Pachydermoperiostosis : Causes and Symptoms

What are the features of osteoskleroticheskoy reconstruction of bones and joints with pachydermoperiostosis and the main manifestations of the disease.

pachydermoperiostosis or hypertrophic osteoarthropathy (Touraine syndrome - Solanta - Gol) - hereditary form osteoskleroticheskoy reconstruction of bones and joints, the main manifestation of which is the thickening of the periosteum (periostoz) with the formation of osteophytes, bilateral symmetric thickening (hyperostosis) bone limb deformityfingers and toes, thickening of the skin and sebaceous gland hyperplasia.

Features of pachydermoperiostosis

disease is still not fully understood.It is more common in adolescents and young men (15-30let) is inherited in an autosomal dominant pattern, there are cases of family illness.The first symptoms are already visible by the third year of life, but most of the development of the disease reaches the maximum in adolescence, it is believed that it was important hormonal changes.

Changes in the bones an
d joints in pachydermoperiostosis

When pachydermoperiostosis marked thickening of the superficial layer of bone - the periosteum, it becomes rough at the edges of the flat bones are formed by local thickenings - nodules, there is bone growth in width, and in the joints, in placesfixing them tendons and ligaments are formed bony spikes - osteophytes.Thickening of nail phalanxes of fingers attached to the form of drum sticks, nails at the same time take the form of time windows.Thickening of nail phalanxes of fingers attached to the form of drum sticks, nails at the same time take the form of time windows.

simultaneously observed deformation of the vertebral bodies with symptoms of radiculitis.When long-existing disease in the lumbar-sacral spine and joints formed ankylosis.

main clinical manifestations pachydermoperiostosis.

disease begins with subtle common symptoms gradually.At the same time patients worried about the growing weakness in children decreases physical activity, they quickly get tired.For several years, formed a thickening of nail phalanges, while the skin thickens, it becomes less elastic and forms wrinkles, coarse wrinkles, more severe on the face and scalp, hands and feet - seemingly patient ages.The sebaceous and sweat glands are functioning actively, which leads to hyperhidrosis (excessive sweating), to inflammation of the follicles (folliculitis), seborrheic dermatitis, scarring.Simultaneously there is a thickening of the cartilage tissue in the upper eyelids, with the formation of cysts and granulomas, accompanied by changes in the conjunctiva (the mucous membranes of the eyes),

The joints at this time may develop swelling, leading to restriction of range of motion in their cavity accumulates fluid, disturb pains injoints.Osteoarthritis arthritis prone to chronic recurrence.Forecast favorable disease.

Diagnostics pachydermoperiostosis

Diagnosis of the disease is difficult, because in some cases, similar to other diseases of metabolism in bone tissue.Is generally carried out:

  • Radiological research (including computed tomography), MRI reveal thickening of the periosteum and distal strain - remote sections of long bones, the calluses in areas ligament fixation, tendon, bone marrow filling channels cancellous bone).
  • The blood may be increasing the level of 17-keto steroids, cortisone, and reduced allotetrahondrokortizola and aldosterone, increased activity of alkaline phosphatase.
  • Biopsy synovium of affected joints can detect the proliferation of the synovial membrane of the type of chronic synovitis, as well as the expansion of surface vessels in it, the deposition of iron-containing pigment hemosiderin.


pachydermoperiostosis

Treatment is mainly symptomatic treatment.Arthritis treatment is carried NSAIDs, glucocorticoids, including in the form blockages.When a cluster of effusion in the joint cavity, periarticular spend their bags with a puncture and aspiration of content administration with corticosteroids, immunosuppressants.With the ineffectiveness of the conservative conduct joint surgery with excision of the hyperplastic synovium, periarticular bag (synovectomy).

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