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CASE REPORT
Year : 2014  |  Volume : 3  |  Issue : 4  |  Page : 270-272

Eruptive syringoma - A great mimicker: An uncommon presentation in males


Department of Dermatology, Raja Rajeswari Medical College and Hospital, Kambipura, Bengaluru, Karnataka, India

Date of Web Publication16-Oct-2014

Correspondence Address:
Yadalla Hari Kishan Kumar
Department of Dermatology, 70, Padma Nivasa, Skin Care Clinic, 3rd Cross MG Extension, HV Halli, Raja Rajeswari Nagar, Bengaluru - 560 098, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2278-344X.143073

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  Abstract 

Syringomas are common benign neoplasms of the eccrine glands. It, usually, affects adult females with its usual presentation; small smooth skin-colored or yellowish papules with flattened or rounded tops on the face, especially in the periorbital area particularly around the lower eyelid. Eruptive syringoma, a distinctive form of syringoma presents as successive crops of numerous, disseminated papules appearing in atypical sites. There are a few reports of its widespread distribution as eruptive syringoma in the literature. Herein, we report an uncommon presentation in a teenage boy aged 20 years presenting with multiple asymptomatic, skin-colored papules predominantly over the trunk clinically mimicking numerous conditions presenting similarly. Histopathology showed multiple ducts and solid nest or cords of epithelial proliferation within the dermis, embedded in fibrous stroma. Some ducts had comma-like extensions or tadpole-like appearance confirming a diagnosis of eruptive syringomas.

Keywords: Eruptive, males, syringoma


How to cite this article:
Kumar YH, Keethi S. Eruptive syringoma - A great mimicker: An uncommon presentation in males. Int J Health Allied Sci 2014;3:270-2

How to cite this URL:
Kumar YH, Keethi S. Eruptive syringoma - A great mimicker: An uncommon presentation in males. Int J Health Allied Sci [serial online] 2014 [cited 2019 Sep 20];3:270-2. Available from: http://www.ijhas.in/text.asp?2014/3/4/270/143073


  Introduction Top


The word syringoma is derived from the Greek word syrinx meaning pipe or tube. The term syringoma refers to a group of benign adnexal neoplasm with a tendency to ductal (acrosyringeal) differentiation. Though, adult women are most commonly affected, can occur in both the sexes. [1] Although occasionally solitary, the lesions are, usually, multiple and may be present in a great number. Clinically lesions are small, firm, smooth, skin-colored or slightly yellowish, 1-2 mm papules and tend to occur in the periorbital area, particularly around the lower eyelid. [1] In eruptive syringoma, a rare variant first described by Jacquet and Darier in 1887, the lesions occurred in successive crops on the anterior chest, neck, upper abdomen, axillae, and the periumbilical region at puberty or during childhood. They may remain stationary throughout the life or may disappear spontaneously. There is also a familial variant. There are only few reports of its widespread distribution as eruptive syringoma in the literature. [2],[3],[4],[5],[6] We, therefore, report this rare variant in a teenage male.


  Case report Top


A 19-year-old male, an engineering student, presented with multiple asymptomatic, skin-colored lesions of 2 years duration with increasing cosmetic disfigurement. Lesions started erupting over the neck, spreading to the chest, both shoulders, and of recent onset minimal lesions over chin and infra-orbital area of the face. Patient was not on any medication. Family history was unremarkable. Examination revealed skin-colored papules in the above-mentioned areas of sizes varying from 1 to 4 mm [Figure 1]. Clinically, a differential diagnosis of acneiform eruptions, steatocystoma multiplex, disseminated granuloma annulare, plane warts, and syringoma were considered.
Figure 1: Numerous skin-colored papules of varying sizes over neck, chest, and shoulders suggestive of eruptive syringoma

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Histopathological examination revealed normal epidermis. Upper and mid dermis showed numerous small ducts embedded in fibrous stroma. The ducts are lined by two rows of epithelial cells with occasional vacuolations in the inner row. The lumina of these ducts contain amorphous debris. Some of the ducts possess small, comma-like tails of epithelial cells. Also seen are strands of basophilic epithelial cells close to the ducts [Figure 2]. The histopathological findings were consistent with the clinical diagnosis of syringoma.
Figure 2: Histopathology showing numerous tubular structures in dermis, embedded in a collagenous stroma
and lined by two rows of epithelial cells resembling typical tadpole body of sryingoma (H and E, ×100)


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  Discussion Top


Syringomas are benign adnexal tumor originating from intraepidermal portion of eccrine sweat duct. Initial description of syringoma was given by Kaposi, [1] later on Jacquet and Darier [2] described the eruptive form. Since its description, few cases have been reported. [3] Syringoma has been classified into four variants: Localized, lesions associated with Down's syndrome, generalized eruptive syringoma and a familial autosomal dominant form. [1],[6] Women are affected more commonly. [1] Clinically, lesions are small, firm, smooth, skin-colored or slightly yellowish, 1-5 mm papules tending to occur in the periorbital area, particularly around the lower eyelid. They can also develop in other anatomical sites such as thigh, axilla, abdomen, and vulva. Most commonly, they occur around puberty or in third to fourth decade. [1],[4] In cases of eruptive syringomas there are successive crops of numerous disseminated papules, with a predilection to occur over the anterior trunk, that is, on anterior aspect of the neck, chest, trunk, axilla, and inner aspect of upper arm and around axilla. [3],[4] There is an association of eruptive syringoma with heat stimuli. [5] There have been reports of unilateral, nevoid, bathing trunk, and generalized distribution of syringoma. [6]

Estrogen and progesterone receptors have been detected within syringoma with histochemical study. This finding may explain why syringomas are more common in females and peak incidence during puberty. [7] The aim of this presentation was to highlight this uncommon entity occurring in males around puberty.

On histopathology of syringoma, epidermis is unremarkable. Upper and mid dermis show multiple ducts and small solid epithelial nests, cords, or tubules embedded in a sclerotic stroma. Lumina of the ducts are filled with amorphous debris. Ducts are lined by two rows of flat epithelial cells. Some of the ducts possess small, comma-like tails of epithelial cells giving them the appearance of tadpole. [8] Cells of epithelial proliferation had pale, eosinophilic cytoplasm and rounded monomorphic nucleus. Histochemical studies have shown that all eccrine type of enzymes and glycogen are present in the tumor cells of syringoma. Eccrine-specific monoclonal antibody positively stains syringoma lesions. Hence, although formerly thought to be of mixed origin, now syringoma is considered to be a benign appendage tumor of intra-epidermal eccrine sweat duct. [8]

Clinical diagnosis of syringomas is often difficult, due to a similar presentation of widespread papular eruptions such as lichen planus, flat warts, papular mucinosis, xanthoma disseminatum, steatocystoma multiplex, Acneiform eruptions, and mastocytosis. Histopathology of the lesions often confirms the diagnosis with its definite classical findings.

Our patients sought medical advice because of cosmetic concern. Multiple treatment options are currently available including surgical excision, dermabrasion, and electrodessication with curettage, laser resurfacing, chemical peeling, cryotherapy, fulguration, oral and topical retinoids, and 1% topical atropine. But none of them are satisfactory, and recurrence is common because these tumors are situated deep in the dermis and are numerous in numbers. [9],[10],[11] We advised the patient topical tretinoin 0.05% cream for lesions over the chest and shoulders, whereas electrocautery was done for small new lesions which started over the chin and infra-orbital areas. The prognosis of the disease of possible recurrence was explained to the patient upon treatment, but later was lost for follow-up after initial treatment.

Syringomas occur more frequently in females on the face in a localized manner, but it is important to consider this as a differential diagnosis of any eruptive papular lesions occurring throughout the body. The specific histopathological features would clinch a final diagnosis and would be the most important diagnostic tool. This case is one of the few cases being reported, presenting with eruptive syringoma in male patients and not associated with medications. Most of the cases reported in male patients were familial, in pediatric age group, postwaxing, in association with medications or mainly in the genital area. [2],[3],[4],[5],[6] Progressive eruptive syringoma in males is a rare condition, further studies are required to establish its etiology, associations, and treatment outcome.

 
  References Top

1.
Srivastava D, Taylor R. Appendage tumors and hamartomas of the skin. In: Goldsmith L, Katz S, Gilchrest B, Paller A, Leffell D, Wolff K, et al. editors. Fitzpatrick′s Dermatology in General Medicine. 8 th ed. USA: McGraw Hill; 2012. p. 1348-9.  Back to cited text no. 1
    
2.
Soler-Carrillo J, Estrach T, Mascaró JM. Eruptive syringoma: 27 new cases and review of the literature. J Eur Acad Dermatol Venereol 2001;15:242-6.  Back to cited text no. 2
    
3.
Hassan S, Rizvi N, Bhengra M, Chaudhary SS. Generalized eruptive syringoma. J Pak Assoc Dermatol 2012;22:153-6.  Back to cited text no. 3
    
4.
Sarifakioglu E, Gorpelioglu C, Bayrak R. Numerous yellow-brown papules over the trunk. Indian J Dermatol Venereol Leprol 2006;72:247-8.  Back to cited text no. 4
[PUBMED]  Medknow Journal  
5.
Pruzan DL, Esterly NB, Prose NS. Eruptive syringoma. Arch Dermatol 1989;125:1119-20.  Back to cited text no. 5
    
6.
Lau J, Haber RM. Familial eruptive syringomas: Case report and review of the literature. J Cutan Med Surg 2013;17:84-8.  Back to cited text no. 6
    
7.
Wallace ML, Smoller BR. Progesterone receptor positivity supports hormonal control of syringomas. J Cutan Pathol 1995;22:442-5.  Back to cited text no. 7
    
8.
Hashimoto K, DiBella RJ, Borsuk GM, Lever WF. Eruptive hidradenoma and syringoma. Histological, histochemical, and electron microscopic studies. Arch Dermatol 1967;96:500-19.  Back to cited text no. 8
[PUBMED]    
9.
Gómez MI, Pérez B, Azaña JM, Núñez M, Ledo A. Eruptive syringoma: Treatment with topical tretinoin. Dermatology 1994;189:105-6.  Back to cited text no. 9
    
10.
Al Aradi IK. Periorbital syringoma: A pilot study of the efficacy of low-voltage electrocoagulation. Dermatol Surg 2006;32:1244-50.  Back to cited text no. 10
[PUBMED]    
11.
Sánchez TS, Daudén E, Casas AP, García-Díez A. Eruptive pruritic syringomas: Treatment with topical atropine. J Am Acad Dermatol 2001;44:148-9.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2]



 

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