Multiple Trichoepitheliomas: A Disfiguring Lesion Successfully Treated with Excision and Skin Grafting.

BACKGROUND: Multiple trichoepitheliomas are rare benign adnexal tumours that present a unique challenge both to the patient and the managing physician. The multiple nature of the lesion and face being a common location often causes cosmetic concern and psychosocial challenges. Physicians on the other hand face the challenge of providing an ideal treatment with a satisfactory outcome. Dermabrasion and laser therapy have been used to treat this lesion successfully, though they require multiple sessions, and recurrence is common. These options are however either not available or unaffordable in low-resource countries such as Nigeria. Surgical excision though an option, has rarely been advocated due to scarring, leading some experts to offer no treatment at all in developing nations. We report a challenging case of sporadic multiple trichoepitheliomas successfully treated with surgical excision and full-thickness skin graft. METHOD: Following diagnosis, the patient was counselled on the procedure, the risks and benefits. She had en-bloc excision of the lesion, and full-thickness skin graft harvested from the right groin was transplanted and anchored with Monocryl 5-0. All wounds were dressed, and the graft site was reviewed on day 10. She was discharged for outpatient follow-up. RESULT: Graft take was 95%. Epidermolysis which was seen on postoperative day 10 resolved. Hypertrophic scar on the ala nasi is softening on scar massage, and the patient is very satisfied with the outcome. CONCLUSION: Surgical excision can be a valuable tool in low-resource settings for the management of multiple trichoepitheliomas.

CONTEXTE: Les trichoépithéliomes multiples sont des tumeurs annexielles bénignes rares qui présentent un défi unique à la fois pour le patient et le médecin traitant. La nature multiple de la lésion et le visage étant un site commun entraînent souvent des préoccupations esthétiques et des défis psychosociaux. Les médecins, de leur côté, sont confrontés au défi de fournir un traitement idéal avec un résultat satisfaisant. La dermabrasion et la thérapie au laser ont été utilisées avec succès pour traiter cette lésion, bien qu'elles nécessitent plusieurs séances et que la récidive soit fréquente. Ces options ne sont cependant pas disponibles ou abordables dans les pays à faibles ressources tel que le Nigeria. L'exérèse chirurgicale, bien qu'une option, a rarement été préconisée en raison des cicatrices, conduisant certains experts à ne proposer aucun traitement du tout dans les pays en dével oppement . Nous rappor tons un cas difficile de trichoépithéliomes multiples sporadiques traités avec succès par exérèse chirurgicale et greffe de peau totale. MÉTHODE: Après le diagnostic, la patiente a été informée de la procédure, des risques et des avantages. Elle a subi une exérèse en bloc de la lésion, et une greffe de peau totale prélevée dans l'aine droite a été transplantée et fixée avec du Monocryl 5-0. Toutes les plaies ont été habillées, et le site de la greffe a été examiné le 10e jour. Elle a été renvoyée pour un suivi en consultation externe. RÉSULTAT: La prise de greffe était de 95 %. L'épidermolyse observée le 10e jour postopératoire a disparu. La cicatrice hypertrophique sur l'aile du nez s'assouplit avec le massage de la cicatrice, et la patiente est très satisfaite du résultat. CONCLUSION: L'exérèse chirurgicale peut être un outil précieux dans les contextes à faibles ressources pour la prise en charge des trichoépithéliomes multiples. MOTS-CLÉS: Trichoépithéliomes multiples, Thérapie au laser, Électrocautérisation, Exérèse chirurgicale, Greffe de peau totale.

Carbon dioxide laser ablation for trichoepitheliomas: The largest reported series.

Trichoepitheliomas are benign cutaneous tumours often occurring on the face and can lead to considerable psychological distress to its sufferers. Treatment is often difficult, and surgery is limited by the obvious scars and multiple numbers of lesions. Carbon dioxide laser ablation can be used safely with good cosmetic outcome and low recurrence rate, and in this article, we describe our experience in the treatment of these tumours with the carbon dioxide laser. This is the largest reported series in the literature.

Síndrome de Brooke-Spiegler a propósito de un caso clínico

El síndrome de Brooke Spiegler es resultante de la mutación del gen CYLD, con expresión fenotípica variable, es una enfermedad autosómica dominante asociada a múltiples tumores de piel, con predilección por cabeza y cuello. Las lesiones aparecen a temprana edad con progresión en tamaño y número en el transcurso del tiempo; tiene predilección por el sexo femenino. La presentación clínica incluye cilindromas, espiroadenomas, y tricoepiteliomas1,2.

Broke-Spiegler syndrome is a condition resulting from mutation in the CYLD gene, autosomal dominant dermatological disease, with variable phenotypical and multiple associated skin tumors, most frequently in head and neck. Lesion can appear from early age, are progressive in number and size, and more prevalent in females. Pathological findings include cylindromas, spiradenomas and trichoepitheliomas. Patients can develop basal cell carcinomas, salivary gland tumors, trichoblastomas, milium, and organoid nevi. Around 80-85 % of patients have mutation in gene CYLD , located in chromosome 16. Familial cylindromatosis and multiple familial trichoethelioma are phenotypical variants. Its current "CYLD cutaneous syndrome".

Basal Cell Carcinoma Developing from Trichoepithelioma: Review of Three Cases.

Trichoepitheliomas (TE) are benign tumours but occasionally can undergo transformation to malignant neoplasms more commonly as Basal Cell Carcinoma (BCC). The correct diagnosis between these tumours is very important because basal cell carcinoma is locally aggressive neoplasm and requires total surgical excision with wide healthy margins while trichoepithelioma needs simple excision. We describe three patients who developed basal cell carcinoma with facial trichoepitheliomas. The only clinical feature that distinguished the carcinomas from the trichoepitheliomas was their larger size, in all three patients, one patient with recurrent, hyper pigmented swelling with surface ulceration and in another patient there are multiple trichoepitheliomas, and other family members are also affected. The history, clinical features and histopathological findings were suggestive of the evolution of basal cell carcinoma directly from trichoepithelioma in our first two cases, but in the third case TE and BCC were separate lesions on face and we are uncertain about whether the BCC developed independently or by transformation from a trichoepithelioma. Based on our clinicopathological observations in the three patients and reports in the recent literature, BCC with follicular differentiation and trichoepithelioma are considered to be highly related.

Multiple trichofolliculomas mimicking multiple trichoepitheliomas.

Trichofolliculomas are benign hair follicle hamartomas which were initially considered as hair follicle tumors. Usually presenting as a solitary lesion associated with a tuft of vellus hairs, multiple trichofolliculomas are rare. Trichofolliculomas are characterized by a histopathological feature of dermal keratin cyst with cyst wall showing radiating hair follicles. We report this case for the rare presentation of multiple trichofolliculomas on the face which clinically mimicked multiple trichoepitheliomas.

Multiple Familial Trichoepithelioma with an Adjacent Basal Cell Carcinoma, Transformation or Collision - A Case Report and Review of Literature.

Trichoepithelioma is a benign tumor of follicular origin that presents as small, skin-colored papules predominantly on the face. When more than one family member is affected, the disease is known as multiple familial trichoepithelioma (MFT). It is a rare autosomal dominant skin disease. Malignant transformation is very rare. We describe here a case that developed malignant neoplasm in a setting of multiple trichoepithelioma.