Congenital desmoplastic trichoepithelioma.

Area of Science:

• Dermatology
• Pediatric Oncology
• Pathology

Background:

• Desmoplastic trichoepithelioma (DT) is a rare benign adnexal neoplasm.
• It typically presents as an asymptomatic plaque, most commonly on the face.
• Previous reports indicate adult onset, with a wide age range.

Observation:

• This case details a female infant born with congenital desmoplastic trichoepithelioma.
• The infant presented with widespread erythematous plaques and milia-like lesions on the scalp, face, and neck.
• Areas of atrophic scarring were also noted.

Findings:

• Histology and immunohistochemistry confirmed the diagnosis of desmoplastic trichoepithelioma.
• This represents the first documented instance of congenital DT.
• The lesions exhibited characteristics consistent with DT, despite the congenital presentation.

Implications:

• This case expands the known clinical spectrum of desmoplastic trichoepithelioma.
• It highlights the possibility of congenital presentation, requiring consideration in neonatal dermatology.
• Further research may elucidate the etiology and long-term prognosis of congenital DT.