Abstract
Primary hypertrophic osteoarthropathy or pachydermoperiostosis is a rare congenital disease characterized by clubbing of the fingers, periostitis of the distal long bones, and hypertrophic skin changes (pachydermia) including thick folds in the skin of the face, forehead, scalp and extremities, and also joint pain. Clinical manifestations of this disease generally appear among the young and middle-aged. In this article we report a case of a 43-year-old man with pachydermoperiostosis. His skin and joint manifestations were prominent. He had also anemia, and bone marrow biopsy showed myelofibrosis.
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References
Touraine A, Solente G, Gole L (1935) Un syndrome osteodermopathique. La pachydermie plicaturee avec pachyperiostose des extremities. Press Med 43:1820–1824
Gilliland BC (2005) Hypertrophic osteoarthropathy and clubbing. In: Kasper DL et al (eds) Harrison’s principles of internal medicine, 16th edn. Mc Graw-Hill, New York, pp 2061–2063
Cooper RG, Freemont AJ, Riley M et al (1992) Bone abnormalities and server arthritis in pachydermoperiostosis. Ann Rheum Dis 51:416–419
Burns T et al (2004) Rook’s text book of dermatology, 7th edn Black well, Oxford, pp 12-72–3
Odom RB, James WD, Berger TG (2000) Andrews diseases of the skin, 9th edn. Saunders, Philadelphia pp 724–726
Altman RD, Tenenbaum J (2005) Hypertrophic osteoarthropathy. In: Harris DE Jr et al (eds) Kelley’s text book of rheumatology, 7th edn. Elsevier, Philadelphia, pp 1748–1753
Matucci-Cerinic M, Lotti T, Jajic I et al (1991) The clinical spectrum of primary hypertrophic osteoarthropathy. Medicine 70:208–214
Resnick D (ed) (2002) Diagnosis of bone and joint disorders, 4th edn. Saunders, Philadelphia, pp 4870–4885
Moschella SL, Hurley HJ (1992) Dermatology, 3rd edn. Saunders. Philadelphia, pp 1362–1364
Martinez-Lavin M, Pineda C, Valdez T et al (1988) Primary hypertrophic osteoarthropathy. Semin Arthritis Rheum 17:156–162
Hedayati H, Barmada R, Skosey JL (1980) Acrolysis in pachydermoperiostosis, primary or idiopathic hypertrophic osteoarthropathy. Arch Intern Med 140:1087
Sega AM, Mackenzie AH (1982) Hypertrophic osteoarthropathy: a ten year retrospective analysis. Semin Arthritis Rheum 12:220
Lauter SA, Vasey FB, Huttner I, Osterland Ck (1976) Pachydermoperiotosis: studies on the synoviom. J Rheumatol 5:85
Lam SK, Hui WKK, Ho J et al (1983) Pachydermoperiostosis, hypertrophic gastropathy, and peptic ulcer. Gastroenterology 84:834–839
Venencie PY, Boffa GA, Delmas PD et al (1988) Pachydermoperiostosis with gastric hypertrophy, anemia, and increased serum bone Gla-protein levels. Arch Dermatol 124:1831–1834
Neiman HL, Gompels BM, Martel W (1974) Pachydermoperiostosis with bone marrow failure and gross extramedullary hematopoiesis. Report of a case Radiology 110:553
Tanaka H, Maehamaf S, Imanaka F et al (1991) Pachydermoperiostosis with myelofibrosis and anemia: report of a case of anemia of multifactorial causes and its improvement with steroid pulse and iron therapy. Jpn J Med 30:73–80
Fontenay-Roupie M,Dupuy E, Berrou E et al (1995) Increased proliferation of bone marrow—derived fibroblasts in primitive hypertrophic osteoarthropathy with severe myelofibrosis. Blood 85:3229–3238
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Saghafi, M., Azarian, A. & Nohesara, N. Primary hypertrophic osteoarthropathy with myelofibrosis. Rheumatol Int 28, 597–600 (2008). https://doi.org/10.1007/s00296-007-0477-4
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DOI: https://doi.org/10.1007/s00296-007-0477-4