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Cases journal 2008-Aug

Rapidly evolving purpuric lesions to massive hemorrhagic bullae, with rapid improvement by Prednisolone: as a coetaneous manifestation of Systemic Lupus Erythematosus: a case report.

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Farzin Khorvash
Alireza Emami Naeini
Mohaddeseh Behjati
Mansoor Karimifar
Fariborz Khorvash
Koorosh Dialami

Nøgleord

Abstrakt

BACKGROUND

Systemic lupus Erythematosus is a chronic autodestructive disease, with loss of immune tolerance to nucleic acid and other cross reactive antigens. Despite of the numerous studies, the presence of some new manifestations indicates the greater proportion of unknown data.

METHODS

Our case, is a 26-year-old female, by the chief complaint of headache, vomiting, fever and arthralgia. Some hemorrhagic ulcers in her mouth with fulminant pethechia/purpura on her limbs and buttocks were prominent. On admission, she was in hypotensive state. By the clinical suspicion to meningococcal septicemia, lumbar puncture was performed, and antibiotic therapy was started. Cerebrospinal fluid was normal. Suddenly, on the 3rd day of admission, hemorrhagic bullae were evolved from those purpuric lesions. Leukocytosis, immune hemolytic anemia, thrombocytopenia and high antinuclear antibody/double stranded DNA level and hypocomplemania were present simultaneously. In skin biopsy, immune complex deposition in dermoepidermal junction was seen. The diagnosis of Systemic lupus Erythematosus was made. The patient responded well to corticosteroid therapy.

CONCLUSIONS

Coetaneous manifestations are very common in Systemic lupus Erythematosus, and help the physician making differential diagnoses and proper diagnosis. The rapidly evolving hemorrhagic bulla from primary purpuric lesions, with rapid response to Prednisolone, is a rare manifestation of Systemic lupus Erythematosus, which should be considered in such a disease setting.

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