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\begin{document}

\title{Cutis verticis gyrata: three cases illustrating three different
etiologies}



\author[1]{Amal Chamli}%
\author[1]{Meriem  Jones}%
\author[1]{Takwa Bacha}%
\author[1]{Noureddine Litaiem }%
\author[1]{Faten Zeglaoui}%
\affil[1]{University of Tunis El Manar Faculty of Medicine of Tunis}%


\vspace{-1em}



  
  \date{June 07, 2024}


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\selectlanguage{english}
\begin{abstract}
Cutis Vertcis gyrata is an uncommon neurocutaneous syndrome
characterized by excessive growth of the skin of the scalp or the face,
forming folds of similar aspect to cerebral cortex gyri. Three
categories have been individualized: the primary form, essential or
non-essential, and the secondary form.%
\end{abstract}%



\sloppy


\emph{Title:} \textbf{Cutis verticis gyrata: three cases illustrating
three different etiologies}

\emph{Running head title:} \textbf{Cutis verticis gyrata}

\emph{Authors name and affiliation:}

Amal Chamli\textsuperscript{\textbf{1,2}} , MD, Meriem
Jones\textsuperscript{\textbf{1,2}} , MD, Takwa
Bacha\textsuperscript{\textbf{1,2}} , MD, Noureddine
Litaiem\textsuperscript{\textbf{1,2}} , MD, Faten
Zeglaoui\textsuperscript{\textbf{1,2}} , PhD

1-Department of Dermatology, Charles Nicolle Hospital, Tunis, Tunisia

2-University of Tunis El Manar, Faculty of medicine of Tunis, Tunisia

\emph{Corresponding Author:}

Amal CHAMLI, MD

Adress: Department of Dermatology, Charles Nicolle Hospital, Tunis,
Tunisia

Email: \emph{amal.fmt@gmail.com}

Tel: +21654085688

\emph{Conflict of interest Disclosure:} None declared

\emph{Funding Sources:} None

\emph{Word count:} 1050

\emph{\textbf{Figures}:} 2; \emph{tables} : 0

\emph{References:} 7

\textbf{Cutis verticis gyrata: three cases illustrating three different
etiologies}

\textbf{Abstract:}

Cutis Vertcis gyrata is an uncommon neurocutaneous syndrome
characterized by excessive growth of the skin of the scalp or the face,
forming folds of similar aspect to cerebral cortex gyri. Three
categories have been individualized: the primary form, essential or
non-essential, and the secondary form.

\textbf{Key words: cutis verticis gyrata, neurocutaneous syndrome,
folds, hypertrophy}

\textbf{Key Clinical Messages:}

- Cutis verticis gyrata is a rare neurocutaneous syndrome with mainly
anesthetic impact.

- A rigorous clinical examination with a minimal workup is required to
eliminate a secondary or a primary non-essential form.

\textbf{Introduction:}

Cutis verticis gyrata (CVG) is a rare skin disease. affecting 1 of
100,000 males and 0.026 of 100,000 females in the general population
{[}1{]} CVG is characterized by excessive growth of the skin of the
scalp or the face, forming folds of similar aspect to cerebral cortex
gyri. Three forms have been individualized: the primary form, essential
or non-essential, and the secondary form. The main complaint of the
patients is usually cosmetic. The treatment, if requested, is surgical.
We report three cases that illustrate one of each of these three
categories.

\textbf{Observations:}

\textbf{Case 1:} A 28-year-old male presented with multiple trichilemmal
cysts of the scalp. Physical examination revealed, in addition, a
thickened scalp with multiple symmetric skin folds from vertex to
occipital area (figure 1). The systemic examination did not reveal any
neurologic, ophthalmologic, or metabolic anomalies. Laboratory
investigations were within the normal range limit. The diagnosis of
primary essential CVG was retained and the patient required only
excision of the cysts.

\textbf{Case 2:} A 32-year-old male with a history of epilepsy was
referred to our consultation for a drug reaction secondary to
phenobarbital. On examination, apart from the skin lesions consistent
with the drug reaction, the patient had cerebriform folds on his
forehead. The furrows were running horizontally and had a width of 1 to
2 cm (figure 2). He didn't have any lesions on the scalp. No other
anomalies were found. The diagnosis of primary non-essential CVG
associated with epilepsy and involving only the forehead was retained.

\textbf{Case 3:} A 54-year-old female treated for hypertension,
dyslipidemia, and a heart rhythm disorder, presented to our department
with an occipital tumefaction evolving for a few years. The examination
revealed cerebriform transverse occipital skin folds. The remaining
physical examination was otherwise unremarkable. Given her chronic
metabolic pathologies, secondary CVG was diagnosed.

\textbf{Discussion}

CVG is a rare benign condition first described by Alibert in 1837
characterized by proliferation and hypertrophy of the skin of the scalp
or forehead. The affected skin then exhibits folds and furrows
resembling the cerebral cortex convolutions. Unna introduced the term
cutis verticis gyrata in 1907 {[}2{]}.

This entity was afterward divided into different subtypes: a secondary
and a primary form, then the primary form was subdivided into primary
essential and non-essential. The primary essential form is the rarest
form, characterized by an isolated cutaneous involvement with no
associated pathology. It usually begins in adolescence and is more
common in men than in women. Typically, as seen in case 1, primary CVG
presents as symmetric scalp folds which usually extend anteroposteriorly
from the vertex to the occiput and transversally in the occipital
region. Terminal hair density is reduced on the folds, but not in the
furrows. The primary non-essential form has been described in
association with several neuropsychiatric pathologies like mental
retardation, epilepsy, microcephaly, schizophrenia, encephalopathy, and
other neurological malformations in addition to ophthalmic pathologies
such as blindness, strabismus, and congenital cataract, the latter being
the most frequent association. In primary non-essential CVG, the
associated conditions do not have a clear pathophysiological link with
the CVG.

Secondary CVG on the other hand is associated with conditions that may
be implicated in modifications of the trophicity of the skin. It is
slightly more common than the primary form. It can occur at any age
without gender predominance. It is associated with a variety of
underlying disorders. It may be caused by general diseases comprise as
liver affections, paraneoplastic syndromes, and pachydermoperiostosis or
also associated with inflammatory dermatoses most commonly psoriasis and
eczema {[}3{]}. Furthermore, various associated genetic conditions have
been described such as neurofibromatosis, Noonan, Turner, Klinefelter
and Ehlers--Danlos syndrome. It may also occur in association with
endocrine etiologies, as seen in case 3, such as diabetes mellitus,
myxedema, and the most described in the literature is acromegaly
{[}3{]}. Among iatrogenic causes, treatment with minoxidil or misuses of
anabolic substances were reported {[}4,5{]}.

The pathophysiology of CVG remains unclear. On a structural level, it is
suggested that the folds are caused by connective tissue septa formation
between the skin and the galea. These septa prevent the expansion of the
skin which bulges into folds {[}6{]}. In the primary form, an endocrine
origin has been postulated as the disease usually manifests in
postpubertal males. Cases reported in the literature are sporadic
although some familial cases are described suggesting possible genetic
factors in primary CVG. The physiopathology in the secondary form
remains also unclear especially in general and inflammatory diseases.
However, in the physiopathology of endocrine etiology, trophic hormones
such as growth hormone (GH) and insulin growth factor 1 (IGF 1) were
incriminated to be responsible for skin modifications with collagen
thickening and hypertrophy of sebaceous glands.

When performed, histopathology of primary CVG shows normal skin.
Thickened connective tissue with hypertrophy or hyperplasia of adnexal
structures has also been described. Secondary CVG has variable histology
representing the underlying disease. No treatment has proven its
effectiveness in this entity. Abstention is the rule in the primary
form. In all cases, regular rigorous hygiene of the skin folds and
furrows is recommended to avoid maceration and infection. If treatment
is requested by the patient because of an anesthetic complaint, medical
and/or surgical treatment may be suggested. Surgical treatment seems to
be the best option with a regularly reported efficacy. The undermining
and relaxation incisions alter the relationship between the skin and the
deeper structures and so result in the disappearance of the folds and
furrows {[}7{]}. Medical treatment includes isotretinoin or systemic
steroids. No treatment can prevent a recurrence.

In summary, CVG is a rare disease with mainly anesthetic impact. A
rigorous clinical examination with a minimal workup is however required
to eliminate a secondary or a primary non-essential form. If aesthetic
prejudice is important, surgical treatment can be suggested.

\textbf{Conflicts of Interest: None declared}

\textbf{Funding sources: None}

\textbf{Ethics statement:} Appropriate consent has been obtained, prior
to submission, for the publication of images and data.

\textbf{Data availability statement:} Data sharing was not applicable to
this article as no datasets were generated or analyzed during the
current study.

\textbf{Author Contributions:}

Drs Amal Chamli and Meriem Jones contributed to the first draft of the
manuscript. Drs Amal Chamli, Meriem Jones,Takwa Bacha, and Noureddine
Litaiem contributed to the literature search, analysis, and
interpretation of the data. Dr Faten Zeglaoui critically revised the
manuscript and gave final approval. All authors read and approved the
final manuscript and agree to be finally accountable for ensuring the
integrity of and accuracy of the work.

\textbf{Acknowledgement:} none

\textbf{References:}

\textbf{{[}} 1{]} Harish V, Clarke F. Isolated cutis verticis gyrata of
the glabella and nasal bridge: a case report and review of the
literature. \emph{J Plast Reconstr Aesthet Surg.} 2013;66(10):1421-3.

{[}2{]} Samira B, Meriem D, Oumkeltoum E, Driss E, Yassine B, Saloua E.
{[}Primitive essential Cutis verticis gyrata, a rare skin disease: case
report and review of literature{]}. \emph{Pan Afr Med J.} 2014;19:345.

{[}3{]} Chentli F, Terki B, Azzoug S. Cutis verticis gyrata and
acromegaly. \emph{Presse Med} . 2015;44(9):958-61.

{[}4{]} Nguyen KH, Marks JG. Pseudoacromegaly induced by the long-term
use of minoxidil. \emph{J Am Acad Dermatol.} 2003;48(6):962-5.

{[}5{]} Sommer A, Gambichler T, Altmeyer P, Kreuter A. A case of cutis
verticis gyrata, induced by misuse of anabolic substances? \emph{Clin
Exp Dermatol} . 2006;31(1):134-6.

{[}6{]} Tani T, Miyamoto Y, Kurozumi N. Surgical treatment of cutis
verticis gyrata: a report of 2 cases. \emph{Br J Plast Surg} .
1977;30(3):235-9.

{[}7{]} El-Husseiny M, Yarrow J, Moiemen N. Primary cutis gyrata: review
of literature and a successful new surgical approach. \emph{Eur J Plast
Surg.} 2010;33(3):153-7.

\textbf{Figure legends:}

\textbf{Figure 1:} multiple symmetric skin folds extending from the
vertex to the occipital area in a 28-year-old male

\textbf{Figure 2:} Cerebriform folds on the forehead of a 32-year-old
male.\selectlanguage{english}
\begin{figure}[H]
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\includegraphics[width=0.70\columnwidth]{figures/Figure-1/Figure-1}

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\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/figure-2/figure-2}

\end{center}
\end{figure}

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