Rheumatology 2001; 40: 947-948
© 2001 British Society for Rheumatology
Letters to the Editor |
Superficial calcinosis related to bleeding in a patient with undifferentiated connective tissue disease and primary biliary cirrhosis
Division of Rheumatology, ‘Villa Pini’ Clinic, Chieti and
1 First Division of Internal Medicine, ‘Spirito Santo’ Hospital, Pescara, Italy
SIR, Calcifications of soft tissues have frequently been described in association with polymyositis–dermatomyositis [1], scleroderma [2], scleroderma overlap syndromes [3] and systemic lupus erythematosus (SLE) [4]. On the contrary, there have been few descriptions of calcifications related to chronic venous insufficiency (CVI), although a French study reported seven subjects with superficial calcific deposits of the lower limbs among 40 patients affected by CVI [5]. We describe a female patient suffering from undifferentiated connective tissue disease (UCTD) and primary biliary cirrhosis (PBC) in whom venous bleeding episodes in the soft tissues of lower limbs, related to CVI, evolved in diffuse calcinosis. Subcutaneous administration of calcium heparin in the abdominal wall for thrombophlebitis was also followed by calcification of the drug-induced haematoma.
A 66-yr-old Caucasian woman presented in March 1999 with a 10-yr history of worsening Raynaud's phenomenon localized in her hands and feet. During the same period, she also complained of recurrent fever and relapsing arthritis involving her wrists and occasionally the second and third metacarpophalangeal joints of both hands. Since 1996, the patient had noted the progressive appearance of several indurated plaques localized in her legs. The affected areas were those that showed dischromic signs of previous bleeding episodes caused by CVI or bilateral saphenectomy in 1998. The patient had been treated previously with non-steroidal anti-inflammatory drugs and occasionally with low doses of steroids. Examination revealed a painful swelling of the wrists. Dischromic signs were present on the surface of the legs. In these areas, palpation confirmed the presence of indurated plaques and caused moderate pain. A small indurated plaque was also disclosed in the subcutaneous tissues of the abdominal wall where, in 1998, calcium heparin had been injected for a lower leg thrombophlebitis. Raynaud's phenomenon was evident after exposure to cold temperature. Sclerodactyly, digital ulcers and other cutaneous signs of scleroderma, dermatomyositis or SLE were absent. Lachrymal and salivary flow were normal. Positive findings of our extensive laboratory examinations were as follows: ESR 47 mm/h; alkaline phosphatase 334 U/l (normally 98–279); IgM 283 mg/dl (40–230); antinuclear antibodies 1/160 (speckled pattern); presence of anti-52kDa-SSA/Ro, anti-SSB/La (Western blot method) and anti-mitochondrial antibodies (1/320). In particular, calcium and phosphate were normal in serum and urine. Radiographs disclosed soft tissue swelling around the wrists and diffuse osteopenia in the wrists and hands. Erosions were absent. X-rays showed diffuse superficial calcifications in the soft tissues of the legs (Fig. 1
) and a slight calcification in the abdominal wall. Their superficial localization was confirmed by ultrasonography. A percutaneous liver biopsy evidenced a picture compatible with PBC stage 2.
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In subjects with CVI, Piry hypothesized a pathogenesis of calcification based on venous stasis, anoxia and inflammatory processes [5]. In childhood dermatomyositis, in which the calcinosis is much more frequent than in adults, four distinct patterns of calcification were identified: deep or superficial calcareal masses, deep linear deposits and a lacy, reticular calcification that could be widespread [6]. In young patients affected by dermatomyositis, vascular involvement is also more evident than in adults (in capillaries, small arteries and veins [7]) and calcification could be related to this increased damage of the vessels.
The leg calcinosis of our patient was superficial with a singular granular pattern, and it was a direct consequence of bleeding. The close relationship with bleeding rather than other causes connected with CVI was confirmed by the calcification following the injection of calcium heparin in the abdominal wall. The underlying pathogenic mechanisms of this calcinosis remain unidentified but the concurrent roles of bleeding and inflammation are likely. As far as we know, the subject described here represents the second reported case of calcinosis associated with UCTD [8] and the first one with primary biliary cirrhosis. The occurrence of this previously undescribed superficial granular pattern of calcinosis in other connective tissue disease patients and its close relationship with bleeding deserve confirmation.
Notes
Correspondence to: C. Palazzi, Via Legnago 23, 65123 Pescara, Italy. 
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