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August 2009

Annular and Keratotic Papules and Plaques in a Teenager—Diagnosis

Author Affiliations
 

MARY S.STONEMD

 

SOONBAHRAMIMDCARRIE ANN R.CUSACKMDSENAIT W.DYSONMDMOLLY A.HINSHAWMDVINCENTLIUMD

Arch Dermatol. 2009;145(8):931-936. doi:10.1001/archdermatol.2009.151-b

Scanning magnification of histologic sections revealed a focal area of epidermal acanthosis with overlying parakeratosis and, at a higher magnification, basophilic aggregates composed of collagen fibers, inflammatory cells, and swollen and clumped dermal elastin fibers present at the base of an epidermal channel. A Verhoeff–van Gieson stain highlighted the predominant component of the clumps to be abnormal, elastic fibers within the channel (Figure 3).

Elastosis perforans serpiginosa is a rare perforating disorder, which usually begins during adolescence or early adulthood with a male predominance of 4:1, and is characterized by extrusion of altered elastic fibers through the epidermis.1 Approximately one-third of cases occur in patients with genetic disorders, including Down syndrome, Ehlers-Danlos syndrome, osteogenesis imperfecta, Marfan syndrome, pseudoxanthoma elasticum, and Rothmund-Thomson syndrome.2 Penicillamine use may also cause EPS.3 Clinically, EPS presents as keratotic 2- to 5-mm papules, which tend to be arranged in serpiginous or annular patterns, most commonly on the lateral surface of the neck, face, or arms. Elastosis perforans serpiginosa tends to be symmetrically distributed, except in cases associated with Down syndrome or penicillamine use. Although most patients are asymptomatic, mild pruritus may be present. While EPS tends to spontaneously resolve, often within months or a few years, lesions may persist for up to 10 years. The lesions may heal with hypopigmented or atrophic scars.

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