A young male patient presented with painful swollen hands.
The patient presented with a 3 years history of painful swollen hands. Clinically he had lupus pernio with ulcerated, red and inflamed external nares on right side. He had bilateral dactylitis with the fingers swollen, red and tender and there was a large ulcer on both the right and left index fingers. X-ray of both hands (Figure 1) followed by a Chest x-ray (figure 2) were taken. A follow up chest x-ray after 2 years showed pulmonary parenchyma changes and high resolution CT was obtained (figure 3).
Sarcoidosis is a disease that exhibits extremely heterogeneous clinical
manifestations. Although the lungs are the most commonly involved organs, the
extension of the granulomatous process may also affect the bones. Almost any bone can be involved; curiously, the small bones of the hands and feet are most frequently affected. The nasal bones are involved particularly in patients with lupus pernio. Skull, vertebrae, pelvis, ribs, sternum, and the distal ends of long bones are rarely affected. The frequency of bone involvement varies from 3% to 13% [1].
Sarcoidosis bone lesions are characterized by their bilateral distribution; the site of origin (cortical, preservation of the periosteum); location (hands and feet); position (usually the ends of the affected bones); and the shape (cystic or lacelike with minimal disturbance in the nearby soft tissues). In advanced cases, as subchondral lesions extend into joint spaces, the adjacent joints may be involved. Calcification is absent. The questions regarding the cause and
localization of bone lesions in sarcoidosis remains unanswered [2].
Although bone lesions are often asymptomatic, in many cases the affected part may be tender and painful. Stiffness of the digits, finger deformities, and soft tissue swelling overlying the bone cysts are frequent and often precede the radiologic diagnosis.
James and Williams [3] classified bone lesions into the following:
1.Lytic lesions: these lesions are either minute cortical defects in phalangeal heads or larger rounded punched out lesions involving cortex and medulla, most frequently of the middle and proximal phalanges. It likely reflects an osteoporotic process producing tunnelling that is more local and destructive. The peripheral lesions simulate marginal erosions.
2.Permeative lesions: "tunnelling" of the cortex of the shaft of the phalanx, followed by remodeling of the cortical and trabecular architecture, results in a reticular pattern. The concave phalangeal shafts become tubular. The lesions are usually accompanied by soft tissue swelling.
3.Destructive lesions: in an advanced sclerotic phase, the bone may develop multiple fractures of devitalized cortex resulting in a sequestrum. Joint destruction is localized to the subchondral areas of the bone.
As a rule, the recognition of typical punched-out lesions is relatively easy if the patient presents with multisystem features of sarcoidosis. However, diagnosis may be difficult if the bone lesion occurs in the absence of the typical pulmonary and extrapulmonary features of sarcoidosis. Many clinical disorders, including tuberculosis, histoplasmosis, coccidoidomycosis, leprosy, brucellosis, syphilis, Wegener granulomatosis, eosinophilic granuloma, multiple
myeloma, lymphoma and metastasis [4] can cause bony lesions indistinguishable from those due to sarcoidosis. Nevertheless, in daily practice only a few diseases need to be differentiated from sarcoidosis as far as bone involvement is concerned. In difficult situations a bone biopsy is needed to demonstrate the presence of noncaseating granuloma and exclude other conditions by appropriate laboratory tests and cultures.
Osseous sarcoidosis responds poorly to corticosteroids as well as to other drugs used in treating the illness [5]. Corticosteroids often decrease pain and ameliorate soft tissue swelling but do not completely normalize the bone abnormality. Symptomatic relief may be obtained by nonsteroidal anti-inflammatory agents.
Sarcoidosis
1. X-ray of both hands: Visible multiple “punched-out” or “mesh-like” bony changes in the phalanges of both hands, especially in the (middle) phalanx region, with varying degrees of cystic lucent areas in shape. The cortical bone may show localized lucency or thinning resembling a “tunnel-like” change. Some mild involvement of the bony structures adjacent to joint surfaces is noted, with evident swelling of the surrounding soft tissues.
2. Chest X-ray and CT: On the chest radiograph, there is increased opacity in both hilar regions or suspicious hilar lymphadenopathy. On CT, scattered small nodules, reticular density increases and/or hilar lymphadenopathy are observed bilaterally and symmetrically, suggesting a possible systemic granulomatous process.
Based on the young male patient, multiple lucent or erosive lesions in the phalanges of both hands, and the hilar lymph node enlargement or pulmonary reticular/small nodule lesions noted on chest imaging, the following should be considered:
In summary, the key differential here is distinguishing between granulomatous disease (primarily sarcoidosis) and infectious or neoplastic conditions. Further clinical tests (serum ACE level, immunological indicators, tuberculin skin test, biopsy, etc.) can aid in making a definitive diagnosis.
Considering the patient’s age, the multiple lucent lytic lesions primarily in the phalanges, the bilateral pulmonary and hilar lymph node changes on imaging, and the clinical symptoms of “hand pain and swelling,” the most likely diagnosis is: Sarcoidosis involving the skeleton.
If diagnostic uncertainties remain, a biopsy of the affected phalangeal lesion or additional laboratory and immunological tests (e.g., serum ACE, glucose tolerance test, sarcoidosis-related autoantibodies) may be performed to further confirm the diagnosis.
1. Treatment Strategies:
2. Rehabilitation/Exercise Prescription:
Because the patient has bony changes in the hands, which may lead to pain, reduced grip strength, and limited range of motion—and may also have some pulmonary function impairment—comprehensive assessment is required in rehabilitation training:
These recommendations align with the FITT-VP principle (Frequency, Intensity, Time, Type, Progression, and Individualization) to ensure progressive exercise within a safe range.
This report is based solely on the available imaging findings and related data, providing a preliminary analysis. The suggested treatment and rehabilitation plans are for reference only and do not replace in-person consultation or professional medical advice. If the patient experiences further changes in symptoms or has any concerns, prompt hospital visits are advised for a more comprehensive evaluation and treatment.
Sarcoidosis
