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Case report
peer-reviewed

Disseminated Syringomas of the Upper Extremities in a Young Woman



Abstract

Syringomas are benign, eccrine sweat gland tumors frequently found on the eyelids and neck in post-pubescent women and may present in healthy individuals or be associated with various medical comorbidities. We present a case of an otherwise healthy 19-year-old female with an abrupt onset of disseminated syringomas on the bilateral forearms and dorsal hands. Eruptive acral syringomas have not been previously reported in adolescents, and this diagnosis should be considered in patients presenting with a papular eruption on the hands and forearms.

Introduction

Syringomas are benign, adnexal tumors of unknown etiology that derive from eccrine sweat ducts [1]. They are most commonly located bilaterally on the inferior eyelids of post-pubescent women and present as multiple, white to yellow, 1 to 3-millimeter papules; on occasion, they are found in alternate locations such as the cheeks, axillae, abdomen, chest, and groin [2]. A clear cell variant, where cells have colorless cytoplasm due to glycogen build-up, is commonly associated with diabetes mellitus [3]. Eruptive syringomas are a less common presentation and may surface abruptly in adolescents, typically on the abdomen. Syringomas have increased incidence in patients with Down syndrome, those with Asian heritage, and in women [2]. Hormone levels are postulated to play a role in driving syringoma development given the increased incidence in women, with proliferation also noted during pregnancy, puberty, and the premenstrual period [4]. There are also reports of eruptive syringomas on medications such as carbamazepine and antiepileptic drugs. Syringomas are not known to have hereditary transmission but some familial occurrences have been reported [5]. Friedman and Butler developed four classifications of syringomas based on clinical features: localized, familial, eruptive, and a form associated with Down’s syndrome [6]. A healthy 19-year-old woman presented with eruptive acral syringomas—the first recorded case of this condition in an adolescent to our knowledge.

Case Presentation

A 19-year-old Hispanic female presented with complaints of an eruption of the hands and forearms that had started one year prior. She reported mild associated pruritus, which increased with exposure to natural sunlight. She denied the involvement of the head, trunk, lower extremities, or genitalia. She took no daily medications and had no chronic medical problems. There was no evidence of conditions associated with syringomas on history, physical exam, or workup. The physical exam revealed numerous, light brown, ovoid papules on the dorsal hands and fingers and on the dorsal and ventral surfaces of the forearms, with some areas of confluence on the lateral dorsal hands (Figures 1-3). Similar lesions were not observed elsewhere, including the groin, on the patient. A punch biopsy was obtained from the right forearm to establish the diagnosis and revealed a proliferation of small eccrine ductal structures lined by cuboidal cells within a fibrous stroma with an unremarkable epidermis, consistent with a diagnosis of syringoma. No cytologic atypia or significant infiltration of the deeper dermis by these ductal structures was appreciated (Figure 4). A limited laboratory analysis was conducted and revealed a normal complete blood count, as well as a glycated hemoglobin test of 5.2% (normal < 5.7%).

Discussion

To our knowledge, our case appears to be the first report of eruptive acral syringomas in an adolescent. Eruptive acral syringomas are extraordinarily rare with a total of 13 previous cases reported in the English-language medical literature (Table 1) [7]. Of these, the average patient age was 48 years, with the youngest report occurring in a 27-year-old and nine of the 13 were in females. The majority of these cases documented no associated disease, although several were associated with malignancy, including carcinoid tumor, melanoma, breast cancer, and promyelocytic leukemia. All 13 cases had an involvement of the upper extremities, and three cases also included involvement in the infraorbital and periorbital regions and the breast. The incidence of acral syringoma may be underreported since these lesions are generally small and asymptomatic and often go unnoticed by the patient and physician [3].

Year Sex Age Location Associated condition Author
1977 Male 31 Dorsal hands None Hughes and Apisarnthanarax [8]
1982 Female 35 Dorsal hands and infraorbital None Asai [9]
1982 Male 52 Forearms and dorsal wrist Clear cell acanthoma Van den Broek [10]
1989 Male 52 Ventral forearms Carcinoid tumor Berbis [11]
1990 Male 69 Ventral forearms and dorsal hands Basal cell carcinoma Metze [12]
1997 Female 43 Dorsal hands and feet Breast cancer Garcia [13]
1998 Male 43 Forearms and wrist None Patrizi [14]
1998 Female 60 Forearms and breast scar Breast tubular adenoma Patrizi [14]
2006 Female 43 Forearms Photosensitivity reaction Martin-Garcia [15]
2008 Female 28 Dorsal hands None Muniesa [16]
2009 Female 27 Dorsal phalanges None Koh [17]
2009 Female 41 Ventral forearms Periorbital trichoepitheliomas Balci [18]
2009 Female 44 Posterior forearms None Valdivieso-Ramos [19]
2015 Female 62 Ventral forearms and periorbital Promyelocytic leukemia Varas-Meis and Prada [7]
2018 Female 19 Dorsal hands and forearms None Patel, Lundgren, Ahmed, Soldano (present study)

Lesional specimens will typically exhibit a “paisley-tie” pattern in the superficial to mid-dermis, with tadpole-shaped ducts embedded in a red, sclerotic stroma and will exhibit positive staining against keratin, carcinoembryonic antigen, and S-100 proteins [3]. A differential diagnosis for the patient based on the clinical examination included hyperpigmented flat warts, lichen planus, atypical polymorphous light eruption, adnexal neoplasms, and acrokeratosis verruciformis of Hopf. Syringomas do not require treatment unless there are cosmetic or symptomatic concerns, and interventions include laser therapy, excision, cryotherapy, dermabrasion, topical atropine, and topical tretinoin, which have been used with variable results [4,7]. The diagnosis and treatment options were discussed with the patient, and she declined medical or surgical intervention. She remains in good health without the development of additional lesions in the six subsequent months following her initial presentation. Based on the short follow-up, there is a possibility that additional lesions will appear in other areas, particularly the groin in a female patient.

Conclusions

Our case demonstrates a unique example of a healthy, 19-year-old female with eruptive syringomas on the upper extremities. This diagnosis should be entertained in patients presenting with a papular eruption on the hands and forearms.


References

  1. Williams K, Shinkai K: Evaluation and management of the patient with multiple syringomas: a systematic review of the literature. J Am Acad Dermatol. 2016, 74:1234-1240. 10.1016/j.jaad.2015.12.006
  2. Resende C, Araújo C, Santos R, Pereira T, Brito C: Late-onset of eruptive syringomas: a diagnostic challenge. An Bras Dermatol. 2015, 90:239-241. 10.1590/abd1806-4841.20153899
  3. Ghanadan A, Khosravi M: Cutaneous syringoma: a clinicopathologic study of 34 new cases and review of the literature. Indian J Dermatol. 2013, 58:326. 10.4103/0019-5154.113956
  4. Vekić Mužević M, Bukvić Mokos Z, Lakoš Jukić I, Radoš J: Eruptive syringoma in the elderly: a case report. Acta Dermatovenerol Croat. 2015, 23:289-292.
  5. Wu WM, Lee YS: Autosomal dominant multiple syringomas linked to chromosome 16q22. Br J Dermatol. 2010, 162:1083-1087. 10.1111/j.1365-2133.2010.09677.x
  6. Lee JH, Chang JY, Lee KH: Syringoma: a clinicopathologic and immunohistologic study and results of treatment. Yonsei Med J. 2007, 48:35-40. 10.3349/ymj.2007.48.1.35
  7. Varas-Meis E, Prada-Garcia C, Samaniego-Gonzalez E, Rodrigriguez Prieto M: Acral syringomas associated with hematological neoplasm. Indian J Dermatol Venereol Leprol. 2017, 83:136. 10.4103/0378-6323.192961
  8. Hughes PS, Apisarnthanarax P: Acral syringoma. Arch Dermatol. 1977, 113:1435-1436. 10.1001/archderm.1977.01640100113023
  9. Asai Y, Ishii M, Hamada T: Acral syringoma: electron microscopic studies on its origin. Acta Derm Venereol. 1982, 62:64-68.
  10. van den Broek H, Lundquist CD: Syringomas of the upper extremities with onset in the sixth decade. J Am Acad Dermatol. 1982, 6:534-536. 10.1016/S0190-9622(82)80368-X
  11. Berbis P, Fabre JF, Jancovici E, Privat Y, Benderitter T: Late-onset syringomas of the upper extremities associated with a carcinoid tumor. Arch Dermatol. 1989, 125:848-849. 10.1001/archderm.1989.01670180120026
  12. Metze D, Jurecka W, Gebhart W: Disseminated syringomas of the upper extremities. Case history and immunohistochemical and ultrastructural study. Dermatologica. 1990, 180:228-235. 10.1159/000248036
  13. Garcia C, Krunic AL, Grichnik J, Viehman G, Clark RE: Multiple acral syringomata with uniform involvement of the hands and feet. Cutis. 1997, 59:213-214, 216.
  14. Patrizi A, Neri I, Marzaduri S, Varotti E, Passarini B: Syringoma: a review of twenty-nine cases. Acta Derm Venereol. 1998, 78:460-462.
  15. Martín-García RF, Muñoz CM: Acral syringomas presenting as a photosensitive papular eruption. Cutis. 2006, 77:33-36.
  16. Muniesa C, Fortuño Y, Moreno A, González J: Papules on the dorsum of the fingers. Actas Dermosifiliogr. 2008, 99:812-813. 10.1016/S1578-2190(08)70371-8
  17. Koh MJ: Multiple acral syringomas involving the hands. Clin Exp Dermatol. 2009, 34:e438. 10.1111/j.1365-2230.2009.03462.x
  18. Balci DD, Atik E, Altintas S: Coexistence of acral syringomas and multiple trichoepitheliomas on the face. J Cutan Med Surg. 2009, 13:169-171.
  19. Valdivielso-Ramos M, de la Cueva P, Gimeno M, Hernanz JM: Acral syringomas. Actas Dermosifiliogr. 2010, 101:458-460. 10.1016/S1578-2190(10)70674-0
Case report
peer-reviewed

Disseminated Syringomas of the Upper Extremities in a Young Woman


Author Information

Kavina Patel Corresponding Author

Dermatology, University of Texas Health Science Center, San Antonio, USA

Ashley D. Lundgren

Dermatology, Dell Medical School - The University of Texas, Austin, USA

Ammar M. Ahmed

Dermatology, Dell Medical School - The University of Texas, Austin, USA

Anthony C. Soldano

Dermatology, Clinical Pathology Associates, Austin, USA


Ethics Statement and Conflict of Interest Disclosures

Human subjects: Consent was obtained by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.


Case report
peer-reviewed

Disseminated Syringomas of the Upper Extremities in a Young Woman


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