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- DOI 10.18231/j.ijpo.2021.059
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Clinicopathological study of 6 cases of idiopathic calcinosis cutis: A case series
- Author Details:
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Tejashwini Kotian *
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Prachi Gaddam
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Susan Cherian
-
Raji Naidu
-
Uma Chaturvedi
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Sruthimayura
Tejashwini Kotian *
Prachi Gaddam
Introduction
Calcinosis cutis is a rare, benign and usually asymptomatic condition which may involve any part of the skin and is characterized histologically by deposition of calcium within the dermis. It can be of metastatic, dystrophic, idiopathic or iatrogenic type. Idiopathic calcinosis cutis is cutaneous calcification of unknown cause in the absence of any metabolic disorder.[1] Few authors have postulated dystrophic calcification of the epithelial inclusion cysts as the cause.[2] Very few case series of this entity have been reported in the literature.[3]
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Metastatic |
Associated with hypercalcemia or hyperphosphatemia |
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Systemic disorders |
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Dystrophic |
Calcium deposition in previously damaged tissue |
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Calcinosis universalis (Calcinosis circumscripta) |
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Idiopathic |
No underlying cause |
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Tumoral calcinosis: large deposits near the joints |
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Idiopathic calcinosis of scrotum |
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Subepidermal calcific nodule |
Cutaneous calculi on face |
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In children |
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Calciphylaxis |
Calcification of cutaneous blood vessels |
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Can be associated with metabolic disorders. |
Case History
We report 6 cases of idiopathic calcinosis cutis. The patient’s age ranged from 25 to 71 years. 3 were males and 3 females. Blood reports were normal. Serum calcium, phosphorus, vitamin D were present within normal limits. Lesions were located on the scrotum, scalp, axilla, iliac region with size ranging from 0.5 to 3 cm. Most cases were clinically diagnosed as sebaceous cyst or lipoma. Cytology was performed in one case which showed amorphous granular material. Grossly excised lesions showed whitish chalky deposits.
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Case no |
Age in years |
Sex |
Site |
Clinical Diagnosis |
Single/ Mutiple |
Size in CM |
Epithelial Lining |
|
1 |
54 |
Male |
Scrotum |
Scrotal calcinosis |
Multiple |
3- 1.5cm |
Absent |
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2 |
71 |
Female |
Right iliac crest |
Cutaneous calcification seen on X ray. Cytology done. |
Multiple |
3-1.5cm |
Absent |
|
3 |
40 |
Male |
Scrotum |
Sebaceous cyst |
Multiple |
1.5 -0.3cm |
Absent |
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4 |
23 |
Female |
Axilla |
Sebaceous cyst |
Single |
2cm |
Present |
|
5 |
30 |
Female |
Scalp |
Sebaceous cyst |
Single |
2cm |
Present |
|
6 |
34 |
Male |
Scrotum |
Sebaceous cyst |
Multiple |
3- 2.5cm |
Present |
Histopathological examination showed a variable amount of calcium deposition with surrounding foreign body reaction. Two cases showed the lining of epidermal inclusion cyst along with calcification. Metabolic and connective tissue workup was normal in all cases. None of the cases have reported recurrence till date.
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|
Muddegowda et al 2011 |
Andola et al 2014 |
Alok et al 2017 |
Present study |
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Number of cases |
4 |
9 |
18 (5 idiopathic) |
6 |
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M:F |
2 Male 2 Female |
9 Male (scrotal calcinosis) |
13Male,5 Female |
3 (Male) 3 ( Female) |
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Size of nodules |
0.4 to 4 cm |
0.5 to 3.5cm |
0.5 to 4.8cm |
0.3cm to 3cm |
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Most common site |
Scrotum, knee, thorax Right arm |
Scrotum |
Scrotum, Hip, scalp |
Scrotum |
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Clinical Diagnosis |
Sebaceous cyst |
Sebaceous cyst |
Sebaceous cyst |
Sebaceous cyst |
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Evidence of epithelial lining |
1 case with calcification within cyst |
Not seen |
- |
3 cases |
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Evidence of calcification elsewhere in body |
No |
No |
No |
No |
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Biochemical values(serum calcium phosphate, uric acid, vitamin D) |
WNL |
WNL |
WNL (in idiopathic cases) |
WNL |
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Recurrence |
Not mentioned |
NIL |
Not mentioned |
NIL |
Discussion
Idiopathic cutaneous calcinosis is a rare, benign, local process, characterized by multiple, painless, hard subcutaneous nodules in the absence of any systemic metabolic disorder.[3] Exact incidence is difficult to report as this entity is usually described in the form of individual case reports and limited numbers of case series are available in the literature.[4] Multiple theories are proposed for pathogenesis of cutaneous calcinosis by various authors.
There are very few case reports on FNA cytology of idiopathic calcinosis cutis.[5], [6] FNA samples yielding abundant calcium have differential diagnosis of calcified fibrous pseudotumor, calcified epidermal cyst, sarcoidosis, tuberculosis, lymphoepithelial lesion, pilomatricoma, osteitis fibrosa cystica, and extra skeletal osteosarcoma etc. Cytological finding of amorphous calcium salts with histiocytes and the appropriate clinical background can help to suspect idiopathic calcinosis cutis which needs confirmation by histology.
Surgical excision is the treatment of choice and has excellent prognosis. Recurrence rate is very low. Incomplete excision may leave microscopic foci of calcification which may recur later.[7]
Conclusion
This case series is reported to create awareness about the rare entity of idiopathic cutaneous calcinosis which is a benign and local process. It can be diagnosed and managed accurately with clinical, pathological and metabolic correlation. Cytology may be performed in larger lesions. Histopathology is the gold standard for diagnosis. It is completely curable by surgical excision and has very low recurrence if completely excised.
Source of Funding
None.
Conflict of Interest
The authors declare that there is no conflict of interest.
References
- PH Muddegowda, JB Lingegowda, RK Ramachandrarao, PG Konapur. Calcinosis Cutis: Report of 4 Cases. J Lab Physicians 2011. [Google Scholar] [Crossref]
- SK Andola, T Tandon, AG Patil. Clinical-epidemiological, Cytological and Histopathological Study of Idiopathic Calcinosis Cutis of the Scrotum. J Clin Diagn Res 2017. [Google Scholar] [Crossref]
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- V Shah, T Shet. Scrotal Calcinosis Results From Calcification of Cysts Derived From Hair Follicles: A Series of 20 Cases Evaluating the Spectrum of Changes Resulting in Scrotal Calcinosis. Am J Dermatopathol 2007. [Google Scholar] [Crossref]
- CSB Viegas, S Cavaco, PL Neves, A Ferreira, A João, MK Williamson. Gla-Rich Protein Is a Novel Vitamin K-Dependent Protein Present in Serum That Accumulates at Sites of Pathological Calcifications. Am J Pathol 2009. [Google Scholar] [Crossref]