Korean J Dermatol.  2020 May;58(4):260-264.

Multiple Cutaneous Plexiform Schwannomas as the First Symptom of Neurofibromatosis Type 2

• Lee MY
• Lee JB
• Lee SC
• Won YH
• Yun SJ

Abstract

Neurofibromatosis type 2 is a rare autosomal dominant disease. Vestibular schwannoma, which causes hearing loss, tinnitus, and imbalance, is the hallmark of the disease and is often the first symptom of neurofibromatosis type 2. Interestingly, children with neurofibromatosis type 2 can present with skin and central nervous system tumors other than vestibular schwannoma. A 10-year-old boy visited our clinic with multiple skin tumors that had been present since he was 1-year-old. Initially, his features did not fulfill the diagnostic criteria of neurofibromatosis type 2, but brain tumors subsequently developed within 2 years. Here, we report a case of neurofibromatosis type 2, that initially presented with multiple cutaneous plexiform schwannomas.

Keyword

Neurofibromatosis type 2; Plexiform schwannoma