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Brooke-Spiegler syndrome
Corresponding author: Dr. Sunmeet Sandhu, Department of Dermatology, Armed Forces Medical College, Pune, Maharashtra, India. sunmeet.sandhu@gmail.com
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Received: ,
Accepted: ,
How to cite this article: Neema S, Sandhu S, Radhakrishnan S. Brooke-Spiegler syndrome. Indian J Dermatol Venereol Leprol 2023;89:139-40.
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.
- 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)
Acknowledgement
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.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
