II. Definition

  1. Excess skin and bone proliferation at distal extremity

III. Pathophysiology

  1. Large Platelets accumulate in distal extremities
  2. Fibroblast growth factors released
  3. Results in edema and excess Collagen deposition
  4. Increased bone formation distally also occurs

V. Symptoms

  1. Often asymptomatic
  2. Deep bone pain at distal extremities (usually legs)

VI. Signs

  1. Digital Clubbing
  2. Bone thickening of wrists and ankles

VII. References

  1. Klippel (1997) Primer Rheumatic Diseases, p. 370-1

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Related Studies

Ontology: Osteoarthropathy, Secondary Hypertrophic (C0029412)

Definition (MSH) Symmetrical osteitis of the four limbs, chiefly localized to the phalanges and the terminal epiphyses of the long bones of the forearm and leg, sometimes extending to the proximal ends of the limbs and the flat bones, and accompanied by dorsal kyphosis and joint involvement. It is often secondary to chronic conditions of the lungs and heart. (Dorland, 27th ed)
Concepts Disease or Syndrome (T047)
MSH D010005
ICD9 731.2
ICD10 M89.4
SnomedCT 222712005, 46922002, 203357004
English Hypertrophic Osteoarthropathies, Secondary, Hypertrophic Osteoarthropathy, Secondary, Marie Bamberger Disease, Marie-Bamberger Disease, Osteoarthropathies, Secondary Hypertrophic, Osteoarthropathy, Secondary Hypertrophic, Secondary Hypertrophic Osteoarthropathies, Secondary Hypertrophic Osteoarthropathy, Disease, Marie-Bamberger, HPOA - Hypert pulm osteoarthr, Hypertroph pulmon osteoarthrop, SECOND HYPERTROPHIC OSTEOARTHROPATHY, MARIE BAMBERGER DIS, HYPERTROPHIC OSTEOARTHROPATHY SECOND, OSTEOARTHROPATHY SECOND HYPERTROPHIC, hypertrophic pulmonary osteoarthropathy, hypertrophic pulmonary osteoarthropathy (diagnosis), Bamberger-Marie disease, Pulmonary osteoarthropathy hypertrophic, Hypertroph osteoarthrop, Marie-Bamberger disease, Osteoarthropathy, Secondary Hypertrophic [Disease/Finding], bamberger-marie disease, acropachy, Hypertrophic pulmonary osteoarthropathy (disorder), Secondary hypertrophic osteoarthropathy, Hypertrophic pulmonary osteoarthropathy, HPOA - Hypertrophic pulmonary osteoarthropathy, Marie Bamberger disease, Pachydermoperiostitis acquired, Marie-Bamberger, Bamberger-Marie, hypertrophic; osteoarthropathy, pulmonary, osteoarthropathy; hypertrophic, pulmonary, osteoarthropathy; secondary hypertrophic, secondary; osteoarthropathy, hypertrophic, Acropachy, Hypertrophic pulmonary osteoarthropathy (disorder) [Ambiguous], secondary; hypertrophic osteoarthropathy
Dutch pulmonale osteoartropathie hypertrofisch, hypertrofische pulmonale osteoartropathie, ziekte van Pierre Marie-Bamberger, hypertrofisch; osteoartropathie, pulmonaal, osteoartropathie; hypertrofisch, pulmonaal, osteoartropathie; secundair hypertrofisch, secundair; osteoartropathie, hypertrofisch, secundair; hypertrofische osteoartropathie, Hypertrofische osteoartropathie, secundaire, Marie-Bamberger-ziekte, Osteoartropathie, hypertrofische, secundaire, Osteoartropathie, secundaire hypertrofische, Secundaire hypertrofische osteoartropathie
French Ostéoarthropathie pulmonaire hypertrophique, Ostéo-arthropathie hypertrophiante pneumique de Pierre Marie, Ostéoarthropathie hypertrophiante de Pierre Marie, Ostéoarthropathie hypertrophiante secondaire, OAH secondaire, Ostéo-arthropathie hypertrophiante secondaire, Ostéoarthropathie hypertrophiante pneumique, Ostéo-arthropathie hypertrophiante de Pierre Marie, Ostéo-arthropathie hypertrophiante pneumique, Ostéoarthropathie hypertrophiante pneumique de Pierre Marie-Bamberger, Syndrome de Pierre Marie-Bamberger
German hypertrophische pulmonaere Osteoarthropathie, pulmonaere Osteoarthropathie, hypertrophisch, Marie-Bamberger-Syndrom, Osteoarthropathie, sekundäre hypertrophische
Italian Osteoartropatia polmonare ipertrofica, Osteoartropatia ipertrofizzante pneumica, Sindrome di Marie-Bamberger, Malattia di Bamberger-Marie, Osteoartropatia ipertrofica secondaria
Portuguese Osteartropatia pulmonar hipertrófica, Doença de Bamberger-Marie, Doença de Marie-Bamberger, Osteoartropatia Hipertrófica Secundária
Spanish Osteoartropatía pulmonar hipertrófica, Enfermedad de Bamberger-Marie, osteoartropatía pulmonar hipertrófica (trastorno), enfermedad de Bamberger-Marie, osteoartropatía hipertrófica secundaria, osteoartropatía hipertrofiante pnéumica (trastorno), osteoartropatía hipertrofiante pnéumica, osteoartropatía pulmonar hipertrófica, enfermedad de Bamberger - Marie, acropaquia, Dedos en Palillos de Tambor, Dedos en Maza, enfermedad de Marie Bamberger, osteoartropatía hipertrofiante pnéumica (concepto no activo), paquidermoperiostitis adquirida, Enfermedad de Marie-Bamberger, Osteoartropatía Hipertrófica Secundaria
Japanese 肺性肥厚性骨関節障害, ハイセイヒコウセイコツカンセツショウガイ, Marie-Bamberger病, 撥指, 続発性肥大性骨関節症, 撥状指, 太鼓撥指, 太鼓ばち指, ばち指, バンベルガー・マリー病, マリー・バンベルガー症候群, バンベルガー-マリー病, マリー-バンベルガー病, 肺性肥大性骨関節症, Marie-Bamberger症候群, マリー-バンベルガー症候群, 大鼓ばち指, 肥大性肺性骨関節症, マリー・バンベルガー病, バンベルガー・マリー病, バンベルガーマリービョウ, 骨関節症-続発性肥大性, Bamberger-Marie病
Swedish Osteoartropati, sekundär hypertrofisk
Czech osteoartropatie sekundární hypertrofická, Pulmonální osteoartropatie hypertrofická, Hypertrofická pulmonální osteoartropatie, Choroba Bambergera-Marieho
Finnish Sekundaarinen hypertrofinen osteoartropatia
Polish Zespół Pierre-Marie-Bamberger, Osteoartropatia wtórna przerostowa
Hungarian Bamberger-Marie betegség, Pulmonalis hypertrophiás osteoarthropathia, Hypertrophiás pulmonalis osteoarthropathia
Norwegian Sekundær hypertrofisk osteoartropati, Marie-Bamberger-sykdom, Marie-Bambergers sykdom

Ontology: Hypertrophic osteoarthropathy (C0178703)

Definition (CSP) two types, primary type is a condition chiefly characterized by thickening of the skin of the head and distal extremities, deep folds and furrows of the skin of the forehead, cheeks, and scalp, seborrhea, hyperhidrosis, periostosis of the long bones, digital clubbing, and spadelike enlargement of the hands and feet, it is more prevalent in the male; secondary type is a condition with symmetrical osteitis of the four limbs, chiefly localized to the phalanges and the terminal epiphyses of the long bones of the forearm and leg, sometimes extending to the proximal ends of the limbs and the flat bones, and accompanied by dorsal kyphosis and joint involvement, and is often secondary to chronic conditions of the lungs and heart.
Concepts Disease or Syndrome (T047)
SnomedCT 156813008, 203357004
English HOA-Hypertrop osteoarthrop, HYPERTROPHIC OSTEOARTHROPATHY, hypertrophic osteoarthropathy, hypertrophic osteoarthropathy (diagnosis), HOA - Hypertrophic osteoarthropathy, Hypertrophic osteoarthropathy, Hypertrophic osteoarthropathy (disorder), hypertrophic; osteoarthropathy, osteoarthropathy; hypertrophic
Italian Osteoartropatia ipertrofica
Japanese 肥厚性骨関節障害, ヒコウセイコツカンセツショウガイ
Czech Hypertrofická osteoartropatie
Hungarian Hypertrophiás osteoarthropathia
Dutch hypertrofisch; osteoartropathie, osteoartropathie; hypertrofisch, hypertrofische osteoartropathie
Spanish osteoartropatía hipertrófica (trastorno), osteoartropatía hipertrófica, Osteoartropatía hipertrófica
Portuguese Osteoartropatia hipertrófica
French Ostéo-arthropathie hypertrophiante
German hypertrophe Osteoarthropathie

Ontology: Disorder of skeletal system (C0263661)

Definition (CSP) disorders of the skeletal system; includes both bone and cartilage.
Concepts Disease or Syndrome (T047)
SnomedCT 88230002
Italian Osteoartropatia, Osteoartropatie
Japanese 骨関節症, 骨関節障害, コツカンセツショウガイ, コツカンセツショウ
English skeletal disorder, Disease of skeletal system, bone joint disorders, disorders skeletal, bone diseases joint, bones joints diseases, osteoarthropathy, skeletal system disease, disease of skeletal system, diseases skeletal system, disorders skeletal system, bones diseases joints, bone joint disease, disease skeletal system, disorder of skeletal system, Osteoarthropathies, Osteoarthropathy, Disease of bone AND/OR joint, Disease of bone and joint, NOS, Disease of skeletal system, NOS, Disorder of skeletal system, NOS, Osteoarthropathy, NOS, Disease of skeletal system (disorder), Disorder of skeletal system, Disorder of skeletal system (disorder)
Spanish enfermedad ósea Y/O articular, enfermedad de hueso Y/O de articulación, enfermedad del sistema esquelético (trastorno), osteoartropatía, Osteoartropatías, Osteoartropatía, trastorno del sistema esquelético, trastorno del sistema esquelético (trastorno), enfermedad del sistema esquelético
Czech Osteoartropatie, Onemocnění kostí a kloubů
Hungarian Osteoarthropathiak, Osteoarthropathia
Portuguese Osteoartropatia, Osteoartropatias
Dutch osteoarthropathieën, osteoartropathie
French Ostéo-arthropathie, Ostéoarthropathies
German Osteoarthropathien, Osteoarthropathie