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White fibrous papulosis of the neck: The first South American case

  • Author(s): Moreira, Ângela Puccini;
  • Souza, Ângela Cristina Leitão de;
  • Sousa, Flávia Feijó;
  • Gutstein, Adriana;
  • Filho, Luiz Peres Quevedo;
  • Rochael, Mayra C;
  • Amaral, Vivian
  • et al.
Main Content

White fibrous papulosis of the neck: The first South American case
Ângela Puccini Moreira, Ângela Cristina Leitão de Souza, Luiz Peres Quevedo Filho, Mayra C Rochael, Vivian Amaral
Dermatology Online Journal 15 (1): 9

Universidade Federal Fluminense, Hospital Universitário Antônio Pedro - Serviço de Dermatologia. Niterói, Rio de Janeiro, Brasil. angelapuccini@bol.com.br

Abstract

A 69-year-old woman presented with white papules on her neck. These papules were multiple, white, discrete, asymptomatic, non-follicular, 2 to 3 mm in diameter, and located on both sides of the neck. On histopathological examination, there was a thickened and abnormal organization of collagen bundles in the papillary to mid-dermis and a decrease in the number of elastic fibers. This clinical and histological picture suggested the diagnosis of white fibrous papulosis of the neck. No treatment was given because of the benign nature of the disease. This is the first case report of this condition in South America.



Introduction

White fibrous papulosis of the neck (WFPN) was first described in 16 patients by Shimizu et al. in 1985 [1]. A few years later, the same authors reported this condition in a group of 32 Japanese patients [2]. Over the last 23 years other cases have been described in the literature [3-14]. Case reports have been made in Japan [1, 2], Iran [3], Italy [4, 8, 9, 10, 11], Saudi Arabia [5], France [7], China [13] and Korea [14]. This is the first case of WFPN report in South America.


Clinical synopsis

A 69-year-old woman from Rio de Janeiro presented with white papules on her neck that she had noted for over five years. These papules were numerous, 2 to 3 mm in diameter, grouped together, asymptomatic, non-follicular; they were located on both sides of her neck (Fig. 1). There was no family history of a similar skin condition and the patient had no underlying illness.


Figure 1
Figure 1. Numerous white papules on the neck

Physical examination and laboratory tests, including an ophthalmological examination, did not show any particular pathological process.

Histopathological examination of H and E and Weigert (elastic stain) stained slides, obtained from a papule on the lateral neck revealed no alteration of the epidermis, but thickened and abnormal organization of collagen bundles in the papillary to mid-dermis and a decrease in number of elastic fibers (Figs. 2 & 3).


Figure 2Figure 3
Figure 2. Thickened and abnormal organization of collagen bundles in the papillary to mid-dermis. (H&E, x40)
Figure 3. Staining shows a decrease in number of elastic fibers. (Weigert)

This clinical and histological picture suggested the diagnosis of white fibrous papulosis of the neck. No treatment was given; there has been no change in the lesions within an observation period of 12 months.


Discussion

White fibrous papulosis of the neck (WFPN) is characterized by multiple, confluent, small white papules on the sides and back of neck in elderly people [10]. The papules are asymptomatic, unassociated with hair follicles, and clearly demarcated; they tend to gradually increase in number [9, 14].

The lesions were characterized histologically by fibrosis of the papillary to mid-dermis [13]. In some cases, there is also a slight paucity of elastic tissue [9]. Ultrastructural changes consist principally of thickened collagen bundles and tightly compacted collagen fibrils. No significant morphological changes in the elastic fibers were observed [9].

The etiopathogenesis of this condition is unknown [10]. Future studies will be required to clarify if ultrastructural alterations are particular age-related features and/or the result of sun-related damage [9].

According to Shimizu et al. (1989) other disorders can be considered in the differential diagnosis, including acrochordons, anetoderma, trichodiscoma, dermatofibrosis lenticularis disseminata, postinflammatory scars, connective tissue nevi, pseudoxanthoma elasticum, eruptive vellus hair cysts, milia, eruptive xanthoma, and perifolicular elastolysis [2]. These conditions can be easily differentiated from WFPN by the age of onset, clinical appearance, preferred site, and histopathologic findings [10].

Rongioletti and Rebora (1992), described two cases of pseudoxanthoma elasticum-like papillary dermal elastolysis (PXELPDE) [8]. This condition shows a similar skin appearance to WFPN, but can be differentiated histologically by a total loss of the elastic fibers in the papillary dermis, absence of thickened collagen bundles in the papillary to mid-dermis, and no calcifications. Pseudoxanthoma elasticum-like papillary dermal elastolysis is very similar to WFPN and both conditions might be interpreted as an expression of photoaging. One study suggested grouping them as fibroelastolytic papulosis on the neck [8].


Conclusion

Considering WFPN as an age-related dermatosis, we think its prevalence is much underestimated at present. Although natural regression does not occur, treatment is not necessary because of the benign nature of the disease.

References

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2. Shimizu H, Kimura S, Harada T, Nishikawa T. White fibrous papulosis of the neck: a new clinicopathologic entity? J Am Acad Dermatol. 1989 Jun;20(6):1073-7. [PubMed]

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14. Kim HS,Yu DS,Kim JW. White fibrous papulosis of the neck. J Eur Acad Dermatol Venereol. 2007 Mar;21(3):419-20. [PubMed]

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