Bilateral calcinosis cutis of orbital walls in CREST syndrome

Abstract

Purpose:

To describe a patient with systemic sclerosis who presented with subcutaneous yellow–white nodules on her lateral orbital rims.

Methods:

Case report.

Results:

A 53-year-old female was referred for management of subcutaneous firm nodules on her lateral orbital rims. She was known to have CREST (calcification, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome, a limited variant of systemic sclerosis. Her drug history included diltiazem, captopril, aspirin, and prednisolone. There were bilateral multiple immobile yellow–white subcutaneous nodules with firm consistency, a few of which were in close proximity of periorbital skin. She was scheduled for surgical resection of the lesions and following tissue dissection, the calcified foci were removed. The incisions healed with some delay but the final postoperative follow-up at third month showed satisfactory appearance.

Conclusion:

Dystrophic calcinosis cutis occurs in approximately one-fourth of patients with systemic sclerosis (scleroderma), particularly in those with the limited variant or CREST syndrome. The most frequently involved sites are the extremities. Occasionally, calcinosis involves the trunk or head, but involvement of bony orbital walls is extremely rare. Surgical excision can be considered for treatment of discrete foci of calcinosis cutis.

Introduction

Calcinosis cutis is defined as the deposition of insoluble calcium salts in the cutaneous and subcutaneous tissue. ¹ Dystrophic calcification due to local tissue damage is the most common type of calcinosis cutis and is most commonly associated with autoimmune connective tissue diseases, particularly systemic sclerosis and dermatomyositis. ² Dystrophic calcinosis cutis occurs in approximately one-fourth of patients with systemic sclerosis (scleroderma), particularly in those with the limited variant or CREST (calcification, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome. ³ The most commonly affected sites include extremities, followed by trunk and head. ² Facial calcinosis is extremely rare ⁴ and periorbital calcification has only been reported in one patient previously. ⁵ Herein, we describe a patient with CREST syndrome who presented with prominent calcification of periorbital tissues.

Case description

The study was approved by ethics committee of our institute and adhered to the tenets of the Declaration of Helsinki as amended in 2008. Written informed consent was obtained from the patient for participation and probable publication of her photographs and medical data and the consent is archived with the authors. A 53-year-old female was referred for management of subcutaneous firm nodules on her lateral orbital rims. She was known to have CREST syndrome, a limited variant of systemic sclerosis. On general physical examination, Raynaud phenomenon was evident and sclerodactyly was observed (Figure 1(a)). Preoperative laboratory tests including serum calcium, phosphorus, and creatinine were normal. Medication history included diltiazem, captopril, aspirin, and prednisolone. There were bilateral multiple immobile yellow–white subcutaneous nodules with firm consistency, a few of which were in close proximity of periorbital skin (Figure 1(b)). Computed tomography revealed multiple round hyperdense lesions on lateral orbital rims (Figure 2). She was scheduled for surgical resection of the lesions. Under local anesthesia, curvilinear incisions were fashioned directly on lateral orbital rims and following tissue dissection, the calcified foci (Figure 3) were removed in piecemeal manner. Histopathological examination of excised lesions revealed fibrosclerotic tissue containing calcified degeneration. The incisions healed with some delay and sutures were removed on postop day 14, but the final postoperative follow-up at third month showed satisfactory appearance and no recurrence was observed (Figure 1(c)).

Figure 1.

The clinical appearance of the patient: (a) photography of the patient’s fingers showing sclerodactyly. (b) Face photograph before operation, revealing subcutaneous yellow–white nodules on lateral orbital rims. (c) Postoperative photograph after 3 months, showing resolution of prominent nodules.

Figure 2.

Axial (a) and three-dimensional (b) orbital CT scan showing multiple nodular masses with density of bony tissue.

Figure 3.

Intraoperative view of the subcutaneous calcified nodules attached to lateral orbital rims (a) which were excised in piecemeal fashion (b).

Discussion

Despite the presence of multiple mechanisms for careful regulation of serum calcium level, insoluble calcium salts may deposit in cutaneous and subcutaneous tissues. ¹ There are five subtypes of calcification, based on the etiology of calcium deposition, namely dystrophic, metastatic, idiopathic, iatrogenic, and calciphylaxis. ⁶ Dystrophic calcinosis cutis, the most common type of calcinosis cutis, is the result of calcium deposition into previously damaged tissue despite normal serum levels of calcium and phosphorus. Although occasionally associated with cutaneous neoplasms ¹ or localized trauma or infection, ² dystrophic calcinosis cutis is most frequently associated with autoimmune connective tissue disease. The two autoimmune connective tissue diseases with highest association with calcinosis cutis are systemic sclerosis and dermatomyositis. ²

Dystrophic calcinosis cutis affects approximately one of every four patients with systemic sclerosis, particularly its limited variant, CREST syndrome. ³ Calcium deposits frequently occur at sites of recurrent microtrauma ⁷ and its development in the periocular area of our patient who had no history of periorbital trauma, including cosmetic procedures or wearing glasses, is unexpected. Generally, the most commonly involved sites are feet and hands (particularly fingers), followed by extremities, trunk, and head. ² Facial calcinosis is extremely uncommon ⁴ and periorbital calcification has only been reported once in the literature. ⁵ Our patient had no other site of calcification, including calcinosis of fingers, despite her sclerodactyly (Figure 1(a)). Compared with the previously reported case with orbital involvement, ⁵ calcinosis was more extensive in the present case, but surgical removal was apparently successful in both instances.

Depending on the severity of signs and symptoms of calcinosis cutis, decision is made regarding therapeutic interventions for calcification. ⁸ The clinical manifestations of calcinosis cutis range in severity from an incidental radiographic finding to subcutaneous nodules or plaques, which can be painful or lead to functional impairment. Exophytic or ulcerated areas of involvement can induce significant discomfort, and local inflammation may also be present. ⁹ The treatment of dystrophic calcinosis cutis is often challenging and because of scarcity of relevant data, there is uncertainty about the best treatment option. ⁸ Adequate treatment of the underlying connective tissue disease is recommended. Medical and surgical treatments are warranted on a case-by-case basis. The most common medications used for calcinosis associated with autoimmune connective tissue diseases are diltiazem, warfarin, minocycline, and colchicine. ⁸ The latter two drugs act through anti-inflammatory mechanisms and are indicated for those patients who have inflammatory signs. Diltiazem is regarded as the first medical choice for patients with calcinosis cutis in the absence of prominent inflammation. ⁸ Our patient lacked inflammatory signs; therefore, minocycline and colchicine were not indicated for her treatment. She was already taking diltiazem as well as other medications for adequate control of her systemic sclerosis, but diltiazem was not effective in prevention or resolution of her calcifications. Treatment with warfarin for 1 year has been shown to be useful against calcinosis in a certain subset of systemic sclerosis patients, especially those with small and relatively new onset calcinosis. ¹⁰ However, our patient was less likely to respond to warfarin because of relatively large foci of long-standing calcinosis. Therefore, surgical option was selected for her.

Surgical removal of foci of calcinosis is an approach that could be considered for patients with discrete symptomatic areas of calcinosis. Although early concerns regarding poor wound healing and sinus track formation led to the historical avoidance of surgical interventions for calcinosis cutis, ⁸ subsequent reports have showed that surgery can be effective. ¹¹ In brief, we presented a patient with systemic sclerosis (CREST syndrome) who was successfully treated with surgical excision of calcinosis cutis of the orbit, an extremely uncommon site for dystrophic calcification.