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Background
This is a common benign tumor of the skin, derived from the sweat glands. It is most common on the head and neck area, often producing small bumps. Under the microscope, there are characteristic comma-shaped ducts in a fibrotic stroma.
OUTLINE
Disease Associations Pathogenesis Gross Appearance and Clinical Variants Histopathological Features and Variants Differential Diagnosis Prognosis Treatment Commonly Used Terms Internet Links
DISEASE ASSOCIATIONS CHARACTERIZATION DIABETES MELLITUS
PATHOGENESIS CHARACTERIZATION CLEAR CELLS Clear cells of eccrine glands in a patient with clear cell syringoma associated with diabetes mellitus.
Saitoh A, Ohtake N, Fukuda S, Tamaki K.
Department of Dermatology, Kambra Hospital, Yamanashi, Japan.
Am J Dermatopathol 1993 Apr;15(2):166-8 Abstract quote We report a patient who presented with clear cell syringomata concomitant with diabetes mellitus. Also prominent were increased numbers of clear cells within the eccrine glands.
This observation of increased numbers of clear cells in eccrine glands of diabetic patients may represent another skin finding that is related to diabetes mellitus.
GROSS APPEARANCE/
CLINICAL VARIANTSCHARACTERIZATION GENERAL
Syringoma: a review of twenty-nine cases.Patrizi A, Neri I, Marzaduri S, Varotti E, Passarini B.
Department of Clinical and Experimental Medicine, University of Bologna, Italy.
Acta Derm Venereol 1998 Nov;78(6):460-2 Abstract quote The purpose of our study was to identify the clinical characteristics, epidemiologic data and histologic features in 29 cases of syringoma with a duration of lesions prior to the observation between 1 and 25 years.
Only one patient complained of moderate itching. In two cases the lesion was solitary, in another the papules formed a lichenified plaque. In six patients only the eyelids were involved and in two patients a symmetrical localization on the forearms was observed. The other 18 patients showed generalized syringoma, 16 with an eruptive onset, 6 of which were familial. One of our cases showed lesions mimicking urticaria pigmentosa and two patients were affected by Down's syndrome.
In two cases, histopathology showed association between syringoma and a melanocytic naevus and in one patient with a solitary lesion a clear cell syringoma was observed.
VARIANTS DISSEMINATED
Multiple syringomas on the abdomen, thighs, and groin.Goyal S, Martins CR.
University of Maryland Medical Services, USA.
Cutis 2000 Oct;66(4):259-62 Abstract quote Syringomas are benign adnexal tumors that occur most commonly in women. They typically present as soft, flesh-colored to slightly yellow papules on the lower eyelids.
We present an unusual case of a healthy 33-year-old male with multiple, reddish brown syringomas located on the lower abdomen, thighs, and groin. Although these lesions can result in significant cosmetic disfigurement, treatment options are limited and generally disappointing.
ERUPTIVE SYRINGOMAS
'Eruptive syringoma': a misnomer for a reactive eccrine gland ductal proliferation?Guitart J, Rosenbaum MM, Requena L.
Department of Dermatology, North-western University Medical School, Department of Dermatology, Rush-Presbyterian Medical Center, Chicago, IL; and Fundacion Jimenez Diaz, Madrid, Spain.
J Cutan Pathol 2003 Mar;30(3):202-5 Abstract quote BACKGROUND: Syringomas have traditionally been categorized as benign neoplasms of the eccrine gland ductal epithelium. However, the variety of clinical presentations reported in the literature and some cases recently observed by the authors cast doubt upon the neoplastic nature of eruptive syringomas. Our goal is to challenge the traditional notion that eruptive syringomas are neoplastic lesions.
RESULTS: We observed two patients who presented with an eczematous process, which resolved leaving residual lesions. Biopsies of the late lesions showed features of eccrine syringoma. Yet a biopsy obtained from an incipient lesion in one of the cases showed a lymphocytic inflammatory reaction of the superficial portion of the eccrine duct resulting in tortuous hyperplastic changes.
CONCLUSION: Based on our observations, some of the so-called 'eruptive syringoma' may represent a hyperplastic response of the eccrine duct to an inflammatory reaction rather than a true adnexal neoplasm. We proposed the term 'syringomatous dermatitis' for such cases.
Eruptive syringoma: 27 new cases and review of the literature.Soler-Carrillo J, Estrach T, Mascaro JM.
Department of Dermatology, Hospital Clinic de Barcelona, Spain.
J Eur Acad Dermatol Venereol 2001 May;15(3):242-6 Abstract quote BACKGROUND: Eruptive syringomas are uncommon eccrine sweat gland tumours. Only 64 cases have been reported in the literature.
OBJECTIVE: Clinical findings of 27 patients with eruptive syringomas were reviewed over a 47-year period.
RESULTS: The tumours appeared as multiple yellow-brown-coloured papules localized on the neck, anterior trunk, axillae, shoulders, abdomen or pubic area. This disorder occurs more frequently among women, and is frequent in the prepubertal as well as in the postpubertal age. The diagnosis was not clinically suspected in most cases.
CONCLUSIONS: Eruptive syringomas have to be considered in differential diagnosis of papular dermatosis at any age.
VULVA
Vulvar syringoma: A clinicopathologic and immunohistologic study of 18 patients and results of treatment.Huang YH, Chuang YH, Kuo TT, Yang LC, Hong HS.
Departments of Dermatology and Pathology, Chang Gung Memorial Hospital.
J Am Acad Dermatol 2003 May;48(5):735-9 Abstract quote BACKGROUND: Syringoma of the vulva has been rarely reported. No effective treatment modality has been documented.
OBJECTIVE: The purpose of this study was to describe the clinicopathologic features of vulvar syringoma, to investigate the hormonal influence on its growth, and to establish an effective treatment modality.
METHODS: A total of 18 cases of vulvar syringoma were selected from the surgical pathologic file at Chang Gung Memorial Hospital. In all, 15 formalin-fixed, paraffin-embedded specimens were examined by immunohistochemical staining for estrogen receptor and progesterone receptor.
RESULTS: The age of first presentation at our vulvar clinic ranged from 21 to 60 years with a median age of 29.5 years. Of patients, 13 (72%) had vulvar pruritus and 7 noticed aggravation during summer or during menstruation. The most common clinical appearance was multiple flesh-colored or brownish papules on bilateral sides of the vulva (9 of 18). One third of our series was found to have coexisting eyelid syringoma and 4 of them also had a family history of periorbital syringoma. Immunohistochemical stainings for estrogen receptor and progesterone receptor were all negative on the 15 cases studied. Of our patients, 7 with intense pruritus were treated with carbon dioxide laser vaporization. Their lesions resolved and pruritus subsided.
CONCLUSION: Vulvar syringoma is not very rare and should be considered in the differential diagnosis of vulvar pruritus. In our study, estrogen receptor and progesterone receptor were not detected in vulvar syringoma. Carbon dioxide laser was an effective therapeutic modality in treating patients with intractable symptoms.
HISTOLOGICAL TYPES CHARACTERIZATION GENERAL Comma shaped ducts lined by bland squamous cells, embedded in a fibrous stroma
Ductal lumina may contain keratinous debrisOccasional microcalcifications
VARIANTS MILIUM-LIKE SYRINGOMA
- Milium-like syringoma: a case study on histogenesis.
Wang KH, Chu JS, Lin YH, Hu CH, Lee WR.
Department of Dermatology, Taipei Municipal Wan-Fang Hospital, Department of Dermatology, Taipei Medical University Hospital, Department of Pathology, Taipei Medical University, and Graduate Institute of Medical Sciences, Taipei Medical University, Taipei, Taiwan.
J Cutan Pathol. 2004 Apr;31(4):336-340. Abstract quote
BACKGROUND: Milium-like syringoma is a variant of syringoma first described in 1987. Only few cases have been reported in the literature. It may be misleading clinically, and its histogenesis has not been clarified.
CASE REPORT: We present a case of periorbital milium-like syringoma, with studies on the histopathologic, histochemical, and immunohistochemical features.
RESULTS: Histology showed a large keratin-filled cyst in the upper portion of the lesion approximating the epidermis. Serial sections revealed that the cyst connected with the underlying syringomatous epithelial strands. Melanin was absent in the wall of the cyst, as demonstrated by Fontana-Masson stain. Cytokeratin 7 was expressed neither in the milia nor in the solid epithelial parts. Carcinoembryonic antigen (CEA) reactivity was seen in the luminal cells of the keratinous cysts. However, in the largest keratin-filled cyst clinically suggesting a milium, only the lower half of the cyst was positive for CEA. These results proved that the milia were part of syringoma with eccrine duct differentiation. Fusion of the upper half of the largest cyst with the epidermis may explain the absence of CEA positivity in this part, analogous with eccrine duct milia. Review of the literature indicated that this variant of syringoma occurs more often in Asians.
CONCLUSION: We present evidence, and propose the histogenesis of milium-like syringoma, that it is a variant of syringoma with a prominent cystic component showing features of eccrine duct milia. Recognition of its nature is of therapeutic significance. Further studies are required to verify its clinical characteristics as compared with ordinary syringomas.PLAQUE-TYPE
Department of Dermatology, University of Minnesota, Minneapolis, MN 55417, USA.
BACKGROUND: Plaque-type syringoma is a rare variant of syringoma. This benign neoplasm may be easily misdiagnosed as microcystic adnexal carcinoma (MAC), potentially resulting in unnecessary surgery with disfiguring consequences.
METHODS: We report two cases of plaque-type syringoma that were initially diagnosed as MAC. Microscopically, these lesions were composed of nests of cuboidal cells arrayed within sclerotic collagen in the upper dermis. The deep reticular dermis was spared. No perineural involvement was observed.
RESULTS AND CONCLUSIONS: Our cases are discussed in the context of histopathologic diagnosis. Detailed histopathologic findings of syringoma, as well as other considerations in the differential diagnosis, are reviewed. We also include a review of all cases of plaque-type syringoma published to date.
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES CHONDROID SYRINGOMA
Chondroid syringoma: a diagnosis more frequent than expected.Yavuzer R, Basterzi Y, Sari A, Bir F, Sezer C.
Department of Plastic and Reconstructive Surgery, Gazi University, Medical Faculty, Ankara, Turkey.
Dermatol Surg 2003 Feb;29(2):179-81 Abstract quote BACKGROUND: Chondroid syringoma or mixed tumor of the skin is a rare subcutaneous tumor that may be confused with various skin lesions.
OBJECTIVE: To elucidate the incidence of condroid syringomas among skin lesions that were excised under local anesthesia.
METHODS: The histopathologic diagnosis of 16,200 skin lesions that had been operated between 1986 and 2002 were retrospectively evaluated. The cases with condroid syringoma were histopathologically re-examined, and confirmed cases were further analyzed for preoperative diagnosis, age, gender, and lesion location.
RESULTS: Sixteen patients were found to have histopathologic diagnosis of chondroid syringoma constituting 0.098% of the excised skin lesions in this series. All of these 16 cases were misdiagnosed preoperatively. The typical presentation was a solitary skin lesion located in the head and neck region in a middle-aged male patient.
CONCLUSION: In the evaluation of a middle-aged male patient with a small subcutaneous nodule in the head and neck region, chondroid syringoma should be also taken into consideration for differential diagnosis. For such a lesion, excisional biopsy without destroying aesthetic and functional structures is the preferred diagnostic approach.
MICROCYSTIC ADNEXAL CARCINOMA
PROGNOSIS CHARACTERIZATION GENERAL Good, usually a cosmetic issue only
TREATMENT CHARACTERIZATION GENERAL LASER
The treatment of eruptive syringomas in an African American patient with a combination of trichloroacetic acid and CO2 laser destruction.Frazier CC, Camacho AP, Cockerell CJ.
Gold Coast Dermatology and Dermatologic Surgery Center, Bay St. Louis, Mississippi, USA.
Dermatol Surg 2001 May;27(5):489-92 Abstract quote BACKGROUND: Eruptive syringomas are uncommon benign adnexal neoplasms. They are numerous and disseminated and often have a predilection for the neck, face, chest, and axillary fossae. Because they are persistent, usually numerous, and often on exposed sites, the lesions may be disfiguring and often pose significant cosmetic concerns for patients. Many treatment modalities such as dermabrasion, electrodesiccation with curettage, and scissors excision have been tried with some success, but more recently lasers have provided good to excellent results.
OBJECTIVE: To describe an approach to the treatment of eruptive syringomas in an African American patient with a combination of trichloroacetic acid (TCA) and CO2 laser resurfacing, providing acceptable cosmetic results without significant side effects. METHODS: We describe an African American patient with eruptive syringomas of the face treated with a combination of TCA and CO2 laser resurfacing with good results.
RESULTS: While the syringomas were not completely ablated, the combination of TCA and CO2 laser resurfacing provided acceptable cosmetic results without significant side effects.
CONCLUSION: The TCA pretreatment probably removed some of the bulk of the surface of the lesions, thereby reducing the number of laser passes required to flatten the remainder of the lesions and thus lessening the potential for thermal damage at the treated sites and of surrounding normal skin.
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Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 6th Edition. McGraw-Hill. 2003.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fifth Edition. Mosby Elesevier 2008
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Last Updated June 19, 2008
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