|SHORT COMMUNICATION |
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|Year : 1995 | Volume : 61 | Issue : 1 | Page : 28-31 |
MV Rao, BSN Reddy, M Nadeem, Mariette D'souza, Gar
M V Rao
Tumoral calcinosis is an unusual clinical disorder in which large masses of calcium are deposited in the periarticular tissues of the body. The characteristic clinical, radiological and histopathological features of this disorder occurring in three middle aged female patients are reported.
Keywords: Tumoral calcinosis, Dystrophic calcification
|How to cite this article: |
Rao M V, Reddy B, Nadeem M, D'souza M, Gar. Tumoral calcinosis. Indian J Dermatol Venereol Leprol 1995;61:28-31
| Introduction || || |
Tumoral calcinosis (TC) is an uncommon but distinct clinical entity, characterized by the deposition of calcium in the tissues adjacent to the major joints of the body. The eponym TC was coined by Inclan in 1943 although it was discribed initially by Durat as early as 1899. Many dermatologists are not familiar with this entity. We report herein the details of TC in three female patients.
| Case Report || || |
Case 1. A 50-year-old women presented with small swellings of the skin near gluteal cleft of 3½ years duration. Her problem started as small firm, painless papulonodular lesions which gradually progressed to the present size. History of frequent episodes of breakdown of overlying skin resulting in the discharge of milky white material was present. No history suggestive of either sexually transmitted disease or tuberculosis could be obtained. None of her family members had similar lesions. Past treatment with topical and systemic antibiotics resulted in little benefit.
Clinical examination revealed an average built and well-nourished woman with papules, nodules and swellings of various sizes extending on an area of 10x8 cm2 near the gluteal cleft and adjoining sacral area [Figure - 1] The overlying skin was punctated with sinuses discharging chalky material. The lesions were firm, non-tender and not adherent to the underlying bones. Irregular patchy scarring and perilesional hyperpigmentation were evident. Rest of the cutaneous and systemic examination revealed no other abnormality.
Laboratory investigations including complete haemogram, liver function tests, blood urea, creatinine, sugar, electrolytes, VDRL and urine examination were within normal limits. Serum calcium and phosphorus values were 9.8 mg/dl and 5.4 mg/dl respectively. Tuberculin test was negative. Radiographs revealed areas of calcification in the soft tissues near sacrum. Histopathological examination of the skin lesion revealed multiple small cysts with basophilic calcified masses and loose fibrocollagenous tissue in the lower dermis and subcutis [Figure - 2]. A cellular reaction consisting of histiocytes, foreign body giant cells, and lymphocytes was seen around the calcified cystic lesions.
Case 2. A woman aged 50 years came with hard swellings over right hip of 3 years duration. The lesion started as a small nodule which gradually increased in size, followed by similar nodules adjacent to the initial lesion. History of periodic episodes of pain and intermittent discharge of whitish gritty material from the lesion was present. None of her family members had similar lesions. Past history revealed no major illness.
Physical examination revealed three nodular lesions, each 1x1 cm2 size situated 5 cm below the right iliac crest [Figure - 3]. The lesions were bony hard, non-tender and adherent to the overlying skin. Patchy scarring was evident. The surrounding skin showed mild hyperpigmentation and induration. The corresponding site on the left side revealed no visible mass but irregular, hard non-tender nodules, three in number, two measuring 1x1cm2 and one measuring 3x2 cm2 were palpable subcutaneously. Rest of the clinical examination revealed no abnormality. Haematological investigations, liver and kidney function tests were normal. Serum calcium (9.7 mg/dl), phosphorus (4.2 mg/dl) levels were normal. Radiological examination revealed calcium deposition adjacent to both the hip bones [Figure - 4]. Histopathological examination of the lesion revealed features consistent with TC.
Case 3. A 60-year-old female patient presented with swellings over left thigh of 10 years and over right thigh of 2 years duration. The lesions gradually progressed with periodic breakdown of overlying skin resulting in discharge of gritty white fluid. No history of any significant illness or similar lesions in the family was found.
On examination irregular, hard, tender nodular swellings of varying sizes from 6x2 cm2 to 2x1 cm2, present over upper and lateral aspect of both thighs. The lesions were attached to the skin and surface showed mild hyperpigmentation and small puckered scars.
Haematological, hepatic, and renal profiles including serum calcium (9.6 mg/d1) and phosphorus (4.2 mg/dl) were normal. Radiological examination revealed soft tissue calcification around both hip bones. Skin biopsy revealed features similar to the earlier patients.
| Discussion || || |
TC is an unusual clinical entity in which large masses of calcium are deposited in periarticular soft tissues and skin. Since these patients are not frequently encountered in dermatology clinics, there may be some difficulty in making an initial diagnosis, and at times confusing with other skin problems such as tuberculosis, deep mycoses, LGV etc. Nevertheless, the diagnosis of TC can be established with the characteristic radiological and histological features.
Clinically the lesions typically occur in the periarticular regions, especially around the hip, the greater trochanter and behind elbow, as masses of varying sizes with chronically discharging sinuses exuding a chalky material. Radiological features are seen as irregular densely calcified areas in the soft tissue spaces adjacent to joints.
The oetiology of tumoral calcinosis is unknown. The hereditary nature has not been established, although in one third of the cases the disease is believed to be familial. In none of our cases family history was found. Several theories have been postulated including calcifying endothelioma, calcified bursa, aberrant synovial tissue and a clinical form of calciphylaxis due to trauma. The theory attributing TC to a form of dystrophic calcification seems most acceptable and explains its occurrence over pressure points in the poor socioeconomic groups who sleep on the hard ground such as seen in our patients. Focal damage to the collagen by pressure ischaemia possibly leads to calcification and the development of TC.
Normal levels of serum calcium and phosphorus were seen in majority of the patients reported in literature including the cases described herein, thereby excluding the possibility of metastatic calcification. However, in isolated cases elevated serum phosphorus levels were reported and an inborn error of phosphorus metabolism was proposed. Chemical analysis of the deposited material has shown to contain either calcium phosphate or carbonate for the development of this condition.
Treatment of this condition is mainly surgical excision. Results with radiotherapy, cortisone, and various dietary restrictions to lower calcium and phosphorus intake have shown little success.
| References || || |
|1. ||Inclan A. Tumoral calcinosis. JAMA 1943;121:490-5. |
|2. ||Duret MH. Tumeurs multiples et singulieres des bourses sereuses. Bull Soc Anat 1899;74:725-31. |
|3. ||Whiting DA, Simson IW, Kallmeyer JC, Dannheimer IPL. Unusualcutaneous lesions in tumoral calcinosis. Arch Dermatol 1970;102:465-73. |
|4. ||Baldursson H, Evans EB, Dodge WF, el al. Tumoral calcinosis with hyperphosphatemia : A report of a family with incidence in four siblings. J Bone Joint Surg Am 1969;51:913-25. |
|5. ||Veress B, Malik MOZ, Ell Hassan AM. Tumoral lipocalcinosis: A clinicopathological study of 20 cases. J Pathol 1976;119:113-8. |
|6. ||Pursley TV, Prince MJ, Chausmen AB, Raimer SS. Cutaneous manifestations of tumoral calcinosis. Arch Dermatol 1979;115:1100-2. |
|7. ||Lafferty FW, Reynolds ES, Pearson OH. Tumoral calcinosis: A metabolic disease of unknown etiology. Am J Med 1965;38:105-18. [PUBMED] |
Figures [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]