phenylephrine-hydrochloride and Xeroderma-Pigmentosum

Vismodegib is an oral inhibitor of the Hedgehog signaling pathway and has been used to treat basal cell carcinoma (BCC) in adults. This article reports clearance of a nodular BCC of the nasal tip in an 8-year-old boy with xeroderma pigmentosum (XP). BCC can pose therapeutic challenges when located in areas that are not amenable to traditional therapies such as Mohs micrographic surgery or topical agents. Vismodegib was used at a dose of 150 mg/day to treat the boy's BCC. After 4 months of therapy, we achieved complete clinical clearance. During 21 months of follow-up, the patient's nose remained clinically clear of tumor. Vismodegib was successfully used to treat a child with XP and nodular BCC. Our goal in using vismodegib was tumor regression while avoiding cosmetic and functional disfigurement. Vismodegib was effective in clinically clearing the tumor, and the patient has shown no signs of recurrence. Further studies are warranted.

Topics: Anilides; Carcinoma, Basal Cell; Child; Facial Neoplasms; Humans; Male; Nose; Pyridines; Skin Neoplasms; Xeroderma Pigmentosum

Xeroderma pigmentosum is a rare genetic disorder, characterized by cutaneous, ocular and neurological symptoms. Squamous cell carcinoma and melanoma are also its secondary characters. This case report is about maxillofacial prosthetic management of a 10 years old child presented with xeroderma pigmentosum. The nose of the patient was excised surgically due to melanoma. This case report elaborates the role of prosthodontist and the whole procedure of constructing the nasal prosthesis via conventional technique by using the patient's sibling nasal form as template. Regular follow up revealed marked improvement in esthetics, function and ultimately patient's quality of life.

Topics: Carcinoma, Squamous Cell; Child; Face; Humans; Male; Melanoma; Nose; Plastic Surgery Procedures; Prostheses and Implants; Prosthesis Design; Skin Neoplasms; Treatment Outcome; Xeroderma Pigmentosum

Topics: Anesthesia, General; Anesthetics, Inhalation; Anesthetics, Intravenous; Cheek; Child; Female; Fentanyl; Humans; Intubation, Intratracheal; Isoquinolines; Methyl Ethers; Mivacurium; Neuromuscular Nondepolarizing Agents; Nose; Plastic Surgery Procedures; Sevoflurane; Skin Transplantation; Xeroderma Pigmentosum

Topics: Adolescent; Anesthesia; Humans; Intubation, Intratracheal; Male; Nose; Xeroderma Pigmentosum

Topics: Carcinoma, Squamous Cell; Humans; Lip; Nose; Skin Neoplasms; Xeroderma Pigmentosum