Facial Plexiform Neurofibromatosis in a 16 Year-Old
Female from Eastern Nigeria: A Rare Presentation
John E Arute1, Paul JC Nwosu2,
Patrick O Erah3*
1Department
of Clinical Pharmacy, Faculty of Pharmacy, Madonna
University, Elele Campus, Rivers State, Nigeria.
E-mail:
arute4john@yahoo.com
2Department
of Surgery, Faculty of Medicine, College of Medicine,
Madonna University, Elele Campus, Rivers State, Nigeria.
3Department
of Clinical Pharmacy and Pharmacy Practice, Faculty of
Pharmacy, University of Benin, Benin City, Nigeria.
*For
Correspondence:
E-mail:
p_erah@yahoo.com
Tel:
+234-802-336-0318
International Journal of Health Research,
September
2008; 1(3):
105-110
Received: 30-Jul-08 Revision received:
13-Aug-08 Accepted for publication: 14-Aug-08
Case Report
Abstract
Plexiform
neurofibromatosis or von Pleckinghausen’s disease is an
uncommon variety of neurofibromatosis type I, usually
associated with trigeminal nerve. We report a rare case
of the disease on the face of a 16 year-old female
patient. The patient presented with overhanging mass of
skin folds on the face, completely covering one eye and
partially covering the second eye, as well as occasional
pain and itching. Physical examination revealed the
presence of café au lait macules, freckling in the
axillary, optic glioma and iris hamartomas. Family
history was not contributory. An interventional
reconstructive surgical procedure with excision of
surplus skin folds on the face and lip margin was
performed on the patient. Doloneurobion® (paracetamol
and vitamins B1, B6 and B12 combination) as well as
Vecuten® (neomycin sulphate, clortrimazole
and dexamethosone combination) cream were used to
successfully manage the occasional pain and itching.
There was evidence of re-growth of the tumours over a
one year follow-up period.
Keywords:
Facial
plexiform neurofibromatosis, von Pleckinghausen’s
disease, neurofibromatosis type I, Eastern Nigeria.
