Adjei, Susuana

Mattia, Alexzandra

Miller, Austinn C

Temiz, Laurie A

Thompson, Anthony

Tyring, Stephen K

English

DigitalCommons@The Texas Medical Center

CASE REPORTFrom theFloridasee, FlMeharrment oTexas.eFunding sIRB approPsoriasiform mycosis fungoidesecutaneousT-cell lymphoma in an African AmericanAustinn C. Miller, MD,a Alexzandra Mattia, BS,b Anthony Thompson, BS,b Laurie A. Temiz, BA,c,dSusuana Adjei, MD,c and Stephen K. Tyring, MD, PhD, MBAc,eKey words: cutaneous T-cell lymphoma; CTCL; mycosis fungoides; MF; skin of color; psoriasis.INTRODUCTIONCutaneous T-cell lymphomas (CTCLs) are a groupof neoplasms characterized by monoclonal prolifer-ation of skin-homing T cells that show considerablevariation in clinical presentation, histologic appear-ance, immunophenotype, and prognosis.1 CTCL isoften divided into mycosis fungoides (MF)-CTCLsand noneMF-CTCLs. MF-CTCL is a rare disease,occurring in approximately 0.5 per 100,000 individ-uals in the United States.2 Despite the rate of MF-CTCL being highest among African Americans, thereis a lack of clinical images demonstrating this condi-tion in skin of color. Here, we describe a case ofpsoriasiform MF-CTCL misdiagnosed as psoriasis inan African American patient and provide excellentteaching images. Consent for publication of all pa-tient photographs and medical information wasprovided by the authors at the time of articlesubmission to the journal stating that all patientsgave consent for their photographs and medicalinformation to be published in print and online, withthe understanding that this information may bepublicly available.CASE REPORTA 45-year-old African-American man presentedwith well-circumscribed plaques, exhibiting finescale on his back, abdomen, groin, and axilla thathad progressively increased in size and number overthe last 3 years (Fig 1). The lesions first appeared inhis right groin, and within 2 weeks, the lesions hadspread to his back. Besides pruritus, the patient hadno other symptoms. Six months after the rashDermatology Associates of Tallahassee, Tallahassee,a; Florida State University College of Medicine, Tallahas-oridab; Centers for Clinical Studies, Webster, Texasc;y Medical College, Nashville, Tennesseed; and Depart-f Dermatology, University of Texas Houston, Houston,ources: None.val status: Not applicable.appeared, a biopsy was performed, which returnedwith indeterminate results. Given the characteristicscaly plaques, the patient was diagnosedwith plaquepsoriasis by a dermatologist and was treated withapremilast. Due to lack of improvement and sideeffects, the medication was discontinued. The pa-tient received no treatment over the next 6 months,and the plaques spread to his left groin, abdomen,and bilateral axilla. The patient was prescribedguselkumab and remained on this medication for6 months, noting that the plaques continued toincrease in number, become scalier, and developfissures. The patient was then switched to secukinu-mab but experienced similar results and the medi-cation was discontinued.Given the lack of improvement on psoriasistreatments, Wood’s lamp examination was per-formed, and fluorescence was detected on his skinlesions. The patient was prescribed terbinafine,antifungal spray, and shampoo/body wash. Thepatient noted decreased scale within 2 weeks oftreatment initiation. After 6 weeks, the patient beganexperiencing refractory pruritus and developedmore lesions. The antifungal medications werediscontinued, and the patient was started on bothoral/topical steroids and hydroxyzine.After 3 years of failed immunosuppressive andantifungal treatments from a multidisciplinary teamof primary care physicians and dermatologists, thepatient was referred to our institution. A detailedhistory and physical examination was performed.Given the treatment-resistant nature of thepsoriasiform-appearing rash, another biopsy wasCorrespondence to: Austinn C. Miller, MD, Dermatology Associatesof Tallahassee, 1707 Riggins Road, Tallahassee, FL 32308. E-mail:millerpublication@gmail.com.JAAD Case Reports 2023;38:17-9.2352-5126 2023 by the American Academy of Dermatology, Inc. Publishedby Elsevier, Inc. This is an open access article under the CC BYlicense (http://creativecommons.org/licenses/by/4.0/).https://doi.org/10.1016/j.jdcr.2023.05.03517Fig 1. A, Plaques with varying degrees of hyperpigmen-tation and fine scale, located on the right medial thigh. Thethickness of scaling in this image is reduced secondary torecent topical corticosteroid use. B, Admixture of pigmen-tation within each plaque. C, Arcuate lesions with finescale and admixture of pigmentation. Prominent lichen-ification is present on the proximal lesions. D, Arcuatelesions with scale and patches and plaques.Fig 2. A, Band-like papillary dermal lymphoid infiltratecomposed of small- to medium-sized hyperchromaticlymphocytes mixed with histiocytes and plasma cells.(H&E 2003) B, Lymphocytes are predominantlycomposed of T cells (CD31), with a CD4:CD8 ratio ofapproximately 4:1 and loss of CD7 in the CD41 lympho-cytes. (Top left: CD3 stain 403, top right: CD4 stain 403,bottom left: CD7 stain 403, bottom right: CD8 stain 403).H&E, Hematoxylin and eosin.JAAD CASE REPORTSAUGUST 202318 Miller et alperformed (Fig 2). Biopsy revealed dermal lymphoidinfiltrate with small- to medium-sized hyperchro-matic lymphocytes, mixed with histiocytes andplasma cells. Lymphocytes were predominatelyCD31, with a CD4:CD8 ratio of 4:1. The CD41lymphocytes did not react with CD7. Histologicalfindings, in conjunction with a T-cell receptor betagene rearrangement, were consistent with MF-CTCL.The patient was referred to MD Anderson CancerCenter for further treatment.DISCUSSIONOver 20 variants of MF-CTCL have been docu-mented on the basis of distinctive clinicopathologicfeatures, clinical behavior, and/or prognosis.1 Manypatients have nonspecific forms that may mimicother dermatoses (ie, chronic eczema or atopicdermatitis) with nondiagnostic biopsies. Rarely,MF-CTCL presents with psoriasiform plaques.Although the exact incidence is unknown, 1 studyof 436 MF-CTCL patients reported psoriasiformvariant frequency as 4.7%.3 Of the 8 case reportspublished on psoriasiform MF-CTCL misdiagnosedas psoriasis, this is the first documented in an AfricanAmerican.4In skin of color, 3 common and often intertwinedpresentations of MF-CTCL usually occur.5 First,dyspigmentation may result in a mixture of asymp-tomatic hypopigmented and hyperpigmented le-sions (Fig 1, A-D).5 In addition, some lesions maymimic a more common dermatosis, such as tinea,psoriasis, atopic dermatitis, vitiligo, or lichen pla-nus.5 The third presentation involves pruritus andsecondary lichenification and hyperpigmentation,which often masks characteristic MF-CTCL clues(Fig 1, C).5 Classic MF-CTCL morphology in skin ofcolor includes arcuate lesions with scale and poiki-lodermatous patches and plaques with atrophy (Fig1, A-D).6 The minimum size of MF-CTCL is generally5 cm, whereas many of the histologic mimics aresmaller lesions.5In psoriasiform MF-CTCL, clinical presentationincludes thick, scaly, well-demarcated, erythema-tous psoriasiform plaques, which are difficult todifferentiate from psoriasis clinically.6 More specificclues to psoriasiform MF-CTCL include accompa-nying alopecia, ulcerative lesions, and lymph nodeenlargement. Distribution may also be helpful.Classic MF-CTCL occurs in the ‘‘swimsuit’’ distribu-tion (buttocks, breasts, and intertriginous regions),whereas psoriasis most commonly occurs on thescalp and extensor surfaces.5 In contrast to psoriasis,psoriasiform MF-CTCL may demonstrate epidermo-tropism with infiltration of abnormal lymphocytes.6Gene rearrangement studies may also help differen-tiate both; however, inadequate samples may pro-duce false negative results.6 Biopsies from differentanatomic sites that show monoclonal T cells are alsohelpful in establishing a diagnosis in indeterminatecases.7JAAD CASE REPORTSVOLUME 38Miller et al 19Correct and timely diagnosis is crucial, givenpsoriasis treatment may exacerbate MF-CTCL. Mostcases of exacerbation following immunosuppressivetreatment have occurred secondary to cyclosporine,methotrexate, or tumor necrosis factor-a inhibitors.A review of patients who developed MF after sys-temic or biological therapy for psoriasis revealed 30cases.8 Of these, 25 patients were treated with atumor necrosis factor-a inhibitor8 and 6 patientswere treated with either an IL-17 or IL-23 inhibitor.8Staging is important because it directs manage-ment and carries prognostic significance. Althoughdiagnosis of MF-CTCL is typically prolonged andcertain treatments may exacerbate disease, it rarelyprogresses beyond the plaque stage in many pa-tients. Approximately 10% of patients develop nod-ules or tumors.5 However, African Americans have asignificantly increased risk of presenting with ahigher T-stage (T3-T4) and a higher rate of mortalitycompared with Whites.9 The reason for higher stageat presentation is unknown but inability to recognizesigns and symptoms of MF-CTCL and differentiate itfrom other conditions in those with skin of colorlikely contributes. Regardless, given the potential forpoor clinical outcomes relating to higher diseasestage, this is concerning.9The key to early treatment is a timely diagnosis.Although MF-CTCL’s qualities as a mimic causeconfusion, it is important to keep it on the differen-tial, especially in patients diagnosed with otherconditions refractory to treatment. Moreover, specialattention should be paid to MF-CTCL in skin of colorpresenting with classic features. Biopsy should beconsidered, especially if previously indeterminate.This has the potential to reduce burden associatedwith incorrect treatments and disease progressionand ultimately improve outcomes.Conflicts of interestNone disclosed.REFERENCES1. Hodak E, Amitay-Laish I. Mycosis fungoides: a great imitator.Clin Dermatol. 2019;37(3):255-267.2. Peterson E, Weed J, Lo Sicco K, Latkowski JA. Cutaneous T celllymphoma: a difficult diagnosis demystified. Dermatol Clin.2019;37(4):455-469.3. Sidiropoulou P, Nikolaou V, Marinos L, et al. The different facesof mycosis fungoides: results of a single-center study. Int JDermatol. 2020;59(3):314-320.4. Azizpour A, Ghanadan A, Nasimi M, Etesami I. Psoriasiformmycosis fungoides: a rare form of the disease with review ofthe literature. Dermatol Online J. 2017;23(9):13030/qt9mv8r0j7.5. Hinds GA, Heald P. Cutaneous T-cell lymphoma in skin of color.J Am Acad Dermatol. 2009;60(3):359-375; quiz 376-378.6. Yamaguchi Y, Fujita Y, Hirata Y, et al. Psoriasiform mycosisfungoides masquerading as tumourous plaques. Eur J Dermatol.2017;27(3):295-296.7. Elston DM, Stratman EJ, Miller SJ. Skin biopsy: biopsy issues inspecific diseases. J Am Acad Dermatol. 2016;74(1):1-16; quiz17-18.8. Biondo G, Cerroni L, Brunasso AMG, et al. Risk of mycosisfungoides in psoriatic patients: a critical review. J Eur AcadDermatol Venereol. 2020;34(6):1186-1195.9. Wilson LD, Hinds GA, Yu JB. Age, race, sex, stage and theincidence of cutaneous lymphoma. Clin Lymphoma MyelomaLeuk. 2012;12(5):291-296.

Psoriasiform Mycosis Fungoides-cutaneous T-cell Lymphoma in an African American

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Psoriasiform Mycosis Fungoides-cutaneous T-cell Lymphoma in an African American

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