A family with a variant form of primary hypertrophic osteoarthropathy restricted to the lower extremities.
We report a case of a family with a variant form of primary hypertrophic osteoarthropathy (HOA) restricted to the lower extremities without digital clubbing or cutaneous changes. Three family members suffered from pain, swelling and hyperhidrosis of both feet. X-rays showed destruction and osteoproliferative changes of the metatarsal bones with periostal hyperostosis close to the talus. There was an increased 99m-Tc-MDP uptake in the early phase of bone scintigraphy. Fibrosis of the marrow with stimulated osteoclastic resorption and the presence of detritus synovialitis were visible in a bone and joint biopsy. All known infectious, neurologic, metabolic and malignant diseases, which affect the bone and joints, were excluded.
Seggewiss
Ruth
R
Department of Internal Medicine V, University of Heidelberg, Hospitalstr. 3, 69115, Heidelberg, Germany. Ruth_Seggeweis@med.uni-heidelberg.de
Hess
Thomas
T
Fiehn
Christoph
C
D000328:Adult; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D006945:Hyperhidrosis / Q000150:complications / Q000473:pathology; D008297:Male; D008682:Metatarsal Bones / Q000000981:diagnostic imaging; D010004:Osteoarthropathy, Primary Hypertrophic / Q000150:complications / Q000235:genetics / Q000473:pathology*; D010375:Pedigree; D011859:Radiography; D013977:Tibia / Q000000981:diagnostic imaging