Tumoral Calcinosis Of Hand: A Rare Location With Unusual Presentation

摘要

Tumoral Calcinosis is an entity characterised by large peri-articualar deposition of calcium phosphate that resembles a neoplasm. Although the aetiology remains obscure, this clinical entity is well established in literature. More than 300 case reports have been published in English literature, but isolated involvement of hand is extremely rare. We herein report a rare case of Tumoral Calcinosis in hand with a very unusual presentation. The clinical presentation posed a diagnostic problem, as it appeared to be infection till the radiograph revealed calcified nodular deposits. The calcific deposits were successfully removed surgically and patient had dramatic relief of symptoms. There was no disturbance of Calcium or phosphate metabolism and no family history of similar disorder was present. After two years of follow up, there was no recurrence and patient is symptom free. Introduction Any abnormal deposition of calcium salt in the soft tissue is called Calcinosis 1,2. There are three varieties of calcinosis 1,2, viz.: Calcinosis universalis Calcinosis circumscripta Tumoral calcinosis Calcinosis universalis is confined to children with deposition of calcium salts as nodules or plaques in skin, subcutaneous tissues and superficial muscles. This is associated with scleroderma in 40% of cases. The prognosis is usually bad. Calcinosis circumscripta has a benign course and occurs in middle-aged women. The deposits are small, nodular or streaky and affect the flexor tendon sheaths of hands and wrist. Scleroderma, sclerodactyly or Raynaud's disease is associated with 30-40% of cases.The third variety is rare and is characterised by large nodular peri-articualar deposits of calcium phosphate that resembles a neoplasm. Inclan (1943) 3 named it as “Tumoral calcinosis”. This lesion is different from the universal and circumscribed types. The lesions are more common among blacks and about 2/3rd of the cases reported are in blacks. More than 1/2 of the patients have an affected sibling. It is usually seen in adolescents and young adults. The lesions are usually asymptomatic and only rarely cause discomfort, pain and tenderness. The underlying joints are unaffected and as a rule the patients are in a good general health. This usually results in delayed presentation when the lesions have attained a large size. The masses usually are firmly attached to the underlying fascia, muscle or tendon. Rarely the lesion might ulcerate and discharge yellow-white chalky fluid, it may get secondarily infected and result in a fistula.There has been a lot of confusion about the aetiology of this condition, which has resulted in many names for the same condition. Although the aetiology is not yet known, the term Tumoral calcinosis is generally accepted to describe this condition. It is classically known to affect large joints and involvement of hand is extremely rare. We herein report such a rare case of Tumoral calcinosis in hand. The presentation of this case was like an infection, which makes this case a unique one. The aim of this report is to make the hand surgeons aware about the unusual presentation of this rare condition, which can cause a diagnostic confusion. Case Report A 22 years old housewife, who was apparently healthy, presented with complaints of severe pain and swelling over the second web space of seven days duration. She noticed swelling seven days back associated with pain, which worsened rapidly in next five days. There were no other such lesions in the body. Patient reported to a local general physician who suspected infection and referred the patient to us. The pain was severe enough to prevent her using the hand. But surprisingly, the patient was afebrile and in good general health. At first instance it appeared to be infection but the absence of fever, and general good health raised suspicion in our mind. There were no signs of tenosynovitis, palmer space infection or flexor sheath infection. There was no regional lymphadenopathy. Mov