Dermatology: Practical and Conceptual


Letter  |  Dermatol Pract Concept 2018;8(2):10 123

DERMATOLOGY PRACTICAL & CONCEPTUAL
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Mycosis fungoides (MF), the most frequent primary cutane-

ous lymphoma, belongs to the group of extranodal non-

Hodgkin lymphomas with a mean age at onset of >40 years 

[1]. The manifestation of MF in children is rare and has only 

been studied by a number of case series [2].

We report on a 15-year-old female adolescent presenting 

with pruritic eczematous lesions of the neck and trunk that 

had persisted for one year (Figure 1A). Clinical examination 

revealed erythematous, oval-shaped to polycyclic, eczematous 

macules and plaques with a cigarette paper-like cutaneous 

atrophy and moderate scaling (Figure 1A-D). The histo-

pathological as well as immunohistochemical investigations 

included stains for hematoxylin-eosin, CD3, CD4, CD5, CD8, 

CD20, and CD30, which showed a dense infiltrate of mainly 

CD3 and CD4 positive atypical lymphocytes with epidermal 

exocytosis forming Pautrier’s microabscesses (Figure 1E-G). 

Expression of CD5 as a marker of potential loss of differen-

tiation was retained. Expectedly, flow cytometry analysis of 

peripheral blood cells showed results within normal limits 

(36% CD3-positive lymphocytes, CD4:CD8 ratio of 1.5). At 

the molecular level, a T-cell-receptor (TCR) gene rearrange-

ment analysis from lesional skin showed two monoclonal 

gene rearrangements, agreeing well with the clinical and 

histopathological diagnosis of stage IA MF. A topical treat-

ment with 0.1% mometasone furoate cream was initiated and 

achieved a partial response at the time of the first follow-up 

examination (6-week interval).

MF is the most common primary cutaneous T-cell lym-

phoma. However, in children and adolescents MF is very rare 

with an incidence of 0.05 new cases per year per 100,000 

[3]. While in adults the female to male ratio was reported 

to be 1:2, Nanda et al described a 1:1 ratio in children and 

adolescents [4]. The difficulties in diagnosing early stage MF 

in children arise from the multitude of differential diagnoses 

with similar clinical morphology but much higher incidences 

in this specific age group. In MF three stages may be differ-

entiated clinically. The patch stage presents with eczematous 

skin lesions, moderate desquamation, cutaneous atrophy, and 

predilection for non-sun-exposed skin areas [5]. In children 

such lesions are often erroneously diagnosed as (atopic) 

eczema, dermatophyte infection, or early onset psoriasis 

vulgaris [2]. Therefore, diagnosis is often delayed until the 

patches evolve into infiltrative plaques (plaque stage) or 

tumors (tumor stage), with all three types of skin patholo-

Mycosis fungoides in a 15-year-old adolescent
Sarah Estelmann1, Anna Neuberger1, Ferdinand Toberer1, Christine Fink1,  

Alexander Enk1, Holger A. Haenssle1

1 Department of Dermatology, Venereology, and Allergology; University Medical Center, Ruprecht-Karls University, Heidelberg, Germany

Key words: mycosis fungoides, childhood, adolescent

Citation: Estelmann S, Neuberger A, Toberer F, Fink C, Enk A, Haenssle HA. Mycosis fungoides in a 15-year-old adolescent. Dermatol 
Pract Concept. 2018;8(2):123-125. DOI: https://doi.org/10.5826/dpc.0802a10

Received: November 12, 2017; Accepted: December 12, 2017; Published: April 30, 2018

Copyright: ©2018 Estelmann et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, 
which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Funding: None.

Competing interests: The authors have no conflicts of interest to disclose.

All authors have contributed significantly to this publication.

Corresponding author: Holger A. Haenssle, MD, Department of Dermatology, Venereology and Allergology, University Medical Center, 
Ruprecht-Karls University, Im Neuenheimer Feld 440, 69120 Heidelberg, Germany. Tel. +49-6221-56-39555; Fax. +49-6221-56-7771. 
Email: Holger.Haenssle@med.uni-heidelberg.de



124 Letter  |  Dermatol Pract Concept 2018;8(2):10

Figure 1. Clinical images and histopathological examination of patches and plaques in a 15-year-old girl with mycosis fungoides. The over-

view image shows disseminated, bizarrely shaped, erythematous lesions on the back (A). Close-up images reveal more infiltrated erythema-

tous plaques with scaling (B) and patches with epidermal atrophy, sharply demarcated borders and moderate scaling (C, D). Hematoxylin and 

eosin staining (E) reveals psoriasiform epidermal hyperplasia and a superficial band-like lymphocytic infiltrate. Some of the atypical lympho-

cytes are present within the epidermis (original magnification x 100). At higher magnification typical Pautrier microabscesses show atypical 

lymphocytes with hyperchromatic and irregular nuclei (F, original magnification x 630). Immunostaining shows positivity for CD3 marker 

in epidermotropic, intraepidermal T lymphocytes (G, original magnification x 200). [Copyright: ©2018 Estelmann et al.]



Letter  |  Dermatol Pract Concept 2018;8(2):10 125

ally add up to a relevant increase of the risk of UV-induced 

skin cancers [3].

References

 1. Trautinger F, Eder J, Assaf C, et al. European Organisation for 

Research and Treatment of Cancer consensus recommendations 

for the treatment of mycosis fungoides/Sézary syndrome—Up-

date 2017. Eur J Cancer. 2017;77:57-74.

 2. Boulos S, Vaid R, Aladily TN, Ivan DS, Talpur R, Duvic M. Clini-

cal presentation, immunopathology, and treatment of juvenile-

onset mycosis fungoides: a case series of 34 patients. J Am Acad 

Dermatol. 2014;71(6):1117-1126.

 3. Ai WZ, Keegan TH, Press DJ, et al. Outcomes after diagnosis of 

mycosis fungoides and Sézary syndrome before 30 years of age: a 

population-based study. JAMA Dermatol. 2014;150(7):709-715.

 4. Nanda A, Al-Ajmi H. Mycosis fungoides in children and adoles-

cents. Expert Rev Dermatol. 2013;8(3):309-320.

 5. Girardi M, Heald PW, Wilson LD. The pathogenesis of mycosis 

fungoides. N Engl J Med. 2004;350(19):1978-1988.

 6. Jawed SI, Myskowski PL, Horwitz S, Moskowitz A, Querfeld 

C. Primary cutaneous T-cell lymphoma (mycosis fungoides and 

Sézary syndrome): part I. Diagnosis: clinical and histopathologic 

features and new molecular and biologic markers. J Am Acad 

Dermatol. 2014;70(2):205-216.

 7. Stadler R, Assaf C, Klemke CD, et al. Brief S2k guidelines—cuta-

neous lymphomas. J Dtsch Dermatol Ges. 2013;11 Suppl 3:19-

30.

 8. Wain EM, Orchard GE, Whittaker SJ, Spittle MF, Russell-Jones 

R.. Outcome in 34 patients with juvenile-onset mycosis fungoi-

des: a clinical, immunophenotypic, and molecular study. Cancer. 

2003;98(10):2282-2290.

 9. Ceppi F, Pope E, Ngan B, et  al. Primary cutaneous lym-

phomas in children and adolescents. Pediatr Blood Cancer. 

2016;63(11):1886-1894.

10. Laws PM, Shear NH, Pope E. Childhood mycosis fungoides: 

experience of 28 patients and response to phototherapy. Pediatr 

Dermatol. 2014;31(4):459-464.

gies possibly existing simultaneously [6]. In contrast to most 

adult cases of MF that show a predominance of CD4-positive 

pathologic T-cells, many pediatric cases (approximately 50%) 

are characterized by CD8-positive epidermotropic infiltrates 

[2]. While a number of relevant molecular mechanisms for the 

manifestation of MF were reported [5] the exact pathogenesis 

of MF remains unknown.

Many of the affected children are diagnosed at early stages 

of the disease (stage IA: 50%, stage IB: 47%) [7,8]; however, 

a mean time interval from first symptoms until a final diag-

nosis of five years was reported [8]. The prognosis of MF in 

children and adolescents is more favorable than in adults, 

with 5-year and 10-year survival rates of 95% and 93%, 

respectively [3,8]. Skin lesions of the patient presented as 

erythematous macules and plaques. In contrast, other authors 

described a high frequency of hypopigmented MF lesions in 

children often associated with a predominance of CD8-posi-

tive atypical T-cells [8] and a non-Caucasian skin phenotype 

[4]. The literature is somewhat discordant in terms of TCR 

clonality when assessed in skin biopsies. While Wain et al 

[8] described monoclonality of TCR genes in 26 of 34 cases 

(76.5%), Ceppi et al [9] reported of only 3 out of 14 cases 

(21.4%). There are no specific guidelines for the therapy of 

juvenile MF. Recommendations for first-line treatment of MF 

stages IA, IB, and IIA are either emollients plus observation 

only or, as used in our case, topical corticosteroids. In refrac-

tory cases, topical corticosteroids may be combined with 

phototherapy (broadband/narrowband ultraviolet B [UVB], 

psoralen and ultraviolet A [PUVA])[1]. Most authors prefer 

narrowband UVB to broadband UVB or PUVA because of its 

decreased carcinogenic potential and less side effects [1,10]. 

As for any phototherapy in children, it should be kept in mind 

that MF is a chronic disease with slow progression. Repeated 

phototherapies along the course of the disease may eventu-