Kotian, Gaddam, Cherian, Naidu, Chaturvedi, and Sruthimayura: Clinicopathological study of 6 cases of idiopathic calcinosis cutis: A case series


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

Table 1

Types of calcinosis cutis

Metastatic

Associated with hypercalcemia or hyperphosphatemia

Systemic disorders

Dystrophic

Calcium deposition in previously damaged tissue

Calcinosis universalis (Calcinosis circumscripta)

Idiopathic

No underlying cause

Tumoral calcinosis: large deposits near the joints

Idiopathic calcinosis of scrotum

Subepidermal calcific nodule

Cutaneous calculi on face

In children

Calciphylaxis

Calcification of cutaneous blood vessels

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.

Table 2

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

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

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.

Figure 1

Well circumscribed amorphous basophilic deposits in dermis. H&E 100X

https://typeset-prod-media-server.s3.amazonaws.com/article_uploads/eb90f906-f589-4fa2-b02b-993c807dc03e/image/046e5d07-ca2e-4c5a-839c-1a3d2e6c77dd-uimage.png

Figure 2

Giant cell reaction around the deposits. H&E 400X

https://typeset-prod-media-server.s3.amazonaws.com/article_uploads/eb90f906-f589-4fa2-b02b-993c807dc03e/image/1d193759-f60e-46ce-9576-2f5501223283-uimage.png

Figure 3

Epidermal inclusion cyst undergoing calcification. H&E 400X

https://s3-us-west-2.amazonaws.com/typeset-prod-media-server/61c33dd4-5dd3-41c1-bc92-f5a4454b4279image3.jpeg

Figure 4

FNAC: Amorphous basophilic material. Giemsa 400X

https://typeset-prod-media-server.s3.amazonaws.com/article_uploads/eb90f906-f589-4fa2-b02b-993c807dc03e/image/e58725a8-0acf-412d-aab7-23f066fb428a-uimage.png

Figure 5

FNAC: Giant cells. Giemsa 400X

https://s3-us-west-2.amazonaws.com/typeset-prod-media-server/61c33dd4-5dd3-41c1-bc92-f5a4454b4279image5.png

Table 3

Muddegowda et al 2011

Andola et al 2014

Alok et al 2017

Present study

Number of cases

4

9

18 (5 idiopathic)

6

M:F

2 Male 2 Female

9 Male (scrotal calcinosis)

13Male,5 Female

3 (Male) 3 ( Female)

Size of nodules

0.4 to 4 cm

0.5 to 3.5cm

0.5 to 4.8cm

0.3cm to 3cm

Most common site

Scrotum, knee, thorax Right arm

Scrotum

Scrotum, Hip, scalp

Scrotum

Clinical Diagnosis

Sebaceous cyst

Sebaceous cyst

Sebaceous cyst

Sebaceous cyst

Evidence of epithelial lining

1 case with calcification within cyst

Not seen

-

3 cases

Evidence of calcification elsewhere in body

No

No

No

No

Biochemical values(serum calcium phosphate, uric acid, vitamin D)

WNL

WNL

WNL (in idiopathic cases)

WNL

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

1 

PH Muddegowda JB Lingegowda RK Ramachandrarao PG Konapur Calcinosis Cutis: Report of 4 CasesJ Lab Physicians20113125610.4103/0974-2727.86849

2 

SK Andola T Tandon AG Patil Clinical-epidemiological, Cytological and Histopathological Study of Idiopathic Calcinosis Cutis of the ScrotumJ Clin Diagn Res201711111410.7860/jcdr/2017/27741.10814

3 

A Mohan S Singh VK Sharma P Sharma S Kaur Calcinosis cutis of usual and unusual sites: An eight year retro-prospective study in a tertiary teaching hospital in Western Uttar Pradesh, IndiaIndian J Pathol Oncol2017421615

4 

S Dubey R Sharma V Maheshwari Scrotal calcinosis: idiopathic or dystrophic?Dermatol Online2010165

5 

P Agrawal T Banik P Dey Calcinosis cutis: Diagnosis by fine needle aspiration cytology-A rare case reportDiagn Cytopathol20113912917810.1002/dc.21577

6 

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 CalcinosisAm J Dermatopathol2007292172510.1097/01.dad.0000246465.25986.68

7 

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 CalcificationsAm J Pathol2009175622889810.2353/ajpath.2009.090474



jats-html.xsl

© This is an open access article distributed under the terms of the Creative Commons Attribution License Attribution 4.0 International (CC BY 4.0). which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.


  • Article highlights
  • Article tables
  • Article images

Article History

Received : 18-11-2020

Accepted : 05-02-2021

Available online : 19-05-2021


View Article

PDF File   Full Text Article


Copyright permission

Get article permission for commercial use

Downlaod

PDF File   XML File   ePub File


Digital Object Identifier (DOI)

Article DOI

https://doi.org/10.18231/j.ijpo.2021.059


Article Metrics






Article Access statistics

Viewed: 104

PDF Downloaded: 37