Diagnostic Dilemma

Urticaria with shock and elevated D-Dimers

A 16 month old boy born of non consanguineous marriage presented with boil over scalp since 15 days, urticarial rash since 3 days and fever since 2 days. Patient was treated with some medications for the boil following which he developed the rash. There were no similar episodes in the past. He was a full term unimmunized child. On examination, he was febrile, had tachycardia with low volume pulses {heart rate = 160, min} and palpable purpura with central clearing. There was an abscess over left fronto parietal region of scalp without bony involvement. Blood pressure was 90 systolic. On systemic examination, he had hepatomegaly. Other systemic examination was normal. He was diagnosed as septic shock and treated with IV Fluids, ionotropes, Hydrocortisone {for urticaria} and Vancomycin and Ciprofloxacin to which he improved. Investigations showed:
? Hemoglobin = 9.2 gm percent
? WBC count = 13,800, cumm {63 percent polymorphs, 34 percent lymphocytes}
? Platelet count = 2,88,000, cumm
? Reticulocyte count = 0..3 percent
? Total proteins = 5.4 mg, dl, Albumin = 2.4 gm, dl
? Blood culture = No growth
? Venous blood gas = pH – 7.42, bicarbonate = 6.4 mmoL, L
? D-Dimers = More than 10,000 units
? Prothrombin Time = 12.9, Partial thromboplastin Time = 20.7 sec. {Normal}.

Again after 1 week, he had similar urticarial rash with fever. At that time, there was no sepsis but he had hepatomegaly. The abscess over scalp had resolved. Investigations at that time showed :
? Hemoglobin = 8.8 gm percent
? WBC count = 24,500, cumm {23 percent polymorphs, 73 percent lymphocytes}
? Platelet count = 5,95,000, cumm {elevated}
? ESR = 71
? D-Dimers = More than 10,000 units
? ANA, dsDNA, APLA, ACLA = Negative

He was again treated with steroids and antibiotics to which he responded.

What is the diagnosis _?
Answer Discussion :
PAVAI Arunachalam
13 years ago
prajakta morathkar
chronic urticari
13 years ago

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