Osteopoikilosis: what does the rheumatologist must know about it?

Abstract

Osteopoikilosis (OPK) is a rare, benign, and asymptomatic bone dysplasia that is developed during childhood and persists throughout life. This condition is generally found incidentally on plain radiographies made by other reasons. The main differential diagnosis is osteoblastic metastasis. So, OPK must be in differential diagnosis when bone lesions are identified on plain radiograph to avoid alarming the patient with more serious disease and misdiagnosis. In this paper, we review the clinical manifestation, pathophysiology, diagnosis, and treatment of OPK.

Familial osteopoikilosis

“…Acetaminophen and opioids can also be used. Rare active lesions have been treated with bisphosphonate therapy [6]. Due to recurrent pain, our patient was treated with indomethacin.…”

“…In our case, we found numerous symmetric, small, well-circumcised, sclerotic lesions of various degrees in the femoral heads, acetabulum, symphysis pubis and hands. OPK is gen-erally asymptomatic and is frequently diagnosed incidentally during radiologic examinations; however, 15-20% of patients may endure slight articular pain and joint effusions [6,14]. The origin of articular pain is unknown, but hypotheses include increased localized bone metabolism at the location of the lesion, or increased intraosseous pressure due to venous stasis in the lesion areas [8].…”

“…Patchy condensations of compact lamellar bone are observed within the spongiosa in epiphyses and metaphyses of long tubular bones and the carpi, tarsi, pelvis and scapulae [4,5]. Patients are asymptomatic and laboratory tests and bone scintigraphy are usually normal [6].…”

Cystinosis with Sclerotic Bone Lesions

“…Hematologic disorders, including systemic mastocytosis (Fritz et al 2012), Langerhans cell histiocytosis, and Erdheim-Chester disease (Wilejto and Abla 2012), can also present with sclerotic bone changes, although there are usually other features on history of imaging to suggest these and the negative S100 immunostaining of the histiocytes from the biopsy exclude Langerhans cell histiocytosis. Nonmalignant causes of sclerotic bone lesions include Paget's disease, osteopoikilosis (Woyciechowsky et al 2012), and parathyroid-related bone disease. There is a single case (Quinn et al 2004) where marrow involvement with cystine crystals was accompanied by changes suggestive of parathyroid-related bone disease in a patient with marked elevation (20-fold upper limit of normal) of parathyroid hormone levels.…”

Osteopoikilosis masquerading as osseous metastases in breast cancer

“…The main differential diagnoses of OPK are mastocytosis, tuberous sclerosis and osteoblastic metastasis. 2 Tc-99 m bone scan is important to distinguish OPK from osteoblastic metastasis. Typically, there is no increase in uptake on bone scan suggesting activity.…”