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Primary Hypertrophic Osteoarthropathy (Hypertrophic Osteoarthropathy, Primary)

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A conditioned chiefly characterized by thickening of the skin of the head and distal extremities, deep folds and furrows of the skin of the forehead, cheeks, and scalp, seborrhea, hyperhidrosis, periostosis of the long bones, digital clubbing, and spadelike enlargement of the hands and feet. It is more prevalent in the male, and is usually first evident during adolescence. It is believed to be inherited as an autosomal dominant trait. (From Dorland, 27th ed)

Also Known As:

Hypertrophic Osteoarthropathy, Primary; Pachydermoperiostosis; Osteoarthropathy, Primary Hypertrophic; Idiopathic Hypertrophic Osteoarthropathy; Hypertrophic Osteoarthropathies, Idiopathic; Hypertrophic Osteoarthropathies, Primary; Hypertrophic Osteoarthropathy, Idiopathic; Idiopathic Hypertrophic Osteoarthropathies; Osteoarthropathies, Idiopathic Hypertrophic; Osteoarthropathies, Primary Hypertrophic; Osteoarthropathy, Idiopathic Hypertrophic; Pachydermoperiostoses; Primary Hypertrophic Osteoarthropathies

Networked: 55 relevant articles (2 outcomes, 2 trials/studies)

Relationship Network

Disease Context: Research Results

Musculoskeletal Diseases: 179
- Bone Diseases: 3566
  - Primary Hypertrophic Osteoarthropathy: 55
    - Reginato Schiapachasse syndrome
- Joint Diseases: 1919
  - Primary Hypertrophic Osteoarthropathy: 55
    - Reginato Schiapachasse syndrome

Related Diseases

1.	Primary Hypertrophic Osteoarthropathy (Hypertrophic Osteoarthropathy, Primary)
2.	Primary Myelofibrosis (Myelosclerosis)
3.	Arthritis (Polyarthritis)
4.	Anemia
5.	Arthralgia (Joint Pain)

Experts

1.	Diggle, Christine P: 3 articles (08/2012 - 06/2008)
2.	Bonthron, David T: 3 articles (08/2012 - 06/2008)
3.	Carr, Ian M: 3 articles (08/2012 - 06/2008)
4.	Markham, Alexander F: 3 articles (08/2012 - 06/2008)
5.	Niizeki, Hironori: 2 articles (05/2015 - 01/2013)
6.	Hirakiyama, A: 2 articles (09/2014 - 05/2014)
7.	Kabashima, K: 2 articles (09/2014 - 05/2014)
8.	Seki, A: 2 articles (09/2014 - 05/2014)
9.	Niizeki, H: 2 articles (09/2014 - 05/2014)
10.	Okuyama, T: 2 articles (09/2014 - 05/2014)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Primary Hypertrophic Osteoarthropathy:

1.	Colchicine FDA LinkGeneric 01/01/1988 - "Colchicine treatment in a case of pachydermoperiostosis with acroosteolysis." 05/01/1992 - "In fourteen patients affected with pachydermoperiostosis (PDP), or primary hypertrophic osteoarthropathy, the efficacy of colchicine (0.5 mg day for one month) versus placebo on the main clinical features of the disease (finger clubbing, arthritis and pachydermia) was evaluated. " 01/01/1988 - "We report a case of pachydermoperiostosis with arthralgia, acroosteolysis, and recurrent staphylococcal folliculitis of the face, treated with colchicine (0.5 mg once daily for the 1st week and 0.5 mg twice daily for the next 3 weeks). "
2.	IronIBA 01/01/1991 - "Pachydermoperiostosis with myelofibrosis and anemia: report of a case of anemia of multifactorial causes and its improvement with steroid pulse and iron therapy."
3.	Isotretinoin (Accutane)FDA LinkGeneric 01/01/1988 - "Pachydermoperiostosis: trial with isotretinoin."
4.	Steroid Receptors (Steroid Receptor)IBA 01/01/1995 - "Pachydermoperiostosis: study of epidermal growth factor and steroid receptors." 01/01/1995 - "We studied a patient with the incomplete form of pachydermoperiostosis, assessing epidermal growth factor receptor (EGF-R) and sex hormone steroid receptors (SR) in the affected skin, and also evaluating the urinary excretion of EGF. "
5.	Epidermal Growth Factor (EGF)IBA 01/01/1995 - "Pachydermoperiostosis: study of epidermal growth factor and steroid receptors." 01/01/1995 - "We studied a patient with the incomplete form of pachydermoperiostosis, assessing epidermal growth factor receptor (EGF-R) and sex hormone steroid receptors (SR) in the affected skin, and also evaluating the urinary excretion of EGF. "
6.	4-hexyloxyaniline (HOA)IBA 09/01/2009 - "The familial or idiopathic HOA (pachydermoperiostosis) appears at puberty and is not associated with other underlying diseases. " 06/01/2003 - "We report a case of a family with a variant form of primary hypertrophic osteoarthropathy (HOA) restricted to the lower extremities without digital clubbing or cutaneous changes. " 07/01/1993 - "Primary hypertrophic osteoarthropathy (HOA) is a rare heritable syndrome in which digital clubbing and periostosis become evident without any underlying illness. " 09/01/2006 - "The pachydermoperiostosis (PDP) or primitive hypertrophic osteoarthropathy (HOA) is a rare hereditary disease. " 01/01/2006 - "Primary hypertrophic osteoarthropathy (HOA) is a rare cause ofjoint pain. "
7.	15-hydroxyprostaglandin dehydrogenaseIBA 03/01/2015 - "A novel recessive 15-hydroxyprostaglandin dehydrogenase mutation in a family with primary hypertrophic osteoarthropathy." 02/01/2013 - "Pachydermoperiostosis occurs owing to mutations of the gene encoding for 15-hydroxyprostaglandin dehydrogenase (15HPGD). " 03/01/2010 - "Homozygous mutations in HPGD gene, encoding 15-hydroxyprostaglandin dehydrogenase, have recently been associated with primary hypertrophic osteoarthropathy (PHO). " 12/01/2009 - "Recently, mutations in the gene HPGD, which encodes the NAD(+)-dependent 15-hydroxyprostaglandin dehydrogenase, were reported in four families affected with primary hypertrophic osteoarthropathy and one family with autosomal-recessive isolated nail clubbing. " 11/01/2009 - "Homozygous mutations in the 15-hydroxyprostaglandin dehydrogenase gene in patients with primary hypertrophic osteoarthropathy."
8.	Dinoprostone (PGE2)FDA Link 05/01/2015 - "Involvement of prostaglandin E2 in the first Japanese case of pachydermoperiostosis with HPGD mutation and recalcitrant leg ulcer." 10/01/2009 - "Prostaglandin E2 and bone turnover markers in the evaluation of primary hypertrophic osteoarthropathy (pachydermoperiostosis): a case report." 01/01/2013 - "Prostaglandin E2 increase in pachydermoperiostosis without 15-hydroprostaglandin dehydrogenase mutations."
9.	CosmeticsIBA 01/01/2008 - "The treatment of pachydermoperiostosis is usually centered around improving the cosmetic appearance through plastic surgery. " 01/18/1988 - "We report on a 38-year-old male patient suffering from pachydermoperiostosis (Touraine-Solente-Golé Syndrome), who underwent a cosmetic operation without success." 11/01/2005 - "The thickened, folded skin of Touraine-Solente-Golé syndrome (pachydermoperiostosis) can result in cosmetic and fuctional deformities. "
10.	Nonsense Codon (Nonsense Mutation)IBA 09/01/2014 - "The complete type of pachydermoperiostosis: a novel nonsense mutation p.E141* of the SLCO2A1 gene." 05/01/2014 - "The novel SLCO2A1 heterozygous missense mutation p.E427K and nonsense mutation p.R603* in a female patient with pachydermoperiostosis with an atypical phenotype."

Therapies and Procedures

1.	Rhytidoplasty (Facelift) 11/01/2014 - "Many techniques for addressing and correcting aesthetic defects associated with pachydermoperiostosis have been introduced, including facelift surgery. " 01/01/2008 - "Frontal rhytidectomy as surgical treatment for pachydermoperiostosis: a case report." 11/01/2003 - "Facelift was performed on five patients with pachydermoperiostosis. " 09/15/2003 - "Frontal rhytidectomy: a new approach to improve deep wrinkles in a case of pachydermoperiostosis." 01/01/2008 - "We describe the case of a 27-year-old male who had pachydermoperiostosis with a leonine facies that was managed with frontal rhytidectomy. "
2.	Oral Administration 01/01/2007 - "A case of pachydermoperiostosis treated by oral administration of a bisphosphonate and arthroscopic synovectomy."