Case reports in dermatology · 2026
Squamous Cell Carcinoma of the External Auditory Canal in a Patient with Netherton Syndrome.
Клинический случайСтатьяНазвание и аннотация: Перевод в очереди
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Hayashi Misaki, Kumai Takumi, Ominato Hisataka, Sato Ryosuke, Wakisaka Risa, Hayashi Ryusuke, Kono Michihisa, Yamaki Hidekiyo, Ohara Kenzo, Kishibe Kan, Takahara Miki
Аннотация
INTRODUCTION: Netherton syndrome is a rare autosomal recessive disorder characterized by congenital ichthyosis, hair shaft abnormalities, atopic diathesis, and a broad spectrum of immunologic dysfunction. Although cutaneous malignancies are uncommon, affected individuals may develop early-onset tumors that can pose diagnostic and therapeutic challenges.
CASE PRESENTATION: We report the case of a 33-year-old woman with Netherton syndrome who presented with persistent left otorrhea and a granulomatous mass in the external auditory canal. Imaging demonstrated a soft-tissue lesion with focal bony erosion and suspected dural involvement, accompanied by fluorodeoxyglucose uptake in a cervical lymph node. Biopsy confirmed squamous cell carcinoma (SCC), staged as T4N1M0. The patient underwent superselective intra-arterial cisplatin infusion combined with radiotherapy, resulting in complete remission of both the primary lesion and nodal metastasis, with no evidence of recurrence over 4 years. Including this case, 9 patients with Netherton syndrome and cutaneous malignancies have been reported, with tumors frequently arising at a young age and showing variable HPV status. Interpretation of SCC antigen may be complicated by elevations associated with atopic dermatitis.
CONCLUSION: This case highlights the elevated risk of cutaneous malignancies in Netherton syndrome and underscores the need for long-term surveillance and multidisciplinary care.
CASE PRESENTATION: We report the case of a 33-year-old woman with Netherton syndrome who presented with persistent left otorrhea and a granulomatous mass in the external auditory canal. Imaging demonstrated a soft-tissue lesion with focal bony erosion and suspected dural involvement, accompanied by fluorodeoxyglucose uptake in a cervical lymph node. Biopsy confirmed squamous cell carcinoma (SCC), staged as T4N1M0. The patient underwent superselective intra-arterial cisplatin infusion combined with radiotherapy, resulting in complete remission of both the primary lesion and nodal metastasis, with no evidence of recurrence over 4 years. Including this case, 9 patients with Netherton syndrome and cutaneous malignancies have been reported, with tumors frequently arising at a young age and showing variable HPV status. Interpretation of SCC antigen may be complicated by elevations associated with atopic dermatitis.
CONCLUSION: This case highlights the elevated risk of cutaneous malignancies in Netherton syndrome and underscores the need for long-term surveillance and multidisciplinary care.