Case Report. 15th June Carolina Ourique Luciana Frade Daniela Alves

Transcription

1 Case Report 15th June 2016 Carolina Ourique Luciana Frade Daniela Alves

2 CASE REPORT Woman 23 year-old Single Profission: Social-cultural animator for children

3 CASE REPORT PERSONAL MEDICAL HISTORY Smoker (8 cigarretes/day, for 4 years) Menarche: 13 years. Oral contraceptive (progesterone and estrogen). Nulliparous. No other chronic medication

4 CASE REPORT FAMILIAR MEDICAL HISTORY Irrelevant No autoimune disease, thrombosis events, neoplasic history.

5 CASE REPORT 10 th NOVEMBER 12 th NOVEMBER Sudden onset of milimetric dark spot in the tip of the nose purplish color Slightly painful ER 2 days of progressive increase in the lesion area, with local paresthesia, pain and cold. Non hemorrhagic or ulcerated. Without exudate. No fever. Without other symptoms.

6 CASE REPORT MEDICAL EXAMINATION IN ER: Hemodynamic stability. No fever. Anterior Rhinoscopy: Poor perfusion lesion extending to bilateral anterior nasal vestibule and septum. REMAINING CLINICAL EXAMINATION UNREMARKABLE: No other poor perfusion areas. No livedo reticularis, other cutaneous lesions or arthritis signs. No visible cartilage deformity. No ocular abnormalities NASAL BIOPSY Normal cardiovascular, respiratory and neurological examination. No palpable lymphadenopathy, or hepatosplenomegaly.

7 CASE REPORT Analysis in ER RESULT REFERENCE VALUE/ UNIT Hemoglobin g/dl Hematocrit % Mean Corpuscular Volume fl Mean Cell Hb concentration g/dl Leukocytes x10 9 /L Neutrophils % Eosinophils % Basophils % Lymphocytes % Monocytes % Platelets x10 9 /L

8 CASE REPORT RESULT REFERENCE VALUE/ UNIT Urea mg/dl Creatinine mg/dl AST Aspartate Aminotransferase ALT Alanine Aminotransferase CK Creatinina kinase U/L U/L U/L Myoglobin 12.4 <146.9 ng/ml CRP C-Reactive Protein <3 mg/l Glucose mg/dl aptt sec PT sec Fibrinogen mg/dl Urinary sediment analysis Normal

9 Question 1. Which are the main diagnostic hypothesis?

10 Question 1. A) Trauma, infection or cocaine abuse. B) Vasculitis. C) Relapsing Polychondritis. D) Thinking nothing by now.

11 CASE REPORT No history of Nose manipulation/ trauma. Cocaine abuse, cosmetic products/ plastic surgery. Recent travels, tuberculosis contact, risk for sexual transmited diseases. Other medication (besides OAC). Autoimmune disease, asthma, rhinosinusitis. Constitutional symptoms.

12 CASE REPORT 13 th November ADMISSION Day 1

13 Question 2. Which therapy would you start?

14 Question 2. A) Sistemic steroids. B) Low molecular weight heparin. C) Empiric antimicrobial therapy. D) A and B options. E) Wait and see.

15 CASE REPORT Enoxaparin 40 mg SC Methylprednisolone 1 g EV ADMISSION IN INTERNAL MEDICINE DEPARTMENT Nasal Necrosis

16 Question 3. Which diagnostic studies would you demand first?

17 Question 3. A) Lung and upper airway imaging study. B) Viral markers (HCV, HBV and HIV) and syphilis test. C) Autoimmune study, serum complement levels and prothrombotic study. D) All options above.

18 CASE REPORT RESULT HIV (1 & 2) HCV antibody HBs antigen, HBc antibody TPPA/ TP CMV, EBV, Parvovirus B19 Negative Negative Negative Negative Negative for acute infection

19 CASE REPORT CERVICAL AND THORACIC CT - No changes in nasopharynx, oropharynx or larynx. - No changes in submandibular, parotid and thyroid glands. - Homogeneous pulmonary parenchyma, without signs of interstitium disease. -No pleural or pericardial effusion. -No cervical, axilary, hilar and mediastinal lymphadenopathy.

20 CASE REPORT AUTOIMMUNE STUDY Antinuclear antibody Anti-DsDNA antibody ANCA (PR3, MPO) Antineutrophil cytoplasmic antibodies RESULT Rheumatoid Factor 594 (<30 UL/mL) COMPLEMENT RESULT C4 7 (12-36 mg/dl) CH UA Anti-ENA (anti-jo 1, anti-rnp, anti-scl70, anti-sm, anti-ssa, anti-ssb) Anticardiolipin antibodies Antibodies to Beta-2-GP-1 Lupus Anticoagulant Serum crioglobulins RESULT PROTHROMBOTIC STUDY RESULT C, S proteins Normal Factor V mutation Prothrombine mutation gene AT-III Antithrombin III Negative Normal Homozigotic Normal

21 CASE REPORT ESR Erythrocyte Sedimentation Rate CRP C-Reactive Protein RESULT REFERENCE VALUE/ UNIT mm/1 st h <3 mg/l

22 CASE REPORT NEGATIVE HISTOCHEMICAL STUDY FOR MICROORGANISMS SEARCH IN BIOPSY.

23 CLINICAL PROGRESSION TIME ADMISSION THERAPY DAY 2 3 rd Pulse MethylPDN + LMWH full dose DAY 3 PDN 50 mg + LMWH full dose

24 Question 4. Would you keep previous therapy (sistemic steroids and LMWH in full dose)?

25 Question 4. A) Yes, keep therapy. B) No, start immediatly agressive immunosuppression with cyclophosphamide/ rituximab. C) Stop steroids and begin dapsone. D) Still have no idea.

26 Vessels filled with homogeneous, eosinophilic material. No evidence of vasculitis.

27 epidermis Fibrin material in the lumen of a small vein Scattered perivascular infiltrate Vessel affected in the papillary dermis

28 CASE REPORT AUTOIMMUNE STUDY Antibuclear antibody RESULT Negative Anti-DsDNA antibody <10 ANCA (PR3, MPO) Antineutrophil cytoplasmic antibodies <20 Rheumatoid Factor 594 (<30 UL/mL) Anti-ENA (anti-jo 1, anti-rnp, anti-scl70, anti-sm, anti-ssa, anti- SSb) Anticardiolipin antibodies Negative Negative Antibodies to Beta-2-GP-1 Lupus Anticoagulant Negative Positive Serum crioglobulins RESULT Positive

29 LESS LIKELY DIAGNOSIS TRAUMA COCAIN ABUSE COSMETIC PRODUCTS INFECTION -No immunossupression. -No travel history. No risk contact for tuberculosis/ sífilis. -No microorganisms were seen in biopsy. - HIV, HCV and HBV were excluded. VASCULITIS - ANCA negative. - No pulmonar, upper airway or renal involvement. -No eosinophilia. LYMPHOPROLIFERATIVE DISORDERS -Absence lymphadenophathy, hemogram abnormalities, hepatosplenomegaly. - Absence of fever, important constitutional symptoms, low LDH. RELAPSING POLYCHONDRITIS - No involvement of other cartilaginous areas. -No ocular inflammation or vestibulo/cochlear dysfuntion. -No eosinophilia or ANCA positive. -CH50 and C4 are usually normal.

30 Question 5. What is the final diagnosis?

31 Question 5. A) Cryoglobulinemia. B) Antiphospholipid syndrome. C) Both.

32 Day 6 Day 8 Day 9 Day 10 Day 13 Day 16

33 Question 6. What should be the treatment after discharge?

34 Question 6. A) Sistemic steroids only. B) Anticoagulants only. C) Rituximab. D) A and B options.

35 1 YEAR HAS PASSED Assintomatic Tapering oral steroids- progressive reduction of PDN to 10 mg/day. Anticoagulation NOVEMBER JANUARY MARCH APRIL MAY JULY Rheumatoid Factor (UL/mL) apl / AL negative

36 PORTO LISBON CASTELO BRANCO AZORES Thank you for listening!