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Authors: Dr Akshay Flora, Resident Medical Officer, Royal Prince Alfred Hospital, University of Sydney, Central Clinical School, Sydney, NSW, Australia; Dr Roger (Hyun Joon) Kim, Anatomical Pathology Registrar, Douglas Hanly Moir Pathology, Sydney, NSW, Australia. DermNet NZ Editor in Chief: Adjunct A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. Copy edited by Gus Mitchell. June 2020.
Epithelial sheath neuroma is an uncommon benign cutaneous tumour composed of dermal nerve fibres surrounded by squamous epithelium [1,2].
Epithelial sheath neuroma has so far only been reported in 13 people; all were older than 40 years of age. It is more common in females .
The pathogenesis of epithelial sheath neuroma has not been fully established. However, several possible mechanisms have been suggested.
Epithelial sheath neuroma presents as a persistent erythematous papule or nodule located on the upper or mid-back. Some patients report tenderness, pruritus, or paraesthesia when the lesion is palpated .
Epithelial sheath neuroma is diagnosed by histopathological examination of a skin biopsy. It is characterised by multiple enlarged peripheral nerve fibres that are sheathed by mature squamous epithelium. Epithelial sheath neuroma is sometimes surrounded by myxoid (mucus-like) stroma and a lymphocytic infiltrate.
Histology of epithelial sheath neuroma
The clinical differential diagnosis for epithelial sheath neuroma includes:
The histological differential diagnosis for epithelial sheath neuroma includes reactive neuroepithelial aggregates, perineural invasion of a well-differentiated cutaneous carcinoma, or after a previous biopsy [7,8].
Epithelial sheath neuroma is benign. The treatment of choice is excision .
To date, there have been no reports of recurrence after excision .
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