Dr Anakha V Ajay DrCJayakumar Dr DrSuma Balan DrVineetha Panicker
Dr Praveena N Bhaskaran Dr Navya George
AIMS KOCHI
8 year old female presented with high grade fever ,vomiting and bifrontal headache as
and aversion to light for 6 days .Child also had non pruritic erythematous tender rashes over the right forehead and adjacent scalp region
Vitals were normal
He had multiple discrete tender cervical submandibular, posterior occipital and post auricular lymph nodes
Swollen lids, periorbital edema, mild chemosis & discharge was also present
Differentials considered were
Infectious mononucleosis
Orbital cellulitis
Viral exanthem
Hereditary angioedema
Kikuchi Fuji Moto
Partially treated bacterial meningitis
Viral meningitis
Lab TC 16,000/cmm ,P70.1%,L 16.7%Plt3.3.7L,ESR78mm
CRP(54.43mg/L).
IgM EBV done was negative.
C3. 219 mg%C427mg%were within normal limits .Further evaluation with USG Abdomen done was within normal limits. C1 esterase inhibitor done to rule out hereditary angioedema was negative. MRI Neck & chest no features of myositis.CSF Analysis showed
TC-122, DC-93% mononuclear cells, Protein-24.2, Sugar-57.4 with cytology showed lymphocytic predominant mixed pleocytosis and negative result for meningoencephalitis panel.
Child was initially started on vancomycin and ceftriaxone. But child continued to develop new crops of lesions on different areas which were extremely painful and stingy which even disrupted the sleep.
Punch biopsy of cutaneous rash done and showed vasculopathic changes with leucocytocklastia .ANA showed 2+. With all these diagnosis of urticarial vasculitis was considered.
Pulse dose methyl prednisolone(10mg/kg x 3 days)was started .Child improved with treatment and was discharged on oral steroids.The patient is doing well on follow up with significant improvement in the rash.
Urticarial vasculitis can be considered
If: (1) Clinical features of urticaria
(2) HPE suggesting cutaneous leukocytoclstic vasculitis of small vessel largely involving the post cappilary venules. It is due to formation of immune complexes in the blood and that then deposit in the vessel.
Presentation includes erythematous urticarial papules and plaques with angioedema, dermographism and annular erythema.Diagnosis is confirmatory with skin biopsy showing leucocytoclastic vasculitis.Treatment is with systemic glucocorticoids .Mild disease can be managed with antihistaminics and NSAIDS. Severe disease require glucocorticoids with MMF/Methotreaxate/Azathiprine/Cyclosporine.
Prognosis: Urticarial vasculitis carries a good prognosis with most occurences resolving in months to years.
Take home message: All cases of urticaria can’t be treated with 1st or 2nd Gen Antihistamines andH2RA (Ranitidine).In some biopsy be needed as in this case and steroids which we do not start intially hearing infection may be necessary