Papular elastorrhexis: an acquired disorder of elastic tissue

ARTICLE

Auteur(s) : Paulina Belmar Flores¹, Jesus Cuevas², Consuelo Sánchez¹, Esther De Eusebio¹, Aranzazu Vergara¹

¹Departments of Dermatology
²Pathology, Universidad Alcalá de Henares, Hospital Universitario de Guadalajara, Spain

Papular elastorrhexis (PE) is a rare disorder of elastic tissue, characterized by asymptomatic papules and intense fragmentation of the elastic bundles in the reticular dermis. It was first described by Bordas in 1987 [1] and since then, approximately 18 cases have been published in the literature [2-4].

We report a 37-year-old woman who presented multiple, asymptomatic, white, non-follicullar papules on her trunk and upper arms (figure 1A). She had no prior history of trauma or local inflammation and no relevant family history. The histopathological examination of a cutaneous specimen showed a normal epidermis and a focal area of homogenized collagen in the reticular dermis with hematoxylin-eosin stain (figure 1B). Orcein stain revealed a decrease and significant fragmentation of elastic fibers in these areas (figure 1C). The electron microscope findings were in agreement with those of the optical microscope.

X-ray examinations, routine laboratory tests, ANA, rheumatoid factor and immunoelectrophoretic studies were normal. Luetic serology and tests for antibodies against B. burgdoferi were negative. PE was diagnosed and no treatment was instituted, due to the asymptomatic character of the lesions.

PE is an uncommon cutaneous disorder of elastic fibers that presents as multiple, whitish or skin-colored, non-follicular papules, with symmetrical distribution on the trunk and upper extremities. It is an acquired disorder that usually appears in the second decade of life without any history of trauma or infection. Hereditary transmission has been described [5] in only one family and extracutaneous changes have not been reported. Histopathologically, the most characteristic finding is the decrease and substantial fragmentation of elastic fibers within the reticular dermis. The differential diagnosis includes nevus anelasticus, papular acne scars, collagenoma, anetodermas, white fibrous Papulosis of the neck and middermal elastolysis.

Since the first descriptions of PE, two nosological considerations have been debated in the literature: its relation to nevus anelasticus and to Buschke-Ollendorf syndrome (BOS). Bordas suggested that PE was a variant of nevus anelasticus, due to the reduction and fragmentation of elastic fibers [1]. However, some features distinguish it from PE. Nevus anelasticus is a predominantly congenital disorder that presents as asymmetrical and perifollicular papules, typically grouped into patches on the anterior aspect of the chest. Moreover, the most striking histopathological feature is the remarkable loss of elastic fibers, whereas fragmentation is the most prominent feature of PE [3-5].

Later, Schirren et al. described one family with PE and proposed that it was an abortive form of BOS [6]. BOS is an autosomal dominant disease characterized by disseminated connective tissue nevi and osteopoikilosis. Elastoma is the condition most commonly found in this syndrome. Patients present with larger, and often grouped, nodules or plaques and most cases show increased elastic fibrils. The nosological confusion between the entities is based on three facts: i) incomplete forms of BOS without osteopoikilosis, ii) infrequent cases with a decrease of elastic bundles in biopsy specimens of BOS and iii) one report of familiar PE. However, those elements only show a certain grade of overlap among the different elastic tissue disorders. As in our patient, a negative family history, the absence of any radiological abnormalities and the histological evidence of a decrease and fragmentation of elastic fibers established the diagnosis of PE as likely.

According to these considerations, Buecher [3] and Martinez [2] upheld that PE is a separate entity, different from nevus anelasticus and Buschke-Ollendorf syndrome. Moreover, Lewis et al. [4] in an extensive review of elastic fiber disorders, classified PE as a distinctive entity in acquired elastic tissue disorders.

In conclusion, we report a new case of papular elastorrhexis. We believe that PE is a distinctive entity with characteristic clinical and histopathological features.

Acknowledgements

References

2 Martínez W, Del Pozo J, Sacristán F, Fernández-Jorge B, Fonseca E. Popular elastorrhexis, a distinctive entity? Am J Dermatopathol 2008; 30: 188-90.

3 Buecher SA, Itin P. Papular elastorrhexis. Report of five cases. Dermatology 2002; 205: 198-200.

4 Lewis K, Bercovitch L, Dill S, Robinson-Boston L. Acquired disorders of elastic tissue: Part II. Decreased elastic tissue. J Am Acad Dermatol 2004; 51: 165-85.

5 Choonhakarn C. Jirarattanapochai. Papular elastorrhexis: a distinct variant of connective tissue nevi or an incomplete form of Buschke-Ollendorf syndrome? Clin Expl Dermatol 2002; 27: 454-7.

6 Schirren H, Schirren CG, Stolz W, Kind P, Plewig G. Papular elastorrhexis: a variant of dermatofibrosis lenticularis disseminata (Buschke-OllendorfSyndrome)? Dermatology 1994; 189: 368-72.