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Congenital blaschkoid eccrine spiradenoma on the face

European Journal of Dermatology. Volume 15, Number 2, 73-4, March-April 2005, Genes and skin

Summary

Author(s) : Tugba Rezan Ekmekci, Adem Koslu, Damlanur Sakiz , Department of Dermatology, Sisli Etfal Research and Training Hospital, Istanbul, TurkeyFax: (+90) 212 234 11 21., Department of Pathology, Sisli Etfal Research and Training Hospital, Istanbul, Turkey.

Summary : Eccrine spiradenoma is an uncommon tumour of the sweat gland(s) and generally occurs as a solitary small painful nodule. Very rarely, it may present as multiple tumours arranged in a linear/blaschkoid pattern. An 8-year-old girl had many papulonodular lesions, arranged in a linear pattern on the left half of the face, involving the forehead and lateral side of the nose. The lesions were along Blaschko’s lines. Lesions were reported to be present at birth\; no family member had similar lesion(s), either in solitary or multiple form. Histopathologic examination of a biopsy specimen revealed characteristic features of eccrine spiradenoma. The case is reported because of the rare occurrence of this presentation.

Keywords : Blaschko’s lines on the face, eccrine spiradenoma, multiple eccrine spiradenomas, linear distribution

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ARTICLE

Auteur(s) :, Tugba Rezan Ekmekci^1,*, Adem Koslu¹, Damlanur Sakiz²

¹Department of Dermatology, Sisli Etfal Research and Training Hospital, Istanbul, TurkeyFax: (+90) 212 234 11 21.
²Department of Pathology, Sisli Etfal Research and Training Hospital, Istanbul, Turkey

accepté le 29 Novembre 2004

Eccrine spiradenoma (ES) is an uncommon tumour of eccrine sweat gland(s) [1]. Typically, it presents as a bluish, painful, slow-growing, solitary nodule, ranging from 0.3 to 5 cm in diameter, located above the waistline on the anterior surface of the body [2-4]. Nodules can appear at any age, but the highest incidence is seen in young adults. The sex incidence is equal. Spontaneous pain or tenderness on palpation is a frequent and striking symptom of ES [1]. It presents rarely as multiple tumors [4]. In this report, a child with multiple ES following Blaschko’s lines is described.

Case report

An 8-year-old girl presented with asymptomatic papulonodular lesions on the left half of the face. Lesions were reported to be present at birth; no family member had similar lesion(s), in multiple or solitary form. Examination revealed multiple, pink-purple, grouped, papulonodular lesions ranging from 1 to 5 mm in size on the left half of the nose and forehead following the lines of Blaschko. The total size of the involved area was 4 × 5 cm on the forehead and 4 × 2 cm on the nose ( (figure 1) ). On palpation, the lesions were firm and nontender. The patient was otherwise well. Examination of a biopsy specimen taken from a papule on the forehead revealed a dermal neoplasm characterized by well-defined nests without connection to the epidermis. Each dermal nest was surrounded by an ill-defined fibrous pseudocapsule ( (figure 2) ). The lobules contained two types of epithelial cells arranged in intertwining cords and small lumina. Cells with small dark nuclei were present at the periphery of the cords, while cells with large, pale nuclei were located in the center of the cord and around small lumina ( (figure 3) ). Based upon these clinical and histological findings the diagnosis was established as multiple ES.

Discussion

Multiple ES are uncommon, accounting for less than 2% of all cases of spiradenoma [5]. They can occur in localized groups, linear or segmental pattern [4, 6-8].

On the forehead the lines of Blaschko run in a funnel-like configuration from the scalp to the eyebrows and converge on the nasal root. From the nasal root they run in 4 main directions. In the first direction they follow a perpendicular direction to the tip of the nose and the philtrum [9]. We named this case “Blaschkoid ES” because the lesions follow exactly this direction. The first case of multiple ES within the lines of Blaschko was described by Bourrat et al. [10]. In our opinion, the cases reported in a zosteriform distribution fit into the Blaschko’s lines [5, 6, 11].

Linear/segmental ES has been proposed to be an organic hamartoma because of its association with other appendageal components [5]. We do not think that this case is an organic hamartoma.

The histological feaures of ES are quite characteristic. The tumor consists of multiple sharply demarcated lobules. Two types of epithelial cells are present within the lobules and are arranged in intertwining bands [4]. In the literature, neoplasms that feature spiradenomas and dermal cylindromas either in the same patient and/or even within the same tumor mass are described [12].

Most of multiple ES are acquired and sporadic. However, to our knowledge, two cases of congenital and three cases of familial ES have been reported [2, 3, 6].

Malignant transformation of ES is rare. Typically, sudden growth is noted in a tumor that has been present for many years. There is no age, gender, or site predilection [13]. The incidence of malignant transformation may increase with the tumor burden of a pre-existing benign spiradenoma. Likewise, 6% of malignant spiradenomas develop from multiple benign spiradenomas [14].

The presented case is an extremely unusual form of ES, because the lesions are multiple, nontender, congenital and follow Blaschko’s lines.

The most effective treatment of ES is complete surgical excision; however, this is sometimes not feasible if tumors are multiple or extensive [5]. Radiotherapy, has been reported to be effective in two sisters with multiple ES [1]. Carbon dioxide laser treatment has been proposed in one case of familial ES [2]. The tissue expansion technique of healthy, neighbouring skin on the forehead and cheek may be useful for wider or complete excision of the lesion in this case. It was decided that therapy should be postponed considering the young age of our patient and likely advances in medical technology.

References

1 Bedlow AJ, Cook MG, Kurwa A. Extensive naevoid eccrine spiradenoma. Br J Dermatol 1999; 140: 154-7.

2 Ter Poorten MC, Barrett K, Cook J. Familial Eccrine Spiradenoma: A Case Report and Review. Dermatol Surg 2003; 29: 411-4.

3 Noto G, Bongiorno MR, Pravata G, Arico M. Multiple nevoid spiradenomas. Am J Dermatopathol 1994; 16: 280-4.

4 Revis P, Chyu J, Medenica M. Multiple eccrine spiradenoma: case report and review. J Cutan Pathol 1988; 15: 226-9.

5 Gupta S, Jain VK, Singh U, Gupta S. Multiple eccrine spiradenomas in zosteriform distribution in a child. Pediatr Dermatol 2000; 17: 384-6.

6 Shelley WB, Wood MG. A zosteriform network of spiradenomas. J Am Acad Dermatol 1980; 2: 59-61.

7 Tsur H, Lipskier E, Fisher BK. Multiple linear spiradenomas. Plast Reconstr Surg 1981; 68: 100-2.

8 Ohtsuka H, Tezuka K, Kumakiri M, Ohtsuki Y. Multiple eccrine spiradenomas on the hand, forearm and head. Dermatology 2002; 205: 401-4.

9 Happle R, Assim A. The lines of Blaschko on the head and neck. J Am Acad Dermatol 2001; 44: 612-5.

10 Bourrat P, Theodore-Lefevre C, Beltzer-Garelly E, Rabary G, Binet O. Multiple eccrine spiradenoma with Blaschko’s lines distribution. Ann Dermatol Venereol 1992; 119: 897-8.

11 Altinyazar HC, Kargi E, Ozen O, Koca R, Babuccu O. Multiple eccrine spiradenoma in zosteriform distribution. Plast Reconstr Surg 2003; 112: 927-8.

12 Michal M, Lamovec J, Mukensnabl P, Pizinge K. Spiradenocylindromas of the skin: Tumors with morphological features of spiradenoma and cylindroma in the same lesion: Report of 12 cases. Pathol Int 1999; 49: 419-25.

13 Leonard N, Smith D, McNamara P. Low-grade malignant eccrine spiradenoma with systemic metastases. Am J Dermatopathol 2003; 25: 253-5.

14 Braun-Falco M, Bonel H, Ring J, Hein R. Linear spiradenoma with focal malignant transformation. J Eur Acad Dermatol Venereol 2003; 17: 308-12.