Arthritis mutilans appearance of the hands clinically
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Diffuse acro-osteolysis involving all digits of the hands and feet. Focal cysts centered on the necks of the proximal phalanges of all fingers bilaterally (curiously the thumbs are spared) with smaller cystic foci within the middle phalanges, proximally and distally. No cysts within the distal radius/ulna, carpus or metacarpals.
Lace-like osteopenia in a periarticular distribution.
Some mild narrowing of some of the joint spaces of the interphalangeal articulations. However generally articulations are preserved without evidence of an erosive arthropathy. No joint subluxation on the AP views.
No soft tissue calcification.
Vessels are calcified although this may be age-related.
Musculoskeletal manifestations of sarcoidosis are relatively rare, when compared to the common pulmonary form of the disease, and estimated to occur in only 5-10% cases 2. Isolated extrapulmonary sarcoidosis is rare, found to be only 2% of all sarcoidosis cases in an epidemiological study in the US of 736 patients with known sarcoidosis 3.
Musculoskeletal symptoms, e.g. arthralgia, are more common than actual visible radiographic pathology.
The appearances of the hands and feet in this case is classic:
- cyst-like lesions of the phalanges
- hands more commonly affected than the feet
- lace-like osteopenia
This patient's bony changes of sarcoid did not extend to the vertebrae or other structures of the axial skeleton.
When the appearances are typical, as here, the radiological diagnosis is usually fairly clear-cut. However when only one or two cyst-like lesions are present, it can be difficult to rule out differentials, such as metastases.
- 1. Palmucci S, Torrisi SE, Caltabiano DC, Puglisi S, Lentini V, Grassedonio E, Vindigni V, Reggio E, Giuliano R, Micali G, Caltabiano R, Andreula C, Foti PV, Ettorre GC, Walsh SL, Vancheri C. Clinical and radiological features of extra-pulmonary sarcoidosis: a pictorial essay. (2016) Insights into imaging. 7 (4): 571-87. doi:10.1007/s13244-016-0495-4 - Pubmed
- 2. Koyama T, Ueda H, Togashi K, Umeoka S, Kataoka M, Nagai S. Radiologic manifestations of sarcoidosis in various organs. (2004) Radiographics : a review publication of the Radiological Society of North America, Inc. 24 (1): 87-104. doi:10.1148/rg.241035076 - Pubmed
- 3. Baughman RP, Teirstein AS, Judson MA, Rossman MD, Yeager H, Bresnitz EA, DePalo L, Hunninghake G, Iannuzzi MC, Johns CJ, McLennan G, Moller DR, Newman LS, Rabin DL, Rose C, Rybicki B, Weinberger SE, Terrin ML, Knatterud GL, Cherniak R. Clinical characteristics of patients in a case control study of sarcoidosis. (2001) American journal of respiratory and critical care medicine. 164 (10 Pt 1): 1885-9. doi:10.1164/ajrccm.164.10.2104046 - Pubmed