A 60 y.o. male presents with painful ankles and a rash
what could be wrong?
hint: he also had pericarditis
Our patient had leukocytoclastic vasculitis or hypersensitivity vasculitis. Immune complexes deposit in small vessels and the resultant white blood cell break down manifests as palpable purpura. There are many causes of this small vessel vasculitis including: drug hypersensitivity, amyloid, antiphospholipid syndrome, atrial myxoma, autoimmune diseases like Wegeners and Behcet’s, ITP, meningococcemia, and relapsing polychondritis.
Our patient had a history of relapsing polychondritis. This is an inflammation of cartilage throughout the body which often occurs with an associated vasculitis or myelodysplastic syndrome. Ear involvement is most common, which was the initial presentation of our patient. 90% of patients with the disease eventually have ear inflammation. The airways can also be involved resulting in tracheomalacia. Antibodies to type II collagen trigger this disease
in all cases of small vessel vasculitis some sort of antibody production occurs . Hep C causes this by producing cryoglobulins.
It is important to take a good history with small vessel vasculitis to distinguish between IgA and non –IgA vasculitis. IgA vasculitis is more likely to present with bloody urine, abdominal pain and joint pain. IgA vasculitis is referred to as Henoch-Schonlein and often occurs in children.
Henoch Schonlein
One of the types of small vessel vasculitis is ANCA positive vasculitis. Anticytoplasmic antibodies are yet another autoimmune disease associated with pathology. This disease used to be called Wegener’s granulomatosis but is now referred to as granulomatosis with polyangiitis. The biopsy of our patients skin lesions were positive for ANCA which occurs in about 14% of people with relapsing polychondritis. More typically in Wegener’s the nose and sinuses, lungs and kidneys are involved.
saddle nose from Wegener’s
Our patient presented with joint pain and was found to have worsening renal failure with his ANCA positive vasculitis. He was treated with rituxan and improved.
Ban B, Crowe J, Tudor M. Saddle nose deformity and granulomatosis with polyangiitis. QJM: an international journal of medicine, Vol 111, issue 1, Jan 2018; 55
Balgrie D, Bansal P, Goyal A, Crane J. Leukocytoclastic vasculitis StatPearls Publishing;2020Janhttps://www.ncbi.nlm.nih.gov/books/NBK482159/
Callen J, Jorizzo J, Zone J, et al. 2016 Dermatological signs of systemic disease. Callen, Jeffrey 1947-(fifth ed) Edinburgh
Dermatopathology Busam, K (1st ed) (Philadelphia) Saunders/Elsevier 2010.