The n e w e ng l a n d j o u r na l
Images in Clinical Medicine
Naveen Yadav, M.D.
Uday Yanamandra, M.D., D.M.
Armed Forces Medical College
Pune, India
naveeny7000@gmail.com
e22
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of m e dic i n e
Stephanie V. Sherman, M.D., Editor
Primary Hypertrophic Osteoarthropathy
A B
C D
A
31-year-old man presented to the internal medicine clinic
with 10 years of progressive enlargement of his fingertips and toes and
intermittent aches in his distal forearms and lower legs. He had no clini-
cally significant medical history or family history of similar symptoms. Finger and
toe clubbing, with a supranormal angle of 210 degrees between the nail bed and
the proximal nail fold, was observed on examination. Hyperpigmentation of the
proximal nail folds; tenderness to palpation of the distal forearms and lower legs,
fingertips, and toes; and widening of the distal forearms and lower legs (Panels A
and B, respectively) were also observed. There was no skin thickening or hyperhi-
drosis. Radiographs of the fingers showed bony proliferation at the tips of the
phalanges (Panel C), and radiographs of the ankles showed increased periosteal
bone formation at the distal tibia and fibula (Panel D). Serum laboratory tests
(including thyroid-function studies), an echocardiogram, and whole-body positron-
emission tomography–computed tomography were normal. The patient received a
diagnosis of primary hypertrophic osteoarthropathy, a hereditary syndrome char-
acterized by clubbing, periosteal bone proliferation, and (not seen in this case)
facial skin thickening and hyperhidrosis, in the absence of systemic conditions. The
patient was initially treated with nonsteroidal antiinflammatory drugs for 3 months.
Given persistent symptoms, zoledronic acid was administered, and his pain had
abated at follow-up 2 months later.
DOI: 10.1056/NEJMicm2113457
Copyright © 2022 Massachusetts Medical Society.
n engl j med 386;9 nejm.org March 3, 2022
The New England Journal of Medicine
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