Calcinosis and rheumatic disease 4.25/5 (4)

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Definition

Calcinosis in the skin is characterized by deposits of calcium salts in the skin, muscles and other organs. The lime can feel like rock-hard lumps or be more liquid. Calculus under the skin can be a sign of serious underlying disease such as dermatomyositis in children, less often in adults or systemic sclerosis (limited form) or a harmless phenomenon.

Disease Causes

Calcinosis is seen associated with a number of, but also without any such condition being detectable (idiopathic):

Lime under the skin (calcineosis) by dermatomyositis in a child. Doh EJ, Ann DermatolCC BY NC. 4.0
  • Dermatomyositis
  • Familial Tumoral Calcinosis (FTC)
  • Hypercalcemia (High blood level)
    • PTH (parathormone) and ionized calcium are measured in blood
  • Hyperphosphatemia
    • High phosphate levels and often low calcium in blood
  • Idiopathic calcinosis has an unknown cause, no previous injuries or current illness. 
  • Calciphylaxis is a serious condition of unclear cause. Vascular calcification and necrosis in the skin. Risk factors are chronic kidney failure and dialysis. 
  • Cutaneous calcinosis
    • Most often after injuries
  • Metastatic calcinosis not necessarily due to cancer. The most common cause is chronic kidney failure. Metabolic calcinosis is also seen in hyperparathyroidism, vitamin D overdose (rare), Sarcoidosis, milk-alkali syndrome (excessive intake of food or antacids containing calcium) and cancer.
  • Iatrogenic causes (related to other treatment) are calcium- or phosphate-containing infusions, venipunctures. Also tumor lysis syndrome and calcinosis cutis after organ transplantation
  • Idiopathic calcinosis has an unknown cause, no previous injuries or current illness. Laboratory tests show normal calcium and phosphate. Idiopathic calcinosis can be divided into three types: 1. Familial calcinosis: healthy, young patients with increased phosphorus uptake in proximal renal tubules. Calcium deposits around large joints, under the skin and in muscles. 2. Calcareous nodules under the skin (Subepidermal calcified nodules, Winder nodular calcinosis) debuts in children, sometimes from birth. Localization is on the head and extremities as limited, hard, white-yellow papules. 3. Scrotal calcinosis have calcareous nodules or larger accumulations on the scrotum in men
  • Osteodystrophy (Albright Syndrome)
    • Lack of response to parathymone, growth disorders
  • Systemic sclerosis
    • Limited form is associated with calcium under the skin

Symptoms

Calcareous changes are rock hard or liquid with a toothpaste consistency. They can develop gradually from being painless to being very painful. The knots can vary in shape and size and can lead to reduced physical function. Perforation of the skin can occur spontaneously, self-inflicted or by healthcare personnel, but carries a risk of infection. The distribution and localization of the lesions may indicate something about the underlying disease: Ved Systemic sclerosis attacked skin in forearms, elbows, fingers and knees.-Ved dermatomyositis (most often children) calcinosis is seen at the elbows and knees and in areas that have been inflammatoryly attacked by the underlying disease. Lupus (SLE) can cause calcium deposits in the extremities, gluteal, under lupus lesions and around joints. Calcification around joints is also seen in metastatic cancer. The face can be attacked in children with calcification nodules due to calcification of unknown cause. Calcification at sites of previous venipunctures is seen in iatrogenic calcinosis.

Examinations

Medical history ask for symptoms of systemic sclerosis, dermatomyositis and other related conditions (see above).

Clinical a thorough assessment is made to uncover findings compatible with the underlying condition.

Laboratory. Cell counts (Hgb, leukocytes with differential counts, platelets), liver, kidney, thyroid function tests, electrolytes with ionized calcium and phosphate, PTH (parathyroid hormone), vitamin D, calcium, phosphate, albumin/protein, CK, LD and ACE (sarcoidosis). Urine sting. Immunological tests can include ANA and a-DNA and in case of positive ANA, ENA and subgroups including myositis specific antibody are done. In milk-alkali syndrome, bicarbonate and arterial PH are determined.

Imaging. Conventional X-ray will clearly show the extent of calcinosis. CT and ultrasound examinations can also be useful. Calcinosis is not shown very well on MRI (Reiter N, 2016).

Similar conditions / differential diagnoses

Similar conditions do not cause lumps as hard as calcinosis; Infection in the skin (cutaneous mycetoma /bacterium actinomycetoma or fungus (eumycetoma). Genital warts: human papillomavirus (HPV). Milia (keratin-filled small point-shaped cysts on the eyelids or in the mouth). molluscum contagiosum (molluscum contagiosum virus, MCV). Calcifications (osteoma cutis) after injuries are new formations of bone structure in the skin. Xanthomas (cholesterol-containing nodules, often hypercholesterolemia)

Treatment

Effective treatment of calcinosis is a challenge. A number of non-pharmacological and medicinal measures are used, but the effects are not very well documented. The choice of treatment depends on the underlying cause (reference; Le C, 2022).

Literature

Bindevevssykdommer.no

Revmakompendium.no


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