Late-onset focal dermal elastosis

ARTICLE

ejd.2012.1664

Auteur(s) : Shan Tian, Zhou Chen chenzhou@medmail.com.cn

Department of Dermatology, Peking University People's hospital, 11# Xizhimen South Street, 100044 Beijing, China

A 72-year-old woman had a 3-year history of slowly progressing skin lesions on the neck. The patient sometimes felt mild itching but no pain. There was no history of gastric bleeding, coronary disease or stroke, or diminished visual acuity. There was no family history of similar skin lesions. Physical examination revealed multiple, pale yellow, 2-5 mm diameter papules on both sides of her neck, as well as the anterior part of her chest (figure 1A-B). A full body examination did not reveal similar lesions in other locations. Laboratory tests, including blood cell count, urea test, liver function, serum lipids and fasting serum glucose, were normal. Ophthalmological examination did not reveal any retinal abnormalities. Electrocardiography, echocardiography and chest radiograph were normal. Her blood pressure was normal. Histopathologic examination of the neck lesion demonstrated that the epidermis was atrophic. The papillary dermis contained pink collagen bundles without amorphous elastotic material, which is a reference to actinic elastosis (figure 1C). Gomori's staining showed a focal increase in the number of elastic fibers. They appeared structurally normal and the increase was confined to the reticular dermis (figure 1D); they were not apparent on the hematoxylin-eosin staining sections. There was no fragmentation, calcification, or phagocytosis of elastic fibers in Gomori's staining sections. Above all, the late age of onset, the absence of systemic involvement and the histopathological findings suggested the diagnosis of late-onset focal dermal elastosis.

Late-onset focal dermal elastosis was first reported as a distinct clinicopathological entity in two elderly Japanese patients by Tajima et al. in 1995 [1]. Since then, only six cases have been reported in the literature. To our knowledge, this case is the first one in China. According to these cases [1-4], this disorder is mostly seen in 65-85 year old men or women with 2-10 year histories. The patients present slowly progressing, asymptomatic, yellow papules on the neck, thighs, groin, axillae, antecubital and popliteal fossae. The most prominent histopathological feature is an increase in normal-appearing elastic tissue in the mid and deep reticular dermis.

The main and most important differential diagnosis of this entity is inherited pseudoxanthoma elasticum (PXE) [5]. PXE clinically presents as cobblestone yellow papules, typically located on the neck and flexural areas, but it usually develops during childhood, characterized by calcification of the elastic fibers of the skin, retina and cardiovascular system, which can lead to serious complications. The other differential diagnosis of late-onset focal dermal elastosis is PXE-like papillary dermal elastolysis. This is characterized by whitish-yellow papules resembling late-onset focal dermal elastosis, but the histological examination shows loss of or a decrease in the number of elastic fibers in the papillary dermis.

The pathogenesis of late-onset focal dermal elastosis has not been determined. However, the advanced age of onset, the absence of solar elastosis and distinct histopathologic findings suggests that the disorder may be one of intrinsic ageing. Kossard [2] postulated that there may be some loss of age-related homeostatic growth regulating gene control mechanism attributed to the pathogenesis. Limas [3] thought the predilection for the neck and flexural creases could be a structural response to the repetitive mechanical stress these areas are subjected to, in conjunction with genetically-determined differences in the composition and turnover of the elastic fibers. It is also possible that these lesions are induced by unidentified environmental factors (e.g., drugs and cosmetics). To date, there is no satisfactory remedy for late-onset focal dermal elastosis.

Dislosure

Financial support: none. Conflict of interest: none

References

1. Tajima S, Shimizu K, Izumi T, et al. Late-onset focal dermal elastosis: clinical and histological features. Br J Dermatol 1995; 133:303-305.

2. Kossard S. Pseudoxanthoma-like late-onset focal dermal elastosis. Australas J Dermatol 2005; 46: 47-50.

3. Limas C. Late onset focal dermal elastosis: a distinct clinicopathologic entity? Am J Dermatopath 1999; 21:381-3.

4. Higgins JH, Whitworth WM. Late-onset focal dermal elastosisi: a case report and review of the literature. Cutis 2010(4); 85:195-197.

5. Kevan G, Lewis, Lionel Bercovitch, et al. Acquired disorders of elastic tissue: Part I. Increased elastic tissue and solar elastotic syndromes. J Am Acad Dermatol 2004; 51:1-21.