Regression of cutis calcinosis with diltiazem in adult dermatomyositis

ARTICLE

Auteur(s) :, M Abdallah-Lotf¹, A Grasland², P Vinceneux², M Sigal-Grinberg^1,*

¹Department of Dermatology, Victor Dupouy General Hospital, 69 rue du Lieutenant Colonel Prudhon, 95107 Argenteuil, FranceFax: (+33) 1 34 23 22 61.
²Department of Internal Medecine, General Hospital, Colombes, France

accepté le 26 Juin 2004

Case report

The erythrocyte sediment rate was elevated at 33 mm in the first hour. Exhaustive exploration for a potential associated neoplasm was negative. Electromyography showed a myositic process. Diagnosis of dermatomyositis was made and systemic prednisone (1 mg/kg/day) was administered. With this therapy, muscular weakness and muscular enzymes rapidly improved and the interstitial lung syndrome disappeared in 8 months. Erythematous cutaneous lesions vanished but severe poikilodermia progressed on the lower neck, the axillary areas, the upper back. Prednisone was progressivly tapered to 10 mg a day and definitively stopped in 1997. Despite the fact that myositis was in remission 5 years after presentation, calcic plaques and nodules developed on the arms, axillary areas and lower abdomen. Calcic nodules increased in size and number, and coalesced to form a stony armour on the trunk and the upper extremities. In some instances ulcerated lesions exuded chalky white material. Joint motions were severely limited with contracture of the elbows in flexion (110°), disturbing everyday activities such as dressing, cooking or eating. Radiographs confirmed calcic depositions in soft tissues ( (figure 1) ). Bone scintigraphy made in October 1997 showed an hyperfixation on the upper extremities and abdomen. Colchicine was first proposed, improving the inflammatory aspect but it did not lead to a real improvement of the calcinosis and the functional disability. In December 1997, diltiazem was started and gradually increased to 330 mg a day (3 mg/kg) with an excellent tolerance.

Functional improvment and well being was noted during the following year with an increasing amplitude of the elbows (140°) allowing current life activities. Calcifications and exudings dramatically decreased. This improvement was attested by a control radiograph (( figure 2 )). Diltiazem was continued with a constant progressive efficacity.

Discussion

CC results from hydroxyapatite calcium phosphate deposits in the dermis and hypodermis without abnormality in calcium and phosphorus levels. Mechanism of these calcium deposits is not clear but it could result from an intracellular accumulation of calcium secondary to an alteration of the cellular membrane by traumatism and inflammation. The calcinosis can progress whereas the underlying disease is controlled as in our patient. CC may cause pain, secondary infection and severe functional disability preventing everyday life activities.

Medical therapy of calcinosis is not highly effective: intralesionnal injections of corticosteroids have limited success [3], bisphophonates, or low dose warfarin have been tried with discordant results [4-8], probenecid and colchicine have led to improvement in many cases by decreasing the inflammation [9], hydroxyde aluminium has been used with success in several children’s dermatomyositis [10, 11]. In some cases, surgical management was used.

More recently diltiazem has been proposed with the hypothesis that a calcium channel blocker could reduce calcium entrance in cells and so the accumulation of it.

Our patient obtained an amazing beneficial effect of diltiazem with a decrease of the motor limitation allowing resumption of everyday activity which had become impossible. The improvement of calcinosis was assessed by radiographs. This efficacity continued as long as diltiazem was used during the following years. These results are supported by several previous reports in the recent literature in which diltiazem was used for childhood and adult calcinosis associated with dermatomyositis, scleroderma or lupus [12-15]. In these reports efficacity was evaluated by clinical decrease of the calcifications, functional improvment and objective studies including radiographs, bone scintigraphy, DEXA, or scan. The efficacity of diltiazem goes on as long as it is used. No adverse effect was reported.

Diltiazem seems to be an interesting therapy for treatment of calcinosis with few adverse effects.

References

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