Turban Tumor: A Classical Presentation of Brooke-Spiegler Syndrome.

A 40-year-old male had multiple gradually progressive asymptomatic papulonodular lesions over head for the past 20 years and similar lesions in two of three children. Cutaneous examination revealed multiple confluent nodules over the scalp, forehead, and lateral cheek [Figures 1 and 2]. The lesions had a smooth surface with telangiectasia and were devoid of hairs. He also had many small (1–2 mm) firm, skin-colored papules over the centro-facial area involving nose, nasolabial fold, and cheek [Figure 3]. Skin biopsy from forehead showed islands of basaloid cells surrounded by hyalinized material arranged in jigsaw-puzzle-like architecture without retraction cleft suggestive of cylindroma [Figure 4]. The facial papules showed basaloid epithelial elements arranged to form immature hair germ cells, follicular papillae, and horn cysts classical of trichoepithelioma [Figure 5].

Figure 1

Front view showing multiple smooth nodules and plaques over forehead and frontal scalp

Figure 2

Lateral view showing large nodules over preauricular area and scalp

Figure 3

Multiple small discrete and confluent skin-colored papules over nose, cheek, and chin

Figure 4

10× magnification in H and E stain showing multiple basaloid proliferations, closely packed in jigsaw puzzle arrangement with no retraction cleft

Figure 5

10× magnification in H and E showing islands of basaloid cells, immature hair germ along with cystic structures lined by squamous epithelium suggestive of papillary mesenchymal bodies

The diagnosis of Brooke–Spiegler syndrome (BSS) was suggested on the basis of coexistence of cylindroma and trichoepithelioma. It is an autosomal dominant condition involving CYLD gene on chromosome 16q12-q13. Co-presentation of cylindromas, spiradenomas, and cylindro-spiradenomas over scalp along with trichoeptiheliomas over face is classic feature of BSS although multiple familial trichoepitheliomas and familial cylindromatosis are also considered as the phenotypic variant of the same.[1] There is 5%–10% risk of development of basal cell carcinoma over the lesions. But our patient did not have any lesion clinically suspicious of BCC, but he was followed up and planned for surgical resection of larger portion of tumor for cosmetic concern.[2]

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