Müzeyyen Gönül1, Seçil Soylu1*, Ülker Gül1, İlhan Kaya1, Levent Albayrak2 and Tuba Ünal3

12nd Dermatology Clinic, 21st Pathology Clinic, and 32nd Pathology Clinic, Ankara Numune Education and Research Hospital, Sıhhiye, TR-06100 Ankara, Turkey. E-mail: secil.soylu@isnet.net.tr

Accepted March 25, 2008.

Sir,

Syringocystadenoma papilliferum (SCAP) is a rare benign adnexal skin tumour of apocrine or apoeccrine type with characteristic histopathological features, and varied and non-distinct clinical findings (1). The vast majority of lesions are solitary and occur in the head and neck region. Their occurrence at other anatomical sites is uncommon, and linear arrangements of these lesions are particularly rare (2). We report here an unusual case of multiple SCAP in a linear distribution presenting on the arm.

CASE REPORT

An otherwise healthy 19-year-old woman presented with papules present since birth. Physical examination revealed multiple linear arranged, discrete, erythematous, 0,5–1 cm sized pseudovesicular papules on the extensor site of proximal part of right upper extremity, close to the shoulder (Fig. 1a). Routine laboratory investigations were within normal limits. Histopathological examination of punch biopsy specimens taken from the lesions showed epidermal invaginations lined by a stratified epithelium in the superficial portions and double layered rows with basal cuboidal cells and luminal columnar cells in the lower portions. Papillary projections protruding into the lumen and within a fibrovascular stroma containing large numbers of plasma cells were present. There were no epidermal hyperplasia, nor abnormal hair follicles, or sebaceous glands (Fig. 1b). The diagnosis was consistent with SCAP. The lesion was totally excised, and the histopathology revealed SCAP.

DISCUSSION

SCAP is a sweat gland tumour that is not clinically distinct; a biopsy is usually required for diagnosis. Two different primary lesions have been described: a solitary plaque or one to several papules. The plaques are usually less than 4 cm in diameter and skin-coloured to dark brown. They may be flat and smooth, or raised with a papillomatous or verrucous surface. The less common papular lesions are skin-coloured to pink and less than 1 cm in diameter (1).

SCAP is usually observed as a warty plaque most commonly located on the head or neck region, where it may occur de novo or within a naevus sebaceous (1–3). However, it was also reported that 20% of lesions occurred on the trunk and 5% on the extremities, almost all on the lower extremities (3). Other unusual locations have included the breast (4, 5), buttock (6), inguinal and perianal regions, and scrotum (2) and on a postoperative scar (7). In about half of cases the lesions are present at birth (1, 3). As far as we know, there has been only one case report published previously with upper extremity involvement (8). Our case is the second reported case of SCAP on the arm.

As far as we have observed, there have been only 8 previous cases of linear SCAP reported in the literature in English (Table I) (2, 3, 8–13). The most recent of these occurred on the scalp and neck (9, 13) and the lower extremity (2).

It appears that SCAP can present rarely as multiple lesions in a linear array, unassociated with a naevus sebaceous or an epidermal naevus. We therefore agree with the suggestion of Patterson et al. (2) that SCAP should be included among the other adnexal tumours that are capable of forming linear arrangements on their own.

REFERENCES

1. Mammino JJ, Vidmar DA. Syringocystadenoma papilliferum. Int J Dermatol 1991; 30: 763–765.

2. Patterson JW, Straka BF, Wick MR. Linear syringocystadenoma papilliferum of the thigh. J Am Acad Dermatol 2001; 45: 139–141.

3. de Bliek JP, Starink TM. Multiple linear syringocystadenoma papilliferum. J Eur Acad Dermatol Venereol 1999; 12: 74–76.

4. Nowak M, Pahtan A, Fatteh S, Lopez J. Syringocystadenoma papilliferum of the male breast. Am J Dermatopathol 1998; 20: 422–424.

5. Subramony C. Bilateral breast tumors resembling syringocystadenoma papilliferum. Am J Clin Pathol 1987; 87: 656–659.

6. Skelton HG III, Smith KJ, Young D, Lupton GP. Condyloma associated with syringocystadenoma papilliferum. Am J Dermatopathol 1994; 16: 628–630.

7. Saricaoğlu H, Bulbul Baskan E, Ozuysal S, Tunali S. A case of syringocystadenoma papilliferum: an unusual localization on postoperative scar. J Eur Acad Dermatol Venereol 2002; 16: 532–548.

8. Rostan SE, Waller JD. Syringocystadenoma papilliferum in an unusual location: report of a case. Arch Dermatol 1976; 112: 835–836.

9. Laxmisha C, Thappa DM, Mishra MM, Verma SK. Linear syringocystadenoma papilliferum of the scalp. J Eur Acad Dermatol Venereol 2007; 21: 275–276.

10. Premalatha S, Rao NR, Yesudian P, Razack A, Zahra A. Segmental syringocystadenoma papilliferum in an unusual location. Int J Dermatol 1985; 24: 520–521.

11. Goldberg NS, Esterly NB. Linear papules on the neck of a child: syringocystadenoma papilliferum. Arch Dermatol 1985; 12: 1198–1201.

12. Epstein BA, Argenyi ZB, Goldstein G, Whitaker D. An unusual presentation of a congenital benign apocrine hamartoma. J Cutan Pathol 1990; 17: 53–58.

13. Dawn G, Gupta G. Linear warty papules on the neck of a young woman: syringocystadenoma papilliferum (SP) in a sebaceous nevus (SN). Arch Dermatol 2002; 138: 1091–1096.