A 34 y.o. comes in with knee pain
What is wrong?
Hint : she cannot open her mouth.
Our patient had systemic scleroderma. She presented with calcinosis cutis which is one of the abnormalities found in scleroderma. Scleroderma is a connective tissue disorder characterized by hardening of the skin. Sclero comes from the Greek for “stone” which describes the process of thickening of the skin that occurs in scleroderma.
tightening of the skin in scleroderma can result in difficulty opening the mouth
PARTS OF THE BODY AFFECTED BY SCLERODERMA
Vascular system-constricts the veins and arteries causing Raynaud’s and pulmonary HT
Mouth-tightening of the skin of the mouth
Hands-digital ulcers and contractures of the fingers occur
sclerodactyly
Musculoskeletal/derm-calcinosis can occur anywhere in the body
Heart and lungs-pulmonary fibrosis, overproduction of collagen in the heart causes conduction abnormalities
Kidneys-renal failure and ht
Digestive system-excessive collagen can affect the GI tract from the mouth to the anus; cause difficulty swallowing or constipation
Eyes-Sjogrens syndrome
The disease is 13 times higher in first-degree relatives of scleroderma patients than in the general population. Women are 5 times more likely than to be affected than men. Scleroderma may also have an environmental component. Silica has been implicated in some cases.
The most common calcinosis we see in the ED is calciphylaxis which is associated with an abnormal calcium/phosphorus product( and decreased circulating pyrophosphate levels) , often in renal failure. It can also occur in Paget’s, hyperparathyroidism, paraneoplastic hypercalcemia, sarcoidosis or excessive Vit D intake.
calciphylaxis
Our pt was in the ED for constipation having been given narcotics for pain after removal of calcinosis from her knee. She was given methylnaltrexone and an enema with a successful result.
Odonwodo A, Badri T, Heriz A. Scleroderma(Systemic Sclerosis) StatPearls(Internet)
Careta MF, Romiti R. Localized scleroderma. Clinical spectrum and therapeutic update. An Bras Dermatol. 2015 Jan-Reb;90(1):62-73.
Schoenfeld S, Castelino F. Interstitial lung disease in scleroderma. Rheum Dis. Clin. North Am. 2015 May;41(2):237-48.
O’Neill W. The fallacy of the calcium-phosphorus product, 2007 Kidney International ;72(7) Kidney International:792-796
calcinosis can occur in other autoimmune diseases as well