Penile syringoma in a 14-year-old boy

ARTICLE

Auteur(s) : Minou HERTL-YAZDI, Andrea NIEDERMEIER, Stefan HÖRSTER, Walter KRAUSE,

Dept. of Andrology and Venereology University Hospital Philipp University Deutschhausstr. 9 D-35033 Marburg Germany
<krause@med.uni-marburg.de>

Syringomas, benign tumors of the eccrine sweat glands, appear at all sites of the body. Involvement of the male genitalia is rare; however, syringomas should be considered as a clinical differential diagnosis to condylomata, lichen planus, and bowenoid papulosis. A proper diagnosis is possible only by histologic examination.
Familial occurrence of multiple syringomas seems to be common, thus autosomal dominant inheritance is likely. An interesting hypothesis of etiology is the suggestion that some of the eruptive lesions may start as an inflammatory process. Treatment options of syringoma are generally unsatisfactory.
Syringomas are most common on the eyelids, the nipples and the female genitalia. Different sites may be affected at the same time. Syringomas are typically skin-coloured to pink, but may appear yellow, translucent, or cystic. They are usually observed in symmetric, bilaterally distributed clusters.
They may occur in successive crops at any age, an increased frequency is observed at puberty [1. The mean age of patients was 29 years, the mean delay from onset to demand for treatment was 6 years. An association with hormonal factors has been suggested, in particular the size of vulvar syringomas may increase during pregnancy.
Involvement of the male genitalia is rare, only few cases have been reported [2-4]. A proper diagnosis of syringoma is possible only by histologic examination. Soler-Carrillo et al. [5] described 27 syringomas among 67,000 histologic specimens (1:2,500) from skin biopsies. In only 11 cases, was the clinical diagnosis correct «syringoma».
Histologically, syringomas are easily diagnosed by the accumulation of small ducts, some of which have comma-like tails, lined by two rows of epithelial cells. The histological differential diagnosis includes adnexal carcinoma, clear cell syringoma, and chondroid syringoma.

Case report

A 14-year-old boy presented in our department with skin-coloured and hyaline translucent papules of 1-2 mm diameter on the skin of the shaft of the penis (figure 1). The lesions were aggregated to a field of about 1 × 2 cm at the dorsum penis near to the foreskin, which was shortened due to earlier circumcision. He did not feel any sensations in the lesions, but asked for treatment for cosmetic reasons.
Histopathology showed normal epidermis and cutis. Within the cutis some small cysts of the acrosyringium and thin epithelial strands of tadpole-like or comma-like appearance were visible. Immunohistochemistry revealed strong expression of cytokeratin MNF 116 and CEA in the epithelial cells of the cysts and the strands, thus indicating their origin from the sweat glands.

Discussion

Familial occurrence of multiple syringomas (MIM 186600) seems to be common, thus autosomal dominant inheritance is likely. Reed [6]) described a family in which 7 females and 1 male in 4 generations were affected. However, eruptive syringomas are not associated with other genodermatoses except the Nicolau-Balus syndrome. They are also described more frequently in patients with Down’s syndrome and patients with Ehlers-Danlos syndrome.
Guitart et al. [7] proposed an interesting hypothesis. They stated «The clinical presentation of multiple small papules with scanty proliferation is unusual for a neoplastic process. Reports on a variety of atypical clinical presentations added credibility to our conclusion that eruptive syringomas are not neoplasias.» They suggested that some of the eruptive lesions may start as an inflammatory process, inducing a hyperplastic reaction of the eccrine duct. The hypothesis is also suitable to explain syringoma-like changes observed in a variety of cutanous conditions such as alopecia areata, melanocytic nevi, or basal cell carcinoma. The pruritus, which is sometimes associated with syringomas, is another argument in favour of this hypothesis. The hypothesis makes it diffecult, however, to explain the familial occurrence of syringoma, but one may speculate on a specific reaction to inflammatory influences in the affected individuals.
Treatment options of syringoma are generally unsatisfactory [1]. In restricted areas, surgical excision or laser treatment may be attempted. The patient presented here also asked for treatment. After discussion of the known possibilities, he refrained from treatment. n

1. Hsiung SH. Eruptive syringoma. Dermatol Online J 2003; 9(4): 14.

2. Zalla JA, Poerry HO. An unusual case of syringoma. Arch Derm 1971; 103: 215-7.

3. Yalisova BL, Stolar EH, Williams CM Multiple penile papules. Arch Dermatol 1987; 123: 1391-96.

4. Cassarino DS, Keahey TM, Stern JB. Puzzling penile papules. Int J Dermatol 2003; 42(12): 954-6.

5. Soler-Carrillo J, Estrach T, Mascaro JM. Eruptive syringoma: 27 new cases and review of the literature. J Eur Acad Dermatol Venereol 2001; 15(3): 242-6.

6. Reed WB. Genetische Aspekte in der Dermatologie. Hautarzt 1970; 21: 8-16.

7. Guitart J, Rosenbaum MM, Requena L. ’Eruptive syringoma’: a misnomer for a reactive eccrine gland ductal proliferation? J Cutan Pathol 2003; 30(3): 202-5.