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CC BY-NC-ND 4.0 · Arq Neuropsiquiatr 2022; 80(04): 450-451
DOI: 10.1590/0004-282X-ANP-2021-0315
Images in Neurology

Atypical cutaneous presentation of tuberous sclerosis complex: Giant angiofibroma on the scalp

Apresentação cutânea atípica da esclerose tuberosa: angiofibroma gigante no couro cabeludo
Leonardo Furtado Freitas 
1   Universidade Federal de São Paulo, Departamento de Radiologia, São Paulo SP, Brazil.
,
Leomar Benicio Maia Segundo 
1   Universidade Federal de São Paulo, Departamento de Radiologia, São Paulo SP, Brazil.
,
Danilo Manuel Cerqueira Costa 
1   Universidade Federal de São Paulo, Departamento de Radiologia, São Paulo SP, Brazil.
,
Márcio Luís Duarte 
2   Universidade Federal de São Paulo, Departamento de Saúde Baseada em Evidências, São Paulo SP, Brazil.
,
Luís Antônio Tobaru Tibana 
1   Universidade Federal de São Paulo, Departamento de Radiologia, São Paulo SP, Brazil.
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Tuberous sclerosis (TSC) is an autosomal dominant neurocutaneous syndrome characterized by several abnormalities, including benign tumors of the embryonic ectoderm in multiple organs, such as skin, eyes, and central nervous system[1]. The main dermatological manifestations of TSC are hypochromic macules (ash leaf spots), facial angiofibromas, fibrous cephalic plaques, periungual fibroids, shagreen patch, and confetti lesions[2].

A 26-year-old woman presented with a giant angiofibroma with an atypical and rare symptom of TSC, the main symptom being the skin lesions ([Figures 1], [2], [3] and [4]). The giant and asymmetric form is described as a rare presentation in the literature[3].

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Figure 1Massive lesion of soft parts in the occipital region presenting fibroelastic consistency, compatible with giant angiofibroma.
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Figure 2Hyperchromic papule on the left forehead (A) and small hyperchromic papular lesions in the malar regions (B).
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Figure 3Typical lesions of tuberous sclerosis. In CT scans (A and B), it is possible to recognize subependymal nodules, some of which are calcified (arrow in A) and found in the topography of the left Monro foramen (arrow in B). Also, note the presence of giant occipital angiofibroma in these CT scans (A and B). FLAIR-weighted MRI image (C) showing evidence of hypersignal in the white and gray matters compatible with tubers.
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Figure 4CT (A) and MRI scans (B) showing a soft tissue lesion characterized by marked cutaneous thickening in the occipital region.

Authors’ contributions:

LFF, LATT, DMCC: manuscript composition; LBMS, MLD: manuscript review.




Publication History

Received: 09 August 2021

Accepted: 19 August 2021

Article published online:
31 January 2023

© 2021. Academia Brasileira de Neurologia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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