Ingenol Mebutate Treatment in a Patient with Gorlin Syndrome.

Stieger, Marco; Hunger, Robert (2016). Ingenol Mebutate Treatment in a Patient with Gorlin Syndrome. Dermatology, 232 Suppl 1, pp. 29-31. Karger 10.1159/000447394

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BACKGROUND Gorlin syndrome, also known as the basal cell nevus syndrome (OMIM #109400), is a rare autosomal-dominant genetic disease. The disease, which shows mutation of the patched receptor gene (PTCH1) of the sonic hedgehog pathway, is characterized by developing multiple basal cell carcinomas (BCCs) in adolescent patients. Other clinical features include mandibular keratocysts, palmar and plantar pits, skeletal abnormalities and malformations central nervous system and genital tract. Gorlin-Goltz patients need multidisciplinary medical care and follow-up as well as genetic counseling if the patients want to have children. The treatment of multiple BCCs includes conventional surgery, micrographic Mohs surgery, cryotherapy, laser ablation, photodynamic therapy, imiquimod 5% cream, 5-fluorouracil cream as well as the sonic hedgehog pathway inhibitor vismodegib. CASE REPORT We report the case of a 30-year-old woman seen in our dermatological department since 2003. All the above-mentioned modalities had been employed for her numerous BCCs. The patient grew wary of the surgical procedures because of the countless scars. We successfully treated multiple BCCs with ingenol mebutate without post-inflammatory scarring. At 8-month follow-up, the patient shows no recurrence of the treated lesions. CONCLUSION Ingenol mebutate can be used to treat (superficial) BCCs in patients with Gorlin-Goltz syndrome as an additional modality. Close clinical follow-up is recommended.

Item Type:

Journal Article (Further Contribution)

Division/Institute:

04 Faculty of Medicine > Department of Dermatology, Urology, Rheumatology, Nephrology, Osteoporosis (DURN) > Clinic of Dermatology

UniBE Contributor:

Stieger, Marco and Hunger, Robert

Subjects:

600 Technology > 610 Medicine & health

ISSN:

1018-8665

Publisher:

Karger

Language:

English

Submitter:

Sandra Nyffenegger

Date Deposited:

01 Mar 2017 10:22

Last Modified:

12 Aug 2017 02:30

Publisher DOI:

10.1159/000447394

PubMed ID:

27513585

BORIS DOI:

10.7892/boris.93470

URI:

https://boris.unibe.ch/id/eprint/93470

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