The phenotypic findings in HCS that particularly relate to abnormal bone turnover are acro-osteolysis
and wormian bones in the skull sutures.
. Eur J Rheumatol 2019; 6(2): 109-10.
Abnormality in X-ray hands was found in 18 (33.3%) cases, of which 8 (14.8%) cases showed acro-osteolysis
, 5 (9.3%) cases showed contracture and another 5 (9.3%) cases showed both these features.
A syndrome of keratosis palmo-plantaris congenita, pes planus, onychogryphosis, periodontosis, arachnodactyly and a peculiar acro-osteolysis
. Br J Dermatol 1986; 115: 243-48.
Films of the hands showed aplastic terminal phalanges in keeping with acro-osteolysis
Primary HOA or pachydermoperiostosis is a rare hereditary disorder characterized by digital clubbing, subperiosteal new bone formation, acro-osteolysis
, hypertrophy of soft tissues and glands, particularly in the face and scalp resulting in wrinkling of skin.
: a complication of Jadassohn-Lewandowsky syndrome.
Bone resorption (osteopaenia) * Sub-periosteal Radial aspect of middle phalanges, medial side of humerus, tibia * Intracortical Terminal tufts(acro-osteolysis
) * Endosteal Hands and long bones * Trabecular Skull (salt and pepper appearance) * Sub-ligamentous Trochanters, calcaneus, ischial/humeral tuberosities, lateral clavicle * Sub-chondral Acromioclavicular, sacro-iliac and sternoclavicular joints 2.
in 1991 proposed diagnostic criteria for PDP i) major criteria: digital clubbing, periostosis, and pachydermia and ii) minor criteria: seborrhea, hyperhidrosis, folliculitis, arthritis or arthralgia, gastritis or gastric ulcer, acro-osteolysis
, neurovegetative syndrome, hypertrophic gastropathy and cutis verticis gyrate.
(4) Radiologically, acro-osteolysis
, periosteal changes of short, long and flat bones, and ossification of ligaments and interosseous membranes have been reported.
X ray AP views of both hands (c, d) showing short metacarpals, phalanges with distal tuft resorption (acro-osteolysis
A patient with acro-osteolysis