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\begin{document}

\title{Late onset of monomorphous flesh-colored papules of the neck}



\author[1]{Massara Baklouti}%
\author[2]{Khadija Sellami}%
\author[3]{Chiraz Chaari}%
\author[3]{Tahya Sellami}%
\author[1]{Hamida Turki}%
\affil[1]{Hedi Chaker Hospital}%
\affil[2]{Hopital Hedi Chaker}%
\affil[3]{Habib Bourguiba Hospital}%


\vspace{-1em}



  
  \date{May 24, 2024}


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\selectlanguage{english}
\begin{abstract}
Papular elastorrhexis is a rare acquired disorder of elastic tissue,
characterized by multiple, flesh-colored monomorphous papules usually
located on the trunk and the proximal portion of the extremities,
occurring in adolescent females. We report a case of PE in an old woman
with atypically isolated localization on the neck.%
\end{abstract}%



\sloppy


\textbf{Introduction}

Papular elastorrhexis (PE) which was first described by Bordas et al. in
1987, is a rare acquired elastic tissue disorder characterized by
multiple asymptomatic, non-follicular, flesh-colored or hypopigmented,
monomorphous papules (1, 2). PE is usually located on the trunk and the
proximal portion of the extremities in female children or adolescent
girls (2). The nosologic position of PE is controversial. Clinical and
histological characteristics classify PE as a distinctive entity
different from nevus anelasticus and Buschke--Ollendorff syndrome (2,3).
The exact etiology of this condition is still unknown. No history of
trauma or local inflammation, nor systemic associations or family
history are present.

\textbf{Case report~}

A 59-year-old woman presented with a one-year history of asymptomatic
small papules on the neck. They were slowly increasing in number. The
patient had a history of squamous cell carcinoma of the cheek that had
been excised. She had not inflammatory dermatosis or trauma on the
involved area. There were no similar lesions in her family members.
Dermatologic examination showed multiple, 1-4 mm in diameter,
flesh-colored, non-follicular, non-confluent and firm papules
distributed over the posterolateral sides of the neck (Figure 1).

There were no lesions elsewhere and ophthalmologic examination was
normal. Biopsy was performed. Histopathologic analysis showed a normal
epidermis and mild thickened collagen bundles in the dermis (Figure 2A).
Orcein stains showed a significant loss and fragmentation of elastic
fibers in the reticular dermis (Figure 2B). Von Kossa staining was
negative. Based on the clinical and histopathological features, the
diagnosis of PE was made. We did not prescribe any treatment.

\textbf{Discussion}

PE is a rare disorder of elastic tissue infrequently reported in the
literature. The onset is usually within the first or second decade of
life with female predominance (75\%) (3). The lesions were described as
multiple well-defined, soft, hypopigmented or skin-colored,
non-follicular oval to round papules with no tendency to group (4). The
lesions are symmetrically distributed throughout the chest, abdomen,
back and shoulders, upper extremities and rarely thighs (4). There has
been no report of extracutaneous manifestation. Uncommon locations such
as involvement of the mandibular, retro-auricular, occipito-cervical
regions and face have been reported (5,6). Neck involvement is described
in two cases and it was associated to other locations (6,7).

The prominent histopathological feature is reduction, fragmentation or
complete loss of elastic bundles in the reticular dermis. A perivascular
infiltrate composed of lymphocytes and macrophages in the superficial
and deep dermis is present in some cases. Thickened collagen could be
seen (8). The pathogenic mechanism of this elastic tissue alteration is
unknown, and no local inflammation, trauma or systemic associations have
been described. A recent study suggests that abnormal fibroblasts might
be involved (9).

Differential diagnosis of PE includes perifollicular elastolysis,
middermal elastolysis, pseudoxanthoma elasticum, pseudoxanthoma
elasticum-like papillary dermal elastolysis, and white fibrous papulosis
of the neck, like in our case.

Oral antibiotics, oral isotretinoin, topical tretinoin, and dibenzoyl
peroxide had not been efficient in treating PE. A report has shown
anecdotal improvement after intralesional injection of triamcinolone.
(5,7,10).

In summary, we report a case of PE in an old woman with atypically
isolated localization on the neck.

The diagnosis of PE can be challenging because of the heterogeneous
group of elastic tissue disorders. This condition is generally
asymptomatic but could cause pruritus and aesthetic discomfort.

\textbf{Key Clinical Message}

* Papular elastorrhexis is a rare acquired disorder of elastic tissue
mostly occurring in adolescent females.

* Papular elastorrhexis is characterized by multiple, flesh-colored
monomorphous papules usually located on the trunk and the proximal
portion of the extremities.

* Neck involvement is described in two cases and it was associated to
other locations. We report a case in an old woman with atypically
isolated localization on the neck.

References

\begin{enumerate}
\tightlist
\item
  Bordas X, Ferrandiz C, Ribera M, et al. Papular elastorrhexis: a
  variety of nevus anelasticus? Arch Dermatol 1987; 123: 433-4.
\item
  Del Pozo J, Mart\selectlanguage{ngerman}ínez W, Sacristán F, Fernández-Jorge B, Fonseca E.
  Papular~elastorrhexis, a distinctive entity? Am J Dermatopathol. 2008
  Apr;30(2):188-90.
\item
  Lewis KG, Bercovitch L, Dill SW, et al. Acquired disorders of elastic
  tissue: part II. Decreased elastic tissue. J Am Acad Dermatol.
  2004;51: 165--185.
\item
  Sezer E, Öztürk E, \selectlanguage{polish}Ş\selectlanguage{english}ahin DS.Papular~elastorrhexis: clinical
  perspectives. Clin Cosmet Investig Dermatol. 2018 Oct 26;11:541-544.
\item
  \selectlanguage{polish}Ş\selectlanguage{english}ahin S, Durmaz E\selectlanguage{ngerman}Ö, Sezer E, Çetin ED.
  Eruptive~papular~elastorrhexis~of the face and scalp.J Am Acad
  Dermatol. 2013 Nov;69(5):e251-e252.
\item
  Luo DQ, Liu JH, Chen MC, Wang Z, Xie WL.Papular~elastorrhexis: report
  of four cases and review of literature. Arch Dermatol Res. 2015
  Mar;307(2):99-108.
\item
  Thome´ EP, Steglich RB, Meotti CD, Schwartz J, Boff AL (2012) Case for
  diagnosis. Papular elastorrhexis. An Bras Dermatol 87:651--653.
\item
  Buechner SA, Itin P. Papular~elastorrhexis. report of five cases.
  Dermatology. 2002;205(2):198-200.
\item
  Li SJ, Peng J, Yang PP, Sheng P, Fan YM.J. Immunopathological and
  ultrastructural features in a case of~papular~elastorrhexis. Dtsch
  Dermatol Ges. 2017 Feb;15(2):212-214.
\item
  Bem LCCP, Coppini A, Padilha VV, Lima BZ. Papular~elastorrhexis: a
  rare and benign disease. An Bras Dermatol. 2018 Jul-Aug;93(4):573-575.
\end{enumerate}

Legend

Figure 1: Multiple flesh-colored non-follicular, 1-4 mm in diameter
papules on the posterolateral side of the neck.

Figure 2: (A) Histopathological examination showed hyperplasia of
collagen fiber in the dermis (HEX 100). (B) Orcein staining showed a
significant reduction and fragmentation of elastic tissue in the
reticular dermis (HEX 200).\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/Figure-1/Figure-1}

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\end{figure}\selectlanguage{english}
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\includegraphics[width=0.70\columnwidth]{figures/Figure-2/Figure-2}

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\end{figure}

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