Brooke-Spiegler syndrome is a rare autosomal dominant genetic disease with predisposition to many adnexal tumors, including trichoepithelioma, cylindroma and spiroadenoma. Tumors appear in the second decade of life, progressively increase with age, and their prevalence is higher in women. It is caused by a mutation in the CYLD gene, localized in the chromosome 16q12-q13. We report a exuberant case of giant eccrine spiradenoma associated to this syndrome.

Keywords: Dermatologic Surgical Procedures; Facial Neoplasms; Neoplasms, Adnexal and Skin Appendage

INTRODUCTION: Hidradenitis supurativa (HS) is a chronic skin disease whose mechanism is not yet fully understood. It has a direct impact on the quality of life of affected patients, but there is no well-established therapy for its treatment.
OBJECTIVE: To assess the quality of life of patients diagnosed with HS on adalimumab using the Dermatology Life Quality Index (DLQI) questionnaire.
METHODS: Information was collected from patients with HS on adalimumab and standard DLQI questionnaires were applied before the beginning of treatment and at least 12 weeks after.
RESULTS: Of the three patients included, two presented decreased DLQI. Of these two, one showed significant improvement after treatment with adalimumab. The third patient showed worsening of this index.
CONCLUSIONS: The impact of hidradenitis supurativa on the quality of life of affected patients is significant and is related to factors other than adequate therapy. Life habits such as smoking and alcoholism, as well as the presence of other comorbidities, probably impact these indices. Affected patients should be assessed globally to determine the actual impact on the quality of life related to the disease in question. Thus, we suggest further studies with a larger number of patients.

Keywords: Hidradenitis suppurativa; Quality of life; Indicators of quality of life