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.
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
Calcinosis cutis is an uncommon disorder caused by an abnormal deposit of calcium phosphate in the skin in various parts of the body. Four main types of calcinosis cutis have been recognized according to etiology: associated with localized or widespread tissue changes or damage (dystrophic calcification), that associated with an abnormal calcium and phosphorus metabolism (metastatic calcification), not associated with any tissue damage or demonstrable metabolic disorder (idiopathic calcification), and Iatrogenic. Very few cases of idiopathic calcinosis cutis are reported in early childhood in the literature. We report one such case of idiopathic calcinosis cutis over elbow in a 12-year-old female child. Histological examinations of the lesions resected in this case reveal calcium deposits in the dermis, surrounded by foreign body giant cells. Idiopathic calcinosis cutis is a rare phenomenon and occurs in the absence of known tissue injury or systemic metabolic defect. It is important to delineate it from other calcification disorders for further plan of management. Medical therapy in calcinosis cutis is of limited benefit in pediatric age group and poses a challenging problem of postsurgical management.
Idiopathic calcinosis cutis is a condition involving the deposition of calcium salts in the skin and subcutaneous tissue. The disease is a pathological condition of unknown origin and hence is idiopathic. The salt deposition is confined to areas such as the breast and vulva in females and scrotum and penis in males. Diffuse calcification with multiple complications in an adult is a rare entity. Only one such case has been reported in literature. A 59-year-old female presented to us with swelling of the right elbow, multiple calcific nodular lesions all over her fingers approximately 0.5x0.5 cm in size, and ulcers on her left great toe and right thumb with pain for the past two months. The ulcers were 2x2 cm and were observed to be healing without active discharge or signs of inflammation. The elbow was diffusely swollen and tender. Flexion deformity was present at the elbow. X-ray of hand and feet revealed calcinosis of the elbow and interphalangeal joints of the foot and hand. Blood tests revealed elevated C-reactive protein levels of 24 mg/dL, elevated Erythrocyte Sedimentation Rate (ESR) of 52 mm/hr., serum calcium of 9.7 mg/dL and a serum phosphorous of 5 mg/dL. Cultures from the foot ulcer were positive for methicillin-resistant staphylococcus aureus (MRSA). Workup for collagen vascular disease was negative. Histopathology confirmed calcinosis cutis. Treatment involved a conservative approach, including physiotherapy for the flexion deformity, antibiotics for MRSA, analgesics for pain relief and daily dressings. This case demonstrates that if a patient presents with multiple chalky nodular lesions with or without ulceration, pain and discharge involving areas of the upper limb or lower limb, diagnosis of idiopathic calcinosis cutis could be considered as a differential, despite its common confinement to the scrotum, breast, vulva and penis.
Calcinosis cutis; idiopathic; diffuse; adult
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 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
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
In the present study, calcinosis cutis (CC) is defined as the deposition of amorphous calcium and phosphate salts under epidermis and it may be caused by a pre-existing event such as extravasation injury or hypercalcemic conditions. Idiopathic CC cases have no underlying disease or pre-existing cause.
A demostrative vulvar idiopathic CC case presentation and review of the related literature.
A 42-year-old multiparous female presented with vulvar nodular masses. She was keen on surgical removal of the lesions, as the masses caused dyscomfort during sexual intercourse. The lesions were removed and sent for histopathological examination. There was neither a history of trauma nor any inflammatory process in the vulvar skin prior to the development of lesions and no systemic abnormality was detected.
Results and Conclusions:
The histhopathologic evaluation of the biopsy specimen showed amorphous calcium deposits without any inflammatory infiltration in the dermis. There was no recurrence at 1 year's follow-up. This case shows that idiopathic CC may develop slowly at labio-vulvar region in a sexually active female with normal systemic or laboratory findings
Idiopathic calcinosis cutis; vulva; subepidermal nodules
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
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
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
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.
A 61-year-old female received intravenous injection of calcium chloride after common iliac artery bypass surgery. A red flare appeared at the site of the intravenous infusion on the left forearm and gradually progressed to induration. Seven weeks later, she was referred to the Department of Dermatology for management. Physical examination showed an indurated plaque measuring 13 × 65 mm in size, with linearly distributed ulcers covered by yellowish-white substance, surrounded by reddish skin. Laboratory tests showed no significant abnormalities including serum calcium, phosphate and thyroid hormones. Cultures were negative for microorganisms. Histopathological examination showed calcium deposition confined to the dermis. The lesion healed spontaneously within 2 months with scar formation. A review of the Japanese literature showed confinement of calcium deposits to the dermis in most of the reported cases. We speculate that the pathomechanism of dermal calcinosis includes needle-induced tissue injury with capillary destruction, leading to release of excess calcium between collagen fibers, and its binding to phosphate in the dermis and deposition as calcium phosphate crystals.
Iatrogenic calcinosis; Calcium chloride
Tumoral calcinosis is a disorder of phosphate metabolism characterized by ectopic calcification around major joints. Surgery is the current treatment of choice, but a suboptimal choice in recurrent and multicentric lesions.
To evaluate the efficacy of bisphosphonates for the management of tumoral calcinosis on optimized medical treatment.
Settings and Design:
The study was done in the endocrine department of a tertiary care hospital in South India. We prospectively studied two patients with recurrent tumoral calcinosis who had failed therapy with phosphate lowering measures.
Materials and Methods:
After informed consent, we treated both patients with standard age adjusted doses of bisphosphonates for 18 months. The response was assessed by X ray and whole body 99mTc-methylene diphosphonate bone scan at the beginning of therapy and at the end of 1 year. We also estimated serum phosphate levels and urinary phosphate to document serial changes.
Two patients (aged 19 and 5 years) with recurrent idiopathic hyperphosphatemic tumoral calcinosis, following surgery were studied. Both patients had failed therapy with conventional medical management − low phosphate diet and phosphate binders. They had restriction of joint mobility. Both were given standard doses of oral alendronate and parenteral pamidronate respectively for more than a year, along with phosphate lowering measures. At the end of 1 year, one of the patients had more than 95% and 90% reduction in the size of the lesions in right and left shoulder joints respectively with total improvement in range of motion. In contrast, the other patient (5-year-old) had shown no improvement, despite continuing to maintain normophosphatemia following treatment.
Bisphosphonate therapy in tumoral calcinosis is associated with lesion resolution and may be used as a viable alternative to surgery, especially in cases with multicentric recurrence or treatment failure to other drugs.
Bisphosphonates; fibroblast growth factor 23; hyperphosphatemia; tumoral calcinosis
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.
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.
Objective: Tumoral calcinosis is an uncommon lesion, composed of ectopic calcified tissue, most commonly seen in the large joints of the hips, knees, shoulders, and elbows. The involvement of the hand in a healthy patient is extremely rare, and therefore this condition can cause diagnostic confusion. The purpose of this report is to describe one case of idiopathic tumoral calcinosis that occurred in the left hand of a 35-year-old healthy female patient. Methods: The patient presented with 2-day history of acutely swelling and painful left hand middle finger metacarpal phalangeal joint without any precipitants. Results: All biochemical, radiological, and histopathological evidence suggested idiopathic tumoral calcinosis of the hand. Conclusions: In this case, surgery provided the patient with instant symptomatic relief and full functional recovery of that joint.
hand; tumoral calcinosis; surgery; idiopathic; calciphylaxis
Cutis marmorata telangiectatica congenita (CMTC) is a rare congenital disorder with persistent cutis marmorata, telengiectasia, and phlebectesia, which may be associated with cutaneaus atrophy and ulceration of the involved skin. We herewith report a full-term newborn female baby with CMTC at birth with ulceration over the extensor aspects of both the knee joints and right elbow joint. CMTC is a benign vascular anomaly representing dilatation of capillaries and veins of dermis and is apparent at birth. The baby had a reticulated bluish purple skin changes all over the body including the face and limb. Although it resembled physiological cutis marmorata, it was strikingly pronounced and definitely was unvarying and permanent. A variety of vascular malformation has been described along with this disorder. Etiology is not very clear and may be multifactorial, teratogens and genes are also been suggested. Prognoses in uncomplicated cases are good.
Cutis marmorata telangiectatica congenita; congenital vascular skin disorder; skin lesion improves
Aplasia cutis congenita (ACC) is a rare condition characterized by a localized absence of skin and in some cases, the subcutaneous tissues. The majority of cases occur in the scalp; however, the lesion may occur anywhere in the trunk and extremities. ACC is most often an isolated defect, but it can be associated with other anomalies. Most reported cases are sporadic with a few reports of familial occurrence. Neither the pathogenesis nor the aetiology is clarified. Healing is spontaneous in most cases, and apart from keeping the lesion clean, no specific treatment is required. In this report, two cases of non-scalp ACC occurring in the lower limbs are presented and a brief review of the literature is conducted.
Aplasia cutis congenita; congenital skin defect; neonatal scalp defects
Background. Cutis laxa (CL) is a rare disorder of elastic tissue characterized by loose, sagging skin with reduced elasticity, and resilience without resulting scarring. CL may be inherited as a dominant, recessive, or X-linked recessive disease, or acquired. The heritable forms of CL predominantly begin at birth, but it may be delayed until puberty or age of 30 years with extracutaneous manifestations including pulmonary emphysema, umbilical and inguinal hernias, and gastrointestinal and vesicourinary tract diverticuli. An acquired form of the disease occurs in adults with no evidence of internal organ involvement.
Objective. The aim of this case report was to present our patient suffering from CL, and to evaluate clinical presentation, diagnostic and therapeutic difficulties in this rare condition.
Case Report. A 30-year-old female patient was admitted to our Hospital due to localized loose and sagging skin of abdomen, induced by prior cesarean section 6 years ago. CL has been diagnosed based on the clinical picture and pathohistological appearance. Conclusion. Reconstructive surgery provides a dramatic cosmetic improvement with significant psychosocial benefit. Repeated surgical procedures may be required to correct the lax skin, which worsens with age.
Cutis verticis gyrata (CVG) is a descriptive term for a scalp condition that is convoluted folds and deep furrows that resemble the surface of the cerebral cortex. It is categorized by the underlying etiology, as primary essential, primary non-essential and secondary. Alopecia areata (AA) is a common, organ specific autoimmune disease, and most AA cases are sporadic. There is clearly a strong genetic component. There is no established relationship between CVG and AA. We report one case which was affected with essential primary CVG and alopecia areata, and suggest a possibility of genetic association between CVG and AA, possibly both being related to mutations in the fibroblast growth factor receptor 2 (FGFR2).
Alopecia areata; cutis verticis gyrata; fibroblast growth factor receptor 2 (FGFR2)