Skip to main content

Advertisement

SpringerLink
Log in

Prostaglandin E2 and bone turnover markers in the evaluation of primary hypertrophic osteoarthropathy (pachydermoperiostosis): a case report

  • Case Report
  • Published:
Clinical Rheumatology Aims and scope Submit manuscript

Abstract

Primary hypertrophic osteoarthropathy, or pachydermoperiostosis (PDP), is an infrequent genetic condition characterized by digital clubbing, periostosis, and pachydermia and is distinct from a more common form, secondary hypertrophic osteoarthropathy, which always associates with an underlying cause (frequently pulmonary or cardiac disease). The diagnosis of this disorder as well as its clinical evaluation can be difficult. We report a 15-year-old boy presenting with intermittent arthralgias and clubbing of fingers and toes for the previous 2 years. The ankles and knees were enlarged, and X-rays showed periosteal apposition. The search for a secondary cause was negative. The skin appearance was normal, but a skin biopsy was indicative of pachydermia, further confirming the diagnosis of PDP. Bone turnover markers were increased at diagnosis and progressively decreased during follow-up; prostaglandin E2, a recently implicated mediator of this disorder, was markedly elevated. In the present case, carrying out a skin biopsy helped us to diagnose this condition. In addition, bone turnover markers were useful for monitoring the disease activity; whereas, increased prostaglandin E2 levels seems to confirm the role of this mediator in the etiopathogenesis of this disorder.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

References

  1. Okten A, Mungan I, Kalyoncu M, Orbak Z (2007) Two cases with pachydermoperiostosis and discussion of tamoxifen citrate treatment for arthralgia. Clin Rheumatol 26:8–11

    Article  PubMed  Google Scholar 

  2. Santos-Durán JC, Yuste-Chaves M, Martínez-González O, Alonso-San Pablo MT, Sánchez-Estella J (2007) Pachydermoperiostosis (Touraine-Solente-Golé syndrome). Case report. Actas Dermosifiliogr 98:116–120

    Article  PubMed  Google Scholar 

  3. Kumar U, Prakash Bhatt S, Misra A (2008) Unusual associations of pachydermoperiostosis: a case report. Indian J Med Sci 62:65–68

    Article  PubMed  Google Scholar 

  4. Castori M, Sinibaldi L, Mingarelli R, Lachman RS, Rimoin DL, Dallapiccola B (2005) Pachydermoperiostosis: an update. Clin Genet 68:477–486

    Article  PubMed  CAS  Google Scholar 

  5. Uppal S, Diggle CP, Carr IM, Fishwick CWG, Ahmed M, Ibrahim GH et al (2008) Mutation in 15-hydroxyprostaglandin dehydrogenase cause primary hypertrophic osteoarthropathy. Nat Genet 40:789–793

    Article  PubMed  CAS  Google Scholar 

  6. Rendina D, De Filippo G, Viceconti R, Soscia E, Sirignano C, Salvatore M et al (2008) Interleukin (IL)-6 and receptor activator of nuclear factor (NF)-κB ligand (RANKL) are increased in the serum of a patient with primary pachydermoperiostosis. Scand J Rheumatol 37:225–229

    Article  PubMed  CAS  Google Scholar 

  7. Rubini G, Lauriero F, Rubini D, D’Addabbo A (1993) 99Tcm-MDP global skeletal uptake and markers of bone metabolism in patients with bone diseases. Nucl Med Commun 14:567–572

    Article  PubMed  CAS  Google Scholar 

  8. Jojima H, Kinoshita K, Naito M (2007) A case of pachydermoperiostosis treated by oral administration of a bisphosphonate and arthroscopic synovectomy. Mod Rheumatol 17:330–332

    Article  PubMed  Google Scholar 

  9. Crofton PM, Evans N, Taylor MRH, Holland CV (2004) Procollagen type I amino-terminal propeptide: pediatric reference data and relationship with procollagen type I carboxyl-terminal propeptide. Clin Chem 50:2173–2176

    Article  PubMed  CAS  Google Scholar 

  10. Yang L, Grey V (2006) Pediatric intervals for bone markers. Clin Biochem 39:561–568

    Article  PubMed  CAS  Google Scholar 

  11. Van Coeverden SCCM, Netelenbos JC, de Ridder CM, Roos JC, Popp-Snijders C, Delemarre-van de Waal HA (2002) Bone metabolism markers and bone mass in healthy puberal boys and girls. Clin Endocrinol 57:107–116

    Article  Google Scholar 

  12. Rauchenzauner M, Schmid A, Heinz-Erian P, Kapelari K, Falkensammer G, Griesmacher A et al (2007) Sex- and age-specific reference curves for serum markers of bone turnover in healthy children from 2 months to 18 years. J Clin Endocrinol Metab 92:443–449

    Article  PubMed  CAS  Google Scholar 

  13. Kabi F, Mkinsi O, Janani S, Raissouni N (2006) Pachydermoperiostosis. A case report. Rev Med Interne 27:710–712

    Article  PubMed  CAS  Google Scholar 

  14. Matsumoto T, Tsurumoto T, Shindo H (2003) A case of pachydermoperiostosis associated with arthritis. Mod Rheumatol 13:371–373

    Article  Google Scholar 

  15. Sasaki T, Nakajima H (1989) Prolyl hydroxylase and procollagen III levels in the sera from patients with collagen diseases and psoriasis. J Dermatol 16:207–211

    PubMed  CAS  Google Scholar 

Download references

Acknowledgement

The authors thank Dr. M. Whyte for his invaluable help.

Disclosures

None.

Author information

Authors and Affiliations

  1. Rheumatology Service, Hospital Clinic, IDIBAPS, University of Barcelona, Villarroel 170, 08036, Barcelona, Spain

    A. Martínez-Ferrer, P. Peris & N. Guañabens

  2. Department of Pathology, Hospital Clinic, IDIBAPS, University of Barcelona, Villarroel 170, Barcelona, Spain

    Ll. Alós

  3. Biochemistry and Molecular Genetics Department, Hospital Clinic, IDIBAPS, University of Barcelona, Villarroel 170, Barcelona, Spain

    M. Morales-Ruiz

Authors
  1. A. Martínez-Ferrer
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. P. Peris
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Ll. Alós
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. M. Morales-Ruiz
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. N. Guañabens
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to A. Martínez-Ferrer.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Martínez-Ferrer, A., Peris, P., Alós, L. et al. Prostaglandin E2 and bone turnover markers in the evaluation of primary hypertrophic osteoarthropathy (pachydermoperiostosis): a case report. Clin Rheumatol 28, 1229–1233 (2009). https://doi.org/10.1007/s10067-009-1197-9

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10067-009-1197-9

Keywords

Search

Navigation