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Extremely rare clinical finding from my daily practice: Brooke-Spiegler syndrome with Turban tumour ..
“Turban tumor” is a historical and descriptive term for the appearance of multiple, coalescing cylindromas on the scalp, which is a classic presentation of Brooke-Spiegler syndrome (BSS).

Brooke-Spiegler syndrome is a rare, inherited, autosomal dominant disorder caused by mutations in the CYLD tumor suppressor gene. The condition is characterized by a predisposition to developing multiple benign skin adnexal tumors, predominantly on the head and neck.
The classic triad of tumors associated with BSS includes:
Cylindromas: Benign tumors that commonly appear on the scalp, where they can grow large and numerous enough to cover the entire area, leading to the “turban tumor” appearance.
Trichoepitheliomas: Skin-colored papules or nodules, usually found on the central face, particularly around the nose and nasolabial folds.
Spiradenomas: Painful, bluish nodules that can appear on various parts of the body, including the face, trunk, and extremities.
These tumors typically begin to appear during adolescence or early adulthood and increase in size and number over time.
Malignancy Risk and Management
While the tumors associated with BSS are generally benign, there is a 5% to 10% risk of malignant transformation. Patients also have an increased risk of developing tumors in other areas, such as the major and minor salivary glands.
Management of BSS often involves:
Close clinical follow-up to monitor for signs of malignant change, such as rapid enlargement, ulceration, or bleeding.
Treatment options for the benign tumors, which are often for cosmetic or functional reasons, and can include surgical excision, laser therapy, or dermabrasion.
Genetic testing for the CYLD gene mutation to confirm the diagnosis and for family counseling.
I have planned serial excisions with my team in order to remove each and every scalp tumour in that patient.
Histopathologically the diagnosis was confirmed.
This is the first case of multiple cylindromas for my 26 years work in dermatology.
The most interesting will be if the patient agrees for several sessions surgical interventions – with the purpose to obtain best quality of life.