Neurofibromatosis type 5

Rare type of neurofibromatosis characterized by benign tumors that occur on or just under the skin. Affected individuals may also have CAFE-AU-LAIT SPOTS and other features similar to those of NEUROFIBROMATOSIS I. The affected area can vary in size from a narrow strip to nearly half the body and may occur on either or both sides of the body.

Also Known As:

Neurofibromatosis type V; Segmental neurofibromatosis

Networked: 5 relevant articles (0 outcomes, 0 trials/studies)

Disease Context: Research Results

Related Diseases

1.	Neurofibromatoses (Neurofibromatosis)
2.	Psoriasis (Pustulosis Palmaris et Plantaris)
3.	Pityriasis Rubra Pilaris
4.	Nevus (Nevi)
5.	Drug Eruptions (Drug Eruption)

Experts

1.	Friedrich, Reinhard E: 1 article (06/2020)
2.	Hagel, Christian: 1 article (06/2020)
3.	Kohlrusch, Felix K: 1 article (06/2020)
4.	Schanze, Ina: 1 article (06/2020)
5.	Wieland, Ilse: 1 article (06/2020)
6.	Zenker, Martin: 1 article (06/2020)
7.	Arrese, Jorge E: 1 article (05/2017)
8.	Lebas, Eve: 1 article (05/2017)
9.	Nikkels, Arjen F: 1 article (05/2017)
10.	Quatresooz, Pascale: 1 article (05/2017)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Neurofibromatosis type 5:

1.	Neurofibromin 1 (Neurofibromin)IBA 04/01/2015 - "Segmental neurofibromatosis (SNF) is a rare type of neurofibromatosis (NF-1) resulting from post-zygotic somatic mutations in the neurofibromin gene that leads to mosaicism. "
2.	Nonsense Codon (Nonsense Mutation)IBA 09/01/2005 - "Gonosomal mosaicism for a nonsense mutation (R1947X) in the NF1 gene in segmental neurofibromatosis type 1."
3.	trans-crotonin (CTN)IBA 06/01/2016 - "Clinically, zosteriform CTN has similar morphology and distribution to nevus lipomatosus superficialis (NLS) or segmental neurofibromatosis. "

Therapies and Procedures

1.	Therapeutics 06/01/2020 - "Surgical therapy for segmental neurofibromatosis does not differ from the concepts for treating nerve sheath tumours in NF1 patients with a germline NF1 mutation."
2.	Drug Therapy (Chemotherapy) 05/01/2017 - "ESK may also be simultaneously observed with hepatic angiomas, chemotherapy, segmental neurofibromatosis, HIV or erythrodermic pityriasis rubra pilaris, psoriasis, and drug eruption. "