Publication: Pachydermoperiostosis And An Eyelid Ptosis Associated With Spiky Keratoderma – An Unusual Presentation
Date
2014
Authors
Journal Title
Journal ISSN
Volume Title
Publisher
Society of Dermatologists, Venereologists and Leprologists of Nepal (SODVELON)
Abstract
Abstract:
Pachydermoperiostosis, ( PDP), a rare hereditary disorder that is characterized by digital clubbing, pachydermia, subperiosteal new bone formation, associated with pain, polyarthritis, cutis verticis gyrata, seborrhoea and hyperhidrosis. It mainly presents due to disfiguring facial features, widening of the limbs and bone and joint pain. It affects male population predominantly and transmitted as an autosomal dominant trait. There are many constant features associated with it as well as rarer features. Increased proliferation of the fibroblast and dysregulation of matrix protein play a central role in the pathogenesis of the disease. They are diagnosed primarily on clinical and radiological grounds. Histopathology plays a supportive role in the diagnosis. Such a case has to be differentiated from several other conditions like acromegaly, neurofibroma,,myxedema, primary systemic amyloidosis as wel as from other causes of digital clubbing.
The case which is described here is a complete form of PDP which is a rare disease reported in Indian context and that too with the a rarer presentation of spiky palmoplanter keratoderma and severe mechanical ptosis leading to severe visual impairment which was corrected after surgical manipulation of the ptosis.
Keywords: Keratoderma, Pachydermoperiostosis, Ptosis
Description
S Das
Assosciate Professor, Department of Dermatology NRS Medical College and Hospital
S Chakraborty
RMO, Department of Dermatology, NRS Medical College and Hospital, Kolkata
NS Sarkar
Assistant Professor, Department of Ophthalmology, NRS Medical College and Hospital, Kolkata