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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 a rare 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 have been older than 40 years of age. It has been reported most commonly in females .
The pathogenesis of epithelial sheath neuroma has not been determined, 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 may include:
The histological differential diagnosis for epithelial sheath neuroma includes reactive neuroepithelial aggregates, perineural invasion of a well-differentiated cutaneous carcinoma, or re-excision perineural invasion [7,8].
Epithelial sheath neuroma is benign.
The treatment of choice is excision, with no reports of recurrence after excision to date .
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