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.