Disease Information for Osteoarthropathy, hypertrophic primary

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Clinical Manifestations
Signs & Symptoms
Furrowed forehead
Leonine Facies/Jowles
Clubbing
Clubbing of fingers/toes
Grooves/Depressions in Scalp
Hyperhidrosis/Chronic sweating excess
Pachyderma Facial Skin
Scalp rash/Oily scales/pruritic
Sweating Excess Perspiration
Thickened skin/diffuse
Hand Pains
Hip Inner Thigh Pain
Leg/lower extremity pains
Pain in Extremities/Melalgias
Sweating palms
Arthralgias Polyarthralgias
Bone Pains
Hip Pain
Joint deformities
Joint Pains
Joint pains Arthralgias in Elderly
Joint Swelling
Multiple Joint Arthritis
Polyarticular Joint Pain
Skeletal problems/symptoms/signs
Tender Bones (Long Bones)
Coarsened facial features
Typical Clinical Presentation
Males sicker than females/characteristic
Disease Progression
Onset/Young adult
Demographics & Risk Factors
Ethnic or Racial Factors
African black population
Black/African american population
Sex & Age Groups
Population/Male
Diagnostic Test Results
Isotope Scan
Isotope/Bone scan abnormality
X-RAY
Xray/Enlarged sinuses/Head
Xray/Skeletal findings
Xray/Subperiosteal new bone growth/diffuse
Associated Diseases & Rule outs
Rule Outs
Osteoarthritis, degenerative
Polyarthritis syndrome/Multiple joints/weeks
PolyArthritis
Associated Disease & Complications
Dermatitis/Chronic rash
Hypertrophic osteoarthropathy
Kyphoscoliosis
Kyphosis
Osteoarthropathy, hypertrophic primary
Periostitis
Polyarthritis syndrome/Multiple joints/weeks
Seborrhea/Seborrhea dermatitis
Congenital clubbing, digital
PolyArthritis
Disease Mechanism & Classification
Class
CLASS/Bone disorder (ex)
CLASS/Joint disorder (ex).
CLASS/Skeletal (category)
Process
PROCESS/Autosomal dominant hereditary disease (ex).
PROCESS/Hereditary dominance/incomplete penetrance (ex).
PROCESS/Hereditofamilial (category)
PROCESS/Hypertrophic/Hyperplastic disorder (ex)
PROCESS/INCIDENCE/Rare disease (ex)
PROCESS/Inflammatory/Traumatic/regeneration (ex)
PROCESS/Reference organ/system (category)
PROCESS/Vegetative-Autonomic/Endocrine (category)
Synonyms
Synonym
Clubbed nail pachydermoperiostosis, Clubbed pachydermoperiostosis, Friedrich Erb Arnold syndrome, Hypertrophic Osteoarthropathies Primary, Hypertrophic Osteoarthropathy Primary, HYPERTROPHIC OSTEOARTHROPATHY PRIMARY OR IDIOPATHIC, Marie Bamberger syndrome idiopathic, Osteoarthropathies Primary Hypertrophic, Osteoarthropathy Primary Hypertrophic, Pachydermoperiostoses, Pachydermoperiostosis, Pachydermoperiostosis of nail, Pachydermoperiostosis of nail (disorder), Pachydermoperiostosis syndrome, Pachydermoperiostosis syndrome (disorder), PDP, Primary Hypertrophic Osteoarthropathies, Primary hypertrophic osteoarthropathy, Roy Jutras syndrome, Touraine Solente Gole syndrome, Touraine Solente Golé syndrome, UEHLINGER SYNDROME, Uehlinger's syndrome, Synonym/Hereditary Clubbing Syndrome, Synonym/Primary pachydermoperiostosis, Synonym/Touraine-Solente-Gole syndrome, Synonym/Uehlinger's syndrome
Treatment
Drug Therapy - Indication
RX/Non-steroidal anti-inflammatory drugs
RX/Prednisone (Deltasone)
Surgical Procedures or Treatments
SX/Vagotomy Indications
Other Treatments
TX/Expectant/supportive treatment.
Definition

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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