Patient presenting to the emergency department with an open wound on the ulnar side of her left hand. The clinician asked for an X-ray of the hand to eliminate the hypothesis of bone injury.
The left hand PA and oblique views revealed no fracture but multiple small (2-10 mm) sclerotic, peri-articular bony opacities. These involved the bones of the wrist, as well as the metacarpophalangeal joints.
The hospital's electronic archives contained older radiographs of the same patient:
-Chest and cervical spine x-rays (after a fall).
-Right ankle x-rays for an Achilles’ tendon rupture.
-Left shoulder x-rays for unknown reason.
All the radiographs revealed similar sclerotic bony opacities, always in a peri-articular location.
Osteopoïkilosis is an asymptomatic osteosclerotic dysplasia. It was first described by Alberg-Schönberg and Ledoux-Lebard in 1916 on the basis of its radiographic appearance [1].
Osteopoïkilosis can be considered as a dysplastic disorder, rather than a disease. It is more common in men than in women and it is attributed to an autosomal dominant inheritance, although sporadic cases have been described. When it is inherited, it is associated in 25% with the Buschke-Ollendorf syndrome (dermatofibrosis lenticularis disseminata). This latter syndrome predisposes to the development of keloids and also to a skin disease similar to scleroderma [2, 3].
Although the natural course of osteopoïkilosis is benign, the coexisting pathologic conditions require medical attention. This disorder can be associated with other abnormalities such as: skeletal disorders, organ anomalies (aortic coarctation, double ureter), endocrine dysfunction, dental abnormalities, facial abnormalities, dacryocystitis and pre-myelopathic syndrome due to spinal stenosis.
Radiographic findings are diagnostic for osteopoïkilosis and show multiple small (2-10 mm), well-defined and homogeneous bones islands clustered in periarticular areas. These radiodense and generally uniform lesions have a symmetrical distribution and a propensity for the epiphyseal and metaphyseal regions of long tubular bones [4, 5]. The articulations of the shoulders, the wrists, the ankles and the hips are more frequently involved. Nuclear medicine imaging, such as scintigraphy, will usually show no anomalies because the lesions are not hypermetabolic.
Osteopoïkilosis can be associated with or even mistaken for other osteosclerotic skeletal disorders such as osteopathia striata, melorheostosis and tuberous sclerosis. It is important to differentiate osteopoïkilosis from diffuse osteoblastic metastases [1, 6]. Radiologically, blastic metastases tend to be presented with larger and more irregular opacities, in a less uniform pattern. They usually vary with time; furthermore, scintigraphy is usually positive because metastatic lesions are hypermetabolic. In spite of these differences, sometimes only bone biopsy permits to distinguish between osteopoïkilosis and bone metastases.
Final diagnosis: osteopoïkilosis (=osteopathy condensans disseminata)
Osteopoïkilosis (=osteopathy condensans disseminata)
Based on the X-ray images of the patient’s left hand and part of the upper limb, multiple regularly shaped, high-density shadows (“bone islands”) measuring approximately 2–10 mm in size with clear boundaries can be observed in several bone structures around the metaphyses and epiphyses (e.g., distal radius, wrist joint, metacarpal and phalangeal bones, shoulder joint, etc.). These bone islands appear in a relatively symmetrical distribution, with uniform lesion morphology and without evident bone destruction or reactive new bone formation. In addition, no definite fracture lines, bone collapse, or obvious periosteal reaction were identified. The patient presented with an open wound on the ulnar side of the left hand, but no bony damage or signs of fracture were apparent on the imaging.
This condition can manifest as multiple sclerotic lesions of varying sizes and irregular shapes, often accompanied by highly active tracer uptake (positive bone scan). In this case, the bone islands show a relatively regular distribution, well-defined margins, and no localized destruction, making metastatic tumors less likely.
This disorder can present as striped or linear areas of increased density in specific bones, but in this case, the lesions lean more toward patchy or island-like increased density, which does not match the typical striped pattern.
Commonly appears as “dripping candle wax” or long streaks of dense bone with irregular bone overgrowth, which differs from the regular, island-like sclerotic lesions presented in this case.
Often presents as multiple bone islands located near metaphyses and epiphyses, with lesions distributed symmetrically and having well-defined margins and relatively uniform sizes. The imaging features in this case are highly consistent with osteopoikilosis.
Considering the patient’s clinical status (36-year-old female with no significant bone pain, functional impairment, or other systemic symptoms) and the imaging findings (multiple, symmetrically distributed, homogeneous bone islands), a benign condition is highly suspected. After comprehensive evaluation, the most likely diagnosis is Osteopoikilosis. This condition can be hereditary or sporadic, generally has a good prognosis, and malignancy is rare. If no special complications are present, invasive examinations or surgical interventions are typically unnecessary.
1. Treatment Strategy:
2. Rehabilitation/Exercise Prescription Recommendations (FITT-VP principle):
Disclaimer: This report is only a preliminary analysis based on the existing imaging and clinical information and cannot replace an in-person consultation or the opinion of a professional physician. If you experience any discomfort or uncertainties, please consult a relevant specialist or visit a medical institution for a face-to-face examination.
Osteopoïkilosis (=osteopathy condensans disseminata)
