Pachydermoperiostosis And An Eyelid Ptosis Associated With Spiky Keratoderma – An Unusual Presentation
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, widen...
Saved in:
| Main Authors: | , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Society of Dermatologists, Venereologists and Leprologists of Nepal (SODVELON)
2016-01-01
|
| Series: | Nepal Journal of Dermatology, Venereology & Leprology |
| Subjects: | |
| Online Access: | https://nepjol.info/index.php/NJDVL/article/view/10603 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1849226763830296576 |
|---|---|
| author | S Das S Chakraborty NS Sarkar |
| author_facet | S Das S Chakraborty NS Sarkar |
| author_sort | S Das |
| collection | DOAJ |
| description |
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.
DOI: http://dx.doi.org/10.3126/njdvl.v12i1.10603
Nepal Journal of Dermatology, Venereology & Leprology Vol.12(1) 2014 pp.48-52
|
| format | Article |
| id | doaj-art-fbea5c1fbf7c40af8dac08c20e5bd589 |
| institution | Kabale University |
| issn | 2091-0231 2091-167X |
| language | English |
| publishDate | 2016-01-01 |
| publisher | Society of Dermatologists, Venereologists and Leprologists of Nepal (SODVELON) |
| record_format | Article |
| series | Nepal Journal of Dermatology, Venereology & Leprology |
| spelling | doaj-art-fbea5c1fbf7c40af8dac08c20e5bd5892025-08-24T10:06:43ZengSociety of Dermatologists, Venereologists and Leprologists of Nepal (SODVELON)Nepal Journal of Dermatology, Venereology & Leprology2091-02312091-167X2016-01-01121Pachydermoperiostosis And An Eyelid Ptosis Associated With Spiky Keratoderma – An Unusual PresentationS Das0S Chakraborty1NS Sarkar2Assosciate Professor, Department of Dermatology NRS Medical College and HospitalRMO, Department of Dermatology, NRS Medical College and Hospital, KolkataAssistant Professor, Department of Ophthalmology, NRS Medical College and Hospital, Kolkata 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. DOI: http://dx.doi.org/10.3126/njdvl.v12i1.10603 Nepal Journal of Dermatology, Venereology & Leprology Vol.12(1) 2014 pp.48-52 https://nepjol.info/index.php/NJDVL/article/view/10603KeratodermaPachydermoperiostosisPtosis |
| spellingShingle | S Das S Chakraborty NS Sarkar Pachydermoperiostosis And An Eyelid Ptosis Associated With Spiky Keratoderma – An Unusual Presentation Nepal Journal of Dermatology, Venereology & Leprology Keratoderma Pachydermoperiostosis Ptosis |
| title | Pachydermoperiostosis And An Eyelid Ptosis Associated With Spiky Keratoderma – An Unusual Presentation |
| title_full | Pachydermoperiostosis And An Eyelid Ptosis Associated With Spiky Keratoderma – An Unusual Presentation |
| title_fullStr | Pachydermoperiostosis And An Eyelid Ptosis Associated With Spiky Keratoderma – An Unusual Presentation |
| title_full_unstemmed | Pachydermoperiostosis And An Eyelid Ptosis Associated With Spiky Keratoderma – An Unusual Presentation |
| title_short | Pachydermoperiostosis And An Eyelid Ptosis Associated With Spiky Keratoderma – An Unusual Presentation |
| title_sort | pachydermoperiostosis and an eyelid ptosis associated with spiky keratoderma an unusual presentation |
| topic | Keratoderma Pachydermoperiostosis Ptosis |
| url | https://nepjol.info/index.php/NJDVL/article/view/10603 |
| work_keys_str_mv | AT sdas pachydermoperiostosisandaneyelidptosisassociatedwithspikykeratodermaanunusualpresentation AT schakraborty pachydermoperiostosisandaneyelidptosisassociatedwithspikykeratodermaanunusualpresentation AT nssarkar pachydermoperiostosisandaneyelidptosisassociatedwithspikykeratodermaanunusualpresentation |