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.