Original article
Syringoma: A clinicopathological study of 244 casesSyringomes : étude anatomoclinique de 244 cas

https://doi.org/10.1016/j.annder.2015.06.025Get rights and content

Summary

Background

Syringomas are benign tumours of the sweat glands, the most familiar clinical presentation of which is the presence of multiple lesions on the eyelids. The aim of our study was to determine the clinical and histological characteristics of a large series of patients and to examine anatomoclinical correlations.

Patients and methods

This was a retrospective study conducted in all of the cases of syringoma analysed at the cutaneous histopathology laboratory in Strasbourg between 1970 and 2008. The clinical elements, patient history and diagnostic data were collated. All slides were re-read in order to determine the microscopic characteristics of the lesions.

Results

Two hundred and forty-four lesions were included. The sex ratio was 0.27 and the mean age was 42 years (8 to 85 years). Multiple syringomas were noted in 76% of cases, of which 29.2% were eruptive, and one case occurred in a setting of metastatic melanoma. The sites of predilection were the face (56.7%, of which 36.3% on the eyelids), the chest (18.1%) and the neck (17.5%) for the multiple forms. The lesions were in the form of papules (67%), either brown (34.2%) or flesh-coloured (19.8%). Pruritus was reported in 14 cases, including 4 at vulvar sites (out of a total of 8). A diagnosis of syringoma was made by the clinician in only 30.2% of the multiple forms, with mastocytosis being proposed in 7.1% of cases. The clear-cell forms (18 cases) presented no special clinical features.

Conclusion

Syringomas are frequently multiple and are seen mainly in women. They are found predominantly on the face and trunk, and lesions are generally brown and pruritic, a little-known feature that accounts for the degree of diagnostic confusion with mastocytosis. The vulvar forms, which are often pruritic, are poorly known. The eruptive forms may include a hormonal component.

Résumé

Introduction

Les syringomes sont des tumeurs eccrines bénignes, dont l’aspect clinique le mieux connu est celui de lésions multiples des paupières. Le but de cette étude était de déterminer les caractéristiques cliniques et histologiques d’une grande série de cas et d’en étudier la corrélation anatomoclinique.

Matériel et méthodes

Il s’agit d’une étude rétrospective réalisée sur tous les cas de syringome analysés au laboratoire d’histopathologie cutanée de Strasbourg entre 1970 et 2008. Les éléments cliniques, anamnestiques et les diagnostics évoqués ont été recueillis. Toutes les lames ont été relues pour déterminer les caractéristiques microscopiques des lésions.

Résultats

Deux cent quarante-quatre lésions ont été incluses. Le sex-ratio était de 0,27 et l’âge moyen de 42 ans (8 à 85 ans). Les syringomes étaient multiples dans 76 % des cas, dont 29,2 % étaient éruptifs, avec un cas survenu au cours d’un mélanome métastatique. Les localisations préférentielles étaient le visage (56,7 %, dont 36,3 % aux paupières), le thorax (18,1 %) et le cou (17,5 %) pour les formes multiples. Il s’agissait de papules (67 %), de couleur brune (34,2 %) ou chair (19,8 %). Un prurit était signalé dans 14 cas, dont 4 cas de localisation vulvaire (sur 8 au total). Le diagnostic de syringome était évoqué par le clinicien dans 30,2 % des formes multiples seulement, une mastocytose étant proposée dans 7,1 % des cas. Les formes à cellules claires (18 cas) n’avaient pas de particularité clinique.

Conclusion

Les syringomes sont le plus souvent multiples et prédominent chez la femme. Leurs localisations électives sont le visage et le tronc et les lésions sont souvent brunes et prurigineuses, notion mal connue, expliquant la confusion avec une mastocytose. Les formes vulvaires, souvent prurigineuses, sont méconnues. Les formes éruptives pourraient avoir des déterminants hormonaux.

Section snippets

Patients and methods

This is a retrospective study concerning all of the samples submitted to the skin histology laboratory of the dermatology clinic at the university hospitals of Strasbourg between 1970 and 2008. Based on histological diagnosis of syringoma, the available clinical files and clinical data submitted alongside the samples at the time of the skin biopsies were analysed. The clinical data comprised: age at the time of examination, gender, clinical description of the lesions (semiology, site, whether

Results

For this 39-year period, we identified 254 samples corresponding to syringomas. For three of these samples, clinical data were absent or inconsistent and these cases were thus excluded. Seven patients underwent two biopsies for the same lesion and they were consequently counted only once. The total number of patients is thus 244. The number of cases per year ranged from 1 to 15: syringomas account for a mean 0.1% of samples each year (range: 0.02% to 0.25%, depending o the year).

Discussion

The specific value of this study is the large number of cases examined, with large patient populations being somewhat rare in this field: 29 cases in Italy in 1998 [12], 61 in Korea in 2007 [13], 27 in Spain in 2001 [14] and 34 in Iran in 2013 [15]. To our knowledge, this is the largest such worldwide study, and the only extant French study.

Our study is based on the analysis of rigorously performed examinations, reports and histopathology slides, which might have resulted in selection bias

Conclusion

To date, our study constitutes the largest worldwide series of syringomas. We confirm that the vulva constitutes a preferential site, and vulvar pruritus results. We also noted rare forms such as familial syringoma, and association with pregnancy and with metastatic melanoma. Brown colouring of lesions and pruritus are both common, and clinicians should be aware of this.

Disclosure of interest

The authors declare that they have no competing interest.

References (28)

  • P. Fernandez-Crehuet et al.

    [Generalized syringoma: a case study]

    Actas Dermosifiliogr

    (2007)
  • S.J. Friedman et al.

    Syringoma presenting as milia

    J Am Acad Dermatol

    (1987)
  • Y.H. Huang et al.

    Vulvar syringoma: a clinicopathologic and immunohistologic study of 18 patients and results of treatment

    J Am Acad Dermatol

    (2003)
  • Y. Scrivener et al.

    Morphologie des glandes sudorales

    Morphologie

    (2002)
  • B. Cribier

    Maladies associées aux tumeurs annexielles. II – Tumeurs sébacées, tumeurs sudorales

    Ann Dermatol Venereol

    (1999)
  • L. Langbein et al.

    New concepts on the histogenesis of eccrine neoplasia from keratin expression in the normal eccrine gland, syringoma and poroma

    Br J Dermatol

    (2008)
  • N.A. Obaidat et al.

    Skin adnexal neoplasms – part 2: an approach to tumours of cutaneous sweat glands

    J Clin Pathol

    (2007)
  • L. Jaquet et al.

    Hidradénomes éruptifs

    Ann Dermatol Venereol Syph

    (1887)
  • M. Teixeira et al.

    Eruptive syringomas

    Dermatol Online J

    (2005)
  • M.S. Henner et al.

    Solitary syringoma. Report of five cases and clinicopathologic comparison with microcystic adnexal carcinoma of the skin

    Am J Dermatopathol

    (1995)
  • C.L. Martyn-Simmons et al.

    Papular eruption on a patient with Down syndrome

    Arch Dermatol

    (2004)
  • L. Thomas et al.

    Syringomes et trisomie 21

    Ann Dermatol Venereol

    (1993)
  • M. Kavala et al.

    Vulvar pruritus caused by syringoma of the vulva

    Int J Dermatol

    (2008)
  • A. Patrizi et al.

    Syringoma: a review of twenty-nine cases

    Acta Derm Venereol

    (1998)
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