Pachydermoperiostosis (PDP) refers to rare osteo-arthro-dermopathic syndrome with familial and idiopathic forms. It is also known as primary hypertrophic osteoarthropathy because it is not due to a secondary cause such as lung disease.
Also known as Touraine-Solente-Golé syndrome, named after the three French physicians who described it in 1935 (see below).
PDP most commonly affects black people and adolescent boys.
PDP is characterised by marked thickening of the skin of the extremities, face, and scalp (including cutis verticis gyrata).
PDP is an autosomal dominant inherited disorder that is self-limiting and progresses for several years by stabilising.
Skeletal findings include symmetric, shaggy subperiosteal bone formation in the long bones, especially of the forearm and leg bones, but also of the metacarpals, metatarsals, and phalanges.
Involvement of the epiphyseal region distinguishes it from the secondary form, in which the epiphyses are usually spared 2.
There can be a widening of the ends of bones, especially in the wrist and knee joints which are due to increased bone formation. The generalised and symmetric periosteal reaction in pachydermoperiostosis tends to blend with the cortex and primarily involves the distal ends of the radius, ulna, tibia, and fibula.
History and etymology
Although not the first to describe the entity, the French physicians, Albert Touraine (1883-1961), G Solente and L Golé, were the first to recognise pachydermoperiostosis as a primary form of hypertrophic osteoarthropathy in 1935 4,5.
- 1. Rana RS, Wu JS, Eisenberg RL. Periosteal reaction. AJR Am J Roentgenol. 2009;193 (4): W259-72. doi:10.2214/AJR.09.3300 - Pubmed citation
- 2. Rastogi R, Suma GN, Prakash R et-al. Pachydermoperiostosis or primary hypertrophic osteoarthropathy: A rare clinicoradiologic case. Indian J Radiol Imaging. 19 (2): 123-6. doi:10.4103/0971-3026.50829 - Free text at pubmed - Pubmed citation
- 3. Chew FS. Skeletal Radiology. Lippincott Williams & Wilkins. (2010) ISBN:1608317064. Read it at Google Books - Find it at Amazon
- 4. Touraine A, Solente G, Gole L. Un syndrome osteoderopatique: La pachydermie plicaturee avec pachyeriostostose des extremites. Presse Med. 1935;43:1820–4.
- 5. Rajan TM, Sreekumar NC, Sarita S, Thushara KR. Touraine Solente Gole syndrome: The elephant skin disease. Indian journal of plastic surgery : official publication of the Association of Plastic Surgeons of India. 46 (3): 577-80. doi:10.4103/0970-0358.122025 - Pubmed