INTRODUCTION
Over the past decade, what is known about skin of color in the field of dermatology has gained an increasing amount of attention among both clinicians and the general public. Recent efforts have continued to shed light on the role of skin color in health disparities and the development of actionable strategies to improve outcomes for patients of color. While research has largely focused on conditions such as atopic dermatitis, psoriasis, and skin cancer, uncommon dermatologic conditions have been less emphasized in skin of color discussions thus far. Nevertheless, it is important to highlight manifestations of less common skin conditions in skin of color patients that residents and physicians may not encounter frequently in clinical practice.
Although rare, mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma (CTCL) among all racial groups.1 MF has a relatively low incidence of 0.41 per 100,000 person-years.1 Despite being more commonly diagnosed in White patients, African Americans are reported to have an incidence of 1.5 times that of Caucasians.2,3 Further, Black patients with MF are diagnosed at later stages and have higher body surface area involvement at the time of diagnosis when compared to white patients.2,4 MF in Black patients has been shown to be associated with poorer prognosis and survival outcomes.1,2,5 However, studies examining the underlying mechanisms contributing to these differences have yielded conflicting results.1,4 The marked variability of MF and its broad differential diagnosis pose a significant diagnostic challenge, particularly early in the course of disease. The diagnosis may be unrecognized or misdiagnosed for years, delaying treatment while the disease follows its indolent progression. Since the prognosis is generally favorable in early stages, early diagnosis by dermatologists and community physicians is essential.6
MF is recognized as a “wild card†in dermatology, closely mimicking a wide variety of inflammatory and pigmentation disorders.7 With respect to classic MF, initial presentation is characterized by progressive erythematous patches or plaques of varying shape and size with a scaling, atrophic surface appearance.8 In skin of color patients, hypopigmented MF is the most common variant, although lesions often exhibit significant variability in pigmentation.7,9 Hypopigmented MF mimics include vitiligo, tinea corporis, tinea versicolor, and postinflammatory hypopigmentation, among others.8 Further, patients with hyperpigmented and erythematous lesions have been shown to have poorer outcomes and more disease progression when compared to hypopigmented forms.7,9 While the clinical heterogeneity of MF has long been known to present diagnostic challenges irrespective of race, it is particularly important to emphasize manifestations and subtleties more commonly seen in darker skin patients to ensure early recognition and treatment for all patients regardless of racial or ethnic background. Increasing awareness of the diverse presentations and differential diagnosis of MF in skin of color patients may help train physicians to know what to look for. Additionally, prompt and definitive diagnosis has important treatment implications, as misdiagnosis and incorrect treatment may result in harmful effects.
Although many studies have proposed that differences in socioeconomic measures and lack of access to medical care may play a role in race-related outcomes in MF, the factors responsible for racial disparities remain unclear.1,9,10 Ultimately, further investigation of demographic trends, socioeconomic parameters, differences in disease biology, and response to treatment is warranted. From a broader health disparity perspective, this highlights the need for more involvement of skin of color patients in research efforts and clinical trials. Regardless of the mechanisms at play, increased attention to the broad clinicopathologic spectrum of MF and differences in disease presentation in skin of color patients may improve diagnostic recognition and patient outcomes. Particularly with respect to rare diseases that residents and training clinicians may not frequently see firsthand, awareness and educational efforts serve as promising targets for change as we continue to shed light on health disparities in skin of color.
Although rare, mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma (CTCL) among all racial groups.1 MF has a relatively low incidence of 0.41 per 100,000 person-years.1 Despite being more commonly diagnosed in White patients, African Americans are reported to have an incidence of 1.5 times that of Caucasians.2,3 Further, Black patients with MF are diagnosed at later stages and have higher body surface area involvement at the time of diagnosis when compared to white patients.2,4 MF in Black patients has been shown to be associated with poorer prognosis and survival outcomes.1,2,5 However, studies examining the underlying mechanisms contributing to these differences have yielded conflicting results.1,4 The marked variability of MF and its broad differential diagnosis pose a significant diagnostic challenge, particularly early in the course of disease. The diagnosis may be unrecognized or misdiagnosed for years, delaying treatment while the disease follows its indolent progression. Since the prognosis is generally favorable in early stages, early diagnosis by dermatologists and community physicians is essential.6
MF is recognized as a “wild card†in dermatology, closely mimicking a wide variety of inflammatory and pigmentation disorders.7 With respect to classic MF, initial presentation is characterized by progressive erythematous patches or plaques of varying shape and size with a scaling, atrophic surface appearance.8 In skin of color patients, hypopigmented MF is the most common variant, although lesions often exhibit significant variability in pigmentation.7,9 Hypopigmented MF mimics include vitiligo, tinea corporis, tinea versicolor, and postinflammatory hypopigmentation, among others.8 Further, patients with hyperpigmented and erythematous lesions have been shown to have poorer outcomes and more disease progression when compared to hypopigmented forms.7,9 While the clinical heterogeneity of MF has long been known to present diagnostic challenges irrespective of race, it is particularly important to emphasize manifestations and subtleties more commonly seen in darker skin patients to ensure early recognition and treatment for all patients regardless of racial or ethnic background. Increasing awareness of the diverse presentations and differential diagnosis of MF in skin of color patients may help train physicians to know what to look for. Additionally, prompt and definitive diagnosis has important treatment implications, as misdiagnosis and incorrect treatment may result in harmful effects.
Although many studies have proposed that differences in socioeconomic measures and lack of access to medical care may play a role in race-related outcomes in MF, the factors responsible for racial disparities remain unclear.1,9,10 Ultimately, further investigation of demographic trends, socioeconomic parameters, differences in disease biology, and response to treatment is warranted. From a broader health disparity perspective, this highlights the need for more involvement of skin of color patients in research efforts and clinical trials. Regardless of the mechanisms at play, increased attention to the broad clinicopathologic spectrum of MF and differences in disease presentation in skin of color patients may improve diagnostic recognition and patient outcomes. Particularly with respect to rare diseases that residents and training clinicians may not frequently see firsthand, awareness and educational efforts serve as promising targets for change as we continue to shed light on health disparities in skin of color.
DISCLOSURES
The authors have no conflicts of interest to declare.
REFERENCES
1. Su C, Nguyen KA, Bai HX, et al. Racial disparity in mycosis fungoides: An analysis of 4495 cases from the US National Cancer Database. J Am Acad Dermatol. 2017;77(3):497-502.e2. doi:10.1016/j.jaad.2017.04.1137
2. Huang AH, Kwatra SG, Khanna R, et al. Racial disparities in the clinical presentation and prognosis of patients with mycosis fungoides. J Natl Med Assoc. 2019;111(6):633-639. doi:10.1016/j.jnma.2019.08.006
3. Agbai ON, Buster K, Sanchez M, et al. Skin cancer and photoprotection in people of color: a review and recommendations for physicians and the public. J Am Acad Dermatol. 2014;70(4):748-762. doi:10.1016/j.jaad.2013.11.038
4. Nath SK, Yu JB, Wilson LD. Poorer prognosis of African-American patients with mycosis fungoides: an analysis of the SEER dataset, 1988 to 2008. Clin Lymphoma Myeloma Leuk. 2014;14(5):419-423. doi:10.1016/j.clml.2013.12.018
5. Sun G, Berthelot C, Li Y, et al. Poor prognosis in non-Caucasian patients with early-onset mycosis fungoides. J Am Acad Dermatol. 2009;60(2):231-235. doi:10.1016/j.jaad.2008.09.063
6. Kelati A, Gallouj S, Tahiri L, et al. Defining the mimics and clinico-histological diagnosis criteria for mycosis fungoides to minimize misdiagnosis. Int J Womens Dermatol. 2017;3(2):100-106. Published 2017 Jan 30. doi:10.1016/j.ijwd.2016.11.006
7. Hinds GA, Heald P. Cutaneous T-cell lymphoma in skin of color. J Am Acad Dermatol. 2009;60(3):359-378. doi:10.1016/j.jaad.2008.10.031
8. Hodak E, Amitay-Laish I. Mycosis fungoides: A great imitator. Clin Dermatol. 2019;37(3):255- 267. doi:10.1016/j.clindermatol.2019.01.004
9. Geller S, Lebowitz E, Pulitzer M, Myskowski PL. Understanding racial disparities in mycosis fungoides through international collaborative studies. Br J Dermatol. 2019;180(5):1263-1264. doi:10.1111/bjd.17598
10. Geller S, Lebowitz E, Pulitzer MP, et al. Outcomes and prognostic factors in African American and black patients with mycosis fungoides/Sezary syndrome: Retrospective analysis of 157 patients from a referral cancer center. J Am Acad Dermatol. 2020;83(2):430-439. doi:10.1016/j. jaad.2019.08.073
2. Huang AH, Kwatra SG, Khanna R, et al. Racial disparities in the clinical presentation and prognosis of patients with mycosis fungoides. J Natl Med Assoc. 2019;111(6):633-639. doi:10.1016/j.jnma.2019.08.006
3. Agbai ON, Buster K, Sanchez M, et al. Skin cancer and photoprotection in people of color: a review and recommendations for physicians and the public. J Am Acad Dermatol. 2014;70(4):748-762. doi:10.1016/j.jaad.2013.11.038
4. Nath SK, Yu JB, Wilson LD. Poorer prognosis of African-American patients with mycosis fungoides: an analysis of the SEER dataset, 1988 to 2008. Clin Lymphoma Myeloma Leuk. 2014;14(5):419-423. doi:10.1016/j.clml.2013.12.018
5. Sun G, Berthelot C, Li Y, et al. Poor prognosis in non-Caucasian patients with early-onset mycosis fungoides. J Am Acad Dermatol. 2009;60(2):231-235. doi:10.1016/j.jaad.2008.09.063
6. Kelati A, Gallouj S, Tahiri L, et al. Defining the mimics and clinico-histological diagnosis criteria for mycosis fungoides to minimize misdiagnosis. Int J Womens Dermatol. 2017;3(2):100-106. Published 2017 Jan 30. doi:10.1016/j.ijwd.2016.11.006
7. Hinds GA, Heald P. Cutaneous T-cell lymphoma in skin of color. J Am Acad Dermatol. 2009;60(3):359-378. doi:10.1016/j.jaad.2008.10.031
8. Hodak E, Amitay-Laish I. Mycosis fungoides: A great imitator. Clin Dermatol. 2019;37(3):255- 267. doi:10.1016/j.clindermatol.2019.01.004
9. Geller S, Lebowitz E, Pulitzer M, Myskowski PL. Understanding racial disparities in mycosis fungoides through international collaborative studies. Br J Dermatol. 2019;180(5):1263-1264. doi:10.1111/bjd.17598
10. Geller S, Lebowitz E, Pulitzer MP, et al. Outcomes and prognostic factors in African American and black patients with mycosis fungoides/Sezary syndrome: Retrospective analysis of 157 patients from a referral cancer center. J Am Acad Dermatol. 2020;83(2):430-439. doi:10.1016/j. jaad.2019.08.073
AUTHOR CORRESPONDENCE
Kendall McDaniel BS kmcdaniel@tulane.edu
