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\begin{document}

\title{Cutis verticis gyrata in a 24 year-old young man revealing a T-cell
lymphoblastic lymphoma}



\author[1]{Sarra Saad}%
\author[1]{Nadia Ghariani Fetoui}%
\author[1]{Jacem Rouatbi}%
\author[1]{Sana Mokni}%
\author[1]{Najet Ghariani}%
\author[1]{Baderedine Sriha}%
\author[1]{Mohamed Denguezli}%
\affil[1]{Farhat Hached University Hospital of Sousse}%


\vspace{-1em}



  
  \date{May 24, 2024}


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\selectlanguage{english}
\begin{abstract}
T-cell lymphoblastic lymphoma (T-LBL) is frequently revealed by a
mediastinal mass or peripheral lymphadenopathy. Skin lesions in T-LBL
usually present as multiple nodules associated with multiple peripheral
lymphadenopathy and bone marrow invasion. Our patient is particular by
the revealing presentation of the lesions as Cutis verticis gyrate.%
\end{abstract}%



\sloppy


\emph{The title of the article} : \textbf{Cutis verticis gyrata in a 24
year-old young man revealing a T-cell lymphoblastic lymphoma}

\emph{Running Head}\textbf{: Cutis verticis gyrata revealing a T-cell
lymphoblastic lymphoma}

\emph{The category for which the article is being submitted} :

\textbf{Case Report}

\emph{Key clinical message~:} T-cell lymphoblastic lymphoma (T-LBL) is
frequently revealed by a mediastinal mass or peripheral lymphadenopathy.
Skin lesions in T-LBL usually present as multiple nodules associated
with multiple peripheral lymphadenopathy and bone marrow invasion. Our
patient is particular by the revealing presentation of the lesions as
Cutis verticis gyrate.

\emph{Key Words} : Cutis verticis gyrate, Malignant hemopathy, T-cell
lymphoblastic lymphoma

\emph{Authors}\textbf{~:}

Dr Sarra Saad, MD, Department of Dermatology, Farhat Hached University
Hospital, Sousse, Tunisia.

Dr Nadia Ghariani Fetoui, MD, Department of Dermatology, Farhat Hached
University Hospital, Sousse, Tunisia.

Dr Jacem Rouatbi, MD, Department of Anatomopathology, Farhat Hached
University Hospital, Sousse, Tunisia.

Dr Sana Mokni, MD, Department of Dermatology, Farhat Hached University
Hospital, Sousse, Tunisia.

Dr Najet Ghariani, MD, Department of Dermatology, Farhat Hached
University Hospital, Sousse, Tunisia.

Dr Baderedine Sriha, MD, Department of Anatomopathology, Farhat Hached
University Hospital, Sousse, Tunisia.

Dr Mohamed Denguezli, MD, Department of Dermatology, Farhat Hached
University Hospital, Sousse, Tunisia.

\emph{Corresponding author:}

Dr Sarra Saad, MD

Department of Dermatology

Fathat Hached University Hospital, Sousse

Tunisia.

Mail: \emph{drsaadsarra@gmail.com}

Phone: 00216 98684703

\emph{Authorship:} All authors had access to the data and a role in
writing this manuscript.

\emph{Word counts~for the text :} \textbf{507}

\emph{The total number of pages~} :3

\emph{Total number of figures~} : 2

\emph{Source of support~:} none

\textbf{INTRODUCTION}

Cutis verticis gyrate (CVG) or ''washboard'' pachydermia is a specific
semiological aspect of the scalp and forehead characterized by
hypertrophy and redundancy of the integument forming ``waves'' separated
by deep folds. Secondary CVG have a wide variety of causes:
genodermatoses, endocrinopathies, overload disease, paraneoplastic or
tumor diseases. Lymphoblastic lymphoma (LBL) is a rare neoplasm with a
poor prognosis.\textsuperscript{1} We describe a case of CVG revealing a
T-cell lymphoblastic lymphoma (T-LBL) in a young man.

\textbf{CASE REPORT}

A 24 year-old man presented to our dermatology department for an
asymptomatic deep rippling lesions on the frontal level of the scalp
evolving for 2 months. He had no past medical history. Dermatology
examination revealed multiple large nodules on deeply infiltrated
erythematous-violaceous skin limited to the frontal area of the scalp
resulting in folds that mimick the surface of the cerebral cortex
(Figure1A). Dermoscopy revealed dotted vessels, fine short linear
vessels and scales over a salmon-pink background (Figure 1B). Physical
examination revealed no locoregional adenopathies. Histopathologic
examination revealed a dense infiltration in the dermis, made by medium
sized immature lymphoid cells, with a high mitotic index (Figure 2A).
There was no epidermotropism. Immunohistochemical stains were positive
for terminal deoxynucleotidyl transferase (TdT), CD3, CD4 and CD8 and
negative for CD20 (Figure 2B, 2C). The diagnosis of secondary CVG
associated with T-LBL was made. The patient's general condition worsened
within a few days and he started complaining of debilitating dyspnoea
and productive cough. The patient underwent computed tomography of the
chest, abdomen and pelvis, which revealed a large mediastinal mass
measuring 15.5cm*10cm*22cm associated with pleural and pericardial
effusions as well as multiple bilateral renal masses. A transthoracic
needle biopsy was performed, confirming the diagnosis of pulmonary
involvement with T-cell lymphoblastic lymphoma. The patient received
chemotherapy.

\textbf{DISCUSSION}

T-cell lymphoblastic lymphoma is a very rare form of non-Hodgkin
lymphoma, accounting for approximately 20\% of
LL.\textsuperscript{1,2}It commonly occurs in young male adults, more
than children or elderly.\textsuperscript{2} T-LBL is frequently
revealed by a mediastinal mass or peripheral lymphadenopathy. Skin
localizations of all LBL are noted in fewer than 20\% of affected
patients.\textsuperscript{3} Unlike B-cell LBL (B-LBL), skin involvement
concerns only a minority of patients with T-LBL, as well as lymph node
involvement. To our knowledge, only 15 cases of T-LBL with cutaneous
involvement have been reported in the
literature.\textsuperscript{1,4,5}Skin lesions mostly present as nodular
lesions, located on the scalp, the trunc and the limbs. Skin lesions in
T-LBL usually present as multiple nodules associated with multiple
peripheral lymphadenopathy and bone marrow invasion. Our patient is
particular by the revealing presentation of the lesions as CVG without
other distant lesions. Only four cases of T-LBL diagnosed by cutaneous
involvement have been reported so far.\textsuperscript{1}

To our knowledge, secondary CVG revealing T-LBL has not been reported
previously.

Dermoscopic features of skin l\selectlanguage{ngerman}ésions are not specific for
LBL.\textsuperscript{6} However, they are comparable to that primary
cutaneous CD4+ small/medium T-cell lymphoproliferative disorders
presenting as short fine irregular or serpiginous vessels over
salmon-pink background. The authors correlate the presence of pinkish
background with atypical lymphocytic infiltrates related
angiogenesis.\textsuperscript{6}

\begin{enumerate}
\tightlist
\item
  Y. Chiba,~N.Hirase,~K.Yamasaki, et al. Mediastinal T-cell
  Lymphoblastic Lymphoma Diagnosed with a Skin Biopsy. Intern
  Med.~2020;59(11):1463-1464.
\item
  S.Khurana,~M.Beltran,~L.Jiang, et al. Primary Cutaneous T-Cell
  Lymphoblastic Lymphoma: Case Report and Literature Review. Case Rep
  Hematol,~2019;2019:3540487.
\item
  Lee WJ, Moon HR, Won CH,et al. Precursor B- or T-lymphoblastic
  lymphoma presentingwith cutaneous involvement: a series of 13 cases
  including7 cases of cutaneous T-lymphoblastic lymphoma. J Am Acad
  Dermatol 2014;70(2):318- 325.
\item
  C. Nascimbenia, S. Chantepieb, C. Brugiere, et al. Localisations
  cutanées d'un lymphomalymphoblastique T.Ann DermatolVenereol (2017).
\item
  Montes-Torres A, Llamas-Velasco M, Capusan TM, et al. Cutaneous
  involvement as the first manifestation of T lymphoblastic lymphoma and
  review of the literature. J Cutan Pathol 2019;46(5): 372-375.
\item
  M. S\selectlanguage{polish}ł\selectlanguage{english}awi\_nska, M. Soko\selectlanguage{polish}ł\selectlanguage{english}owska-Wojdy\selectlanguage{polish}ł\selectlanguage{english}o, B. Olszewska, et al.
  Dermoscopic and trichoscopic features of primary cutaneous lymphomas
  -- systematic review. J Eur Acad Dermatol Venereol.2021~;35(7)~:
  1470-1484.
\end{enumerate}

\textbf{Legends:}

\textbf{Figure 1: Clinical and dermoscopic presentation:}

\textbf{A:} Multiple large nodules on deeply infiltrated
erythematous-violaceous skin limited to the frontal area of the scalp
resulting in folds.

\textbf{B:} Dermoscopy: dotted vessels (white circle), fine short linear
vessels (green circle) and scales (blue circle) over a salmon-pink
background.

\textbf{Figure 2: Histology and immunohistochemistry:}

\textbf{A:} Dense infiltration in the dermis, made by medium sized
immature lymphoid cells, with a high mitotic index, (H\&E 400).

\textbf{B,C:} Immunohistochemistry: positive for CD3 (x200) and TdT
(x100).

\textbf{Funding:} None

\textbf{Acknowledgment:} None

\textbf{Conflicts of interest:} None

\textbf{Ethics statement:} None

\textbf{Written Consent from the patient:} Written informed consent was
obtained from the patient to publish this report in accordance with the
journal's patient consent policy.

\textbf{Detailed author's contribution:} Dr Saad and Dr Ghariani Fetoui
contributed to the first draft of the manuscript. Dr Mokni and Dr
Ghariani helped in writing the manuscript and literature search. Dr
Rouatbi and Dr Sriha contributed to the histological data. Dr Denguezli
revised and approved the final version of the manuscript. All the
authors contributed to and have approved the final manuscript.

\textbf{Data availability Statement:} Not applicable\selectlanguage{english}
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