Basal cell carcinoma developing in a nevus flammeus.

Area of Science:

• Dermatology
• Oncology
• Genetics

Background:

• Sturge-Weber syndrome is a rare congenital disorder characterized by a port-wine stain (nevus flammeus) and neurological abnormalities.
• Basal cell carcinoma (BCC) is the most common form of skin cancer, typically arising on sun-exposed areas.
• Nevus flammeus, a type of birthmark, can sometimes be associated with underlying vascular malformations.

Observation:

• A 44-year-old male patient with Sturge-Weber syndrome presented with a basal cell carcinoma on his right cheek.
• The BCC was located at the site of a nevus flammeus, previously treated with grenz rays in childhood.
• The patient also had erythematous papulovesicular lesions on his upper trunk, diagnosed as transient acantholytic dermatosis.

Findings:

• The co-occurrence of nevus flammeus and basal cell carcinoma in this patient is exceedingly rare.
• This case adds to the minimal reported instances of this specific association.
• The presence of transient acantholytic dermatosis represents an additional dermatological finding in this complex case.

Implications:

• This case highlights a potentially under-recognized association between vascular malformations like nevus flammeus and the development of skin cancers.
• Further research may be warranted to explore any potential shared etiological factors or increased risk in patients with Sturge-Weber syndrome.
• Clinicians should maintain a high index of suspicion for skin malignancies in patients with extensive nevus flammeus, particularly those with underlying syndromes.