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A Firm Plaque on the Occipital Scalp

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

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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Article Information

Corresponding Author: Hongxiao Chen, MD, Department of Dermatology, Linyi People’s Hospital, No. 27, Jiefang Road, Lanshan District, Linyi, China, 276003 (hongxiaochen@126.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.

References
1.
Northrup  H, Krueger  DA; International Tuberous Sclerosis Complex Consensus Group.  Tuberous sclerosis complex diagnostic criteria update: recommendations of the 2012 International Tuberous Sclerosis Complex Consensus Conference.  Pediatr Neurol. 2013;49(4):243-254. doi:10.1016/j.pediatrneurol.2013.08.001PubMedGoogle ScholarCrossref
2.
Jóźwiak  S, Schwartz  RA, Janniger  CK, Michałowicz  R, Chmielik  J.  Skin lesions in children with tuberous sclerosis complex: their prevalence, natural course, and diagnostic significance.  Int J Dermatol. 1998;37(12):911-917. doi:10.1046/j.1365-4362.1998.00495.xPubMedGoogle ScholarCrossref
3.
Oyerinde  O, Buccine  D, Treichel  A,  et al.  Fibrous cephalic plaques in tuberous sclerosis complex.  J Am Acad Dermatol. 2018;78(4):717-724. doi:10.1016/j.jaad.2017.12.027PubMedGoogle ScholarCrossref
4.
Leung  AK, Robson  WL.  Tuberous sclerosis complex: a review.  J Pediatr Health Care. 2007;21(2):108-114. doi:10.1016/j.pedhc.2006.05.004PubMedGoogle ScholarCrossref
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Volpi  A, Sala  G, Lesma  E,  et al.  Tuberous sclerosis complex: new insights into clinical and therapeutic approach.  J Nephrol. 2019;32(3):355-363. doi:10.1007/s40620-018-0547-6PubMedGoogle ScholarCrossref
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Bongiorno  MA, Nathan  N, Oyerinde  O,  et al.  Clinical characteristics of connective tissue nevi in tuberous sclerosis complex with special emphasis on shagreen patches.  JAMA Dermatol. 2017;153(7):660-665. doi:10.1001/jamadermatol.2017.0298PubMedGoogle ScholarCrossref
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Phillips  JC, Knautz  MA, Sangueza  OP, Davis  LS.  Familial cutaneous collagenoma.  J Am Acad Dermatol. 1999;40(2 Pt 1):255-257. doi:10.1016/S0190-9622(99)70199-4PubMedGoogle ScholarCrossref
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González-Vela  MC, Val-Bernal  JF, González-López  MA, Drake  M, Fernández-Llaca  JH.  Pure sclerotic neurofibroma: a neurofibroma mimicking sclerotic fibroma.  J Cutan Pathol. 2006;33(1):47-50. doi:10.1111/j.0303-6987.2006.00392.xPubMedGoogle ScholarCrossref
9.
Malissen  N, Vergely  L, Simon  M, Roubertie  A, Malinge  MC, Bessis  D.  Long-term treatment of cutaneous manifestations of tuberous sclerosis complex with topical 1% sirolimus cream: a prospective study of 25 patients.  J Am Acad Dermatol. 2017;77(3):464-472.e3. doi:10.1016/j.jaad.2017.04.005PubMedGoogle ScholarCrossref
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Franz  DN, Krueger  DA.  mTOR inhibitor therapy as a disease modifying therapy for tuberous sclerosis complex.  Am J Med Genet C Semin Med Genet. 2018;178(3):365-373. doi:10.1002/ajmg.c.31655PubMedGoogle ScholarCrossref
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