This is a 28-year-old female patient, with no history of previous diseases, with clinical picture characterized by complete infectious toxic syndrome and pain in the mouth due to polyuria with mild mucormycosis remission, progressive urinary expectulence, with dementia.
On physical examination: bad general conditions, P.A. 110/60 mmHg, F.C. 100 beats/minute, F.R. 24 / minute, with dry skin and mucous membranes pale.
In the skull, parietal region with areas of alopecia, central-facial erythema of greater intensity is observed in cheeks, lungs with incomplete right subscapular condensation and contralateral replacement.
Abdomen blando, painful right hypochondrium and flank, Blumberg positive.
Genitals with white-yellow genital discharge.
In the upper limbs livedo reticularis was observed on the dorsum of the hands, as well as distal bleeding of the fingers of both hands, with decreased local temperature and "mouse bite" lesions on the third hand 1 hand.
Lower limb edema, petechiae on the back of both feet, confluent.
Aware, oriented, with no data on meningeal irritation or neurological focalization.
Laboratory: hemoglobin 10.3 g/dl, hematocrit 31%, VES 105 mm. leukocytes 10,000 mm3, segmented 8300/mm3, lymphocytes 1500/mm3, monocytes 100/mm3, platelets 200,000 mm3, serum nitrogen mmol/ml 159
Urine output: proteins ++, blood ++, leukocytes 50 to 60, pyocytes 40 to 50, presence of biliary strictures and granuloses.
CRP 2.6 mg/dl (< 0.8), rheumatoid factor (+) 1.3 IU/ml (minor 8), ASTO 50 (< 200), serology for hepatitis B, C, HIV negative, TSH 5.54 uU/ml (0.63-4,
Hemoculture, coprocultive and uroculture negative.
24-h urinary protein excretion: 899 mg. Complement 3, 12 mg/dl (70-170) and complement 4, 0 mg/dl (20 patients).
Diffuse and peripheral ANA pattern (+) 1:320 (< 1/40) and anti-dsDNA (+) 64 IU/ml (< 27) core extractable antigen profile (ENA) with positive JO-1 S doubt 0.92.
Antiphospholipid antibodies: IgM anticardiolipin 24 MPL/ml (< 12 ) and IgG 21 GPL/ml (< 13).
Viral serology: positive ELISA IgG toxoplasma 2.1 (> 1.1), positive ELISA IgG cytomegalovirus 4.4 (> 1.1) and positive ELISA IgM 2.8 (> 1.1).
Data on deep venous thrombosis in the left lower limb verified by venous Doppler, severe pericardium effusion corroborated by chest X-ray, electrocardiogram Doppler echocardiography, and both hands due to distal echocardiography due to their internment are added.
Pulses were performed with methylprednisolone 500 mg for 3 consecutive days, continued with prednisone at a dose of 1 mg/kg/day and azathioprine 100 mg/day with heparin and warfarin.
The outcome was unfavourable, with digital necrosis (dry sclerosis), associated signs of lupus nephropathy and cerebral ischemia (left hemiparesis), documented with brain CT and EEG.
She was treated with 3 weekly sessions of plasmapheresis for two weeks, pulses with 1 g of weekly cyclophosphamide for 3 consecutive weeks and immunoglobulin 20 g per day (400 mg/kg/day) with favorable anti-hypertensive evolution.
She was discharged after 6 weeks of hospitalization with neurological sequelae and complete necrosis of the 1st and 2nd distal phalanges of the fingers mentioned in both hands, which were naturally amputated 9 months after admission.
He is currently under regular medical follow-up and treatment with mycophenolate mofetil 1 g/day with favorable outcome.
