Allen P Kaplan MDMedical University of South CarolinaCharlestown SC USAJune 2011 UrticariaAcutePhysicalChronicAnaphylaxisHypotensionDiarrhea1 Systemic Manifestation2 Systemic DiseaseSystemic Man ID: 947161
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Systemic Manifestations of Atopic Urticaria Allen P. Kaplan, MDMedical University of South CarolinaCharlestown, SC, USAJune 2011 UrticariaAcutePhysicalChronicAnaph
ylaxisHypotensionDiarrhea1) Systemic Manifestation2) Systemic DiseaseSystemic Manifestation AnaphylaxisSkinRespiratoryGastrointestinalStridorWheezeHypotensionUrtic
ariaAngioedemaVascularVomitingPainDiarrheaA) Typically antigen-dependent, IgEdependent thru IgEreceptorsB) Skin involvement as early symptom is 90%C)Diagno
sticconsideration: Find the antigen IgE-Dependent Physical Urticaria1)Cold Urticaria2)Dermatographism3)Solar Urticaria light-inducible autoantigen4)Cholinergic
Urticaria1,3,4 Occasionally associated with hypotension Cold urticaria 5 Histamine release in cold urticaria Cholinergic (generalized heat) urticaria Dermatograph
ism 9 Diagnostic Consideration1)Cold Urticaria Occasionally associated with cold agglutinins or Cryoglobulins2)Physical Urticariasoccur spontaneously and are not
associated with systemic diseases3)Solar Urticaria and photosensitive rashes in SLE are different Autoimmune urticaria 12 \n \r\r \n
\r \r 20406080100No UrticariaCU-IdiopathicCU-Autoimmune Percentage Histamine Release Serologic A
bnormalities1)CRP is elevated compared to normal2)IL6 levels are increased3)Metalloproteinase 9 levels are increased4)Prothrombinfragments 1 + 2 are increased5)The
re is no fever, or thrombosis or bleeding Search for Systemic Disease in Chronic UrticariaA)Systemic diseases can have urticariaas a manifestationB)Urticaria can b
e the presenting symptom of a systemic disease, but its uncommonC)What to order, and when Systemic Diseases with Urticaria or UrticarialVasculitisA)Systemic Lupu
s Erythematosisa)Autoimmuneb)CutaneousvasculitisB)DermatomyositisC)Microscopic PolyarteritisNodusaD)Wegeners GranulomatosusE)Churg-StrassSyndromeF)Rheumatoid Vasc
ulitisG)Cryoglobulinemia CasesA)A 30 yr old male presents with urticariaon a daily basis for 3 months. There is no fever, arthralgia, or myalgia. He is somewhat ti
red but indicates that he is not sleeping well because of pruritus. There is no other significant history and physical exam is normal except for generalized urtica
riaB)A 30 yr old female presents with chronic urticariafor 10 weeks accompanied by angioedemaaffecting the lips and eyes. When symptoms are prominent she complains
of fatigue and joint pain. A prior evaluation by a family physician reveals a sedimentationrate of 16 and a positive ANA at 1:80 titer Rationale for Doing the Min
imum1)With the exception of idiopathic urticarialvasculitis, the incidence of chronic urticariabeing the presenting symptom of a rheumatic disease is less than 1%.
2)Patients with chronic urticariafrequently have a (+) ANA (low titer ~15-20%) and elevated CRP as part of the disorder.3)Although arthralgia, if prominent, freque
ntly leads to extensive testing for rheumatic diseases, it can be part of the syndrome of chronic urticariaas long as there is no true arthritis. Rationale for Doi
ng the Minimum4) Drawing ANAsroutinely means that a significant number of patients who have a titer that is less than 1:320 will have tests for anti dsDNA, anti
SLA, s (Ro), anti SSB (La), anti Sm, anti RNP, anti SCL-70 and rheumatoid factor and typically none are positive. Rationale for Doing the Minimum5) If you miss a c
onnective tissue disease that has presented with chronic urticaria, it will become obvious, and if you knew about it earlier, there is nothing you could have done.
6)Patient A-order CBC, CRP, thyroid function tests and antithyroidantibodies, and antibody to IgEreceptor.7)Patient B-Tests for systemic disorders other than SLE a
re unwarranted. An ANA and anti dsDNA are reasonable, but will likely be negative. Rationale for Doing the MinimumA skin biopsy is indicated if the patient has fev
er, palpable purpura, true arthritis, hives lasting 24 hrs, hives that leave a bruisewhen they fade. Then serologiesfor rheumatic diseases are also indicat