A rare case of eccrine spiradenoma-treatment and management

Abstract

A young male patient presented with multiple swellings on his chest and the nape of his neck. Physical examination revealed multiple small papulonodular swellings measuring 0.5 × 0.5 cm to 2 × 2 cm, that were soft without discharge with no surrounding skin changes or induration. Skin biopsy samples were diagnosed as benign adnexal neoplasm consistent with eccrine spiradenoma, trichoepithelioma, and cylindroma, i.e., Brooke-Spiegler syndrome. Having confirmed this to be a case of eccrine spiradenoma, surgical excision was performed and the raw area was covered with a split thickness skin graft taken from the right thigh and sutured over the raw area. The sternal lesion was circumferentially excised and the wound was primarily closed by Z-plasty. Surgical excision is considered the gold standard for the treatment of these cases, with low rates of recurrence. Around 50 such cases have been reported in the literature to date. Although eccrine spiradenomas are usually solitary and small, the findings in our case underscore the fact that a variety of presentations are possible. With strict clinical suspicion and histological criteria, the correct diagnosis can be achieved, especially when combined with pertinent clinical information and laboratory studies.

Level of evidence: Level V, therapeutic study.

Keywords: Brooke–Spiegler syndrome; CYLDI gene; Eccrine unit; Multiple familial trichoepithelioma (MFT); Z-plasty.

Fig. 1

a, b Skin showing multiple ill-defined small nodules

Fig. 2

Surgical excision of the lesion extending between the Occipital Region and in the Nape of Neck measuring 24 cm × 11 cm

Fig. 3

Excised area covered by split thickness skin graft

Fig. 4

Markings at Pre-tracheal area for conducting surgical excision

Fig. 5

Wound at the pre-tracheal and pre-sternal area closed primarily by Z-plasty

Fig. 6

Blue ball tumor in dermis with no connection to the epidermis (HP ×4)

Fig. 7

Tumor lobules composed of two cell types. Peripherally small dark blue cells and centrally pale large cells. Large cells show luminal differentiation containing eosinophilic material at the center (HP ×40)