DrMehak ,DrC Jayakumar DrPraveena Bhaskaran ,DrNavya George
Nine year old girl presented with low grade intermittent fever of 4 days and pin point erythematous well circumscribed non pruritic flat rashes over lower limbs. Over the next 4 days, the rashes increased in size and number, became raised over skin surface and smaller rashes coalesced to form bigger ones ~1*1cm.In between She was asymptomatic. On the 5th day of illness, she had severe colicky abdominal pain followed five episodes of non bilious vomiting followed 3 episodes of greasy foul smelling stools containing dark red blood.
FIG Showing rashes in the legs
At this time child was febrile with stable vitals and bilaterally enlarged non tender anterior cervical lymph nodes with Grade 1 congested tonsils having whitish exudate. She had symmetrically distributed multiple well circumscribed palpable purpuric rashes on lower limbs. Some pin point erythematous lesions were seen on the dorsum of hands also
Differentials
1Vasculitis
2Immune Thrombocyopenic Purpura
3Systemic lupus erythematosis
4Henoch schonlein vasculitis
5.Acute leukaemia
Blood investigations reflected thrombocytosis with elevated CRP of 47.98mg/L and ESR 33mm/hr,Peripheral smear , renal functions USG abdomen and urine routine exam was with in normal limits As the child’s clinical criteria didn’t fit for SLE work up to rule out SLEwas not doneChild was started on Inj Ceftriaxone
USG abdomen was done which showed mild hepatomegaly with borderline splenomegaly. Stool Occult Blood tested at this juncture was positive
She was started on oral prednisolone 2mg/kg with a provisional diagnosis of Henoch schonlein vasculitis and Inj Ceftriaxone was stopped
She was finally discharged on a tapering dose of steroids as her fever and rashes subsided with repeat labs showing reduced CRP.
Child is on routine follow up with-a view to watch for renal abnormalities at home and regular urinalyses studies.
Henoch Schonlein Purpura Is a IgA Vasculitis commonly presents with systemic involvment like abdominal pain, arthritis/arthralgia, hematuria/proteinuria in addition to pursues usually in the lower limb. It is important rule out conditions like intussusception and renal involvement.
Carry home message- As recurrences are common and systemic involvement particularly renal involvement is late, potentially leading to renal failure, regular follow ups with simple measures like BP monitoring and UREs play a pivotal role in the holistic management of the disease.
HSP has excellent prognosis with self limiting course of ~4weeks. However, the long term prognosis is largely dependent on the extent of the involvement of GIT and renal systems.
GIT complications like intusussception and intestinal perforation, though rare, contribute to the mortality associated with the disease. Renal involvement is seen in only 1-2% patients and only <5% go into ESRD and develops upto 6months after initial diagnosis.
Thus, for diagnosis of HSP nephritis, serial BP monitoring and URE upto 6 months from diagnosis is must.