Anemia with a Reticular Rash in a Child (Answers)

Winner of the Clinical Quiz Dr. Sathwik Gangireddy Senior Resident All India Institute of Medical Sciences, RaipurQuestion 1: Identify the rash as depicted in Image 1? Answer: Livedo racemosa. It is a reddish blue mottling of the skin with irregular, broken circular segments.[1] On the other hand, livedo reticularis is a reddish blue mottling of the skin with regular, unbroken, reticular net-like pattern. While the latter occurs due to prominence of the superficial venous plexus due to increased deoxygenated hemoglobin resulting from impaired cutaneous circulation and is completely blanchable and reversible on rewarming, livedo racemosa results from permanent impairment of peripheral blood flow, only partially blanchable, and persists despite rewarming. While livedo reticularis may be physiological or pathological, livedo racemosa is always pathological. Question 2: What is the name of the phenomenon described when the mother gives "history of digits turning blue on exposure to cold?" Answer: Raynaud's phenomenon. Question 3: What is the type of anemia in this child as per the available history and examination? What investigations would you do to support the same? Answer: Autoimmune hemolytic anemia (AIHA). In the index case, the presence of anemia with adequate nutritional history with nonresponse to adequate dose of iron therapy with absence of bleeding with hepatosplenomegaly was suggestive of a hemolytic anemia. A positive direct Coombs test positive was suggestive of AIHA. Investigations to support the diagnosis include a peripheral smear, reticulocyte count, bilirubin, serum lactate dehydrogenase (LDH) levels, and haptoglobin.[2] In the present case, the child had hemoglobin of 6.1 g/dL, raised reticulocyte count (11%), total bilirubin of 0.75 mg/dl (normal), serum LDH of 891 IU/L (increased), and serum haptoglobin of <7 mg/dl (decreased) suggestive of hemolytic anemia. This was corroborated with peripheral smear showing red cell clumping with polychromatophils and microspherocytes. A positive direct Coombs test confirmed AIHA as in our case.Image 1Question 4. Give three most likely differential diagnoses in this case scenario Answer: Differentials could be AIHA associated with antiphospholipid antibody syndrome, systemic lupus erythematosus (SLE) or hepatitis C. In the present case, once a diagnosis of AIHA was established, further tests for hepatitis B, hepatitis C, human immunodeficiency virus, Epstein–Barr virus, COVID-19 serology, parvovirus, typhoid, rickettsia, Leptospira, mycoplasma, and tuberculosis were all negative. However, the antinuclear antibody for the child came strongly positive, in a titer of 1:1280 positive, with positive anti-ds DNA and positive anticardiolipin antibody. The child was, thus, diagnosed as SLE as per the European Alliance of Associations for Rheumatology criteria. There was no microalbuminuria or hematuria. The baseline SLE Disease Activity Index score was 6. The child was started on oral steroids and hydroxychloroquine and was advised for protection from cold temperature. At 2 months of follow-up, the child was well and with no evidence of hemolysis. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.Comments by Dr. Ankita Patel (Section Editor) The current clinical quiz titled "Anemia with a reticular rash in a child" was a tricky one, but still we received many responses. We could not get any answer which was perfectly correct but the enthusiasm was heartening. The first person who gave the maximum number of correct answers is declared as the winner for this quiz.