Folliculotropic Mycosis Fungoides with Comedonal-like Appearances

AUTHORS

Azita Nikoo 1 , Mahtab Rahbar ORCID 1 , *

1 Department of Dermatopathology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran

How to Cite: Nikoo A, Rahbar M. Folliculotropic Mycosis Fungoides with Comedonal-like Appearances, Iran Red Crescent Med J. 2016 ; 18(12):e16542. doi: 10.5812/ircmj.16542.

ARTICLE INFORMATION

Iranian Red Crescent Medical Journal: 18 (12); e16542
Published Online: October 17, 2016
Article Type: Case Report
Received: January 2, 2014
Revised: September 19, 2014
Accepted: October 3, 2016
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Abstract

Introduction: Folliculotropic mycosis fungoides (FMF) is a variant of mycosis fungoides characterized by the presence of folliculotropic atypical Tcells infiltrates, often with sparing of the epidermis and preferential involvement of the head and neck. Since the original designation of folliculotropic mycosisfungoides (FMF) is a distinct entity, there has been an increasing appreciation of the broad clinical and histopathology spectrum, which can present in this disease.

Case Presentation: In July 2013, a 37-year-old male from Tehran (Iran) who had complained of 9 months of progressive erythematous follicular patches and plaques on the trunk and comedones and acne like lesions on his forehead has been referred to us. The histopathology study showed perifollicular and intrafollicular lymphoid cells infiltration predominantly. There was no histopathology evidence of dermal mucin deposit (PAS negative) and alopeciaor syringotropism T cell infiltration. The immunohistochemical analysis was positive for CD3 and D4. The patients received different treatments based on the stage of their disease.

Conclusions: FMF should be considered in patients who present with alopecia, acne like lesions, cysts and comedones in older ages with a spectrum of histologic changes. We emphasize for any time it should be under-recognized.

Keywords

Mycosis Fungoides Hair Follicle Folliculotropic T-cell Lymphocytosis

Copyright © 2016, Iranian Red Crescent Medical Journal. This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License (http://creativecommons.org/licenses/by-nc/4.0/) which permits copy and redistribute the material just in noncommercial usages, provided the original work is properly cited.

1. Case Presentation

A 37-year-old male patient has been presented with 9 months of history of progressive erythematous patches andplaques on his trunk that was followed by an exophytic crusted nodule with comedonal changes on his forehead and acne-like lesions on his forehead and trunk (Figure 1).

The Patient with Erythematous Patches, Plaques and Acne form Lesions on Trunk with Comedonal-Like Lesion on Forehead Was Consistent with Folliculotropic Mycosis Fungoides IIA. (Original Magnification x80)
Figure 1. The Patient with Erythematous Patches, Plaques and Acne form Lesions on Trunk with Comedonal-Like Lesion on Forehead Was Consistent with Folliculotropic Mycosis Fungoides IIA. (Original Magnification x80)

No other cutaneous involvement was observed. Accompanied with these skin lesions, physical examination of cervical and axillary lymphadenopathy have been noted. Histopathology study of the initial skin biopsy of forehead lesion (dated 2013) showed marked epidermal and follicular hyperplasia. The follicles were distorted and their infundibulum were dilated and filled by laminated keratin. All follicles were surrounded and infiltrated by atypical lymphocytes admixed with eosinophils, plasma cells and scattered mast cells (Figure 2).

Skin Biopsy Shows Epidermal and Follicular Hyperplasia with Dilated Follicles that Filled by Laminated Keratin (Comedonal-like in Center) with Epidermotropism of Atypical Lymphocytes and Perifollicular and Intrafollicular Atypical Lymphoid Cells Infiltration. (Original Magnification x80)
Figure 2. Skin Biopsy Shows Epidermal and Follicular Hyperplasia with Dilated Follicles that Filled by Laminated Keratin (Comedonal-like in Center) with Epidermotropism of Atypical Lymphocytes and Perifollicular and Intrafollicular Atypical Lymphoid Cells Infiltration. (Original Magnification x80)

Most skin lesions were in the plaque stage of mycosis fungoides (MF). There was no evidence of mucin deposit in dermis (alcian blue staining, negative), alopecia or syringotropism of T Cells. The original biopsy showed a population of atypical, epidermotropic CD3-positive lymphocytes, positive for CD4 and negative for CD8, which were more aggregated around of hair follicles (Figure 3). The lymph node biopsy showed lymphomatous follicular hyperplasia without infiltration of atypical lymphocytes. The patient was diagnosed with folliculotropic mycosis fungoides stage IIA (T2N0M0).

Immunohistochemical Stains Lymphocytes to be CD4-Positive T Cells. (Original Magnification x80)
Figure 3. Immunohistochemical Stains Lymphocytes to be CD4-Positive T Cells. (Original Magnification x80)

According to world health organization (WHO) and European organization for research and treatment of cancer classification (EORTC) and revised by WHO in 2008, our patient was in Indolent (low-grade/slow growing) clinical behavior.

2. Discussion

Folliculotropic mycosis fungoides (FMF) is a variant of cutaneous T cell lymphoma (CTCL) (1). However, FMF is a distinct entity in the current world health organization-European organization for research and treatment of cancer (WHO-EORTC) consensus classification of cutaneous Lymphomas according to its unique clinical, prognostic and histopathological features (2). FMF is more common in men. Clinically, presentations of the disease include follicular erythematous papules, plaques, cysts and comedone-like lesions and sometimes tumors (3). These lesions are often accompanied by alopecia (4). Most patients experience moderate-to-severe pruritus (5) and in some cases, follicular mucinosis is also present (2). FMF lesions often involve the head and neck (1, 5), in addition to trunk and extremities Gerami et al. has described five characteristic patterns in FMF. They are: 1) basaloidfolliculolymphoid hyperplasia with folliculotropism, 2) granulomatous dermatitis with folliculotropism, 3) eosinophilic folliculitis-like picture with folliculotropism, 4) dilated follicular cysts with folliculotropism (which we did find in our study) and 5) prototypical FMF with / without follicular mucinosis. Multiple patterns can be seen in a single biopsy (1).

In our study, histological patterns were variable because of different clinical features. Our skin biopsy showed significant epidermal and follicular hyperplasia. In dermis, follicles were distorted, sometimes laminated keratin filled the dilated follicular infundibulum, perifollicular and perivascular to diffuse dermal infiltrations with medium-sized to large atypical T cells (rare interfollicular epidermotropism) admixed with eosinophils. Plasma cells and scattered mast cells were noted. Follicular mucinosis is also not present. On immunohistochemistry, FMF comprises of CD3+, CD4+ T-lymphocytes. There is an elevated CD4:CD8 ratio within the follicular infiltrate. This may also be seen in inflammatory disorders, albeit to a lesser extent. Therefore, it is not a conclusive test and has not been performed in our cases (4). FMF is believed to have a worse prognosis (2). Vand Doorn et al. recommended considering and treating FMF patients as a tumor-stage disease regardless of clinical appearance because of the perifollicular localization of the dermal infiltrate (6).

We did not find any reports in Iranian literature, but it could be the fact that FMF is under-recognized, both by dermatologists and pathologists, especially in the early / patch stage. In this report we have attempted to establish this one required on average the greatest number of biopsies to make the diagnosis. Sometimes follicular epithelium is destroyed and only mixed interstitial inflammatory cells are replaced in the adjacent reticular dermis, which makes the diagnosis difficult when it’s only based on one biopsy.

3. Conclusion

FMF is a distinct entity of CTCL. This diagnosis should be considered in older patients who are presented with alopecia, acne like lesions, cysts and comedones. FMF may be presented with a spectrum of histology changes. We emphasize on any time it is under-recognized. However, the interesting point in this case report is different histopathological differences between FMF and conventional type of MF, which can make a difference in planning Therapeutic strategy.

Acknowledgements

Footnote

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