Linear Scleroderma with Calcinosis and its Successful Treatment with Surgical Excision

Sir,

Linear scleroderma is a variant of localized scleroderma.[1] The clinical picture is of a unilateral lesion affecting the extremities, scalp or face. The affected skin shows induration, atrophy and/or pigmentary changes. Cutaneous calcification is common on the fingertips in cases of systemic sclerosis, but occurs rarely in a plaque of localized linear scleroderma. We describe a case of linear scleroderma associated with calcinosis.

A 22-year-old female presented with a single, linear plaque with atrophy over the anterior surface of the right arm and postero-lateral aspect of the forearm [Figure 1]. It had been present for 10 years with tightening of skin, but since the past 2 months the patient complained of pain and swelling in an area of the plaque just above the elbow. The patient gave history of discharge of chalky white material from this area. There was no history of ulceration, Raynaud's phenomenon, joint pain or systemic involvement.

Figure 1.

Linear scleroderma with plaque overlying calcinosis (arrow)

On examination, there was a linear, atrophic plaque over the right upper extremity extending from the anterior aspect of the mid-arm to the lateral aspect of the elbow joint up to the distal third of the extensor aspect of the forearm, of width 2-3 cm, with some areas of hypo- and hyperpigmentation at places. Tightening and atrophy of skin was seen over the plaque, without evidence of muscle weakness, limb atrophy or joint contracture. A well-defined, hard, tender swelling, about 3 × 2 cm in size, was seen over the linear plaque, located on the lateral aspect of the arm just above the elbow joint.

The patient's blood chemistry, including serum calcium, phosphorus and alkaline phosphatase, was within normal limits. Rheumatoid factor and work-up for collagen vascular disorders was negative. X-ray of the right arm and forearm showed localized calcium deposits within the plaque above the elbow [Figure 2]. The computed tomography scan revealed calcinosis in the subcutaneous tissue with no involvement of muscles.

Figure 2.

X-ray (AP view) of the right upper extremity showing localized calcification

The patient consented to removal of the area of calcinosis by surgical excision. At the end of 6 weeks, the area showed complete healing [Figure 3]. The excised tissue along with the overlying skin was sent for histopathological examination. Histopathology showed epidermal atrophy and an increase in the collagen in the dermis with a decrease in the skin appendages, suggestive of morphea. The dermis and subcutaneous tissue showed multiple areas of calcium deposition.

Figure 3.

Same patient after 6 weeks of surgical excision

Localized scleroderma associated with calcification is uncommon, with only a few case reports published. Studies conducted on a large number of patients with localized scleroderma have shown the incidence of calcification to be 0-2.7% in the affected cases. In the study of 235 cases of localized scleroderma by Christianson et al.[2] and 106 cases of scleroderma by Curtis et al.,[3] no case of calcinosis was found. In the study by Muller et al.,[4] of 36 linear scleroderma patients, only one showed calcinosis. Calcification up to the level of muscle has also been reported.[1] Treatment modalities like oral probenecid, aluminum hydroxide, diphosphonates, colchicine, etc., have been tried for cutaneous calcinosis without significant benefit. Surgical excision of the calcified area of the plaque gave our patient satisfactory relief.