Becker's nevus syndrome with quadriparesis

Area of Science:

• Dermatology
• Neurology
• Genetics

Background:

• Becker's nevus syndrome is a rare congenital disorder characterized by a spectrum of abnormalities.
• It is associated with various ectodermal and mesodermal defects, including neurological and musculoskeletal anomalies.

Observation:

• A 12-year-old female presented with quadriparesis, a hyperpigmented chest patch with hypertrichosis, musculoskeletal anomalies, limb asymmetry, and breast hypoplasia.
• Cranio-vertebral junction anomaly and cervical spina bifida occulta were identified via MRI.
• Skin biopsy confirmed findings consistent with Becker's nevus.

Findings:

• The patient was diagnosed with Becker's nevus syndrome based on clinical and radiological findings.
• Conservative management was chosen due to the unsuitability of surgical intervention for her specific presentation.

Implications:

• This case highlights the complex and variable presentation of Becker's nevus syndrome.
• Understanding the syndrome's manifestations is crucial for appropriate diagnosis and management planning.
• Further research into the genetic and developmental pathways of Becker's nevus syndrome is warranted.