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Calcinosis Cutis – a Study of Six Cases

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Calcinosis Cutis – a Study of Six Cases Case Series Calcinosis cutis – A study of six cases Huzaifa N Tak, Prema Saldanha, Pushpalatha Pai Department of Pathology, Yenepoya Medical College, Mangalore, Karnataka. Abstract Background: Calcinosis cutis is a very rare condition where in calcium deposits form in the skin. It occurs in four forms: metastatic, dystrophic, idiopathic and as a subepidermal nodule. Aim: This study was done to analyze the clinical and histological features of calcinosis cutis which have an influence on patient management. Material: A retrospective study of cases diagnosed in the Department of Pathology over a period of six years. Results: Six cases were found during this period, which included two cases of idiopathic calcinosis cutis, two of scrotal calcinosis, one case of calcinosis cutis secondary to systemic sclerosis, and one subepidermal calcified nodule. Conclusion: In the types in which there is an underlying systemic disease it is important to recognize this condition promptly for the proper management of the patient. Key words: Calcinosis cutis, idiopathic, dystrophic; scrotal calcinosis; subepidermal calcified nodule. Introduction an influence on the management of the patient. The deposition of insoluble calcium salts in the skin Material is known as calcinosis cutis. Metastatic, dystrophic, The cases of calcinosis cutis which were diagnosed idiopathic and subepidermalnoduleare four in our institution from 2009 to 2013 were studied subtypes of calcinosis cutis. Metastatic calcification retrospectively. The clinical presentation, relevant results from elevated serum levels of calcium or investigations and morphological findings were phosphorus[1,2,3]. The latter three subtypes are recorded. associated with normal serum calcium levels. Dystrophic calcinosis cutis is the most common. Results Dystrophic calcinosis is calcification associated There were two cases of idiopathic calcinosis cutis, with infection, inflammatory processes, cutaneous two of scrotal calcinosis, one case of calcinosis neoplasms or connective tissue diseases and it is cutis secondary to systemic sclerosis, and one most frequently seen in association with underlying subepidermal calcified nodule. The clinical and autoimmune connective tissue disease. Idiopathic morphological findings are given in Table 1. Routine calcinosis cutis is cutaneous calcification of unknown haematological, biochemical (including serum cause with normal serum calcium. Subepidermal calcium and phosphous levels) and serological calcified nodule, tumoral calcinosis and scrotal investigations were within normal limits in the calcinosis (idiopathic calcinosis of the scrotum) are first five cases. The sixth one was a known case of idiopathic forms of calcification[1,2,3]. Iatrogenic and systemic sclerosis. On gross examination of the traumatic calcinosis are also described, which are histopathological specimens the cut surfaces of associated with medical procedures[2,3]. the nodules had chalky white areas and felt gritty This study was done to analyse the clinical and to cut (Figure 1). Histologically, with the routine histological features of calcinosis cutis which have haematoxylin and eosin stain, the calcified material Address for Correspondence Dr. Prema Saldanha, Professor of Pathology, Yenepoya Medical College, Mangalore-575018, Karnataka, India. E-mail:[email protected] Medica Innovatica December 2014, Volume 3 - Issue 2 107 Prema Saldanha, et al,: Calcinosis cutis – A study of six cases had a basophilic, amorphous granular, and in some specific for calcium. cases also a clumped appearance (Figure 2). This was confirmed by the Alizarin Red S stain which is Discussion Table 1. The clinical and pathological findings of the patients in our study Age/ Clinical presentation and Histolopatholgical Sl.No Microscopic findings gender Diagnosis Diagnosis Subepidermal basophilic Swelling on the trunk since granular clumps of 1. 24/M 1 year - Calcified sebaceous Calcinosis cutis calcification with formation cyst of bone. Dermis and subcutaneous Swelling since 20 years tissue showed a nodules of associated with pain since 2. 65/F calcified material and thick Calcinosis cutis 3 years - Calcified bursa of fibrocollageneous tissue the right iliac crest around. Epidermis had verrucous surface with hyperkeratosis Lesion left arm since 2 and acanthosis. Upper Subepidermal 3. 18/F years - Naevus dermis showed aggregates calcified nodule of calcium globules and masses. Multiple small painless Dermis showed amorphous Idiopathic Calcinosis 4. 38/M firm to hard nodules on the basophilic material. of the Scrotum scrotal skin – 6 months Irregular large masses of Multiple small firm to hard calcified material in the Idiopathic Calcinosis 5. 39/M painless nodules on the dermis. Calcified material of the Scrotum scrotal skin – 1 year surrounded by a foreign- body reaction. Epidermis with Patient diagnosed as hyperkeratosis and Calcinosis cutis (in systemic sclerosis since acanthosis. Dermis showed 6. 35/F a case of systemic 10 years Had features of thick collageneous tissue sclerosis) CREST syndrome with areas of dense calcification 108 December 2014, Volume 3 - Issue 2 Medica Innovatica Prema Saldanha, et al,: Calcinosis cutis – A study of six cases Figure 1. A case of scrotal calcinosis. Skin showing multiple nodules Cut surfaces of the nodules are chalky white and gritty Figure 2. Dermis showing calcified material surrounded by a foreign-body reaction(H&Ex400) Medica Innovatica December 2014, Volume 3 - Issue 2 109 Prema Saldanha, et al,: Calcinosis cutis – A study of six cases Pathologic calcification is the abnormal tissue limbs, especially digits, the neck, and often the deposition of calcium salts. There are two forms nose. This association is seen with the Thibierge- of pathologic calcification. When the deposition Weissenbach syndrome or the CREST (calcinosis occurs locally in dead or degenerating tissues cutis, Raynaud’s phenomenon, esophageal it is known as dystrophic calcification; it occurs dysfunction, sclerodactyly, telangiectasia) despite normal serum levels of calcium and in the syndrome[1,4]. One of the cases in our study was of a absence of derangements in calcium metabolism. 35 year old woman who presented with features of In contrast, the deposition of calcium salts in CREST syndrome. otherwise normal tissues is known as metastatic Dystrophic calcinosis cutis also occurred in cutaneous calcification, and it almost always results from neoplasms such as pilar cyst, basal cell carcinoma, hypercalcemia secondary to some disturbance in intradermal nevi, desmoplastic malignant [1,2,3] calcium metabolism . melanoma, pyogenicgranuloma, hemangioma, Calcinosis cutis is a very rare condition where in trichoepithelioma, and seborrheic keratosis and calcium deposits form in the skin. There are four mixed tumours (chondroidsyringomas)[2]. forms of calcinosis cutis: metastatic, dystrophic, Idiopathic calcification occurs without any idiopathic and subepidermal calcified nodule. The underlying tissue damage or metabolic disorder. deposition can be in the dermis, subcutaneous The calcification is most commonly localized tissue, or vascular endothelium when the local to one general area, but a case of unusually calcium concentration exceeds its solubility in the widespread calcinosis cutis has been reported[5]. [2] tissue . Idiopathic calcinosis cutis comprises subepidermal Metastatic calcification develops as a result calcified nodules, tumoralcalcinosis, and scrotal of hypercalcemia or hyperphosphatemia. calcinosis[1,2,3]. Four of the cases in this study were Hypercalcemia may result from primary idiopathic calcinosis cutis, in which no underlying hyperparathyroidism, excessive intake of milk and cause could be found out. Subepidermal calcified alkali, excessive intake of vitamin D, or extensive nodules occur on the head and extremities, mainly destruction of bone through osteomyelitis or as solitary, hard, white yellowish papules of 3 to 11 metastasis of a carcinoma. Hyperphosphatemia mm size. The disorder usually occurs in children and occurs in chronic renal failure. Metastatic can even be present at birth[6,7]. Some investigators calcification most commonly affects the media of suggest that they represent calcified adnexal the arteries and the kidneys. Instances of cutaneous structures[8,9]. [1,2,3] . metastatic calcinosis are rare . A special form of idiopathic calcinosis cutis is Dystrophic calcification is the most common type tumoral calcinosis which is characterized by large and occurs as a result of local tissue injury. Although periarticular deposits of calcium resembling calcium and phosphate metabolism and their serum neoplasms and is found commonly around hip, levels are normal, local tissue abnormalities, such shoulder, and elbow joints. It usually affects as alterations in collagen, elastin, or subcutaneous adolescents and young adults presenting as multiple fat may trigger calcification[2]. The internal organs lesions. Tumoral calcinosis as intramuscular or usually remain unaffected. There may be numerous subcutaneous calcific masses around major joints in large deposits of calcium (calcinosis universalis) or otherwise healthy adolescent in sporadic or familial only a few deposits (calcinosiscircumscripta). cases had also been described[10]. Calcinosisuniversalis occurs as a rule in patients with Scrotal calcinosis is a rare benign local process in dermatomyositis, but exceptionally it has also been the absence of any systemic metabolic disorder that observed in patients with
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