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CORRESPONDENCE
Year : 2021  |  Volume : 39  |  Issue : 3  |  Page : 145-146

Juvenile folliculotropic mycosis fungoides mimicking tinea capitis: A case report and literature review


1 Department of Dermatology, Chang Gung Memorial Hospital, Linkou; College of Medicine, Chang Gung University, Taoyuan, Taiwan
2 Department of Dermatology, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan
3 College of Medicine, Chang Gung University; Department of Anatomic Pathology, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan

Date of Submission09-Jun-2020
Date of Decision06-Mar-2021
Date of Acceptance10-Mar-2021
Date of Web Publication19-Jul-2021

Correspondence Address:
Dr. Chun-Wei Lu
Department of Dermatology, Chang Gung Memorial Hospital, Linkou, 5, Fuxing St., Guishan Dist, Taoyuan 33305
Taiwan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ds.ds_15_21

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How to cite this article:
Lu LY, Yen H, Sun PL, Huang YL, Lu CW. Juvenile folliculotropic mycosis fungoides mimicking tinea capitis: A case report and literature review. Dermatol Sin 2021;39:145-6

How to cite this URL:
Lu LY, Yen H, Sun PL, Huang YL, Lu CW. Juvenile folliculotropic mycosis fungoides mimicking tinea capitis: A case report and literature review. Dermatol Sin [serial online] 2021 [cited 2021 Oct 19];39:145-6. Available from: https://www.dermsinica.org/text.asp?2021/39/3/145/321874



Dear Editor,

Among primary cutaneous T-cell lymphomas, mycosis fungoides (MF) is the most common and has been demonstrated to effectively mimic other conditions.[1] Folliculotropic MF (FMF) is a distinct variant of MF that is rarely encountered in young adults and is associated with a poor prognosis.[1] In this article, we report an unusual case of juvenile FMF mimicking tinea capitis in a healthy young adult; few similar cases have been previously reported.

A 19-year-old male patient without any underlying disease presented with diffuse hair loss and erythematous scaling patches accompanied by yellowish crusts and pustules on the occipital scalp that had persisted for 4 years [Figure 1]a. A dermoscopic examination revealed numerous black dots and follicular plugging, and therefore, tinea capitis was suspected. Itraconazole (200 mg/day) was initially prescribed as the antifungal therapy. However, an examination of the hair using potassium hydroxide yielded unremarkable results, and the bacterial culture exhibited a high presence of methicillin-sensitive Staphylococcus aureus. Oral amoxicillin clavulanate was prescribed to treat the secondary bacterial infection. Due to poor responses to the antifungal and antibacterial therapy, a skin biopsy was performed.
Figure 1: (a) Large alopecia patch with erythematous scaling plaques and crusts on the occipital scalp for 4 years. (b) Severe indurated erythematous plaque with prominent follicular plugging after methotrexate and acitretin therapy. The patient demonstrated favorable responses at (c) 1 year and (d) 2 years after the initiation of psoralen and ultraviolet A therapy, with reduced erythematous plaques and partial hair regrowth.

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The histopathological examination revealed diffuse infiltrates of atypical lymphocytes in the dermis with extensive folliculotropism [Figure 2]a, [Figure 2]b, [Figure 2]c, [Figure 2]d, [Figure 2]e, [Figure 2]f. These atypical lymphocytes were medium sized with round to mildly irregular nuclear contours. Colloidal iron staining revealed mild mucinosis around the follicles. Periodic acid–Schiff–diastase staining was negative for fungal hyphae. Immunohistochemistry revealed that the folliculotropic atypical lymphocytes expressed the CD3+/CD5+/CD7-/CD4+/CD8- immunophenotype.
Figure 2: (a) Histopathology shows diffuse infiltrates of atypical lymphocytes in the dermis with extensive folliculotropism (H and E, ×40) (b) The atypical lymphocytes are intermediate with round to irregular nuclei (×400, inset × 800) (c-f) Immunohistochemistry reveals that the atypical folliculotropic lymphocytes have CD4(+), CD5(+), CD7(−) and CD8(−) immunophenotype (c: CD4, d: CD5, e: CD7, f: CD8, ×200).

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Serum laboratory data were unremarkable, except for IgE, which was elevated (797 IU/mL). Oral methotrexate (5 mg/week) and acitretin (20 mg/day) were prescribed, but the hair loss pattern with follicular plugging persisted [Figure 1]b. A T-cell receptor (TCR) gene rearrangement study revealed a monoclonal band on the 259nt locus for TCR beta VB-JB2 and a 321nt locus for TCR beta DB-JB, which supported the monoclonality of these atypical lymphocytes. This, the diagnosis of juvenile FMF was reached. The patient was referred to a hematologist for a lymphoma survey. A chest radiograph and computed tomography revealed cutaneous lymphoma limited to the bilateral parietal and occipital scalp.

This patient was initiated on psoralen and ultraviolet A (PUVA) therapy with a starting dose of 1 J/cm2; the dose was increased in increments of 0.5 J until it reached 7.0–7.5 J/cm2. This dose was then maintained along with the administration of topical psoralen. A favorable response to PUVA therapy with reduced erythematous plaques and partial hair growth was demonstrated at 1st- and 2nd-year follow-up [Figure 1]c and [Figure 1]d.

FMF is rare among young adults, especially in those not receiving immunosuppressants.[2] Hodak et al.[3] reported that 36% of their juvenile MF patients exhibited follicular involvement. Keratosis pilaris-like lesions predominantly affect the trunk in juvenile FMF.[1] However, FMF is observed more frequently among adults than among juveniles; hypopigmented MF is commonly seen in pediatric patients. Additional distinguishing features of juvenile FMF relative to adult FMF are the absence of comedo-like plugs, fistulae, or abscesses and the relative absence of pruritus.[3]

One case report of juvenile FMF mimicking tinea capitis was reported in an 8-year-old child who received liver transplantation at the age of 6 years for biliary atresia. He presented with hair loss with hyperkeratotic follicular papules and several black dots for 3 months.[2] He was started on topical clobetasol and tazarotene, and he received excimer laser therapy of the scalp with complete remission and partial hair regrowth.

Treatment for juvenile FMF usually mirrors that for adult FMF, and it can be challenging. Favorable responses to topical steroids and PUVA phototherapy have only been observed for early-stage FMF. For advanced-stage FMF, local radiotherapy combined with PUVA and further systemic therapeutic options should be considered.[1] Compared with adult FMF, early-stage juvenile FMF exhibited an excellent response to PUVA monotherapy in a case series of 50 patients.[3]

The prognosis of FMF has previously been considered poor.[4] However, juvenile FMF has exhibited a more indolent course than adult FMF.[3],[5] Hodak et al.[4] proposed a subclassification system to identify distinct prognoses between indolent/early and aggressive/advanced variants of FMF, with 5-year-survival rates of 92% and 55%, respectively. Our patient received a classification of T1N0M0B0 based on the tumor-node-metastasis-blood staging system. There are still some risk factors associated with a poor prognosis such as head-and-neck region involvement, concomitant bacterial infection, and dense atypical lymphocytes infiltration. After careful evaluation, PUVA was chosen as the primary treatment modality, and favorable treatment response was noted at long-term follow-up.

In conclusion, compared with adult FMF, juvenile FMF may more often present with follicular involvement and a tinea capitis-like presentation. Thus, juvenile FMF should be considered in patients presenting with alopecia with black dots resembling tinea capitis refractory to antifungal treatment. Early-stage juvenile FMF has a more favorable prognosis than adult FMF, with PUVA as the first-line therapeutic option.

Declaration of patient consent

The patient gave his consent for his images and other clinical information to be reported in the journal. The patient understands that his name will not be published but that his anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

Dr. Pei-Lun Sun, an editorial board member at Dermatologica Sinica, had no role in the peer review process or decision to publish this article. The other authors declared no conflicts of interest in writing this paper.



 
  References Top

1.
Mitteldorf C, Stadler R, Sander CA, Kempf W. Folliculotropic mycosis fungoides. J Dtsch Dermatol Ges 2018;16:543-57.  Back to cited text no. 1
    
2.
Wang RF, Sokumbi O, Chiu YE. Folliculotropic mycosis fungoides in a pediatric patient mimicking black dot tinea capitis. Pediatr Dermatol 2019;36:386-7.  Back to cited text no. 2
    
3.
Hodak E, Amitay-Laish I, Feinmesser M, Davidovici B, David M, Zvulunov A, et al. Juvenile mycosis fungoides: Cutaneous T-cell lymphoma with frequent follicular involvement. J Am Acad Dermatol 2014;70:993-1001.  Back to cited text no. 3
    
4.
Hodak E, Amitay-Laish I, Atzmony L, Prag-Naveh H, Yanichkin N, Barzilai A, et al. New insights into folliculotropic mycosis fungoides (FMF): A single-center experience. J Am Acad Dermatol 2016;75:347-55.  Back to cited text no. 4
    
5.
Wain EM, Orchard GE, Whittaker SJ, Spittle MS, Russell-Jones R. Outcome in 34 patients with juvenile-onset mycosis fungoides: A clinical, immunophenotypic, and molecular study. Cancer 2003;98:2282-90.  Back to cited text no. 5
    


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