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Table of Contents
ORIGINAL ARTICLES
Year : 2022  |  Volume : 16  |  Issue : 1  |  Page : 7-11

Mycosis fungoides in children and adolescents: A clinicopathological study in Jordan, Middle East


Mu’tah University, Amman, Mu’tah, Jordan

Date of Submission24-Sep-2021
Date of Decision19-Nov-2021
Date of Acceptance16-Jan-2022
Date of Web Publication09-Mar-2022

Correspondence Address:
Dr. Awad H Al-Tarawneh
Mu’tah University, Amman, Mu’tah
Jordan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/tjd.tjd_114_21

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  Abstract 

Background: Mycosis fungoides usually affects adults but rarely occurs in children and adolescents with a deceptive clinical picture that simulates more common skin diseases at this age; therefore, the diagnosis can be delayed. Objective: To determine the clinical and histopathological features in a group of patients who developed mycosis fungoides during childhood and adolescence to share experience and to highlight the early diagnosis of mycosis fungoides in this age group. Materials and Methods: A retrospective study was performed, and the clinical and histopathological data for all children and adolescent patients with confirmed mycosis fungoides diagnosis for the last five years were retrieved, reviewed, and analyzed. Results: Seven patients were diagnosed with mycosis fungoides with an age ranging from 5 to 17 (mean age, 10) years, comprising five males and two females patients, with a male-to-female ratio of 2.5:1. Three clinical variants of mycosis fungoides were present in our cases: hypopigmented mycosis fungoides in four patients (57%), poikilodermatous mycosis fungoides in two (29%), and classical mycosis fungoides in one (14%). No more than one variant of mycosis fungoides was observed in any patient. Conclusion: Although mycosis fungoides rarely occurs in children and adolescents, sufficient clinical and histopathological features are required to make the diagnosis. Therefore, it should always be considered in our clinical differential diagnosis in any appropriate clinical setting. A skin biopsy should not be delayed. Study Design: Retrospective study.

Keywords: Adolescents, children, clinicopathological, mycosis fungoides


How to cite this article:
Al-Tarawneh AH. Mycosis fungoides in children and adolescents: A clinicopathological study in Jordan, Middle East. Turk J Dermatol 2022;16:7-11

How to cite this URL:
Al-Tarawneh AH. Mycosis fungoides in children and adolescents: A clinicopathological study in Jordan, Middle East. Turk J Dermatol [serial online] 2022 [cited 2022 May 22];16:7-11. Available from: https://www.tjdonline.org/text.asp?2022/16/1/7/339255




  Introduction Top


Mycosis fungoides is the most common subtype of primary T-cell cutaneous lymphoma usually affecting older adults with an incidence of 5.6 per million people with male predominance.[1],[2] Primary cutaneous T-cell lymphoma and mycosis fungoides rarely occur in children and adolescents, but the latter is the most common clinical presentation of the former in this age group.[3] The incidence of mycosis fungoides in children and adolescents varies in different geographical areas, with a higher incidence in Asia than in Western countries, according to different studies.[4],[5],[6] In this study, we will add and share our experiences to add more spotlight to this important subject. The clinical and histopathological features of our cases are presented and analyzed.


  Materials and Methods Top


This was a retrospective study. All patients younger than 18 years with a confirmed diagnosis of mycosis fungoides during our practice at Jordan University Hospital in Amman, Jordan, Al-Karak Governmental Hospital in Al-Karak, Jordan, and our private practice in Amman, Jordan, for the last five years from 2016 to 2021 were included in our study. The clinical and pathological data of all patients diagnosed with mycosis fungoides were retrieved, reviewed, and analyzed. The diagnosis of mycosis fungoides was made according to the International Society for Cutaneous Lymphomas – European Organization of Research and Treatment of Cancer criteria[7] with clinicopathological correlation. Skin biopsies of all patients were interpreted by a dermatopathologist. Due to the presence of sufficient diagnostic criteria for the diagnosis of mycosis fungoides based on unequivocal histopathological findings of the skin biopsy and clinical findings (the presence of more than two clinical criteria) in all our cases, T-cell markers CD4, CD8, CD5, and CD7 were evaluated in only a few cases (three cases with hypopigmented mycosis fungoides and one case with classical mycosis fungoides). A clonal T-cell receptor gene rearrangement analysis was not performed.


  Results Top


A total of seven patients aged <18 years were diagnosed with mycosis fungoides from 2016 to 2021, with ages ranging from 5 to 17 (mean age 10) years, comprising five men and two women with a male-to-female ratio of 2.5: 1. Three variants of mycosis fungoides were present in our study: hypopigmented mycosis fungoides (four patients), accounting for 57% of our cases [Figure 1]; poikilodermatous mycosis fungoides (two patients) [Figure 2], and classical mycosis fungoides (one patient) [Figure 3]. No more than one variant of mycosis fungoides was observed in any of our cases. The mean time before diagnosis was 17 months. All our cases were in the early stages of the disease at the time of diagnosis, Stage IA and Stage IB according to physical examination and imaging studies. [Table 1] shows the clinical characteristics of our patients.
Figure 1: Hypopigmented patch on the buttock of a five-years-old female patient with hypopigmented mycosis fungoides

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Figure 2: Poikilodermatous patch on the forearm of a 14-years-old female with poikilodermatous mycosis fungoides

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Figure 3: Erythematous scaly patch over the trunk in a 17-years-old male with classical mycosis fungoides

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Table 1: Clinical characteristics of patients in the study

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All cases showed unequivocal diagnostic histopathological features, including lymphocytic atypia, epidermotropism, haloed lymphocytes, disproportional spongiosis, and lymphocytic infiltrate, which were superficial perivascular in most cases with hypopigmented mycosis fungoides [Figure 4]; lichenoid with interface changes in cases with poikilodermatous mycosis fungoides [Figure 5]; and band-like features in the case of classical mycosis fungoides [Figure 6]. In a few cases, T-cell markers were used. The CD-8 phenotype was predominant in all three cases with hypopigmented mycosis fungoides with loss of pan-T-cell markers CD5 and CD7. The CD4 phenotype was predominant in classical mycosis fungoides. [Table 2] shows the histopathological features of all patients.
Figure 4: Hypopigmented mycosis fungoides: Mild superficial perivascular lymphoid cell infiltrate with prominent epidermotropism of a typical lymphocyte, haloed lymphocyte, and disproportional spongoisis (HE x400).

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Figure 5: Piokilodermatus mycosis fungoides: Hperkeratosis, epidermal atrophy, lichenoid lymphocytic inflammatory infiltrate, vacuolar changes epidermotropism of atypical lymphocytes, haloed lymphocyte, disproportional spongiosis, and lining up of atypical lymphocytes at the dermo epidermal junction (HE x400).

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Figure 6: Classical mycosis fungoidis: Parakeratosis, hyperkeratosis, epidermal hyperplasia, epidermotropism of atypical lymphocytes, disproportional spongoisis, band like lymphoid cell infiltrate, and papillary dermal fibrosis (HE x100).

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Table 2: Histopathological features of patients in the study

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  Discussion Top


Several reports of mycosis fungoides in children and adolescents have been published in the literature, indicating that mycosis fungoides is not rare in this age as it was previously thought.[8] Mycosis fungoides in children and adolescents account for approximately 4%–5% of cases of mycosis fungoides in the United States.[9] A higher prevalence of mycosis fungoides in this age group has been reported in Asia, with 16.6% of patients with mycosis fungoidesin children and adolescents in Kuwait,[5] and 11% of patients with mycosis fungoides were children and adolescents in Singapore.[10] In Jordan, we do not have scientific statistics about the prevalence of mycosis fungoides in this age group, and we cannot determine its prevalence based on this study alone, but based on the total number of all cases of mycosis fungoides that we have seen during the period of this study, cases of mycosis fungoides in children and adolescents represent around 14% of them which is similar that in Asia.[5],[10] The clinical presentation of mycosis fungoides in this age group can be similar to that of different inflammatory dermatoses, such as eczema, vitiligo, pityriasis alba, and fungal infections. In our study, the initial clinical diagnosis was eczema in three patients, vitiligo in two, and hypopigmented mycosis fungoides in addition to pityriasis alba and tinea versicolor included in the clinical differential diagnosis in the other three. These clinical presentations concur with those of other studies.[4],[11] Because physicians sometimes avoid skin biopsies in children, the diagnosis can be delayed. The mean time before diagnosis was 17 months. Due to the increasing awareness of hypopigmented mycosis fungoides, it was recently presented in the clinical differential diagnosis in three of our cases. Hypopigmented mycosis fungoides is the predominant variant of mycosis fungoides in our cases, which concurs with that of other studies.[4],[5],[6],[8],[11],[12] The hypopigmented and poikilodermatous variants of mycosis fungoides were often found in children and adolescents,[8] and a similar finding was observed in our cases [Table 3]. In our study, a male predominance was observed, which was also documented in other studies.[6],[8],[11] All our cases were in the early disease stages at the time of diagnosis (Stage IA and Stage IB, which were also consistent with those in other studies).[4],[6],[8],[13],[14]
Table 3: Mycosis fungoides variants in the study n = 7 (100%)

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Routine histopathological findings of lymphocytic atypia, epidermotropism, haloed lymphocytes, and disproportional spongiosis were present in all cases [Table 2], similar histopathological findings have been reported.[6],[12],[15] Recent studies have shown that the predominant T-cell phenotype in hypopigmented mycosis fungoides is commonly CD8.[16] Our cases with hypopigmented mycosis fungoides also showed a predominant CD8 T-cell phenotype. Loss of T-cell markers CD3, CD5, and CD7 is considered a helpful diagnostic immunopathologic feature to diagnose mycosis fungoides.[17] In all four cases in which these T-cell markers were performed, at least one of these markers was lost.


  Conclusion Top


Mycosis fungoides is not as rare as it was thought to be. Therefore, it must be considered as a differential diagnosis in children and adolescents. We should not hesitate to perform a skin biopsy if clinical suspicion exists. Clinical, histopathological, and immunopathological features should be determined for the early diagnosis of mycosis fungoides.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Willemze R, Jaffe ES, Burg G, Cerroni L, Berti E, Swerdlow SH, et al. WHO-EORTC classification for cutaneous lymphomas. Blood 2005;105:3768-85.  Back to cited text no. 1
    
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Korgavkar K, Xiong M, Weinstock M. Changing incidence trends of cutaneous T-cell lymphoma. JAMA Dermatol 2013;149:1295-9.  Back to cited text no. 2
    
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Fink-Puches R, Chott A, Ardigo M, Simonitsch I, Ferrara G, Kerl H, et al. The spectrum of mycosis fungoides in patients less than 20 years of age. Pediatric Dermatol 2004;21:525-33.  Back to cited text no. 3
    
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Wu JH, Cohen BA, Sweren RJ. Mycosis fungoides in pediatric patients: Clinical features, diagnostic challenges and advances in therapeutic management. Pediatr Dermatol2020;:18-28.  Back to cited text no. 4
    
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Nanda A, Qasem A, Al-Ajmi H, Al-Sabah H, Elkashlan M, Al-Shemari S, et al. Mycpsis fungoides in Arab children and adolescent: A report of 36 patients from Kuwait. Pediatr Dermatol 2010;27:607-13.  Back to cited text no. 5
    
6.
Heng YK, AanKoh MJ, Giam YC, Tang MBY, Chong WS, Tan SH, et al. Pediatric mycosis fungoides in Singapore: A series of 46 children. Pediatric Dermatol2014;31:477-82.  Back to cited text no. 6
    
7.
Olsen E, Vonderheid E, Pimpinelli N, Willemze R, Kim Y, Knobler R, et al; ISCL/EORTC. Revisions to the staging and classification of mycosis fungoides and sezary syndrome: A proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC). Blood 2007;110:1713-22.  Back to cited text no. 7
    
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Koch SE, Zackheim HS, Williams ML, Fletcher V, LeBoit PE. Mycosis fungoides beginning in childhood and adolescence. J Am Acad Dermatol 1987;17:563-70.  Back to cited text no. 8
    
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Nada A, Al-Ajmi H. Mycosis fungoides in children and adolescents. Expert Rev Dermatol 2013;8:309-20.  Back to cited text no. 9
    
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Tan E, Tay YK, Giam YC. Profile and outcome of childhood mycosis fungoides in Singapore. Pediatr Dermatol 2000;17:352-6.  Back to cited text no. 10
    
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Boulos S, Vaid R, Aladily TN, Ivan DS, Talpur R, Davic M. Clinical presentation, immunopathology, and treatment of juvenile-onset mycosis fungoidees: A case series of 34 patients. J Am Acad Dermatol 2014;71:1117-26. dio:10.1016|j.jaad.2014.07.049.Epub 2014 Sep 26  Back to cited text no. 11
    
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Pope E, Weitzman S, Ngan B, Walsh S, Morel K, Williams J, et al. Mycosis fungoides in the pediatric population: Report from an International Childhood Registryof Cutaneous lymphoma. J Cutan Med Surg 2010;14910:1-6. dio;10.2310/7750.2009.08091.PMID:20128983  Back to cited text no. 12
    
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Wain EM, Orchard GE, Whittaker SJ, Spittle MF, Russell-Jones R. Outcome in 34 patients with juvenile-onset mycosis fungoides: A clinical, immunophenotypic and molecular study. Cancer 2003;98:2282-90. Dio;10.1002/cncr.11780.PMID: 14601100.  Back to cited text no. 13
    
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Alsuwaidan SN. Childhood mycosis fungoides: New observation from the Middle East. J Saudi Soc Dermatol Dermatol Surg 2012;16:5-6.  Back to cited text no. 14
    
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Al-tarawneh AH. Clinical and histopathological spectrum of mycosis fungoides. Bahrain Med Bull 2018;40:103-7.  Back to cited text no. 15
    
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El-Shabrawi-Caelen L, Cerroni L, Mederios LJ, McCalmont TH. Hypopigmented mycosis fungoides: Frequent expression of CD8 T-Cell phenotype. Am J Surg Pathol 2002;26:450-7.  Back to cited text no. 16
    
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Pimpinelli N, Olsen EA, Santucci M, Vonderheid E, Haeffner AC, Stevens S, et al; International Society for Cutaneous Lymphoma. Defining early mycosis fungoides. J Am Acad Dermatol 2005;53:1053-63.  Back to cited text no. 17
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]
 
 
    Tables

  [Table 1], [Table 2], [Table 3]



 

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