Case report

1. Case report Prepared by Dr/ Ahmed fahmy Assistant lecturer Rheumatology , Physical medicine and Rehabilitation department Al-Azhar university 2011

2. Complaint • 15 years old femalepatient, complaining of 2ry difficulty in ascending stairs and inability to standing from sitting position for tow months duration

3. History • The patient was quiet well tell the age of 14 (one year ago) • The condition started by attack of fever for 3 days associated with dull aching pain in both lower limbs of acute onset progressive course not controlled by NSAID • The fever increase at night and decrease at day time • Antibiotic (broad spectrum) and anti pyretic was given empirically and symptoms improved

4. History • After few days the mother sought medical advise and the condition diagnosed as acute rheumatic fever and take long acting penicillin • In the next few months repeated attacks of acute infection (otitis externa and muco purulent conjunctivitis) • Recurrent painful oral ulcers was noticed and diagnosed as fungal infection

5. History • At the same time the patient start to notice falling of scalp hair in excessive manner up to appearance of scalp skin with changes in hair texture • Patient visit dermatologist and local and topical anti biotic was prescribed also topical steroid was used but the condition continue to progress

6. History • Two months ago the patient suffer from sudden attack of shortening of breath with bluish discoloration of fingers and both hands • Maculopapular rash was develop on arms and back • Patient admitted in pediatric department and start high dose steroid 60 mg /day on divided doses

7. History • The condition improved with high dose steroid but shortly after 3 weeks patient start to complaining of proximal muscle weakness in form of difficulty in ascending stairs and inability to standing from sitting position separately

8. History • Also skin pigmentation was notice in both hands in form of mild hyper pigmentation over knuckles and mcps • After this patient referred to rheumatology department for consultation

9. General Examination • Normal vital signs. Bl p 110/80 pulse 100/m temp 37c • Head and neck : • Scalp and hair : area of secatrecial alopecia with coarse hair texture dermatological consultation toxic alopecia may be due to discoid lesion of lupus

10. General Examination • Purple discoloration of upper eye led • Acne • no lymph node or thyroid enlargement

11. General Examination • Eye examination: • Rt 6/60 hand motinsinccebith corrected to 6/24 with glassess with myopic fundus • Lt 6/18 with glassess with normal fundus

12. General Examination • Normal pulmonary, cardiac, and abdominal examination. • Normal skin appearance allover the body except for mild hyper pigmentation over the knuckles

13. Musculoskeletal Examination • No muscle wasting by inspection but there is difficulty in hair combing • Positive Gower’s sign • Manual muscle testing average • Muscle power proximal 3/5 , distal4/5 • Normal Neurological examination

14. Investigations • Abdominal ultrasonography free except for mild splenomegally • Echo cardiography free • X-ray both hands are free

15. Laboratory tests

16. Data Analysis • The previous data match with juvenile lupus with discoid lesion especially with +ve ANA and +ve Anti Ds DNA and low serum c4 • Also the skin manifestation of dermatomyositis must be consider especially with evident proximal muscle weakness with very high CPK • Malignancy also should considered especially with high ESR and un explained elevated SGOT and SGPT

17. Data Analysis • Finally what is your diagnosis and what is your decision • Can we give her a diagnosis like SLE and dermatomyositis or shall we wait and see • Do you expect malignancy in spite of long duration and good general condition or you exclude • At the end • I wish I have answers for you >>>>>>>>>>

18. Thank you