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CASE REPORT
Year : 2021  |  Volume : 24  |  Issue : 8  |  Page : 1252-1254

Generalized Eruptive Syringoma in a Nigerian Woman: A Case Report and a Brief Literature Review


Dermatology Unit, Department of Internal Medicine, Kaduna State University/Barau Dikko Teaching Hospital, Kaduna, Nigeria

Date of Submission17-Aug-2019
Date of Acceptance14-Jan-2021
Date of Web Publication14-Aug-2021

Correspondence Address:
Dr. H Yahya
Dermatology Unit, Department of Internal Medicine, Kaduna State University/Barau Dikko Teaching Hospital, Kaduna
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/njcp.njcp_438_19

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   Abstract 


Generalized eruptive syringomas are a very rare variant of syringoma which appear on the chest, neck, abdomen, upper and lower arms, thighs, legs and back. They often affect adolescent or post-adolescent females, although cases have been described in children and older adults. Generalized eruptive syringomas are mostly sporadic but familial cases, and those associated with eczematous skin conditions, solid organ transplants, renal cell carcinoma and some genodermatoses have also been reported. Although eruptive syringomas may resemble cutaneous mastocytosis, disseminated granuloma annulare, eruptive xanthomata, steatocystoma multiplex, eruptive velus hair syndrome, verruca plana and other skin conditions clinically, the histological appearance characterized by upper dermal epithelial cords with a tendency to ductule formation and tadpole extension of outer epithelial layer is diagnostic. We report the case of a 20-year-old Nigerian woman with generalized eruptive syringoma.

Keywords: Eruptive syringoma, generalized eruptive syringoma, syringoma


How to cite this article:
Yahya H. Generalized Eruptive Syringoma in a Nigerian Woman: A Case Report and a Brief Literature Review. Niger J Clin Pract 2021;24:1252-4

How to cite this URL:
Yahya H. Generalized Eruptive Syringoma in a Nigerian Woman: A Case Report and a Brief Literature Review. Niger J Clin Pract [serial online] 2021 [cited 2022 Dec 3];24:1252-4. Available from: https://www.njcponline.com/text.asp?2021/24/8/1252/323859




   Introduction Top


Syringomas are 1–3 mm, skin-colored to yellow or brown, flat-topped benign skin tumors derived from intraepidermal eccrine ductal epithelium with a characteristic histological appearance.[1] Friedman and Butler[2] have classified syringomas into four clinical types: Localized, generalized (eruptive), variant associated with Down's Syndrome, and familial types. The common (localized) syringomas which affect about 1% of the population, occur as a few lesions, often in females, and are localized on the lower eyelids or periorbital areas, although they may appear on the scalp, axilla, vulva, and penis.[3]

Eruptive syringomas refer to a rare type of syringomas that appear in large numbers over a short period of time and are usually located on two or more anatomic parts of the body such as the chest, neck, abdomen or upper and lower extremities.[2] Both localized and eruptive syringomas are usually sporadic, but familial forms have been described. Syringomas associated with Down's syndrome––found in about a quarter of the patients––usually affect the eye lids.[3] Generalized eruptive syringomas,[4] which refer to widespread involvement of the face, trunk and upper and lower limbs, are even rarer. We report the case of a 20-year-old Nigerian woman who presented with generalized eruptive syringoma. The rarity of the condition may pose diagnostic challenge to clinicians hence the need for a literature review highlighting the various clinical conditions that may be associated with it and the treatments modalities that are available.


   Case Report Top


A 20-year-old Nigerian woman presented to the skin clinic with a 4-year history of large numbers of asymptomatic skin lesions which first appeared on her chest but over a period of several months spread to involve her neck, abdomen, upper back, upper and lower arms, thighs, legs, and axillae. The patient was otherwise healthy. There was no family history of a similar condition. On examination, she had innumerable, symmetrically distributed, 1–3 mm brown, flat-topped, discrete to confluent papules on her chest, neck, abdomen, and upper and lower limbs excluding the hands and feet [Figure 1]a, [Figure 1]b and [Figure 1]c. She also had many scattered lesions on her forehead and upper back and axillae but none on the eyelids or cheeks. There were many small, skin-colored and barely perceptible papules, especially on her arms and back. Darier's sign was negative. Clinical examination of other systems was apparently normal. Basic laboratory tests including a blood count, serum chemistries and urine tests were normal. Histological examination of a 4 mm skin punch biopsy taken from the thigh showed numerous well circumscribed epithelial cords embedded in a collagenized upper dermis, which in many instances formed small ducts. A one-to-two layer of flattened to cuboidal cells, with eosinophilic cytoplasm and regular nuclei, with no nucleoli or mitoses, lined several ducts whose outer layer occasionally extended to form the coma-shaped or tadpole tail typical of syringoma [Figure 2]. Oral isotretinoin taken for three months did not have any effect on the lesions.
Figure 1: Clinical photographs of generalized eruptive syringoma affecting the neck and chest (a), forearms (b) and thighs (c)

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Figure 2: Photomicrograph of syringoma showing numerous upper dermal epithelial cords, with tendency for ducts formation, embedded in a collagenized stroma. Note the typical “tadpole” tail extension of outer epithelial layer

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   Discussion Top


Eruptive syringomas are a rare variant of syringoma which appears in adolescence or immediately after, like the far more common localized type, although cases have been reported in children and older adults.[3] Clinically, eruptive syringomas may resemble[5] maculopapular mastocytosis, the most common form of cutaneous mastocytosis, which is intensely itchy and may blister when rubbed; disseminated granuloma annulare, which presents as rings of skin-colored papules on arms and legs; and eruptive xanthomata, which appear as yellowish papules on extensor surfaces of elbows and knees, lower back and palms and soles. Steatocystoma multiplex (firm yellowish subcutaneous papules and nodules on the chest), Fox Fordyce disease (occasionally itchy, skin-colored papules on the axillae, groin, pubic and submammary area), eruptive velus hair syndrome, (small, dome-shaped, umblicated papules on the front of sternum), trichoepithelioma (shiny firm papules on the face) and verruca plana (flat-topped, single to confluent papules which may show pseudo-kobnerization) are other clinical differentials but the histology is diagnostic.[1]

There are other differences between localized syringoma and eruptive syringomas apart from location and extent: Localized syringomas are usually skin colored or yellowish while eruptive syringomas are distinctly brown and histologically, epithelial cords predominate over ductules in localized syringomas while it is the reverse in eruptive syringomas. An increased amount of melanin in the basal epidermis, associated with pigmentary incontinence in some cases, is said to account for the brown color of eruptive syringomas.[5]

The exact cause of eruptive syringomas remains an enigma. Most eruptive syringomas have been reported in otherwise normal pubertal or post-pubertal females, like our patient, which suggest hormonal factors are important, although cases in children and older adults have been reported.[3] Eruptive syringomas may represent generalized harmatomas of eccrine germ cells.[3] Other factors undoubtedly play a role in specific instances. Familial eruptive syringomas are inherited in an autosomal dominant fashion. Eruptive syringomas have developed in older adults after liver and kidney transplantation which suggests that immunosuppression may be the trigger to the condition;[6] tumors of skin appendages are more common after organ transplantation than in normal people. Guitart et al.,[7] who reported two cases of eruptive syringoma preceded by eczematous skin reaction, have suggested that inflammatory reaction around sweat ducts may be the predisposing factor. Nicolau-Balus syndrome (milia and atrophoderma vermiculata) and  Brooke-Spiegler syndrome More Details (spiradenomas, trichoepitheliomas and cylindromas) are genodermatoses associated with eruptive syringomas in single case reports.[3] Association of eruptive syringoma with bilateral renal cell carcinoma in a 53-year-old man suggested that it might be a paraneoplastic syndrome.[8]

Cosmetic concern is the primary reason patients seek treatment for syringomas, although some patients with a large number of lesions may complain of itching, especially after sweating. Various modalities have been used in treating syringomas with variable result. Williams and Shinkai,[3] in their systematic review of treatments used in 215 cases of syringomas, reported that the most widely used method was CO2 laser (78.6% of cases), used alone or in combination with other locally destructive measures with moderate improvement in a third of cases and total resolution in 14%. Other locally destructive treatments used in a smaller number of patients included low-voltage electrocoagulation and electrodesiccation. Scarring and hyperpigmentation were a significant concern which could be worse in dark-skinned patients.

Gomez et al.[9] reported a good response to topical tretinoin in a patient with eruptive syringoma. Oral isotretinoin has given inconsistent results in eruptive syringoma with two patients showing satisfactory response while another one showed no benefit.[3] Topical atropine 1% aqueous solution relieved the symptoms of intense pruritus within days and resulted in a decrease in the size of lesions in one patient with eruptive syringoma.[10] In view of the problems of availability or expense of treatments that have been shown to be effective and the widespread nature of eruptive syringomas, patients may sometimes opt for no treatment.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Patterson JW. Benign sweat gland tumors: Syringoma. In: Patterson JW, Hosler GA, editors. Weedon's Skin Pathology. 4th ed.. London: Churchill Livingstone; 2016. P 936.  Back to cited text no. 1
    
2.
Friedman SJ, Butler DF. Syringoma presenting as milia. J Am Acad Dermatol 1987;16:310-4.  Back to cited text no. 2
    
3.
Williams K, Shinkai K. Evaluation and management of the patient with multiple syringomas: A systematic review of the literature. J Am Acad Dermatol 2016;74:1234-40.e9.  Back to cited text no. 3
    
4.
Dyall-Smith DJ, Connors TJ, Scurry J. Generalized eruptive syringoma––a papular dermatosis. Australas J Dermatol 1990;31:95-8.  Back to cited text no. 4
    
5.
Kim KJ, Roh KH, Choi JH, Sung KJ, Moon KC, Koh JK. Eruptive syringoma: Clinicopathologic analysis of thirteen cases. Ann Dermatol 2002;14:143-8.  Back to cited text no. 5
    
6.
Maeda T, Natsuga K, Nishie W, Yamashita K, Shimizu H. Extensive eruptive syringoma after liver transplantation. Acta Derm Venereol 2018;98:119-20.  Back to cited text no. 6
    
7.
Guitart J, Rosenbaum MM, Requena L. “Eruptive syringoma”: A misnomer for a reactive eccrine gland ductal proliferation? J Cutan Pathol 2003;30:202-5.  Back to cited text no. 7
    
8.
Uysal PI, Yalcin B, Ozhamam E, Bozdogan O. Co-existence of adult onset eruptive syringoma and bilateral renal cell carcinoma: A case report. Am J Dermatopath 2017;39:56-8.  Back to cited text no. 8
    
9.
Gomez MI, Perez B, Azana JM, Nunez M, Ledo A. Eruptive syringoma: Treatment with topical tretinoin. Dermatology 1994;189:105-6.  Back to cited text no. 9
    
10.
Sanchez TS, Dauden 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. 10
    


    Figures

  [Figure 1], [Figure 2]



 

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