Disease Information for Osteoarthropathy, hypertrophic primary

Hypertrophic osteoarthropathy is divided into primary and secondary forms; Pachydermoperiostosis (PDP), the primary form, accounts for 5% of all cases of hypertrophic osteoarthropathy; Secondary hypertrophic osteoarthropathy,

also called pulmonary hypertrophic osteoarthropathy, is associated with underlying cardiopulmonary diseases and malignancies; Primary PDP is a rare hereditary disorder that was first described in 1868; It is characterized by digital clubbing and subperiosteal new bone formation associated with pain, polyarthritis, cutis verticis gyrata, seborrhea, and hyperhidrosis; Pachydermia o the facial skin and/or scalp) and periostitis, (2) an incomplete form with evidence of bone abnormalities but lacking pachydermia, and (3) a forme fruste with prominent pachydermia and minimal-to-absent skeletal changes; rare in USA; Although progression of PDP typically ceases after 10 years, the disorder can result in significant morbidity; patients may develop disabilities, which include severe kyphosis, restricted motion, and

neurologic manifestations; PDP is more common in African Americans than in whites; The male-to-female case ratio is approximately 7:1; PDP typically begins during childhood or adolescence and progresses gradually over the next decade; Enlargement of the fingers and the toes; Swelling or pain of the large joints; Coarsening of facial features; Grooves or depressions in the scalp; Oily, scaly facial skin; Excessive sweating of the palms, soles, or other areas; Physical: Skin, hair, and nail examination may reveal the following: Digital clubbing and/or paronychial thickening may be observed; Coarse facial features may be reminiscent of acromegaly; Facial skin changes may include sclerodermoid thickening and furrowing of the skin on

the forehead and the cheeks; Leonine facies may occur in advanced stages; Cutis verticis gyrata (undulating grooved and thickened scalp) may become apparent during adolescence; Seborrheic dermatitis of the face and the scalp may be present; Palmoplantar hyperhidrosis or generalized hyperhidrosis characterized by shiny and/or wet skin may be observed; Dermatitis of the hands and the feet may be associated with hyperhidrosis; PDP is often familial; It is believed to be inherited in an autosomal dominant pattern with variable penetrance; however, autosomal recessive forms have been reported;

econdary hypertrophic pulmonary osteoarthropathy must be excluded; Radiographs of the long bones reveal subperiosteal new bone formation, which is a characteristic radiologic feature; This feature is mainly seen in the distal tibia; the fibula; the radius; the ulna; the metacarpals; the phalanges; and, less frequently, the metatarsals; RX: Nonsteroidal anti-inflammatory drugs (NSAIDs) or corticosteroids may alleviate the polyarthritis associated with PDP; Vagotomy may improve the articular pain and swelling associated with PDP; Plastic surgery may improve the appearance of the face

; [EMedicine 2005]

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