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Case 335

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Male patient, 23 years old, presents hyperchromic lesions in the left dimidium and the cervical region, asymptomatic, evidenced at birth and showing progressive evolution with growth and darkening. He denies family history of similar lesions. He presented episodes of diarrhea and involuntary movimentation of the right dimidium since he was 16 years old, when diagnosed with right hemichorea.

Considering the clinical history and the presented images, what is the most likely diagnosis?

a) Lichen striatus

25%

b) Linear psoriasis

25%

c) Epidermal nevus syndrome

25%

d) Darier Disease

25%
   

Image analysis

Image 1: Hyperchromic and brownish macules and plaques in the left hemidimidium, following the Blaschko's lines.

 

Image 2: Hyperchromic, dark brown and well-defined plaque with irregular margins and verrucous surface in the anterior cervical region of the patient, measuring approximately 4,5 cm in its larger diameter. 

 

Image 3: Brownish and well-defined papules and plaques with irregular margins in the posterior region of the patient's left dimidium, following the Blaschko's lines.

 

Highlights

- Epidermal nevus associated with disorders of the central nervous, the ocular and/or the musculoskeletal systems characterize the epidermal nevus syndrome;

- 80% of the epidermal nevus syndrome are present at birth, affecting both sexes equally;

- They appear as linear macules or thin plaques along the Blaschko lines and they may darken and thicken throughout puberty;

- Seizures and intellectual disability are the most common systemic manifestations of the syndrome;

- The diagnosis is clinical, but skin biopsy is an option in cases of diagnostic doubt.

 

References

- Wright ST, Epidermal nevus and epidermal nevus syndrome. In: Uptodate.  Available in: https://www.uptodate.com/contents/epidermal-nevus-and-epidermal-nevus-syndrome?search=verrucous%20nevus%20syndrome&source=search_result&selectedTitle=2~150&usage_type=default&display_rank=2

- Síndrome do Nevo Epidérmico - Relato de um caso. Available in: http://www.jped.com.br/conteudo/99-75-04-287/port.pdf

- Cutaneous mosaicisms: concepts, patterns and classifications. Available in: http://www.scielo.br/pdf/abd/v88n4/0365-0596-abd-88-4-0507.pdf

 

Authors

Gabriel Sousa Santos, 5th year medical student at Universidade Federal de Minas Gerais.

Email: gabrielssantos86[at]gmail.com

 

Elaine Kimie Iwayama Ikematu, 5th year medical student at Universidade Federal de Minas Gerais.

Email: elaineiwayama[at]gmail.com

 

Supervisors

Professor Flávia Vasques Bittencourt, Dermatologist and Associated Professor at Universidade Federal de Minas Gerais, coordinator of the Dermatology Service at Hospital das Clínicas da UFMG.

Email: flaviavbi[at]gmail.com

 

Reviewers

Ariádna Andrade, Amanda Lauar, Professor Viviane Parisotto.

 

Translated by

Gabriella Yuka Shiomatsu, 4th year medical student at Universidade Federal de Minas Gerais.

Email: gabriellashiomatsu[at]gmail.com

 

Test question

(FHEMIG Dermatology Medical Residency - 2009)

The Blaschko lines are a characteristic pattern of linear distribution of skin lesions. About these lines, it is INCORRECT to affirm that:

a) They differ from the Langer lines which are minimum tension lines of the skin.

25%

b) They result from the mosaicism due to the lyonization phenomenon, the somatic mutations, the chromosome nondisjunction and chimerism.

25%

c) They result from the skin cells connection at embryogenesis, representing the division between normal cells and mutant cells populations.

25%

d) They correspond to the dermatomes correlating with the distribution of vascular and nervous structures.

25%

e)

25%
   

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