¹Khalid A. Alnaqbi, ¹Naser Abunamous

¹Tawam

Background(s): COVID-19 has affected millions worldwide, primarily presenting with respiratory symptoms. Dermatological manifestations are rare but have been reported. We present a rare case with hemophilia A presenting with Immunoglobulin A vasculitis (IgAV), also known as Henoch-Schönlein purpura, as a complication of COVID-19.

Method(s): We searched PubMed, Google Scholar and Web of Science on published literature of COVID-19 infection, IgA vasculitis / Henoch Schonlein purpura and hemophilia.

Result(s): A 23-year-old man diagnosed previously with hemophilia A presented with a one-week history of skin rash and abdominal pain. The abdominal pain was colicky, non-radiating, and worsened with eating. It was associated with fatigue, bodyache, and decreased oral intake. He did not respiratory symptoms of COVID-18 infection. Skin rash initially in the lower limbs, spread to the upper extremities (Figure 1). There was no associated itching or pain. He also reported watery diarrhea with no blood or mucus. A review of other symptoms was unremarkable.

On physical examination revealed a purpuric and petechial rash on the lower and upper extremities. (Figure 1a and 1b), Abdominal examination was unremarkable, with no organomegaly.

Laboratory results revealed a slight elevation of CRP (8.3 mg/L, NR 0 – 5), with normal urea and electrolytes, complete blood count, liver function tests, and coagulation profile including Factor VIII activity (3.90%). Immunological tests showed negative ANA, cANCA, pANCA, rheumatoid factor, and cryoglobulin. CT abdomen with contrast showed no signs of vasculitis. A skin biopsy strongly indicated IgA vasculitis. (Figure 2).  On the 4th day of admission, nasopharyngeal swab was positive for COVID-19 infection.

The patient was started on IV methylprednisolone (40mg daily). COVID-19 was managed symptomatically. Hematology advised the continuation of anti-hemophilic factor VIII (1500 units three times per week) due to low disease activity.  The patient was discharged on a tapering dose of prednisolone (40mg daily) with a follow-up plan in the rheumatology clinic.

A literature review revealed only a few case reports about the association between IgAV and hemophilia patients, mostly in pediatric patients.  To our knowledge, this is one of the few reported cases of IgAV associated with COVID-19 in an adult with underlying hemophilia A.

Conclusion(s): Timely recognition and management of dermatological complications, such as IgAV, in COVID-19 patients who have hemophilia can contribute to improved clinical outcomes. Further research is warranted to better understand the pathophysiology and prevalence of IgAV in the context of COVID-19, as well as the long-term implications for affected individuals