Papular elastorrhexis located on occipito-cervical and mandibular regions

ARTICLE

Auteur(s) : Cheng Tan¹, Wen-Yuan Zhu², Zhong Sheng Min¹

¹Department of Dermatology, first Affiliated Hospital of Nanjing University of TCM, HanZhong Road, Jiang Su Province, Nanjing, China, 210029
²Department of Dermatology, first Affiliated Hospital of Nanjing Medical University, GuangZhou Road, Jiang Su Province, Nanjing, China

Papular elastorrhexis (PE) is characterized by non-follicular white papules distributed predominantly over the trunk and extremities [1]. The occipito-cervical and mandibular regions are novel sites of involvement.

A 32-year-old female was seen with a 3-week history of asymptomatic white macules and papules on the neck. She had no prior medical problems and denied antecedent acne or other histories of inflammatory skin conditions on the area involved. Family members had no similar lesions. Physical examination revealed approximately 20 non-follicular white macules and papules on the occipito-cervical and mandibular regions. These lesions were 2 mm ~ 4 mm in diameter, slightly indurated on palpation and round to polygonal (figures 1A and 1B). There was no tendency to coalesce. Pathologically, mild acanthosis was shown, and a perivascular lymphocytic infiltration in the dermis. Basilar vaculor degeneration was absent. Collagen fibers increased slightly in the papillary dermis. Ocein stains demonstrated a significant focal decrease and fragmentation of elastic tissue (figures 1C and D). Laboratory investigations demonstrated normal complete blood count and serum biochemistry tests. Radiographic survey of hands was unremarkable. A diagnosis of PE was finally made. No treatment was introduced due to the asymptomatic feature of the lesions.

To date, about 18 cases of PE (including this one) have been reported in the English literature [1-5]. Most of them were females in the second decade of life. PE presents as multiple, 1- to 5-mm white papules without predilection for the follicular areas. They are slightly indurated on palpation and are scattered predominantly on the trunk and, to a lesser extent, on the limbs. There is no tendency to group, or merge into plaques [2, 3]. The most prominent histopathological changes of PE are focal areas of collagen homogenization with diminution and fragmented elastic fibers within the dermis [4, 5].

Our case was interesting mainly for its aspect [1]. Besides typical white papules, almost half the lesions were hypopigmented firm macules [2]. Most lesions were typical oval to round in shape, a few were polygonal, which has never been reported [3]. The lesions were on the occipito-cervical and mandibular regions, which are new sites of involvement. PE may be underestimated with such variable clinical presentations. We propose the following diagnostic principles and hope it will be helpful for the ultimate diagnosis of PE: (1) Lesions are multiple, discrete, tiny, non-follicular, white papules or macules, their morphology varies from oval to round and sometimes stellated [2]. The trunk and extremities are preferentially affected. Occipito-cervical and mandibular areas may also be involved [3]. Decreased and fragmented elastic fibers are prominent in the dermis, occasionally there is slight focal homogenization of collagen [4]. Osteopoikilosis is excluded.

The prominant fragmentation of the elastic fibers, the absence of osteopoikilosis and non-familial involvement in our patient make the diagnosis of Buschke-Ollendorff syndrome unlikely. Pseudoxanthoma elasticum-like papillary dermal elastolysis (PXEPDE) shows a loss of elastic networks in the papillary dermis. Papules present in PXEPDE are yellow or skin-colored in contrast with whitish in PE. Our case can be easily differentiated from mid-dermal elastolysis (MDE) considering its circumscribed fine wrinkling and discrete perifollicular papular protusions. Moreover, our patient could be differentiated from papular acne scars, white fibrous papulosis of the neck and morphea guttate. The histological features of all of these diseases are distinct from those of PE.

There is no consensus whether PE is a unique entity or just a special demonstration of nevus anesticus, eruptive collagenoma and a mild form of Buschke-Ollendorff syndrome [6]. We believe our patient adds another case supporting PE as a unique entity, according to Del Pozo et al. [4]. No systemic associations have been described with PE to date. Furthermore, there is no reliable treatment for PE.

Acknowledgements

References

2 Ryder HF, Antaya RJ. Nevus anelasticus, papular elastorrhexis, and eruptive collagenoma: clinically similar entities with focal absence of elastic fibers in childhood. Pediatr Dermatol 2005; 22: 153-7.

3 Lee SH, Park SH, Song KY, et al. Papular elastorrhexis in childhood improved by intralesional injections of triamcinolone. J Dermatol 2001; 28: 569-71.

4 Del Pozo J, Martínez W, Sacristán F, et al. Papular elastorrhexis, a distinctive entity? Am J Dermatopathol 2008; 30: 188-90.

5 Buechner SA, Itin P. Papular elastorrhexis. report of five cases. Dermatology 2002; 205: 198-200.

6 Assmann A, Mandt N, Geilen CC. Buschke-Ollendorff syndrome--differential diagnosis of disseminated connective tissue lesions. Eur J Dermatol 2001; 11: 576-9.