatr Dermatol. 2000;17:403-406.
  3. Furlan FC, Sanches JA. Hypopigmented mycosis fungoides: a review of its clinical features and pathophysiology.  An Bras Dermatol. 2013;88:954-960.
  4. El-Shabrawi-Caelen L, Cerroni L, Medeiros LJ, McCalmont TH. Hypopigmented mycosis fungoides: frequent expression of a CD8+ T-cell phenotype.  Am J Surg Pathol. 2002;26:450-457.
  5. Akaraphanth R, Douglass MC, Lim HW. Hypopigmented mycosis fungoides: treatment and a 6(1/2)-year follow-up of 9 patients.  J Am Acad Dermatol. 2000;42:33-39.
  6. Duarte I, Bedrikow R, Aoki S. Mycosis fungoides: epidemiologic study of 17 cases and evaluation of PUVA photochemotherapy.  An Bras Dermatol. 2006;81:40-45.
  7. Bisherwal K, Singal A, Pandhi D, Sharma S. Hypopigmented mycosis fungoides: clinical, histological, and immunohistochemical remission induced by narrow-band ultraviolet B.  Indian J Dermatol. 2017;62(2):203-206.
  8. Onsun N, Kural Y, Su O, Demirkesen C, Büyükbabani N. Hypopigmented mycosis fungoides associated with atopy in two children.  Pediatr Dermatol. 2006;23:493-496.
  9. Yang M-Y, Jin H, You H-S, et al. Hypopigmented mycosis fungoides treated with 308 nm excimer laser.  Ann Dermatol. 2018;30(1):93-95.
  10. Patrone CC, Geskin LJ. Clinical insights and emerging strategies in managing cutaneous T-cell lymphoma. J Drugs Dermatol. 2017;16(5):405-412.
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