Calcinosis cutis is an uncommon disorder characterized by the progressive deposition of crystals of calcium phosphate (hydroxyapatite) in the skin in various areas of the body. It is classified into four types according to etiology, namely as dystrophic if calcium and phosphorus levels are normal and tissue damage is present, as idiopathic if calcium and phosphorus levels are normal and no tissue damage is present, or as metastatic if there is hypercalcemia or hyperphosphatemia. Medical and surgical treatments are options to cure calcinosis cutis. Medical therapy is not very effective. Surgical excision has shown to be beneficial, as it can provide a symptomatic relief. However, since calcinosis cutis limits are not always well defined, a recurrence of the lesions may occur. We dealt with a very rare form of calcinosis cutis in a healthy 6-year-old girl. There was no evidence of connective tissue disorder or abnormal mineral metabolism. Hence, she was diagnosed as idiopathic calcinosis cutis and, although calcifications in idiopathic cutis are most commonly localized to one area, our patient unusually exhibited widespread calcific deposits. Although the existing lesions showed slow improvement, systemic pamidronate therapy was effective in preventing the occurrence of new lesions. Surgical excision proved to be an effective and successful treatment. This report aims to raise doctors’ awareness on the presentation, etiopathogenesis, and course of the relatively rare idiopathic calcinosis cutis.
Calcinosis cutis; Idiopathic; Child; Pamidronate; Surgery
Background: Calcinosis cutis—the deposition of insoluble calcium salts in the skin and the soft tissue—occurs in the following five settings: calciphylaxis, dystrophic, iatrogenic, idiopathic, and metastatic. Idiopathic calcinosis cutis of the penis is rare. Purpose: This paper describes a man with idiopathic calcinosis cutis of the penis, summarizes the clinical features of previously reported men with this condition, and also reviews dystrophic, iatrogenic, and metastatic penile calcinosis. Methods: A 27-year-old Pakistan man presented with concurrent, asymptomatic, individual nodules on the right mid-ventral penile shaft and left side of scrotum and two additional papules on the right side of the scrotum. Evaluation and treatment included the excision of all lesions. Reports of patients with penile calcinosis were identified using a medical search engine (PubMed Central) and referenced citations from the published papers on this subject. Results: Microscopic examination of the patient's nodules showed idiopathic and dystrophic calcinosis cutis of the penis and scrotum, respectively; the scrotal papules were fibroepithelial polyps. Including this individual, idiopathic calcinosis cutis of the penis has only been reported in 11 men. It presents as either an asymptomatic nodule (5 patients) or multiple lesions (6 patients) of less than one-year duration, on either the penile shaft (distal in 4 patients, mid in 2 patients, both in 1 patient, and site unspecified in 1 patient) or the prepuce (3 patients) of uncircumcised men less than 30 years of age. Concurrent scrotal calcification was noted in two patients. Dermal deposits of calcium are found in the dermis—often with surrounding histiocytes and multinucleated giant cells; concurrent features of dystrophic penile shaft calcification, such as calcium within syringomas or transepidermal elimination of calcium through eccrine sweat ducts, was only noted in two men. The nodules do not recur following excision. Conclusion: Idiopathic calcinosis cutis of the penis is extraordinary and has only been reported in 11 men. It presents as an asymptomatic nodule or nodules on mid- to distal penile shaft or foreskin. Concurrent scrotal calcinosis cutis was noted in two men. Microscopic examination shows calcium deposits in the dermis, usually with associated histiocytes and multinucleated giant cells; concurrent changes of dystrophic calcification were also present in two men. Excision of the penile nodules not only provides the diagnosis, but also successfully resolves the condition without recurrence.
Milia-like idiopathic calcinosis cutis is a rare entity. Only 19 cases have been reported so far, the majority of them developed in children with Down's syndrome. The mean age of the patients is 10.3 years, with a sex ratio of nine girls to ten boys. Hands are most commonly affected.
We report a case of a 69-year-old, otherwise healthy woman, who developed milia-like idiopathic calcinosis cutis on her forehead.
To our knowledge, we report the seventh case occurring in a patient without Down's syndrome, and the first case occurring in an elderly person.
Down's syndrome; calcinosis cutis; milia; MICC
Calcification of the skin occurs in four main forms namely dystrophic, metastatic, iatrogenic and idiopathic. Idiopathic calcinosis cutis of the penis is exceedingly rare as only five cases have been reported till date to the best of our knowledge. Herein, we present another case of this rare entity in a 29-year old man and discuss its probable pathogenic origin.
Calcinosis cutis; penis; idiopathic
Calcinosis cutis involves the inappropriate deposition of calcium within the dermis layer of the skin, and is often associated with rheumatoid disease. A 42-year-old woman presented for evaluation of a hard palpable mass on the left upper eyelid. After everting the eyelid, a large papillomatous mass with a broad base was identified on the superior area of the tarsus. The lesion was partially excised posteriorly under local anesthesia, and pathologists identified the mass as calcinosis cutis. The patient had no systemic or trauma history, and the serum levels of calcium and phosphorous were normal. Idiopathic calcinosis cutis should be included in the differential diagnosis for a protruding papillomatous mass of the tarsal plate, and surgical debulking could be a viable option for large protruding lesions, although more follow-up is necessary to monitor regrowth.
Calcinosis cutis; Idiopathic; Papillomatous mass; Tarsus
Milia-like idiopathic calcinosis cutis (MICC) is characterized by smooth, firm, whitish papules resembling milia. Histologically, it appears as a well-defined, round, basophilic nodule within the upper dermis. Although the etiology and treatment remain unclear, it may resolve spontaneously. Some cases have been associated with Down syndrome, and the mean age of MICC patients was 9.9 years old. Herein, we report a rare case of MICC that was not associated with Down syndrome. Noticeably, the patient, a toddler, was born as a premature baby and had an ischemic injury on the right foot at birth. However, the lesions appeared on both feet, including the non-injured left foot. Otherwise he was healthy. After a 21-month follow-up period, the lesions had almost disappeared without any treatment.
Calcinosis cutis; Prematurity
A 23-year-old lady presented with a slowly progressing firm mass on the nasal dorsum since 8 months. Her biochemical, haematological and collagen vascular disease screening tests were normal. Radiographs of the nasal bones showed a subcutaneous calcifying lesion with no evidence of nasal bone erosion. A diagnosis of idiopathic calcinosis cutis (ICC) was made. The mass was excised and soft tissue defect was augmented with silicone prosthesis. The histopathology with the haematoxylin and eosin staining and von Kossa stain confirmed the diagnosis of calcinosis cutis. This is an unusual presentation of ICC involving the nasal dorsum requiring surgery and nasal dorsal augmentation with silicone prosthesis.
Calcinosis cutis; idiopathic; nasal dorsum
Calcinosis cutis is a condition of accumulation of calcium salts within the dermis. We are presenting four cases of calcinosis cutis, with different clinical presentations, occurring in healthy individuals, with normal serum calcium and phosphorus levels. Histologically, all cases showed similar morphology, the lesions were composed of large and small deposits of calcium. Foreign-body giant cell reaction was seen in one case. Another case had intact and ruptured epidermal cysts and calcification within the cyst.
Calcinosis cutis; dystrophic calcinosis; subepidermal calcified nodule; tumoral calcinosis
Calcinosis cutis is a rare complication of adult dermatomyositis. The authors report a case of dystrophic calcinosis cutis that ultimately led to the diagnosis of dermatomyositis and metastatic lung adenocarcinoma.
Calcinosis cutis is a condition characterized by the deposition of calcium salts in the skin and subcutaneous tissues, and patients suffering from it encounter various connective tissue disorders, such as dermatomyositis (DM), scleroderma, and systemic lupus erythematosus. Although calcinosis cutis is frequently accompanied by juvenile dermatomyositis, rare cases have been reported in adult patients with DM. On the other hand, lichen sclerosus (LS) is a chronic inflammatory disease of the skin and mucosal surfaces. In the present report, we present a rare case of a 71-year-old patient with DM accompanied by ulcerated calcinosis cutis and vulvar LS.
Calcinosis cutis; Dermatomyositis; Lichen sclerosus
We report a case of idiopathic tumoral calcinosis localized to the thumb without prior trauma or surgery. Initial physical examination and imaging studies were suggestive of more common etiologies of thumb pain. After treatment failure, a biopsy specimen revealed calcium phosphate salt deposition in the soft tissue around the metacarpophalangeal joint, which was treated by excision of the tumoral calcinosis masses. Tumoral calcinosis can occur idiopathically in the hand and digits and should be considered when other more common pathologies of thumb pain have been ruled out.
Scrotal calcinosis (SC) was a rare and benign condition characterized by multiple calcific substances deposits occurring in scrotum and formed nodules and lumps within scrotal skin. A case of a 49-year-old male patient with a 7-year history of scrotal calcinosis was reported. Histopathological findings had not showed evidences of epithelial structures. In our case, no evidence of cystic structure was found around calcified materials. It was indicated that SC might be idiopathic.
Calcinosis; Scrotal; Case report
Idiopathic scrotal calcinosis is an uncommon benign disorder of the scrotal skin which is characterized by multiple calcified intradermal nodules. We report a 33-year old with asymptomatic multiple calcified scrotal skin nodules. He had wide excision of the lesions and direct closure of the scrotum. We review the pathogenesis and surgical treatment options for this rare disease of the scrotum.
Tumoral calcinosis is a rare disease characterised by deposition of calcified mass near the joints. The pathogenesis of this disease is not exactly defined. A disorder of calcium and inorganic phosphate metabolism may play a role. Here, we report a case of 19-year-old girl who had both cryptogenic cirrhosis and idiopathic tumoral calcinosis. To our knowledge, there is few report of such concurrence.
Scrotal calcinosis is a rare benign entity defined as the presence of multiple calcified nodules within the scrotal skin. There are controversies about the origin of this entity. In fact, it is still debatable whether scrotal calcinosis is an idiopathic growth or dystrophic calcification of dartoic muscles. It is also unclear whether scrotal calcinosis originates from inflammation of epidermal cysts affected by mild to moderate inflammation of mononuclear cells, from foreign body granuloma formation followed by resorption of cyst walls or from eccrine epithelial cysts.
We report a 41-year-old male Turkish patient presenting with a 10-year history of scrotal tumours increasing slowly in size and number. Histopathologically, there was no epithelial lining around the calcified nodules, but there was fibrosis adjacent to atrophic stratified squamous epithelium.
Results of histopathological examinations suggested that scrotal calcinosis might have been due to resorption of cyst walls. Surgery remains the key for this problem. In cases of non-massive scrotal calcinosis, like the case presented here, excision of the nodules from the affected part of the scrotal wall and repairing the defect with horizontal stitches offer good cosmetic results without relapse.
Familial tumoral calcinosis is characterized by ectopic calcifications and hyperphosphatemia due to inactivating mutations in FGF23 or UDP-N-acetyl-α-D-galactosamine:polypeptide N-acetylgalactosaminyltransferase 3 (GALNT3). Herein we report a homozygous missense mutation (H193R) in the KLOTHO (KL) gene of a 13-year-old girl who presented with severe tumoral calcinosis with dural and carotid artery calcifications. This patient exhibited defects in mineral ion homeostasis with marked hyperphosphatemia and hypercalcemia as well as elevated serum levels of parathyroid hormone and FGF23. Mapping of H193R mutation onto the crystal structure of myrosinase, a plant homolog of KL, revealed that this histidine residue was at the base of the deep catalytic cleft and mutation of this histidine to arginine should destabilize the putative glycosidase domain (KL1) of KL, thereby attenuating production of membrane-bound and secreted KL. Indeed, compared with wild-type KL, expression and secretion of H193R KL were markedly reduced in vitro, resulting in diminished ability of FGF23 to signal via its cognate FGF receptors. Taken together, our findings provide what we believe to be the first evidence that loss-of-function mutations in human KL impair FGF23 bioactivity, underscoring the essential role of KL in FGF23-mediated phosphate and vitamin D homeostasis in humans.