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\begin{document}

\title{Folliculotropic mycosis fungoides associated with follicular mucinosis:
a case report and minireview}



\author[1]{Majed Aldayhum}%
\author[1]{Mohammed Alshahrani}%
\author[2]{Mahmoud A. Hussein}%
\author[3]{Abdulmajeed Alshahrani}%
\affil[1]{Armed Forces Hospitals Southern Region}%
\affil[2]{Assiut University Faculty of Medicine}%
\affil[3]{Najran University College of Medicine}%


\vspace{-1em}



  
  \date{June 18, 2024}


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\textbf{INTRODUCTION}

\textbf{Mycosis fungoides:} Mycosis fungoides (MF) is a primary
cutaneous T-cell lymphoma. It represents the most common type of
extranodal non-Hodgkin lymphomas \textsuperscript{12}. Follicular or
folliculotropic MF (F-MF, also known as follicular T-cell lymphoma,
follicular MF, MF-associated mucinosis, or pleiotropic MF) is an
under-recognized subtype of MF. It is characterized histologically by
folliculotropism (histologically analogous to epidermotropism) with
malignant T cells, with or without associated follicular
mucinosis\textsuperscript{3}. In 2005, The WHO/EORTC considered F-MF as
a variant of MF in their classification\textsuperscript{4-7}.

\textbf{Mycosis fungoides in the Kingdom of Saudi Arabia:} In the
Kingdom of Saudi Arabia, few studies are available about MF and
F-MF\textsuperscript{1,8-12}. AlGhamdi et al examined the
clinicopathologic features of MF in 43 cases of immunohistologically
confirmed MF. The average age was 33.5 years was reviewed. The
male-to-female ratio was 2:1. Most of the cases presented in an
early-stage (I and II) MF. Most of the patients had classic MF and other
subtypes included hypopigmented MF and other variants. The patients were
followed up for a duration of 27.6 months. The follow-up data indicated
that few patients recovered (9.5\%) patients recovered; whereas the
majority (83.3\%) had persistent MF skin lesions. A single patient had
the extracutaneous disease; whereas only two patients died of MF
\textsuperscript{8}.

Al-Dawsari et al examined the surgical pathology records of Saudi
patients from 1995 to 2014 at the Johns Hopkins Aramco Healthcare Center
(Eastern Province of KSA). This medical facility is the healthcare
provider for the employees working at Saudi Aramco company and their
dependents in the Eastern Province of Saudi Arabia. The total number of
primary skin tumors was 204 and out of them 22 cases were
MF\textsuperscript{10}. Albasri et al examined the pattern of skin
cancer in the Madinah region of KSA. Among the 202 cancer cases examined
in their series, there were 14 (6.8\%) cases of MF. The mean age of the
patients was 30.7 years with a male-to-female ratio of 11:3. The
distribution of the MF skin lesions included: 4 cases (head and neck), 3
cases (upper limb), and 7 cases (lower limb) \textsuperscript{11}.

Alsaif et al described the Leser-Trelat sign (sudden appearance of
seborrheic keratosis) in a 60-year-old Saudi male patient with MF. The
lesions appeared on the face and back \textsuperscript{12}. Alojail et
reported the clinical features and treatment regimens in 34 cases of MF
in KSA. The cases included both hyperpigmented (11 cases) and
hypopigmented (21 cases) MF, poikilodermatous MF, and pagetoid
reticulosis (a single, each). The treatment regimens included
phototherapy Narrowband UVB (NB-UVB) in combination with topical
corticosteroids, phototherapy NB-UVB in combination with the topical
corticosteroid, and phototherapy NB-UVB with topical corticosteroid and
systemic acitretin \textsuperscript{1}.

Alghubaywi et al examined all MF cases diagnosed at King Abdulaziz
Medical City in KSA (January 2016 to July 2022). A total of 73 patients
were included in their study. The incidence of MF was slightly higher in
females (male to female ratio: 1: 1.3). The average age was 44 years.
The most common MF subtypes included classic MF, followed by
hypopigmented MF. Most of the cases were presented at an early-stage MF
(IA, IB, and IIA). The treatment option was mainly in the form of
topical steroids. Immunohistological staining of CD4+/CD8+ with CD8
predominance was common and this may reason for the favorable disease
outcome \textsuperscript{13}. These authors also indicated that MF was
under-recognized with 20\% of the patients with MF initially
misdiagnosed as atopic dermatitis. The correct diagnosis of MF was
rendered within a mean of 33 months from the time of presentation to the
Dermatology clinics. The authors reasoned the delay in the diagnosis of
MF the fact that chronic dermatitis and MF share several clinical
manifestations such as the presence of pruritic, erythematous, scaly
plaques \textsuperscript{13}. A summary of the previous studies is shown
in Table 1.

\textbf{Follicular mucinosis:} Follicular mucinosis, i.e. accumulation
of mucin in the epithelium of the hair follicles can be either a primary
idiopathic disorder or an associated event with several inflammatory
conditions (such as chronic spongiotic dermatitis), benign or malignant
neoplasms\textsuperscript{14-16}. In 1957, Pinkus first described mucin
deposits in the follicular epithelium (alopecia mucinosa) that are
associated with folliculotropism, i.e., infiltration of the epithelial
cells of the outer hair sheath by atypical
lymphocytes\textsuperscript{17}. In 1959, the term follicular mucinosis
was introduced by Jablonska \textsuperscript{18}. There are two clinical
types of follicular mucinosis: idiopathic follicular mucinosis and
lymphoma-related follicular mucinosis, i.e., F-MF. The latter can be
separated from the classical MF on a clinical and histological
basis\textsuperscript{2,19,20}. Currently, there is no specific therapy
for idiopathic follicular mucinosis and the skin lesions can resolve
spontaneously within a period of 2 to 24. Some therapeutic options
include steroids (topical, intralesional, and systemic steroids),
photodynamic therapy, antimalarials, interferon, and
dapson\textsuperscript{21,22}.

\textbf{Folliculotropic mycosis fungoides:} F-MF represents the most
common non-classic variant of MF, representing approximately 10\% of the
cases of MF \textsuperscript{23}. It usually shows a male predominance
and most of the cases occur in adults \textsuperscript{24}. Its clinical
findings include follicle-based infiltrated erythematous grouped papules
resembling acne or keratosis pilaris, patches, plaques, and tumor-like
lesions with follicular accentuation \textsuperscript{2,20,25}. Other
clinical presentations include prurigo-like lesions, pseudotumors,
lichen spinulosus-like lesions, rosacea-like lesions, and lupus
tumidus-like plaques \textsuperscript{26}. Pruritis is a common
complaint and it is aggravated by superinfection by staphylococcus auras
resulting in pyoderma \textsuperscript{25}. The lesions of F-MF vary
from solitary lesions to extensive lesions giving the appearance of a
leonine face \textsuperscript{23}. The head and neck region is the most
commonly affected site. Other sites include the trunk, and the
extremities \textsuperscript{17}. The features of F-MF are seen in
nearly 10\% of the patients with MF \textsuperscript{2,20,25}. The
salient histological features of F-MF include perivascular and
peri-adnexal dermal lymphocytic infiltrates with variable infiltration
and destruction of the follicular epithelium by small, medium-sized, or
large-sized lymphocytes with hyperconvoluted nuclei (cerebriform

nuclei). The epidermis is usually spared. Other histological findings
include follicular mucinosis, Pautrier's Micro-abscess, follicular
plugging, tagging of the atypical lymphocytes along the follicular
epithelium, peri-eccrine infiltrate of atypical
lymphocytes\textsuperscript{23,26}. Immunohistochemistry usually reveals
prominent CD3 positive infiltrate, with

a predominance of CD4 positive lymphocytes over rare CD8 positive
T-cells. CD7 expression is reduced or completely lost. Molecular
analysis (T-cell receptor-\selectlanguage{greek}γ \selectlanguage{english}gene rearrangement) reveals a monoclonal
T-cell population\textsuperscript{23,26-28}. The therapeutic options in
F-MF include photochemotherapy combined with interferon alpha-2a and
retinoids. Other options include local radiotherapy, topical imiquimod,
and total body electron beam irradiation \textsuperscript{23}.

Several F-MF case reports were presented in the literature. Monopoli et
al described two adult patients with F-MF. The clinical manifestations
included alopecia and follicular erythematous papules, and comedones and
cysts, respectively. The histological findings included folliculotropic
atypical T-cell infiltrate that extends to the epidermis. There was no
associated mucinosis. Clonality analyses revealed the oligo/monoclonal
nature of the T-cell lymphocytic infiltrate \textsuperscript{27}.

Rajalakshmi et al examined four cases of F-MF affecting the skin of the
face. The mean age of the patients was 17.5 years and there were no
differences in gender distribution. The lesions were in the form of
hypopigmented patches or erythematous plaques. Two patients had
associated alopecia. The salient histological finding included
folliculotropism, mucin deposition around the hair follicles, lymphocyte
tagging with haloes, atypical lymphocytes with
hyperconvoluted/cerebriform nuclei, and infiltration of the eccrine and
sebaceous glands by atypical lymphocytes. Other histological features
included the presence of epithelioid cells, parakeratosis, and a
prominent infiltrate of eosinophils \textsuperscript{29}. Magro et al
reported 6 cases of unilesional follicular MF. All the patients were
males with a mean age of 28 years. The patients were presented with a
solitary lesion on the face and scalp (five patients) and trunk (a
single patient) that persisted for a few months. There were follicular
prominence and alopecia. On histology, there was an atypical
folliculotropic lymphocytic infiltrate associated with follicular
mucinosis. Immunohistology revealed a high CD4: CD8 ratio and loss of
CD7 expression. A single untreated case developed similar lesions over
the skin of the thigh and buttock after 3-4 years \textsuperscript{28}.
F-MF usually occurs in adults with an average age at the time of
diagnosis of 60 years \textsuperscript{30}. F-MF is exceptionally rare
in children and adolescents. Mantri et al reported a case of F-MF in a
16-year-old boy who presented with 6x7 cm plaque on the forehead for a
2-month duration. Immunohistological examination revealed dense atypical
T-cell lymphocytes invading and destroying the hair follicles.
Follicular mucinosis was also seen. Most of the neoplastic cells were
CD4-positive T- T-lymphocytes with scarce CD8-positive T cells around
the hair follicles. Treatment was in the form of a spot electron beam
and the lesion completely resolved within 2 months \textsuperscript{30}.
Interestingly, Emge et al reported a case of F-MF in a 6-year-old boy.
The lymphoma was associated with idiopathic follicular
mucinosis\textsuperscript{31}.

Taken collectively, these previous reports indicated that F-MF is a rare
and aggressive form of MF that has a worse prognosis compared to
conventional MF. It usually affects the head and neck region in adults.
It has distinct histological features and may or may not be associated
with follicular mucinosis. F-MF has several clinical presentations and
many histological faces. Therefore, it is often misdiagnosed, or
diagnosed with much delay at an advanced stage as compared to the
conventional MF.

Herein, we report a case of F-MF in a middle-aged male patient. To date
and to be best of our knowledge, this is the first case of F-MF to be
reported in the Southern region (Asir region) of the KSA. The
clinicopathologic features were discussed.

\textbf{CASE REPORT}

\textbf{Clinical features:} A 52-year-old Saudi male presented to the
Dermatology Clinic at the Armed Forces Hospitals Southern Region, KSA in
2020 with an eight-month history of recurrent itchy scalp lesions. These
skin lesions developed suddenly and increased in size with time. There
was no history of fever, night sweating, weight loss, joint pain, muscle
pain, or loss of appetite. The patient was otherwise medically free with
no history of taking medication or other comorbidities. There was no
family history of similar dermatological problems.

Physical examination revealed multiple well-defined erythematous
telangiectatic infiltrated nodules and plaques associated with hair loss
over the scalp and forehead. There was a poikilodermatous patch over the
skin of the chest. The clinical examination revealed no other
abnormalities. A summary of these findings is shown in Figures 1 and 2.
The clinical impressions included F-MF, follicular lymphomatoid
papulosis, follicular eczema, and pseudolymphomatous folliculitis. All
the laboratory investigations including hematological (complete blood
cell count with differential and peripheral blood) and biochemical
(liver function, urea, electrolyte, lactate dehydrogenase) parameters
were within normal limits. CT scan revealed multiple bilateral cervical
lymph nodes the largest measuring 1.1 x 0.9 cm with preserved fatty
hilum. Although itching was slightly ameliorated with the application of
topical steroids, the lesions did not improve on this treatment. The
patient disappeared and refused any further treatment at our medical
facility.

\textbf{Pathological findings:} A skin 6 mm punch biopsy was obtained.
Sections show deposition of mucin in the follicular epithelium both in
hematoxylin-eosin and PAS/Alcian blue-stained preparations. There was a
dense predominantly perifollicular lymphoid infiltrate. folliculotropism
with infiltration of the mildly hyperplastic follicular epithelium by
atypical small-to-medium-sized lymphocytes infiltrating the follicular
epithelium was seen in all cases. The infiltrate was composed
essentially of lymphocytes with few histiocytes but no eosinophils
(Figure 2-A-F). Infiltration was accompanied by eosinophils and
histiocytes at various rates. There was attenuation of the epidermis
without atypia. There was no epidermotropism. Immunophenotyping revealed
dense dermal (predominantly perifollicular) CD3 and CD4 positive
lymphoid infiltrate (Figure 2-G-H). Occasional CD8-positive dermal T
lymphocytes were seen (CD4: CD8 ratio was more than 7:1) (Figure 2-I).
CD20 showed focal expression in some of the diffusely scattered
lymphocytes. CD30 was negative. A summary of these findings is shown in
Figure 3.

\textbf{DISCUSSION}

MF is the most common type of cutaneous T cell lymphoma accounting for
50\% of all primary cutaneous lymphomas \textsuperscript{4,5}. MF
includes several variants based on the clinical and immunohistological
characteristics \textsuperscript{1} \textsuperscript{32}. Some clinical
variants of MF such as bullous MF and hyperpigmented MF or hypopigmented
MF have clinical behavior similar to that of the classic MF.
Accordingly, they are not considered separately. In contrast, some
variants of MF such as F-MF, pagetoid reticulosis, and granulomatous
slack skin have distinctive clinicopathologic features and therefore are
considered distinct entities \textsuperscript{4,5,29,32}.

F-MF is a very aggressive subtype of MF and usually, the patients need
aggressive interventions in contrast to classic MF \textsuperscript{29}.
Gender distribution in F-MF shows a male-to-female ratio of 3:1 and a
mean age of 55 years at the time of presentation. F-MF is very rare in
adolescents and children with few cases being reported in the literature
to date \textsuperscript{20,30}.

The clinical features of the case reported here concur with the previous
studies \textsuperscript{14,27-29}. Bonta et al reported a case of a
44-year-old male patient with a generalized pruritic eczematous
eruption. The patient developed an aggressive, rapidly progressive F-MF
involving the scalp, eyebrows, and axillae. This was associated with
follicular mucinosis, alopecia, and lymphomatous involvement of the
inguinal lymph node. Histological examination of the skin biopsy
revealed follicular mucinosis, folliculotropism of atypical cells, and
intrafollicular Pautrier's microabscesses \textsuperscript{14}.

The histological differential diagnoses in the case presented here
revolved around follicular lymphomatoid papulosis, follicular eczema,
and pseudolymphomatous folliculitis \textsuperscript{18,33}. Follicular
lymphomatoid papulosis (LyP) is characterized clinically by a ``waxing
and waning'' of the skin lesions and histologically by the presence of
eosinophils and expression of CD30 protein \textsuperscript{18,33}.
Follicular eczema is characterized by prominent spongiosis of the
interfollicular epidermis and the lack of lymphocyte atypia.
Pseudolymphomatous folliculitis that can be drug-induced is separated
from F-MF by the presence of lymphoid follicles with germinal centers,
and folliculotropic infiltrate is composed predominantly of CD3 positive
cells admixed with CD20-positive, S100-positive, and CD1a-positive
cells\textsuperscript{17}. Some authorities in Dermatopathology (Gerami
and Guitart, and Mitteldorf) proposed the presence of five
histomorphologic patterns for F-MF including the ``Prototypic'' pattern
with intact hair follicles, folliculotropism with or without follicular
mucinosis, eosinophilic folliculitis, basaloid folliculolymphoid
hyperplasia with folliculotropism, granulomatous dermatitis associated
with follicular destruction, and follicular cysts with
folliculotropism\textsuperscript{17,24}. In agreement with other
studies, we found the deposition of mucin in the follicular epithelium.
Mucin deposition represents a response of the host follicular epithelium
to injurious agents and can be encountered in several conditions such as
follicular eczema, granulomatous rosacea, Ofuji's disease, contact
dermatitis, F-MF, angiolymphoid hyperplasia with eosinophilia. Taken
together, these pathological conditions were lumped under the
terminology of ''Alopecia mucinosa or Follicular mucinosis''. Some
authorities consider follicular mucinosis as an ''abortive cutaneous
lymphoma'' \textsuperscript{4,529}.

To sum up, F-MF is a rare and under-recognized variant of MF. Its
salient clinicopathologic findings include frequent head and neck
involvement, folliculotropism, nuclear atypia, and mucin deposition in
the follicular epithelium. The prognosis of FTMF is poor compared to
classical MF. The early disease has 10 years survival of 82\% and 42\%
by 15 years. The late disease has a similar prognosis as classical MF
(91\% at both 5 and 10 years) \textsuperscript{30}The diagnosis of F-MF
requires a high index of suspicion both on the part of the dermatologist
and dermatopathologists.

\textbf{Table 1: Previous studies about mycosis fungoides in the Kingdom
of Saudi Arabia}\selectlanguage{english}
\begin{longtable}[]{@{}llllll@{}}
\toprule
\begin{minipage}[b]{0.16\columnwidth}\raggedright\strut
Studies\strut
\end{minipage} & \begin{minipage}[b]{0.16\columnwidth}\raggedright\strut
Location of the study\strut
\end{minipage} & \begin{minipage}[b]{0.16\columnwidth}\raggedright\strut
Number of cases\strut
\end{minipage} & \begin{minipage}[b]{0.16\columnwidth}\raggedright\strut
Mean age (Years)\strut
\end{minipage} & \begin{minipage}[b]{0.16\columnwidth}\raggedright\strut
Male to female ratio\strut
\end{minipage} & \begin{minipage}[b]{0.16\columnwidth}\raggedright\strut
References\strut
\end{minipage}\tabularnewline
\midrule
\endhead
\begin{minipage}[t]{0.14\columnwidth}\raggedright\strut
1\strut
\end{minipage} & \begin{minipage}[t]{0.14\columnwidth}\raggedright\strut
King Khalid University Hospital, Riyadh\strut
\end{minipage} & \begin{minipage}[t]{0.14\columnwidth}\raggedright\strut
43\strut
\end{minipage} & \begin{minipage}[t]{0.14\columnwidth}\raggedright\strut
33.5\strut
\end{minipage} & \begin{minipage}[t]{0.14\columnwidth}\raggedright\strut
2:1\strut
\end{minipage} & \begin{minipage}[t]{0.14\columnwidth}\raggedright\strut
\textsuperscript{8}\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.14\columnwidth}\raggedright\strut
2\strut
\end{minipage} & \begin{minipage}[t]{0.14\columnwidth}\raggedright\strut
Johns Hopkins Aramco Healthcare Center (Eastern Province)\strut
\end{minipage} & \begin{minipage}[t]{0.14\columnwidth}\raggedright\strut
22 cases were MF out of 204 skin cancer cases\strut
\end{minipage} & \begin{minipage}[t]{0.14\columnwidth}\raggedright\strut
-\strut
\end{minipage} & \begin{minipage}[t]{0.14\columnwidth}\raggedright\strut
-\strut
\end{minipage} & \begin{minipage}[t]{0.14\columnwidth}\raggedright\strut
\textsuperscript{10}\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.14\columnwidth}\raggedright\strut
3\strut
\end{minipage} & \begin{minipage}[t]{0.14\columnwidth}\raggedright\strut
The western region of KSA\strut
\end{minipage} & \begin{minipage}[t]{0.14\columnwidth}\raggedright\strut
14 cases of MF out of 202 cases of skin cancer\strut
\end{minipage} & \begin{minipage}[t]{0.14\columnwidth}\raggedright\strut
30.7\strut
\end{minipage} & \begin{minipage}[t]{0.14\columnwidth}\raggedright\strut
11:3\strut
\end{minipage} & \begin{minipage}[t]{0.14\columnwidth}\raggedright\strut
\textsuperscript{11}\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.14\columnwidth}\raggedright\strut
4\strut
\end{minipage} & \begin{minipage}[t]{0.14\columnwidth}\raggedright\strut
College of Medicine, King Saud University, Riyadh\strut
\end{minipage} & \begin{minipage}[t]{0.14\columnwidth}\raggedright\strut
A single case of MF\strut
\end{minipage} & \begin{minipage}[t]{0.14\columnwidth}\raggedright\strut
-\strut
\end{minipage} & \begin{minipage}[t]{0.14\columnwidth}\raggedright\strut
-\strut
\end{minipage} & \begin{minipage}[t]{0.14\columnwidth}\raggedright\strut
\textsuperscript{12}\strut
\end{minipage}\tabularnewline
\begin{minipage}[t]{0.14\columnwidth}\raggedright\strut
5\strut
\end{minipage} & \begin{minipage}[t]{0.14\columnwidth}\raggedright\strut
King Faisal University, Al Ahsa\strut
\end{minipage} & \begin{minipage}[t]{0.14\columnwidth}\raggedright\strut
34 cases\strut
\end{minipage} & \begin{minipage}[t]{0.14\columnwidth}\raggedright\strut
44\strut
\end{minipage} & \begin{minipage}[t]{0.14\columnwidth}\raggedright\strut
1: 1.3\strut
\end{minipage} & \begin{minipage}[t]{0.14\columnwidth}\raggedright\strut
\textsuperscript{1}\strut
\end{minipage}\tabularnewline
\bottomrule
\end{longtable}

\textbf{Figure and table legends}

\textbf{Table 1: Previous studies about mycosis fungoides in the Kingdom
of Saudi Arabia}

\textbf{Figure 1: The clinical findings of folliculotropic mycosis
fungoides of the scalp}

(A)- This picture is showing well-defined erythematous non-scally
infiltrated plaque over the forehead with poikilodermatous changes.

(B-C)- This side-picture of the face of the patient shows multiple
well-defined erythematous non-scaly alopecic patches over the temporal
side of the scalp.

(D-E)- This picture of the scalp of the patient shows multiple
erythematous patches with poikilodermatous changes over the vertex.

(F) This picture of the upper chest of the patient shows
poikilodermatous changes.

\textbf{Figure 2:} \textbf{The clinical findings of folliculotropic
mycosis fungoides of the scalp and chest}

(A-C) These are dermoscopic pictures of the skin lesion of the patient
showing areas of hypopigmentation, hyperpigmentation telangiectasia, and
hypotrophy, and are associated with thinning of terminal hair.

(D) This dermoscopic picture shows the comparison of affected skin and
normal skin.

\textbf{Figure 3: Immunohistological features of folliculotropic mycosis
fungoides.}

(A-B) Superficial and deep dense dermal monotonous lymphocytic
infiltrate with predominantly perifollicular/folliculocenteric
distribution extending down into the mid-dermis. There is tagging of the
lymphocyte along the basal layer of the follicular epithelium, with some
lymphocytes enclosed within haloes. There is the attenuation of the
epidermis, but there is no atypia or epidermotropism, Pautrier
microabscess formation., tagging, haloed lymphocytes, lymphocyte atypia,
or pagetoid spread in the spinous layer. The interfollicular epidermis
is not involved. (hematoxylin-eosin stain, original magnification,
A:x20, and B:x40).

(C-D) Exocytosis of small to medium-sized hyperchromatic lymphocytes
into the follicular epithelium of the bulbar and isthmic portions of the
hair follicle with disproportionate spongiosis and destruction of the
hair follicles. The follicular epithelium is focally spongiotic
(hematoxylin-eosin stain, original magnification, C-D: x400).

(E-F) A close-up examination of PAS/Alcian blue stained sections reveals
mucin deposition in the hair follicles and the interstitial spaces
(PAS/Alcian blue, original magnification, E-F: x400)

(G-H) Dense CD4 positive perifollicular lymphoid aggregates with marked
infiltration of the follicular epithelium (folliculotropism) by small to
medium-sized T- T-lymphocytes (original magnification, E: x20, and
F:x400).

(I): Minimal CD8-positive T- T-lymphocytes are seen. The shift of the
CD4 positive folliculotropic T-cells (H) to the CD8 (I) ratio favors the
diagnosis of F-MF (original magnification, I:x400). The lack of
epidermotropism should not deter dermatopathologists from considering
F-MF as a diagnostic possibility.

\emph{Declarations}

-Ethics approval and consent to participate: Enclosed.

-Consent for publication: Enclosed.

-Availability of data and material: All data and materials are included
inside the manuscript.

-Competing interests: None to declare.

-Funding: None to declare.

-Authors' contributions: The authors equally contributed to the
generation of this manuscript (conception and design, acquisition of
data, analysis and Interpretation of data, drafting of the manuscript,
critical revision of the manuscript for important intellectual content,
and statistical analysis.

-Acknowledgements: None to declare

\textbf{REFERENCES}

1. Alojail HY, Alshehri H, Kaliyadan F. Clinical Patterns and Treatment
Response of Patients With Mycosis Fungoides a Retrospective
Study.\emph{Cureus.} 2022;14(1):e21231.

2. Latzka J, Trautinger F. Mycosis fungoides and Sezary syndrome -
Review and outlook. \emph{J Dtsch Dermatol Ges.} 2023;21(4):386-391.

3. Kempf W, Kazakov DV, Schermesser M, et al. Unilesional follicular
mycosis fungoides: report of two cases with progression to tumor stage
and review of the literature. \emph{J Cutan Pathol.} 2012;39(9):853-860.

4. van Doorn R, Van Haselen CW, van Voorst Vader PC, et al. Mycosis
fungoides: disease evolution and prognosis of 309 Dutch
patients.\emph{Arch Dermatol.} 2000;136(4):504-510.

5. van Doorn R, Scheffer E, Willemze R. Follicular mycosis fungoides, a
distinct disease entity with or without associated follicular mucinosis:
a clinicopathologic and follow-up study of 51 patients. \emph{Arch
Dermatol.} 2002;138(2):191-198.

6. Willemze R, Cerroni L, Kempf W, et al. The 2018 update of the
WHO-EORTC classification for primary cutaneous lymphomas.
\emph{Blood.}2019;133(16):1703-1714.

7. Willemze R, Jaffe ES, Burg G, et al. WHO-EORTC classification for
cutaneous lymphomas. \emph{Blood.} 2005;105(10):3768-3785.

8. AlGhamdi KM, Arafah MM, Al-Mubarak LA, Khachemoune A, Al-Saif FM.
Profile of mycosis fungoides in 43 Saudi patients. \emph{Ann Saudi
Med.}2012;32(3):283-287.

9. Arafah M, Zaidi SN, Kfoury HK, Al Rikabi A, Al Ghamdi K. The
Histological Spectrum of Early Mycosis Fungoides: A Study of 58 Saudi
Arab patients. \emph{Oman Med J.} 2012;27(2):134-139.

10. Al-Dawsari NA, Amra N. Pattern of skin cancer among Saudi patients
attending a tertiary care center in Dhahran, Eastern Province of Saudi
Arabia. A 20-year retrospective study. \emph{Int J
Dermatol.}2016;55(12):1396-1401.

11. Albasri AM, Borhan WM. Histopathological pattern of skin cancer in
Western region of Saudi Arabia. An 11 years experience. \emph{Saudi Med
J.} 2018;39(10):994-998.

12. Alsaif F, Alkhayal FA, Aldahash R, Alhumaidi A. Leser-Trelat Sign
Presenting in a Patient with Relapsing Mycosis Fungoides. \emph{Case Rep
Oncol.} 2018;11(2):436-441.

13. Alghubaywi FA, Alharthi SA, Aldharman SS, et al. Clinicopathologic
characteristics and outcomes of patients with mycosis fungoides: A
single tertiary center retrospective analysis in Saudi
Arabia.\emph{Saudi Med J.} 2023;44(4):394-400.

14. Bonta MD, Tannous ZS, Demierre MF, Gonzalez E, Harris NL, Duncan LM.
Rapidly progressing mycosis fungoides presenting as follicular
mucinosis. \emph{J Am Acad Dermatol.} 2000;43(4):635-640.

15. Khalil J, Kurban M, Abbas O. Follicular mucinosis: a
review.\emph{Int J Dermatol.} 2020.

16. Khalil J, Kurban M, Abbas O. Follicular mucinosis: a
review.\emph{Int J Dermatol.} 2021;60(2):159-165.

17. Pinkus H. Alopecia mucinosa; inflammatory plaques with alopecia
characterized by root-sheath mucinosis. \emph{AMA Arch
Derm.}1957;76(4):419-424; discussion 424-416.

18. Jablonska S, Chorzelski T, Lancucki J. {[}Mucinosis
follicularis{]}.\emph{Hautarzt.} 1959;10:27-33.

19. Boone SL, Guitart J, Gerami P. Follicular mycosis fungoides: a
histopathologic, immunohistochemical, and genotypic review. \emph{G Ital
Dermatol Venereol.} 2008;143(6):409-414.

20. Lehman JS, Cook-Norris RH, Weed BR, et al. Folliculotropic mycosis
fungoides: single-center study and systematic review. \emph{Arch
Dermatol.} 2010;146(6):607-613.

21. Esteves M, Nogueira A, Azevedo F, Mota A. Pediatric Follicular
Mucinosis: A Report of Two Cases. \emph{Indian Dermatol Online
J.}2021;12(1):172-173.

22. Lewars M, Levin J, Purcell S. Follicular mucinosis. \emph{Indian
Dermatol Online J.} 2013;4(4):333-335.

23. Tambe SA, Zambare US, Nayak CS. Folliculotropic Mycosis
Fungoides.\emph{Indian Dermatol Online J.} 2021;12(1):178-180.

24. Gerami P, Guitart J. The spectrum of histopathologic and
immunohistochemical findings in folliculotropic mycosis
fungoides.\emph{Am J Surg Pathol.} 2007;31(9):1430-1438.

25. Roccuzzo G, Mastorino L, Gallo G, Fava P, Ribero S, Quaglino P.
Folliculotropic Mycosis Fungoides: Current Guidance and Experience from
Clinical Practice. \emph{Clin Cosmet Investig
Dermatol.}2022;15:1899-1907.

26. Baykal C, Atci T, Ozturk Sari S, Polat Ekinci A, Buyukbabani N.
Underrecognized clinical features of folliculotropic mycosis fungoides:
a large clinical series. \emph{J Dtsch Dermatol Ges.}2017;15(3):289-299.

27. Monopoli A, Annessi G, Lombardo GA, Baliva G, Girolomoni G. Purely
follicular mycosis fungoides without mucinosis: report of two cases with
review of the literature. \emph{J Am Acad Dermatol.} 2003;48(3):448-452.

28. Magro CM, Telang GH, Momtahen S. Unilesional Follicular Mycosis
Fungoides: Report of 6 Cases and Review of the Literature. \emph{Am J
Dermatopathol.} 2018;40(5):329-336.

29. Rajalakshmi T, Inchara YK, Antony M. Follicular mycosis fungoides -
A report of four Indian cases. \emph{Indian J Med Paediatr
Oncol.}2009;30(3):108-112.

30. Mantri MD, Khadke MP, Ameet DL, Rachita DS. Folliculotropic Mycosis
Fungoides in an Adolescent: A Rare Case. \emph{Indian J
Dermatol.}2016;61(4):467.

31. Emge DA, Lewis DJ, Aung PP, Duvic M. How to Discern Folliculotropic
Mycosis Fungoides From Follicular Mucinosis Using a Pediatric
Case.\emph{J Cutan Med Surg.} 2018;22(3):336-340.

32. Saleh JS, Subtil A, Hristov AC. Primary Cutaneous T-cell Lymphoma: A
Review of the Most Common Entities with Focus on Recent
Updates.\emph{Hum Pathol.} 2023.

33. Woringer F, Chapuis JL, Bouteille H. {[}Mucinosis
follicularis{]}.\emph{Bull Soc Fr Dermatol Syphiligr.} 1959;3:273-280.\selectlanguage{english}
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