Syndrome In Question

Brooke-Spiegler syndrome is an autosomal dominant disorder with variable penetrance and expression. It is characterized by a genetic predisposition to develop multiple adnexal neoplasias: cylindromas, trichoepitheliomas, and trichoblastomas. We describe a 54-year-old male patient with cylindromas, trichoepitheliomas, and trichoblastoma.

Neoplasms, adnexal and skin appendage; Skin diseases, genetic; Skin neoplasms

Authorship

Fernanda Guedes Lavorato MAILING ADDRESS: Fernanda Guedes Lavorato, Boulevard 28 de Setembro, 77, Vila Isabel, 20551-030 Rio de Janeiro, RJ, Brazil. Email: fglavorato@yahoo.com.br

MD, dermatologist at private clinic - Rio de Janeiro (RJ), Brazil.MD, dermatologist at private clinic - Rio de Janeiro (RJ), Brazil.MD, dermatologist at private clinic - Rio de Janeiro (RJ), Brazil.

Marcela Duarte Benez Miller

MD, specialist in Dermatology, Teaching Hospital Pedro Ernesto - State University of Rio de Janeiro (Universidade do Estado do Rio de Janeiro) (HUPE-UERJ) - Rio de Janeiro (RJ), Brazil.State University of Rio de JaneiroBrazilRio de Janeiro, RJ, BrazilMD, specialist in Dermatology, Teaching Hospital Pedro Ernesto - State University of Rio de Janeiro (Universidade do Estado do Rio de Janeiro) (HUPE-UERJ) - Rio de Janeiro (RJ), Brazil.

Daniel Lago Obadia

MD, dermatologist - Assistant Professor of Dermatopathology, Dermatology Service, Teaching Hospital Pedro Ernesto - State University of Rio de Janeiro (Universidade do Estado do Rio de Janeiro - HUPE-UERJ). Professor, head of the discipline of Dermatopathology at the Tropical Dermatology Service, Central Army Hospital (Hospital Central do Exército - HCE) - Benfica (RJ), Brazil.State University of Rio de JaneiroBrazilBenfica, RJ, BrazilMD, dermatologist - Assistant Professor of Dermatopathology, Dermatology Service, Teaching Hospital Pedro Ernesto - State University of Rio de Janeiro (Universidade do Estado do Rio de Janeiro - HUPE-UERJ). Professor, head of the discipline of Dermatopathology at the Tropical Dermatology Service, Central Army Hospital (Hospital Central do Exército - HCE) - Benfica (RJ), Brazil.

Central Army Hospital, Tropical Dermatology ServiceCentral Army HospitalCentral Army Hospital, Tropical Dermatology Service

Natália Solon Nery

MD, dermatologist at private clinic - Brasília (DF), Brazil.MD, dermatologist at private clinic - Brasília (DF), Brazil.MD, dermatologist at private clinic - Brasília (DF), Brazil.

Roberto Souto da Silva

MD,Preceptor of the Dermatology Service, Teaching Hospital Pedro Ernesto - State University of Rio de Janeiro (Universidade do Estado do Rio de Janeiro - HUPE-UERJ). Researcher at the Laboratory of Histocompatibility and Criopreservation - State University of Rio de Janeiro (Universidade do Estado do Rio de Janeiro - HLA-UERJ) - Rio de Janeiro (RJ), Brazil.State University of Rio de JaneiroBrazilRio de Janeiro, RJ, BrazilMD,Preceptor of the Dermatology Service, Teaching Hospital Pedro Ernesto - State University of Rio de Janeiro (Universidade do Estado do Rio de Janeiro - HUPE-UERJ). Researcher at the Laboratory of Histocompatibility and Criopreservation - State University of Rio de Janeiro (Universidade do Estado do Rio de Janeiro - HLA-UERJ) - Rio de Janeiro (RJ), Brazil.

Laboratory of Histocompatibility and Criopreservation, State University of Rio de JaneiroState University of Rio de JaneiroLaboratory of Histocompatibility and Criopreservation, State University of Rio de Janeiro

MAILING ADDRESS: Fernanda Guedes Lavorato, Boulevard 28 de Setembro, 77, Vila Isabel, 20551-030 Rio de Janeiro, RJ, Brazil. Email: fglavorato@yahoo.com.br

Conflict of Interests: None.

SCIMAGO INSTITUTIONS RANKINGS

MD, dermatologist at private clinic - Rio de Janeiro (RJ), Brazil.MD, dermatologist at private clinic - Rio de Janeiro (RJ), Brazil.MD, dermatologist at private clinic - Rio de Janeiro (RJ), Brazil.

Central Army Hospital, Tropical Dermatology ServiceCentral Army HospitalCentral Army Hospital, Tropical Dermatology Service

MD, dermatologist at private clinic - Brasília (DF), Brazil.MD, dermatologist at private clinic - Brasília (DF), Brazil.MD, dermatologist at private clinic - Brasília (DF), Brazil.

Figures

Figures (3)

Thumbnail

FIGURE 1
A - Multilobular violaceous nodule in the left retroauricular region; B - erythematous papules and nodules on the scalp, with telangiectasias (turban aspect); C - normochromic papules on eyelids; nodule with a pearlaceous border and telangiectasias in the left nasal region; normochromic papule in the left nasogenian groove; D - normochromic papules on the back

Thumbnail

FIGURE 2
A: Cylindroma (HE 40x): base cell tumour arranged in islands in the dermis, some with sudoriparous ducts; 2B: Cylindroma (HE 400x): hyalinised cylinders surrounding each tumour island. 2C: Trichoepithelioma (HE 40x): islands of well demarcated basaloid tumour cells and some horn cysts; 2D: Trichoepithelioma (HE 100x): peripheral distribution nucleus palisades, without retraction artefact or loose stroma

Thumbnail

FIGURE 3
Trichoblastoma (HE 100x): Dermal proliferation of basaloid cells without atypical fibrotic stroma

FIGURE 1 A - Multilobular violaceous nodule in the left retroauricular region; B - erythematous papules and nodules on the scalp, with telangiectasias (turban aspect); C - normochromic papules on eyelids; nodule with a pearlaceous border and telangiectasias in the left nasal region; normochromic papule in the left nasogenian groove; D - normochromic papules on the back

FIGURE 2 A: Cylindroma (HE 40x): base cell tumour arranged in islands in the dermis, some with sudoriparous ducts; 2B: Cylindroma (HE 400x): hyalinised cylinders surrounding each tumour island. 2C: Trichoepithelioma (HE 40x): islands of well demarcated basaloid tumour cells and some horn cysts; 2D: Trichoepithelioma (HE 100x): peripheral distribution nucleus palisades, without retraction artefact or loose stroma

FIGURE 3 Trichoblastoma (HE 100x): Dermal proliferation of basaloid cells without atypical fibrotic stroma

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