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A man in his 20s presented with a 6-year history of multiple growing papules superimposed on an asymptomatic firm plaque on the occipital scalp. The lesions were never ulcerated or inflamed, but hemorrhage occurred occasionally after rubbing. At 3 years of age, scarlike macules appeared on the occipital scalp and coalesced to form a larger plaque with sparse hair. Simultaneously, multiple brown-red, dome-shaped papules developed on the nose and gradually evolved to involve the cheeks. Rubbery plaques also developed on the sacrococcygeal area, along with hypopigmented patches on the right buttock. The patient has no systemic symptoms or mental impairment and denied a family history of genetic disease.
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D. Fibrous cephalic plaque
One of the superimposed papules from the plaque demonstrated dense fibrosis with thick collagen bundles throughout the dermis and prominent decrease of hair follicles. In some areas, the fibroplasia was arranged in a whorled and onionskin pattern (Figure, B). Capillary vessel proliferation with ectasia between collagen bundles and scattered fibroblasts were noted (Figure, C), and perifollicular fibrosis with distortion of hair follicles was observed (Figure, D).
Fibrous cephalic plaques (FCPs) on the skin are among the diagnostic manifestations of tuberous sclerosis complex (TSC). Other features include facial angiofibromas, hypomelanotic macules, shagreen patches, and periungual fibromas. Fibrous cephalic plaques are included in the major diagnostic criteria for TSC according to the 2012 International TSC Consensus Conference.1 Fibrous cephalic plaques may be present at birth and often become more noticeable in early childhood.2 They are often located on the forehead but may also present on other parts of the face, scalp, or neck. Lesions can be solitary or multiple. According to a recent study, a significant portion (31%) of FCPs occur on the scalp.3 Fibrous cephalic plaques appear as rubbery-to-firm, smooth-to-bumpy, skin-colored, pink, red, or brown plaques. When present on the scalp, decreased hair density can be noted. Most lesions are asymptomatic and rarely cause bleeding or pain.
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Corresponding Author: Hongxiao Chen, MD, Department of Dermatology, Linyi People’s Hospital, No. 27, Jiefang Road, Lanshan District, Linyi, China, 276003 (email@example.com).
Published Online: July 24, 2019. doi:10.1001/jamadermatol.2019.1972
Conflict of Interest Disclosures: None reported.
Additional Contributions: We thank the patient for granting permission to publish this information.
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