How to cite this article: Neema S, Sandhu S, Radhakrishnan S. Brooke-Spiegler syndrome. Indian J Dermatol Venereol Leprol 2020;1-2.
A 42-year-old female presented with insidious onset and gradually progressive growths over the scalp and face since 8 years of age with similar complaints in the sibling. Cutaneous examination revealed multiple hairless, pinkish-red to skin coloured, dome-shaped, firm papules, nodules and tumoral lesions of varying sizes over scalp, forehead and face (predominantly over the nasolabial fold and nasal bridge) [Figure 1]. Histopathology of scalp lesion and facial papule were consistent with cylindroma and trichoepithelioma, respectively. Based on clinical features and histopathologic findings, a diagnosis of Brooke-Spiegler syndrome was made. Lesions that were mechanically hampering her vision were excised. Brooke-Spiegler syndrome is a rare autosomal dominant condition characterised by multiple skin tumours like spiradenoma, trichoepithelioma and cylindroma, most often found over head and neck. It is a disfiguring condition and associated with psychological disturbances.
We are thankful to Dr. B S Sunita, Reader, Department of Pathology, Armed Forces Medical College, Pune for her help in providing histopathological correlation with the clinical diagnosis.
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