Diffuse Idiopathic Calcinosis Cutis in an Adult : A Rare Case

Idiopathic calcinosis cutis is a condition involving the deposition of calcium salts in the skin and subcutaneous tissue. The disease is a pathological condition of unknown origin and hence is idiopathic. The salt deposition is confined to areas such as the breast and vulva in females and scrotum and penis in males. Diffuse calcification with multiple complications in an adult is a rare entity. Only one such case has been reported in literature. A 59-year-old female presented to us with swelling of the right elbow, multiple calcific nodular lesions all over her fingers approximately 0.5x0.5 cm in size, and ulcers on her left great toe and right thumb with pain for the past two months. The ulcers were 2x2 cm and were observed to be healing without active discharge or signs of inflammation. The elbow was diffusely swollen and tender. Flexion deformity was present at the elbow. X-ray of hand and feet revealed calcinosis of the elbow and interphalangeal joints of the foot and hand. Blood tests revealed elevated C-reactive protein levels of 24 mg/dL, elevated Erythrocyte Sedimentation Rate (ESR) of 52 mm/ hr., serum calcium of 9.7 mg/dL and a serum phosphorous of 5 mg/ dL. Cultures from the foot ulcer were positive for methicillin-resistant staphylococcus aureus (MRSA). Workup for collagen vascular disease was negative. Histopathology confirmed calcinosis cutis. Treatment involved a conservative approach, including physiotherapy for the flexion deformity, antibiotics for MRSA, analgesics for pain relief and daily dressings. This case demonstrates that if a patient presents with multiple chalky nodular lesions with or without ulceration, pain and discharge involving areas of the upper limb or lower limb, diagnosis of idiopathic calcinosis cutis could be considered as a differential, despite its common confinement to the scrotum, breast, vulva and penis.


Introduction
Calcinosis cutis is a pathology characterized by the deposition of calcium salts in the skin and subcutaneous tissue.Etiologically, calcinosis cutis is divided into 4 major types: dystrophic calcification (most common, 95-98% cases), metastatic calcification, idiopathic calcification and iatrogenic calcification [1].Idiopathic calcinosis cutis is rare and hence is usually a diagnosis of exclusion [2].The disease is often restricted to the vulva, scrotum, penis, and breast [3].Our patient was an elderly woman who presented with calcinosis cutis in the hands, feet and elbow with ulceration and flexion deformity.Only one such case of diffuse Idiopathic calcinosis cutis (ICC) in an adult has been reported in the literature to the best of the author's knowledge [4].

Case Report
A 59-year-old female agriculturist presented with an ulcer on the ball of left great toe and of the right thumb of 2 months duration with associated pain and purulent discharge for the past 2 weeks.She also complained of pain and swelling of the right elbow and multiple nodular lesions over the hand and the sole (Figure 1).No history of similar lesions over the breast or genitalia was present.There was no history of trauma or pathologic lesions at the site of the nodular lesions.No history of loss of weight or appetite or fever was present.The patient was postmenopausal and had been diagnosed as diabetic and hypertensive and was on oral hypoglycemic drugs and anti-hypertensive medications, respectively, for the past 2 months.There was no significant family history.
On examination, the ulcer at the ball of the toe was approximately 2x2x2 cm (Figure 1) with signs of healing, and the ulcer of the right thumb was 1x1x1 cm (Figure 2).There was no active discharge or signs of inflammation, including redness, temperature increases or tenderness.The multiple nodular lesions of the upper limb digits were approximately 0.5x0.5 cm and calcific in nature.The elbow was diffusely swollen and tender.Fixed flexion deformity was present at the elbow (Figure 3).
Blood Tests revealed C-reactive protein (CRP) levels of 24.0 mg/dL (0-6 mg/dL); ESR of 52 mm/hr.(0-20 mm/hr.);serum Hansen's disease.The biopsy revealed the accumulation of calcium salts in the dermis (Figure 4).Treatment involved a conservative approach that included physiotherapy for the flexion deformity, oral clindamycin 300 mg 1-1-1x10 days for Methicillin-resistant staphylococcus aureus (MRSA), non-steroidal anti-inflammatory drugs 1-0-1x10 days for pain relief and daily dressing.The patient was advised to follow-up and undergo excision of the nodules if ulcer persists.

Discussion
Calcinosis cutis is divided into the following subtypes: dystrophic, metastatic, iatrogenic and idiopathic.Dystrophic calcinosis is the most commonly observed form and usually follows trauma associated with damaged, inflamed and necrotic skin and is also associated with connective tissue disorders such as the limiting form of systemic scleroderma (CREST syndrome) and dermatomyositis [5].Metastatic calcification usually occurs in situation of hypercalcemia and/or hyperphosphatemia when calcium phosphate products exceed 70 mg 2 /dL 2 in conditions like hyperparathyroidism and hypervitaminosis D [6].
Iatrogenic calcinosis is due to the intravenous administration of calcium or phosphate containing infusions like calcium chloride or calcium gluconate [7].
Idiopathic calcinosis cutis (ICC) is a rare form and is a diagnosis of exclusion.Idiopathic calcinosis is further classified into tumoral calcinosis, characterized by masses around the major joints usually appearing in otherwise healthy adolescents; sub-epidermal calcified nodules occurring on the head and extremities, usually observed in children; and scrotal calcinosis and milia-like calcinosis [8].The diagnosis of ICC was made in our patient in the absence of previous trauma, pathologic lesions at site of calcinosis, history of parenteral therapy and presence of calcium in the dermis on histopathology.The calcium and phosphate levels were within normal limits.Collagen vascular screening was normal.
Calcifications in ICC are usually localized to one area but can be seen all over the genitals and the breast [1,2].Our patient presented with calcinosis in the hands and feet.She also exhibited deposition of calcium in the subcutaneous tissue around the elbow leading to a permanent position of flexion at the elbow joint.Calcinosis at the ball of foot resulted in ulceration over a period of time.
There is no single treatment option for ICC.A variety of drugs, mainly bisphosphonates, intralesional corticosteroids, aluminum hydroxide, warfarin and diltiazem, have been tried with limited success [9,10].Local recurrence rates are high with surgical excision and are reserved for painful ulcerated nodules.
A better understanding of the mechanism of calcium deposition in ICC is necessary, especially in cases where it is diffuse, as in our case.Newer treatment options, both medical and surgical, have to be formulated for this rare disease so that complications like flexion deformities, recurrent ulceration and morbidity can be avoided.
Informed Consent: Written informed consent was obtained from the patient who participated in this case.

Figure 1 .Figure 2 ,Figure 3 .
Figure 1.a, b.Plain X-ray of the foot demonstrating calcification at the heel as well as the great toe (a).Clinical photograph revealing a superficial ulcer at the sole of the foot (b).