White fibrous papulosis of the neck

White fibrous papulosis of the neck (WFPN) is a relatively new and rare disease. It was first described by Shimizu et al. in 1985. WFPN is characterized by the appearance of multiple papules with a diameter of 2–3 mm, and not attached to hair follicles. They are smooth, ivory, and often confluent. Most often sides and back of the neck are affected, but lesions may also spread across the upper part of the trunk [1]. Differential diagnosis should include pseudoxanthoma elasticum due to nearly identical clinical presentation and many common features on histological examination. Causes of these both diseases have not been fully elucidated, but aging of the body and environmental factors play a role in their pathogenesis [2].
We present the case of a 57-year-old woman, a teacher with a several-year history of skin lesions on both sides of the neck. The patient denied prolonged exposure to sunlight as well as rubbing or scratching affected areas. Considering other diseases, she suffered from mild and well-controlled arterial hypertension treated in the cardiac clinic. She did not have any symptoms suggestive of vascular, gastro-intestinal, and ocular disorders; they were absent in other family members as well. Dermatological examination revealed numerous, discrete, whitish, papular lesions located predominantly on both sides of the neck and the nape (Figures 1). Biopsy performed 2 years before had shown no typical features of any of the dermatoses. The patient had not undergone any dermatological treatment. The current biopsy revealed a piece of skin with a slightly thinned epidermis and orthokeratosis; only in the central part, foci of mild acanthosis were present. The granular layer was visible along the entire length of the sampled tissue. In the dermis, scanty and diffuse lymphocytic infiltration with a tendency to gather around vessels was present. In the central part of the lesion, the presence of hair follicles and sebaceous glands with more abundant lymphocytic infiltration was visible (Figure 2). It is worth noting that in the papillary layer and in the upper part of the reticular layer, a thinning of collagen fibres without an increased vascular proliferation was present. The elastic fibre pattern assessed with elastic van Gieson (EVG) (Figure 3) and Fontana-Masson stain revealed a reduced number of those fibres (Figure 4). The presented patient has not started any of the proposed treatments for her skin lesions. She remains under observation...


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