Calcinosis cutis universalis with pediatric systemic lupus erythematosus

Abstract

Sirs, Calcinosis cutis (CC) is characterized by the deposition of hydroxyapatite crystals of calcium phosphate in the skin. CC is a common feature of scleroderma (SD), CREST syndrome (calcinosis, Raynoud's phenomenon, esophageal dysfunction, sclerodactyly and telangiectasia), dermatomyositis (DM), systemic lupus erythematosus (SLE), overlap syndromes and malignancies [1,2]. In SLE, mainly the dystrophic form, CC has been described, but it has been rarely reported in children with SLE. Here, we report on a patient who was diagnosed SLE and antiphospholipid syndrome at 3 years of age, and his renal biopsy showed diffuse proliferative glomerulonephritis. He developed extensive areas of skin ulcers and calcifications at 5 years of age, and the lesions resolved with diltiazem and acetylsalicylic acid treatment.A 9-year-old boy presented with symptoms of weakness, abnormal gait, pitting edema, generalized arthralgia, malar rash and dark urine in November 2003. Laboratory investigation revealed antinuclear antibody (ANA) 1/100 (+), anti-nuclear (n)DNA antibody 2 +, anti-doublestranded (ds)DNA antibodies 2 +, anti-Ro and anti-La antibodies, positive, complement C3 101 mg/dl (normal 90-140 mg/dl), complement C4 5.7 mg/dl (normal 10-40 mg/dl), anti-cardiolipin antibody immunoglobulin (Ig)M (+), IgG (−), hematuria (++) and a 24-h protein concentration of 2 g/l. The patient had SLE and antiphospholipid syndrome together, and his renal biopsy showed class IV lupus nephritis. He was treated with five doses of pulse methylprednisone and follow-up oral prednisolone at a dose of 2 mg/kg per day. At the follow-up examination, 1 year after the diagnosis, the patient was readmitted with a malar rash and proteinuria. Azathiopurine (2 mg/kg per day) was added to his steroid treatment. However, during follow-up, the patient did not respond to this therapy and extensive leg ulcers developed. He underwent seven treatments of plasmapheresis and received eight doses of pulse cyclophosphamide in 2004. Five months later, he presented with ulcers on the face and extensor side of his extremities and was hospitalized. His test results for anti-cardiolipin antibody IgM were positive. His therapy was changed to enoxaparin sodium (2 mg/kg per dose) and oral steroids (60 mg/day), and cyclosporine was started at a dose of 5 mg/kg per day. As his lesions resolved, the steroid dose was decreased at the follow-up examination. One year later, the patient presented with a limited range of motion and subcutaneous nodules with a maximum diameter of 1 cm× 0.5 cm in the back and extensor part of the extremities. His plain radiographs revealed multiple calcifications in soft tissues ( Fig. 1) and multiple calcifications with acoustic shadows that were found superficially by ultrasonography. No other calcinosis was determined in his abdominal ultrasonography and computed tomography images. His laboratory studies showed blood urea 20 mg/dl, creatinine 0.7 mg/dl, sodium 132 mEq/l, potassium 3.8 mEq/l, calcium 9.2 mg/dl, phosphorus 4.8 mg/dl, parathormone 42 pg/m...

Mucocutaneous manifestations in juvenile-onset systemic lupus erythematosus: a review of literature

“…Other LE nonspecific lesions Other lesions include: calcinosis cutis, acanthosis nigricans, hypocomplementaemic urticarial vasculitis (very rare in JSLE patients) [62–64], whilst rheumatoid nodules, anetoderma and erythromelalgia have been reported in adult SLE [65–67]. …”

El Parmaklarına Lokalize İdyopatik Kalsinozis Kutis

“…İdyopatik kalsinozis kutisin etkili bir tedavisi bulunmamaktadır. Tedavi olarak bifosfanat, etidronat disodyum, aluminyum hidroksit, kolşisin, warfarin, probenesid, intralezyonel steroid enjeksiyonu, steroid, minosiklin, salisilat ve diltiazem kullanılabilir, en son olarak cerrahi eksizyon ve laser terapisi seçenekler arasında vardır [4][5][6][7][8][9][10][11][12] . İlaç kullanmak istemeyen olgumuz kendisine önerilen eksizyon tedavisini birkaç ay sonra yaptırmak üzere taburcu edildi.…”

Clinical variants of skin and mucous membrane lesions in systemic lupus erythematosus with juvenile onset

“…Другие неспецифические виды поражения кожи, такие как кальциноз кутис, черный акантоз, описаны в единичных случаях, буллезные высыпания на коже и слизистой оболочке представлены в серии наблюдений (их частота составляла 0,35%) [73][74][75].…”