Dermatology

Expanding the clinical spectrum of dermal hyperneury. Report of nine new cases and review of literature (2019)

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Type of publication:
Journal article

Author(s):
Ieremia, Eleni; Marušić, Zlatko; Mudaliar, Vivek; *Kelly, Susan; Gonzalvo Rodriguez, Pablo; McNiff, Jennifer M; LeBoit, Philip E; Calonje, Eduardo

Citation:
Histopathology; Nov 2019; vol. 75 (no. 5); p. 738-745

Abstract:
AIMS Dermal hyperneury is defined as the hypertrophy of small nerves in the dermis. It has been described in a variety of settings. We present a series of nine new cases with distinctive clinical presentation and review the existing literature. The aim of the study is to summarise the clinical, histopathological and immunohistochemical findings in a case series of dermal hyperneury with unique clinical presentation. METHODS AND RESULTS Nine cases were identified from the referral practice of one of the authors. Clinical characteristics, including demographic details were collated. The histopathological features and novel immunohistochemical findings were analysed. Four cases presented with multiple skin lesions. Clinical evaluation revealed no associated syndromic stigmata. The histology in all cases was that of dermal hyperneury. Immunohistochemistry for phosphatase and tensin homolog (PTEN) and RET was supportive of the lack of syndromic association. CONCLUSION The presentation of dermal hyperneury with multiple cutaneous lesions and no syndromic associations is distinctive and the study with PTEN and RET immunohistochemistry is previously undescribed. Comparison to recent reports of multiple nonsyndromic mucocutaneous neuromas is discussed.

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The spectrum of dermal hyperneury. Report of six cases (2014)

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Type of publication:
Conference abstract

Author(s):
Ieremia E., *Mudaliar V., *Kelly S., Grech B., Rodriguez P., Martin B., Calonje E.

Citation:
British Journal of Dermatology, July 2014, vol./is. 171/(90), 0007-0963 (July 2014)

Abstract:
Dermal hyperneury is defined as the presence of increased and hypertrophic myelinated and nonmyelinated nerve fibres in the dermis. Cutaneous nerve hyperplasia is rare and can be seen in lesional skin in multifocal or localized forms. When multifocal, it can be present in a pure cutaneous or mucocutaneous form or it may have syndromic associations. It is fascinating that it is present in the normal skin of patients with multiple endocrine neoplasia type 2b (MEN2b) and Cowden syndrome, but also in the lesional skin of those patients, as well as in neurofibromatosis (type 2), attenuated forms of MEN2b and in medullary thyroid carcinoma with macular amyloidosis. Localized, it may be encountered in areas of trauma, nodular prurigo, notalgia paraesthetica, neurocristic hamartoma and rarely in cases of chronic rubbing/scratching (Schaffer JV, Kamino H, Witkiewitcz A et al. Mucocutaneous neuromas. An underrecognised manifestation of PTEN hamartoma- tumor syndrome. Arch Dermatol 2006; 142: 625-32; Winkelmann RK, Carney JA. Cutaneous neuropathology in multiple endocrine neoplasia, type 2b. J Invest Dermatol 1982; 79: 307-12). We present six cases spanning through the spectrum of conditions described. We describe four patients with multiple cutaneous papules, variably symptomatic. Extensive investigations did not reveal any syndromic associations. Furthermore, we include two localized forms: one case of notalgia paraesthetica and one case of trauma. It is interesting to note that PTEN and RET mutations seen in Cowden and MEN2b syndromes, respectively, are implicated in common pathways of the growth and development of neural-crestderived and nerve tissue. We would like to propose dermal hyperneury as a distinct rare entity specifically in those cases presenting with multiple lesions confined to the skin and no syndromic stigmata, and therefore, no associated risk of malignancy.

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