系统性红斑狼疮并发泛发性皮肤钙质沉着症一例

临床资料患者,女,55岁。主因面部、颈部,前胸V型区散在蝶形的淡紫红色斑片、丘疹15 d,于2014年8月21日就诊。患者确诊系统性红斑狼疮、狼疮性肾炎21年,双侧髋关节起包块15年。长期在武汉市第一医院肾内科就诊,经糖皮质激素、中药等治疗后,病情基本稳定。高血压病病史十余年,口服坎地沙坦酯片和苯磺酸左旋氨氯地平片,血压控制稳定。否认有糖尿病、冠心病和内分泌疾病史,家族     

Bullous systemic lupus erythematosus with milia and calcinosis

Bullous systemic lupus erythematosus (SLE) is a rare skin manifestation of SLE. It shares many features with epidermolysis bullosa acquisita (EBA). We report on a patient with SLE who developed a vesiculobullous eruption followed by findings not typical in bullous SLE, namely milia, mild scarring, and calcinosis. We discuss the relationship between bullous SLE and EBA.     

Dystrophic calcinosis cutis in subacute lupus

Dystrophic calcinosis cutis is known to be associated with various connective tissue disorders but to the best of our knowledge has never been reported in subacute cutaneous lupus erythematosus (SCLE), a distinctive cutaneous subset in the spectrum of lupus erythematosus. It occurs without calcium and phosphorus metabolic abnormalities and may be localized or generalized. We report a patient with SCLE who developed calcinosis cutis and had normal serum calcium and phosphorus levels and, interestingly, a normal concentration of blood ionized calcium. This latter, which represents the active form in the total amount of blood calcium, is a parameter only rarely assessed in patients with dystrophic calcinosis cutis. Thus, other pathogenic factors should be investigated to clarify the pathophysiology of the dystrophic type of calcification.     

Diffuse soft tissue calcifications (calcinosis cutis) in a patient with discoid lupus erythematosus

Soft tissue calcification is known to occur in dermatomyositis, systemic scleroderma and CREST syndrome, but rarely in systemic lupus erythematosus (SLE). Diffuse soft tissue calcifications have not been reported in discoid lupus erythematosus (DLE). In a patient with discoid lupus erythematosus, calcinosis cutis developed about 20 years after the onset of the disease. During the follow-up time of 25 years, manifestations suggestive of a systemic disease were observed in our patient. However, no specific diagnosis could be established. The clinical, light and electron microscopic as well as immunohistochemical findings of our patient are reported. On the basis of electron microscopic findings it is suggested that intracellular calcification occurred in this case.     

Calcinosis cutis universalis associated with systemic lupus erythematosus: an exuberant case

Calcinosis cutis is an uncommon disease of unclear pathophysiology that is often disabling. It is characterized by the formation of calcium deposits in the skin or subcutaneous tissue. It is classified into four subtypes: dystrophic, metastatic, idiopathic or iatrogenic. It may be seen in a variety of systemic diseases such as hyperparathyroidism and hypervitaminosis D, but is most commonly found in dermatomyositis, scleroderma and overlap syndromes and is a rare complication of systemic lupus erythematosus. The management of secondary complications and the success of therapy are constant challenges in the follow-up of these cases.     

Neonatal lupus erythematosus progressing into systemic lupus erythematosus

An infant with neonatal lupus erythematosus developed systemic lupus erythematosus at the age of 13. The clinical distinction between neonatal lupus erythematosus and familial lupus erythematosus may not be as clear cut as previously reported.     

Extensive skin calcifications in an infant with chronic renal failure: metastatic calcinosis cutis

Calcinosis cutis, one of the rare manifestations of systemic calcinosis, is characterized by the deposition of calcium and phosphate salts in the skin. Metastatic calcinosis, usually a late complication of chronic renal failure, arises from increased calcium or phosphate levels in the serum or both. Both sexes and all ages may be affected; however, cutaneous involvement is uncommon, particularly in children. We present the youngest patient, to our knowledge, with end-stage renal disease and cutaneous metastatic calcification resulting from secondary hyperparathyroidism. A 2-month-old infant presented to the pediatric service with anuria and uremia. A renal biopsy specimen showed chronic tubulo-interstitial nephritis. Indurated, firm, tender reddish papules were localized to both lower limbs, and extensive irregular nodules and plaques with ulceration and white stony contents were localized to the right upper limb. Topical antibiotic ointment was applied to the skin lesions to prevent secondary infection. However, acute peritonitis developed during peritoneal dialysis, and death occurred as a result of sepsis.     

Metastatic calcinosis cutis

Calcinosis cutis may be associated with metastatic calcification, dystrophic calcification, tumoral calcinosis, or may be deemed idiopathic. The association of cutaneous calcification with metastatic or tumoral calcinosis is quite rare. A case of metastatic calcinosis cutis in a woman with chronic renal failure and secondary hyperparathyroidism is presented. Other causes of calcinosis cutis are discussed briefly.     

Three siblings with systemic lupus erythematosus

We present the cases of three siblings with systemic lupus erythematosus (SLE). The diagnosis was made when the sisters were of age 21, 25 and 28 years. They shared some clinical features, including typical facial rash, photosensitivity and Raynaud's phenomenon, and tested positive for antinuclear antibodies. However, their symptoms and clinical courses varied. Human leukocyte antigen (HLA) typing revealed that DR4 and A2 were present in all three sisters, while HLA type A11, B35 and B54 were each found in two of the three sisters. The two elder sisters developed lupus glomerulonephritis 8 and 11 years after the onset of SLE. It is suggested that there are genes responsible for the onset of the disease and also unknown regulatory genes other than HLA result in different phenotypes.     

Bullous systemic lupus erythematosus

Bullous systemic lupus erythematosus (BSLE) is a rare bullous dermatosis in patients with systemic lupus erythematosus. It is characterized by clinical and histologic features, resembling either bullous pemphigoid or dermatitis herpetiformis, and a heterogeneous immunologic profile, characterized by autoimmunity to components of type VII collagen, much like epidermolysis bullosa acquisita. As understanding of the pathology of this interesting dermatologic condition has evolved, so too have criteria and profiling of BSLE. The distinct clinical, histologic, and immunologic features of BSLE represent a unique bullous disease phenotype.     

ANA-negative systemic lupus erythematosus

A subset of patients who often satisfy the ARA criteria for SLE but whose sera lack antinuclear antibodies when tested against various standard substrates is reviewed. Such a subset of patients forms a distinct clinical and serological group. The majority of these patients present with cutaneous lesions frequently seen in discoid or systemic lupus erythematosus. Involvement of other organ systems is less frequent in this group compared to ANA-positive SLE. The sera of these patients usually contain anti-Re and anti-La antibodies detectable by double immunodiffusion. While most patients deposit immunoglobulins in the lesional skin, direct immunofluorescence of uninvolved skin is positive only in approximately ten per cent of patients. The clinical course is marked by relapses and remissions and is favourable compared to ANA-positive SLE patients. Ten percent of SLE patients who are initially ANA-negative become ANA-positive on follow-up of approximately 4 years. Topical therapy alone may be helpful when only cutaneous manifestations are present. Systemic steroids and antimalarials are the mainstay of therapy in most cases.     

Bullous systemic lupus erythematosus

Blistering eruptions are rare cutaneous manifestations of lupus erythematosus (LE) that may be caused by different mechanisms. Subepidermal clefting with frank vesiculation may occur in early lesions of chronic-, subacute-, and acute-cutaneous LE due to a severe vacuolar alteration of the dermoepidermal junction (DEJ), dermal edema, and lekocytoclastic vasculitis. An exaggerated example of such changes is rarely seen at the advancing edge of the annular plaques of subacute cutaneous LE with erythema-multiforme (EM)-like appearance, a condition formerly described as Rowell's syndrome. In a recently reported novel variant of LE-associated toxic epidermal necrolysis, dysregulated keratinocyte apoptosis has been proposed as an underlying mechanism. These vesiculobullous lesions are considered to be LE-specific. Blistering may also occur in LE in the context of a coexisting immunobullous disease. Pemphigus, bullous pemphigoid (BP), epidermolysis bullosa acquisita (EBA), dermatitis herpetiformis, and linear IgA bullous dermatosis have been all reported in association with LE. Their differentiation relies upon characteristic clinical, histologic, and immunopathologic features (Table 1). These blistering eruptions are rather non-specific for LE.