Primary Hypertrophic Osteoarthropathy: A Case Report

  • Shima Asadi komeleh Department of Rheumatology, School of Medicine AND Vali-Asr Hospital, Tehran University of Medical Sciences, Tehran, Iran.
  • Abdolrahman Rostamian Department of Rheumatology, School of Medicine AND Vali-Asr Hospital AND Center for Research on Occupational Disease, Tehran University of Medical Sciences, Tehran, Iran.
  • Fatemeh Shahbazi Department of Biology, Payame Noor University, Tehran, Iran.
  • Shafieh Movassagi Department of Rheumatology, School of Medicine AND Vali-Asr Hospital, Tehran University of Medical Sciences, Tehran, Iran.
  • Parviz Soofivand Department of Rheumatology, School of Medicine AND Vali-Asr Hospital, Tehran University of Medical Sciences, Tehran, Iran.
Keywords: Idiopathic hypertrophic osteoarthropathy, Pachydermoperiostosis, Skinfold thickness


The primary hypertrophic osteoarthropathy (PHOA or pachydermoperiostosis) is a rare (5% of total HOA) hereditary disease. One study described that the prevalence of PHOA is 0.16%. PHOA characterized by skin thickening (pachydermia), finger clubbing, and proliferation of periosteum (periostitis) with subperiosteal new bone formation and enlarged extremities secondary to periarticular and bone proliferation. Clinical manifestations are variable; the term complete syndrome is used for the patient with pachydermia, coarsening of the face skin and scalp, periostitis, and cutis verticis gyrata; the incomplete form is used when there is no sparing of the scalp; and the frusted form is used for pachydermia with minimal or absent periostitis. We describe a 29-year-old white man with PHOA, and clinical and radiological characteristics of this syndrome, as well as therapeutic approach of PHOA.


Zanon AB, Faccin MP, Alvarenga Anti SM, Silva Melo CruzI FJ, Rosa RF. Primary hypertrophic osteoarthropathy: Case report and literature review. Rev Bras Reumatol 2009; 49(4): 447-55.

Gomez Rodriguez N, Ibanez Ruan J, Gonzalez P Perez M. Primary hypertrophic osteoarthropathy (pachydermoperiostosis). Report of two familial cases and literature revie. Reumatol Clin 2009; 5(6): 259-63.

Sinha GP, Curtis P, Haigh D, Lealman GT, Dodds W, Bennett CP. Pachydermoperiostosis in childhood. Br J Rheumatol 1997; 36(11): 1224-7.

Auger M, Stavrianeas N. Pachydermoperiostosis. Orphaned encyclopedia [Online]. [cited 2004]; Available from: URL:

Santos-Duran JC, Yuste-Chaves M, MartinezGonzalez O, Alonso San Pablo MT, SanchezEstella J. Pachydermoperiostosis (TouraineSolente-Gole syndrome). Case report. Actas Dermosifiliogr 2007; 98(2): 116-20.

Cavallasca JA, Malah VA, Fernandez DE, Carbia SG, Nasswetter GG. Paquidermoperiostosis (osteoartropatia hipertrofica primaria). Medicina (B Aires) 2006; 66(2): 147-96.

Jajic Z, Jajic I, Nemcic T. Primary hypertrophic osteoarthropathy: Clinical, radiologic, and scintigraphic characteristics. Arch Med Res 2001; 32(2): 136-42.

Matucci-Cerinic M, Lotti T, Jajic I, Pignone A, Bussani C, Cagnoni M. The clinical spectrum of pachydermoperiostosis (primary

hypertrophic osteoarthropathy). Medicine (Baltimore) 1991; 70(3): 208-14.

Carvalho TN, Araujo CR Jr, Fraguas SRF, Costa MAB, Teixeira KS, Ximenes CA. Primary hypertrophic osteoarthropathy (pachydermoperiostosis): Report of cases in two brothers. Radiol Bras 2004; 37(2): 147-9. [In Portuguese].

Resnick D. Diagnosis of bone and joint disorders. Philadelphia, PA: Saunders; 1995.

Diamond S, Momeni M. Primary hypertrophic osteoarthropathy in a patient with rheumatoid arthritis. J Clin Rheumatol 2007; 13(4): 242-3.

Stoker DJ. Pachydermoperiostosis mimicking acromegaly. J R Soc Med 1992; 85(5): 306.

Castori M, Sinibaldi L, Mingarelli R, Lachman RS, Rimoin DL, Dallapiccola B. Pachydermoperiostosis: An update. Clin Genet 2005; 68(6): 477-86.

Angel-Moreno Maroto A, Martinez-Quintana E, Suarez-Castellano L, Perez-Arellano JL. Painful hypertrophic osteoarthropathy successfully treated with octreotide. The pathogenetic role of vascular endothelial growth factor (VEGF). Rheumatology (Oxford) 2005; 44(10): 1326-7.

Silveira LH, Martinez-Lavin M, Pineda C, Fonseca MC, Navarro C, Nava A. Vascular endothelial growth factor and hypertrophic osteoarthropathy. Clin Exp Rheumatol 2000; 18(1): 57-62.

Touraine A, Solente G, Golé L. Osteodermopathic syndrome: plicated pachydermia with pachydermoperiostosis of the extremities. Presse Med 1935; 43: 1820-4. [In French].

Seyhan T, Ozerdem OR, Aliagaoglu C. Severe complete pachydermoperiostosis (TouraineSolente-Gole syndrome). Dermatol Surg 2005; 31(11 Pt 1): 1465-7.

Kerimovic-Morina DJ, Mladenovic V. Primary hypertrophic osteoarthropathy in 32 patients. Clin Exp Rheumatol 1992; 1002(10): 51-6.

Latos-Bielenska A, Marik I, Kuklik M, Materna-Kiryluk A, Povysil C, Kozlowski K. Pachydermoperiostosis-critical analysis with

report of five unusual cases. Eur J Pediatr 2007; 166(12): 1237-43.

Fietta P, Manganelli P. Pachydermoperiostosis and psoriatic onychopathy: An unusual association. J Eur Acad Dermatol Venereol 2003; 17(1): 73-6.

Gaston-Garrette F, Porteau-Cassard L, Marc V, Zabraniecki L, Ginesty E, Andrieu V, et al. A case of primary hypertrophic osteoarthropathy without skin involvement (Currarino's disease). Rev Rhum Engl Ed 1998; 65(10): 591-3.

Shinjo SK, Levy-Neto M, Borba EF. Palindromic rheumatism associated with primary hypertrophic osteoarthropathy. Clinics (Sao Paulo) 2006; 61(6): 581-3.

Pignone A, Calabro A, Rotter JI. Gastric abnormalities in pachydermoperiostosis. Clin Exp Rheumatol 1992; 10(suppl 7): 72.

Ikeda F, Okada H, Mizuno M, Kawamoto H, Okano N, Okazaki H, et al. Pachydermoperiostosis associated with juvenile polyps of the stomach and gastric adenocarcinoma. J Gastroenterol 2004; 39(4): 370-4.

Shim YW, Suh JS. Primary hypertrophic osteoarthropathy accompanied by Crohn's disease: A case report. Yonsei Med J 1997; 38(5): 319-22.

Bianchi L, Lubrano C, Carrozzo AM, Iraci S, Tomassoli M, Spera G, et al. Pachydermoperiostosis: Study of epidermal growth factor and steroid receptors. Br J Dermatol 1995; 132(1): 128-33.

How to Cite
Asadi komeleh S, Rostamian A, Shahbazi F, Movassagi S, Soofivand P. Primary Hypertrophic Osteoarthropathy: A Case Report. CRCP. 3(1):2-6.
Case Report(s)