pachydermoperiostosis

Lost bones:
differential diagnosis of acro-osteolysis seen by the pediatric rheumatologist. Frontal (a) and oblique (b) radiographs of the hands in a 14 year old male patient with primary hypertrophic osteoarthropathy demonstrate bilateral and symmetric tuft resorption and remodeling. Smooth periostitis involves the proximal phalangeal shafts (arrows). Frontal radiograph of the feet (c) shows smooth tapering of the distal tufts
ped-rheum.biomedcentral.com • CC-by-4.0
Pachydermoperiostosis
(Touraine–Solente–Gole syndrome): a case report. Radiography of skull showed mild cortical and subperiosteal thickening
jmedicalcasereports.biomedcentral.com • CC-by-4.0
Pachydermoperiostosis
(Touraine–Solente–Gole syndrome): a case report. Radiograph of bilateral knees showing periosteal hypertrophy with subperiosteal bone formation
jmedicalcasereports.biomedcentral.com • CC-by-4.0
Pachydermoperiostosis
(Touraine–Solente–Gole syndrome): a case report. Radiograph of bilateral ankles showing irregular subperiosteal bone formation and cortical thickening of distal tibia, fibula, calcaneum, and talus
jmedicalcasereports.biomedcentral.com • CC-by-4.0
Pachydermoperiostosis
(Touraine–Solente–Gole syndrome): a case report. Radiograph of bilateral hands showing soft tissue tumefaction, hyperostosis of metacarpal, and proximal phalanges
jmedicalcasereports.biomedcentral.com • CC-by-4.0
Pachydermoperiostosis
- A rare radiologic case. Symmetric, shaggy and exuberant subperiosteal new bone formation. There is expansion of the proximal end of the tibia with a reduction in the femoro-tibial joint spaces.
www.eurorad.org • CC by-nc-sa-4.0
Pachydermoperiostosis
- A rare radiologic case. Exuberant and shaggy subperiosteal new bone formation. There is expansion of the distal end of the radius and ulna with a reduction in the radio-carpal joint space.
www.eurorad.org • CC by-nc-sa-4.0
Pachydermoperiostosis
- A rare radiologic case. There is reduction in PIP and DIP joint spaces. Periarticular osteopaenia and acroosteolysis with associated soft tissue swelling and contracture of distal fingers. Widening of the base of the distal phalanx of all fingers.
www.eurorad.org • CC by-nc-sa-4.0
Pachydermoperiostosis
- A rare radiologic case. There is evidence of collapse of calcaneum and calcification of Achilles tendon.
www.eurorad.org • CC by-nc-sa-4.0
Pachydermoperiostosis
- A rare radiologic case. Symmetrical and shaggy periosteal reaction along lower lateral aspect of iliac bones. There is metaphyseal widening of both femurs. Cortical widening and widening of femoral shafts is seen.
www.eurorad.org • CC by-nc-sa-4.0
Pachydermoperiostosis
- A rare radiologic case. Mild hyperostosis of the skull bones in parieto-occipital region. Sella turcica is normal.
www.eurorad.org • CC by-nc-sa-4.0
Pachydermoperiostosis
- A rare radiologic case. There is reduction in PIP and DIP joint spaces. Periarticular osteopaenia and resorption of the distal phalanges. Associated soft tissue swelling and contractres of toes. Enlargement and deformities of sesamoid bones.
www.eurorad.org • CC by-nc-sa-4.0
Pachydermoperiostosis
- A rare radiologic case. Mild effusion with thickened synovium bilaterally. The effusion was seen to extend into suprapatellar bursae.
www.eurorad.org • CC by-nc-sa-4.0
Radiopaedia • CC-by-nc-sa 3.0 • de

Pachydermoperiostosis (PDP) refers to a rare osteoarthrodermopathic 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.

Terminology

Also known as Touraine-Solente-Golé syndrome, named after the three French physicians who described it in 1935 (see below).

Epidemiology

PDP most commonly affects black people and adolescent boys.

Clinical presentation

PDP is characterized by marked thickening of the skin of the extremities, face, and scalp (including cutis verticis gyrata).

Pathology

PDP is an autosomal dominant inherited disorder that is self-limiting and progresses for several years by stabilizing.

Radiographic features

Plain radiograph/CT

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 .

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 generalized 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 recognize pachydermoperiostosis as a primary form of hypertrophic osteoarthropathy in 1935 .

Siehe auch:
und weiter:
Assoziationen und Differentialdiagnosen zu primäre hypertrophe Osteoarthropathie:
hypertrophe
Osteoarthropathie
Wikipedia • CC BY-SA 3.0