1/36. Generalized lupus erythematosus profundus in a patient with genetic partial deficiency of C4. Lupus erythematosus profundus (LEP) is an unusual variant of cutaneous lupus erythematosus (CLE)that is characterized by chronic, recurrent inflammation of the subcutaneous tissue leading to fibrosis. It is found in the settings of both discoid and systemic lupus erythematosus. Generalized forms are extremely rare. We present a case of generalized LEP associated with genetic partial C4-deficiency. ( info) |
| We present the case of a 57-year-old woman with a 1-year history of a cutaneous eruption clinically and histopathologically characteristic of Degos disease. In one of the two cutaneous biopsy specimens taken from the trunk lesions, the histopathology consisted of necrosis and sclerosis of the subcutaneous lobules, a finding that to our knowledge has not been previously described in the cutaneous lesions of Degos disease. Two months after cutaneous biopsies were taken, the patient developed ptosis and an episode of acute abdominal pain; intestinal perforation and many characteristic lesions of visceral Degos disease involving the entire small bowel were noted at laparotomy. ( info) |
| Generalized chronic cutaneous lupus including lupus panniculitis in childhood is rare and usually occurs in the setting of genetic complement deficiencies. The association with antiphospholipid syndrome is even more rare. We report a 13-year-old girl with extensive lupus panniculitis since the age of 8 months and no evidence of complement deficiency. She recently developed antiphospholipid syndrome characterized by anticardiolipin antibodies and digital necrosis. ( info) |
4/36. Orbital infarction and melting in a patient with systemic lupus erythematosus. OBJECTIVE: To present a patient with systemic lupus erythematosus who developed infarction and melting of the orbit secondary to her systemic disease. DESIGN: A case report. PARTICIPANT: A 61-year-old white woman with a 5-year history of systemic lupus erythematosus. methods: The patient presented with left orbital pain, limitation of extraocular movements, and a fistula from the ethmoid sinus to the upper eyelid. A detailed examination with computerized tomography, ultrasound, and a comprehensive medical evaluation with laboratory testing was performed. Histopathologic analysis with special stains of the orbital tissues was also performed. RESULTS: Histopathologic examination of the biopsy specimens revealed the features of an inflammatory process involving the orbit, similar to a panniculitis. These include a lymphocytic reaction with a predominance of plasma cells, vasculitis with occlusion, and thickening of the vessel walls, necrosis, and hyalinization of fat. CONCLUSION: This is a unique case in which infarction and melting of the entire orbital structures occurred in the presence of systemic lupus erythematosus. The underlying disease process is a lupus-related panniculitis. The authors stress that this is a very rare entity and that other diseases should be ruled out before entertaining this diagnosis. ( info) |
5/36. Maintaining remission of lupus erythematosus profundus (LEP) with cyclosporin A. We report a young female patient with recurrent lupus erythematosus profundus (LEP) who has successfully maintained remission of LEP with cyclosporin A (CsA), although conventional treatments such as systemic corticosteroids (low-dose), dapsone, and other immunosuppressive drugs (azathiopurine, cyclophosphamide) could not maintain remission. ( info) |
6/36. A fatal case of pancreatic panniculitis presenting in a young patient with systemic lupus. BACKGROUND: subcutaneous fat necrosis associated with pancreatic disease is a rare event. The clinical cutaneous findings are non-specific erythematous nodules with central softening located predominantly on the lower extremities. The histopathologic features of these lesions are very characteristic and diagnostic. methods: We present an unusual case of pancreatic panniculitis associated with lupus pancreatitis in a 21-year-old African American female. The patient presented with lower extremity skin nodules, arthralgia, and serositis prior to the diagnosis of systemic lupus and pancreatitis. The skin lesions progressed despite normalization of serum pancreatic enzymes. Following femoral vein catheterization for renal dialysis, she developed a large indurated area over the left lower quadrant, flank, groin, and upper thigh measuring 25 cm. She was treated with repeated debridement, tissue grafts, and hyperbaric oxygen because of a clinical suspicion of necrotizing fasciitis. RESULTS: Examination of skin biopsies and debrided tissue revealed the pathognomonic features of pancreatic panniculitis without any evidence of necrotizing fasciitis. Organisms were not detected by tissue examination or microbiologic cultures. CONCLUSIONS: This case illustrates the potential role of vascular trauma in the pathogenesis of pancreatic panniculitis. ( info) |
7/36. Lupus erythematosus panniculitis in a patient with autoimmune hepatitis. Lupus erythematosus panniculitis (profundus), a rare variant of chronic panniculitis, sometimes develops during the course of discoid lupus erythematosus or systemic lupus erythematosus. A 61-year-old woman had suffered from autoimmune hepatitis type I for 5 years. prednisolone had been administered as maintenance therapy and her hepatitis had been well controlled. However, asymptomatic erythematous indurated nodules developed symmetrically in both pre-auricular regions, and skin biopsy revealed lupus erythematosus panniculitis (profundus). Increase in dosage of prednisolone resolved the skin lesion, leaving depressed atrophic scars. This is the first report of lupus erythematosus panniculitis complicating autoimmune hepatitis. ( info) |
8/36. Lupus profundus: not a benign disease. Lupus profundus is an unusual clinical variant of cutaneous lupus erythematosus that has previously been described as a benign disease that follows a mild course. This report describes the extent of disease and associated comorbidities in patients with severe lupus profundus and systemic lupus erythematosus. Four cases of lupus profundus are reviewed and their associated systemic disease complications are highlighted. All four patients fulfilled at least four of the 11 criteria for systemic lupus erythematosus. One patient suffered from severe facial disfigurement and Parry-Romberg syndrome. Two patients developed nonhealing ulcers on the scalp. All four patients had scarring alopecia as well as depressed areas over large areas of their body surfaces. All patients were resistant to conservative therapy, and required long-term aggressive therapy. Clinical depression secondary to disfigurement was a major problem in three patients. Extensive lupus profundus may be associated with more serious systemic disease and warrants aggressive treatment early on to prevent permanent disfigurement and its resultant psychological consequences. ( info) |
9/36. Calcifying lupus panniculitis in a patient with subacute cutaneous lupus erythematosus: response to diltiazem and chloroquine. Lupus panniculitis has been reported to occur with a frequency of 2-3% in patients with lupus erythematosus (LE). It is most often reported in association with lesions of discoid LE. We describe a patient with subacute cutaneous LE who developed calcified nodules that were histopathologically consistent with lupus panniculitis. She was treated with a combination of chloroquine and diltiazem with a good therapeutic response. The addition of diltiazem may be beneficial in patients with calcified nodules of lupus panniculitis. ( info) |
10/36. Lupus erythematosus profundus with unusual skin manifestation: subcutaneous nodules coexisting with eyelid plaques. A 71-year-old Japanese woman presented with erythematous plaques on the eyelids and subcutaneous indurations or nodules with or without overlying erythema on the hands, thigh, and leg. She also had oral ulcers, arthralgia and a low grade fever. Laboratory tests revealed an elevated titer of antinuclear antibody, an increased erythrocyte sedimentation rate and anemia. skin biopsy specimens from the hand and thigh showed lymphocytic perivascular and periappendageal infiltrates and vacuolar alterations at the basement membrane zone of the skin appendages. Moreover, there was a dense lymphocytic infiltrate deep in the dermis with extension into the subcutaneous fat, which was compatible with the diagnosis of lupus erythematosus profundus. Although the biopsy specimen from the eyelid lesion did not contain the subcutaneous fat, the changes in the dermis were essentially the same as those of the hand and thigh. The eruption as well as the other symptoms promptly responded to oral prednisolone. ( info) |