Connective tissue nevus masquerading as a localized linear epidermal nevus.

Area of Science:

• Dermatology
• Pathology
• Histopathology

Background:

• Atypical localized linear epidermal nevus is a rare congenital skin condition.
• Accurate diagnosis is crucial for appropriate management and to rule out other conditions.

Observation:

• A 22-year-old female presented with hyperpigmented, verrucous papules on her left arm.
• Clinical diagnosis was atypical localized linear epidermal nevus due to soft lesions.
• Histopathological examination revealed fibroblastic proliferation and dermal fibrosis.

Findings:

• Histopathology showed epidermal changes (hyperkeratosis, hypergranulosis, papillomatosis).
• Findings were consistent with connective tissue nevus with epidermal changes.
• Dermal fibrosis and fibroblastic proliferation were significant.

Implications:

• Deep biopsy is recommended for suspected atypical linear epidermal nevus.
• This aids in differentiating from connective tissue nevus.
• Accurate histopathological diagnosis is vital for patient care.