Erythema and Erythema and UrticariaUrticariaAugust 31, 2004August 31, 2004

FlushingFlushing

Transient, diffuse redness of face/neck/trunkTransient, diffuse redness of face/neck/trunk Niacin, Calcium Channel Blockers, cyclosporine, Niacin, Calcium Channel Blockers, cyclosporine,

chemotx, vancomycin, bromocriptine, contrast dye, chemotx, vancomycin, bromocriptine, contrast dye, tamoxifen, leuprolide acetate, high dose tamoxifen, leuprolide acetate, high dose methylprednisolonemethylprednisolone

Capsaicin (red pepper), sodium nitrate, sulfites, Capsaicin (red pepper), sodium nitrate, sulfites, alcohol, food poisoning (ciguatera, scrombroid)alcohol, food poisoning (ciguatera, scrombroid)

Carcinoid, Mastocytosis, PheochromocytomaCarcinoid, Mastocytosis, Pheochromocytoma Menopause, oophorectomyMenopause, oophorectomy

Erythema PalmareErythema Palmare

Hypothenar erythema

Elevated Estrogen

Cirrhosis

Metastatic Liver CA

Pregnancy

Erythema Toxicum NeonatorumErythema Toxicum NeonatorumOccurs in most healthy full term newborns, usually on 2nd - 3rd day.

Multiple papules that rapidly evolve into pustules with an erythematous base

Lesions may become confluent, especially on the face

No fever, gone by 10th day

DDx: Miliaria, Herpes, Bacterial folliculitis, scabies

Pustule smear revealing eosinophils is diagnostic.

Bx shows follliculitis with eosinophils and neutrophils

Erythema Multiforme MinorErythema Multiforme Minor

AKA Herpes Simplex-Associated EM (HAEM)AKA Herpes Simplex-Associated EM (HAEM) Minor is typically associated with orolabial HSVMinor is typically associated with orolabial HSV Major (SJS) is associated with sulfonamides, Major (SJS) is associated with sulfonamides,

NSAIDS, antibiotics, allopurinol, Mycoplasma NSAIDS, antibiotics, allopurinol, Mycoplasma pneumoniae, anticonvulsants, and radiationpneumoniae, anticonvulsants, and radiation

Cause not identified in 20% of casesCause not identified in 20% of cases Self-limited, recurrent, young adults, spring/fallSelf-limited, recurrent, young adults, spring/fall Mild or no prodrome lasting 1-4 weeksMild or no prodrome lasting 1-4 weeks Lesions evolve over 24-48 hoursLesions evolve over 24-48 hours ““Target” or “iris” lesions are diagnosticTarget” or “iris” lesions are diagnostic

Erythema MultiformeErythema Multiforme

1) Central dusky purpuric area1) Central dusky purpuric area2) Elevated edematous pale ring2) Elevated edematous pale ring

3) Surrounding macular erythema3) Surrounding macular erythema

EM: Vacuolar interface with “tagging” of lymphocytes along EM: Vacuolar interface with “tagging” of lymphocytes along DEJ with necrotic and apoptotic keratinocytesDEJ with necrotic and apoptotic keratinocytes

Cytoid BodiesCytoid Bodies

Erythema Multiforme MinorErythema Multiforme Minor

Locations: dorsal feet, limbs, elbows, Locations: dorsal feet, limbs, elbows, knees, palms and solesknees, palms and soles

EMM appears 1-3 weeks after the herpes EMM appears 1-3 weeks after the herpes lesionlesion

Tx: Self-limited, supportive careTx: Self-limited, supportive care If HSV: antivirals improve/steroid worsen; If HSV: antivirals improve/steroid worsen;

sunblocksunblock If SJS or TEN, stop suspect medications, If SJS or TEN, stop suspect medications,

admit to burn unit, IVIG, steroids, etc.admit to burn unit, IVIG, steroids, etc.

Oral Erythema MultiformeOral Erythema Multiforme

Usually limited to orolabial involvementUsually limited to orolabial involvement Concomitant skin involvement in 25% Concomitant skin involvement in 25% Tongue, gingiva and buccal mucosa are the Tongue, gingiva and buccal mucosa are the

most severly affectedmost severly affected Erosions +/- pseudomembraneErosions +/- pseudomembrane Important to r/o Important to r/o Candida, because topical Candida, because topical

antifungal therapy leads to improvement in 40% antifungal therapy leads to improvement in 40% of cases in which Candida is found, otherwise of cases in which Candida is found, otherwise prednisoneprednisone

Oral Erythema MultiformeOral Erythema Multiforme

Erythema Annulare CentrifugumErythema Annulare Centrifugum

Most common gyrate erythemaMost common gyrate erythema Unknown pathogenesisUnknown pathogenesis Polycyclic, trailing scale at inner borderPolycyclic, trailing scale at inner border Eccentric growth 2-3mm per dayEccentric growth 2-3mm per day Asymptomatic but chronic, recurrentAsymptomatic but chronic, recurrent Evaluate for TineaEvaluate for Tinea Recommended: Good H&P, CBC, LFT’s, UA Recommended: Good H&P, CBC, LFT’s, UA

and CXR to r/o internal cancerand CXR to r/o internal cancer Responsive to topical steroidsResponsive to topical steroids

Erythema Annulare CentrifugumErythema Annulare Centrifugum

EAC: “coat in sleeve” = lymphos EAC: “coat in sleeve” = lymphos tightly associated with vesselstightly associated with vessels

Erythema Gyratum RepensErythema Gyratum Repens

RareRare Undulating bands of slightly elevated wavy Undulating bands of slightly elevated wavy

erythema over the entire bodyerythema over the entire body ““Wood grain” with “trailing scale”Wood grain” with “trailing scale” Severe pruritis; eosinophilia often foundSevere pruritis; eosinophilia often found 80% underlying malignancy, MC lung CA80% underlying malignancy, MC lung CA Rash may precede CA by 9 monthsRash may precede CA by 9 months Remove CA, rash resolvesRemove CA, rash resolves

Erythema Gyratum RepensErythema Gyratum Repens

“WOOD GRAIN” APPEARANCE

Annular Erythema of InfancyAnnular Erythema of Infancy

RareRare Lesions are transitory, last 36-48 hoursLesions are transitory, last 36-48 hours Onset: 6 months, resolves by 11 months Onset: 6 months, resolves by 11 months

without treatmentwithout treatment

Necrolytic Migratory ErythemaNecrolytic Migratory Erythema AKA Glucagonoma SyndromeAKA Glucagonoma Syndrome Associated with amino precursor and uptake Associated with amino precursor and uptake

decarboxylation (APUD) tumor of the pancreasdecarboxylation (APUD) tumor of the pancreas Increased glucagon, decreased zinc Increased glucagon, decreased zinc Pancreas scan may be normalPancreas scan may be normal Location: periorificial, flexural, acralLocation: periorificial, flexural, acral Papulovesicular lesions coalesce, form pustules Papulovesicular lesions coalesce, form pustules

then erode. Active erythematous gyrate or then erode. Active erythematous gyrate or circinate borders with central confluencecircinate borders with central confluence

Patients present ill, with hyperglycemia, weight Patients present ill, with hyperglycemia, weight loss, diarrhea, anemia, atrophic glossitis, loss, diarrhea, anemia, atrophic glossitis, angular cheilitisangular cheilitis

Tx: removal of tumorTx: removal of tumor

Necrolytic Migratory Necrolytic Migratory ErythemaErythema

NME path identical to Zinc Defic.NME path identical to Zinc Defic.

Acanthosis with upper Acanthosis with upper epidermal necrolysis. epidermal necrolysis. There is a pallor of There is a pallor of the keratinocytes in the keratinocytes in the granular layer due the granular layer due to intracellular edema, to intracellular edema, thus “loss” of the thus “loss” of the granular cell layer. granular cell layer. Edema correlates Edema correlates with vesicles.with vesicles.

Erythema BrucellumErythema Brucellum

Vets and Cow tendersVets and Cow tenders Starts with itching and erythema of the Starts with itching and erythema of the

upper extremities, sometimes face and upper extremities, sometimes face and neck, then skin thickens and erupts with neck, then skin thickens and erupts with conical follicular papules conical follicular papules

Resolves without Tx in 2 weeksResolves without Tx in 2 weeks BrucellaBrucella organisms not identified in organisms not identified in

lesions, suggesting a sensitization lesions, suggesting a sensitization phenomenonphenomenon

Recurrent Granulomatous Recurrent Granulomatous Dermatitis with EosinophiliaDermatitis with Eosinophilia

Clinical hybrid between cellulitis and urticariaClinical hybrid between cellulitis and urticaria RecurrentRecurrent Reaction pattern to many possible things, Reaction pattern to many possible things,

including bites, onchocerciasis, parasites, including bites, onchocerciasis, parasites, varicella, mumps, tetanus immunization, drug varicella, mumps, tetanus immunization, drug reactions, myeloproliferative dz, atopic diathesis, reactions, myeloproliferative dz, atopic diathesis, hypereosinophilic synd., fungal infectionhypereosinophilic synd., fungal infection

TX: OAH, TCN, UVB, PUVA, Dapsone, TX: OAH, TCN, UVB, PUVA, Dapsone, Prednisone low dosePrednisone low dose

Recurrent Granulomatous Recurrent Granulomatous Dermatits with EosinophiliaDermatits with Eosinophilia

AKA Eosinophilic Cellulitis, Well’s SyndromeAKA Eosinophilic Cellulitis, Well’s Syndrome

Dermal eos and histiocytes surrounding central Dermal eos and histiocytes surrounding central masses of brightly pink collagen that has lost its masses of brightly pink collagen that has lost its

fibrillar appearance and is more amorphousfibrillar appearance and is more amorphous

“FLAME FIGURES”

Erythema NodosumErythema Nodosum

Young adult womenYoung adult women Crops of bilateral deep Crops of bilateral deep

tender nodules, pretibialtender nodules, pretibial Overlying skin shiny, redOverlying skin shiny, red Acute onset with arthralgia, Acute onset with arthralgia,

malaise, edemamalaise, edema In 2-3 days, lesions flatten In 2-3 days, lesions flatten

and have a bruised and have a bruised appearance; may last days appearance; may last days or weeksor weeks

Erythema Nodosum in Sarcoid Erythema Nodosum in Sarcoid

MC nonspecific cutaneous finding in MC nonspecific cutaneous finding in sarcoidosis sarcoidosis

Young females Young females Anterior shins Anterior shins Good prognosis Good prognosis Lofgren’s SyndromeLofgren’s Syndrome = EN with fever, = EN with fever,

arthralgias, hilar adenopathy, & fatiguearthralgias, hilar adenopathy, & fatigue

Erythema NodosumErythema Nodosum

Reactive ProcessReactive Process Strep, Yersinia, Salmonella, Shigella, Strep, Yersinia, Salmonella, Shigella,

Coccidiomycosis, Histoplasmosis, Coccidiomycosis, Histoplasmosis, Sporotrichosis, Blastomycosis, Sporotrichosis, Blastomycosis, Toxoplasmosis, TB, Sarcoidosis, Toxoplasmosis, TB, Sarcoidosis, Hematologic Malignancies, Pregnancy, Hematologic Malignancies, Pregnancy, Oral contraceptivesOral contraceptives

HISTO: Septal panniculitisHISTO: Septal panniculitis

Sweet’s SyndromeSweet’s Syndrome

MAY BURN, BUT DO NOT ITCH

PATHERGY

Sweet's syndrome, or acute febrile neutrophilic Sweet's syndrome, or acute febrile neutrophilic dermatosis, is a condition characterized by the dermatosis, is a condition characterized by the sudden onset of fever, leukocytosis, and tender, sudden onset of fever, leukocytosis, and tender, erythematous, well-demarcated papules and erythematous, well-demarcated papules and plaques.plaques.

71% no known disease71% no known disease 11% hematologic disease (including leukemia)11% hematologic disease (including leukemia) 16% immunologic disease (rheumatoid arthritis, 16% immunologic disease (rheumatoid arthritis,

inflammatory bowel disease)inflammatory bowel disease) 2% pregnancy2% pregnancy TX: systemic corticosteroidsTX: systemic corticosteroids The overlap between sweets and pyoderma The overlap between sweets and pyoderma

gangrenosum well documentedgangrenosum well documented

Marshall’s SyndromeMarshall’s Syndrome

Skin lesions that resemble Sweet’s but is Skin lesions that resemble Sweet’s but is followed by Cutis Laxa changesfollowed by Cutis Laxa changes

Primarily, affects childrenPrimarily, affects children Small red papules expand to urticarial Small red papules expand to urticarial

targetoid plaques with hypopigmented targetoid plaques with hypopigmented centerscenters

Eosinophilic infiltrate may be seenEosinophilic infiltrate may be seen Biopsies demonstrate loss of elastinBiopsies demonstrate loss of elastin

Marked diminution Marked diminution of elastic fibers in of elastic fibers in

lower dermis lower dermis (Verhoeff-van (Verhoeff-van

Gieson stain)Gieson stain)

Pyoderma GangrenosumPyoderma Gangrenosum

Pathergy, as in Sweet’s SyndromePathergy, as in Sweet’s Syndrome Heal with atrophic scarsHeal with atrophic scars Extremely painfulExtremely painful 50% of pts have associated disease50% of pts have associated disease MC: Crohn’s and Ulcerative ColitisMC: Crohn’s and Ulcerative Colitis 1/3 of PG patients have arthritis1/3 of PG patients have arthritis Other associations: leukemia, myeloma, Other associations: leukemia, myeloma,

polycythemia vera, Hep C, SLE, HIV, polycythemia vera, Hep C, SLE, HIV, pregnancy, Takayasu’s arteritis pregnancy, Takayasu’s arteritis

Pyoderma Gangrenosum Pyoderma Gangrenosum ulcers with distinct rolled ulcers with distinct rolled

edges, sharply edges, sharply marginated, undermined marginated, undermined blue to purple bordersblue to purple borders

Pyoderma GangrenosumPyoderma Gangrenosum

Histopathology is not helpfulHistopathology is not helpful Must rule out deep fungal, mycobacterial, Must rule out deep fungal, mycobacterial,

gummatous syphillis, synergistic gummatous syphillis, synergistic gangrene, amebiasisgangrene, amebiasis

Biopsy with special stains and cultures are Biopsy with special stains and cultures are very importantvery important

cANCA to rule out Wegener’s cANCA to rule out Wegener’s granulomatosisgranulomatosis

TX: Pyoderma GangrenosumTX: Pyoderma Gangrenosum

Excise colon segment for IBS, UC, Crohn’sExcise colon segment for IBS, UC, Crohn’s Rule out/treat malignancy or infectionRule out/treat malignancy or infection Steroids: topical, IL or oral depending on Steroids: topical, IL or oral depending on

severity and aggressivenessseverity and aggressiveness Topical 4% cromolyn or tacrolimusTopical 4% cromolyn or tacrolimus Hyperbaric oxygen leads to rapid pain reliefHyperbaric oxygen leads to rapid pain relief Cyclosporine, Sulfasalazine, Dapsone, Cyclosporine, Sulfasalazine, Dapsone,

Clofazimine, Azathioprine, Mycophenolate, IVIG, Clofazimine, Azathioprine, Mycophenolate, IVIG, Plasma exchangePlasma exchange

UrticariaUrticaria

History is KeyHistory is Key

Illness: e.g., fever, sore throat, cough, rhinorrhea, vomiting, diarrhea, Illness: e.g., fever, sore throat, cough, rhinorrhea, vomiting, diarrhea, headache -- INFECTIOUS: STREP, HEP C, H. PYLORIheadache -- INFECTIOUS: STREP, HEP C, H. PYLORI

Medications: ACE inhibitors, which result in angioedema, as well as Medications: ACE inhibitors, which result in angioedema, as well as anesthetics, penicillins, cephalosporins, sulfas, diuretics, aspirin, nonsteroidal anesthetics, penicillins, cephalosporins, sulfas, diuretics, aspirin, nonsteroidal anti-inflammatory drugs [NSAIDs], iodides, bromides, quinidine, chloroquine, anti-inflammatory drugs [NSAIDs], iodides, bromides, quinidine, chloroquine, vancomycin, isoniazid, antiepileptic agentsvancomycin, isoniazid, antiepileptic agents

Travel: rule out amebiasis, malaria, helminthicsTravel: rule out amebiasis, malaria, helminthics New foods: e.g., shellfish, fish, eggs, cheese, chocolate, nuts, berries, New foods: e.g., shellfish, fish, eggs, cheese, chocolate, nuts, berries,

tomatoes, alcoholtomatoes, alcohol Perfumes, detergents, lotions, creams, or clothesPerfumes, detergents, lotions, creams, or clothes Exposure to new pets (dander), dust, mold, chemicals, or plantsExposure to new pets (dander), dust, mold, chemicals, or plants Pregnancy (PUPPP)Pregnancy (PUPPP) Contact with nickel (eg, jewelry, jean stud buttons), rubber (eg, gloves, elastic Contact with nickel (eg, jewelry, jean stud buttons), rubber (eg, gloves, elastic

bands), latex, industrial chemicals, and nail polishbands), latex, industrial chemicals, and nail polish Sun exposure or cold exposure, exerciseSun exposure or cold exposure, exercise

Urticaria PathogenesisUrticaria Pathogenesis

Increased capillary permeability, which Increased capillary permeability, which allows proteins and fluids to extravasateallows proteins and fluids to extravasate

Due to histamine release from mast cells Due to histamine release from mast cells degranulating, which in turn recruits degranulating, which in turn recruits eosinophils, neutrophils and basophilseosinophils, neutrophils and basophils

Other triggers are leukotrienes (slow Other triggers are leukotrienes (slow reacting substances of anaphylaxis), reacting substances of anaphylaxis), prostaglandins, proteases, bradykininsprostaglandins, proteases, bradykinins

Chronic UrticariaChronic Urticaria

1/3 of these patients have circulating 1/3 of these patients have circulating functional histamine-releasing functional histamine-releasing autoantibodies that bind to the high-affinity autoantibodies that bind to the high-affinity IgE receptor producing mast cell-specific IgE receptor producing mast cell-specific histamine releasing activityhistamine releasing activity

Fc epsilon RIFc epsilon RI

H&E: collagen bundles separated H&E: collagen bundles separated by edema, perivascular infiltrateby edema, perivascular infiltrate

Urticaria & Angioedema DDx:Urticaria & Angioedema DDx:

Clinical diagnosisClinical diagnosis DDx: Urticarial Vasculitis, Bullous DDx: Urticarial Vasculitis, Bullous

Pemphigoid, GA, Sarcoidosis, CTCLPemphigoid, GA, Sarcoidosis, CTCL Most of the diseases listed above have Most of the diseases listed above have

lesions that last longer than 24 hourslesions that last longer than 24 hours Biopsy urticarial lesions that last > 24 Biopsy urticarial lesions that last > 24

hourshours

Urticaria EvaluationUrticaria Evaluation

Dental and sinus x-rays can be of benefitDental and sinus x-rays can be of benefit Order laboratory tests based only on Order laboratory tests based only on

symptoms and signs from H&P including:symptoms and signs from H&P including:

TSH, LFTs, Hepatitis panel, ANA, CBCTSH, LFTs, Hepatitis panel, ANA, CBC Eosinophilia: search for parasitesEosinophilia: search for parasites Food skin testsFood skin tests

Urticaria TreatmentUrticaria Treatment

Tx: OAH, multiple if necessaryTx: OAH, multiple if necessary Simons et al., randomized, double blind parallel Simons et al., randomized, double blind parallel

series of 23 “refractory” urticarias found 58% of series of 23 “refractory” urticarias found 58% of patients preferred H1 + H2 combinationspatients preferred H1 + H2 combinations

Atarax + Tagamet much better than Zyrtec and Atarax + Tagamet much better than Zyrtec and Tagamet Tagamet

Cool bathingCool bathing Pramoxine, SarnaPramoxine, Sarna Oral steroids rarely helpfulOral steroids rarely helpful

Urticaria TreatmentUrticaria Treatment

Foods to avoid: Fish and shellfishFoods to avoid: Fish and shellfish PorkPork Garlic, onionsGarlic, onions MushroomsMushrooms Tomatoes, melons, strawberries, citrus fruits, Tomatoes, melons, strawberries, citrus fruits,

pickles and relishespickles and relishes Nuts, peanuts, cheeseNuts, peanuts, cheese Remove suspected food x 3 weeks then resumeRemove suspected food x 3 weeks then resume

Anaphylaxis Anaphylaxis

Acute, life threateningAcute, life threatening urticaria/angioedema 90%, SOB 60%urticaria/angioedema 90%, SOB 60% Onset: peak severity within 5-30 minutesOnset: peak severity within 5-30 minutes MC causes of serious anaphylactic MC causes of serious anaphylactic

reactions are: antibiotics, especially PCNs, reactions are: antibiotics, especially PCNs, NSAIDS, radiographic contrast dyesNSAIDS, radiographic contrast dyes

22ndnd MC cause – hymenoptera, shellfish MC cause – hymenoptera, shellfish

AnaphylaxisAnaphylaxis Mortality rate less than 10%Mortality rate less than 10% Still account for vast majority of fatal Still account for vast majority of fatal

reactions, peak onset 5-30 minutesreactions, peak onset 5-30 minutes One of every 2700 hospital patientsOne of every 2700 hospital patients 500 annual fatalities500 annual fatalities Tx: 0.3 - 0.5mL dose of 1:1000 dilution of Tx: 0.3 - 0.5mL dose of 1:1000 dilution of

epinephrine SQ q 10-20 minutes epinephrine SQ q 10-20 minutes IV Solumedrol 50mg q6h x 2-4 dosesIV Solumedrol 50mg q6h x 2-4 doses Benadryl, aminophyliine, neb. Benadryl, aminophyliine, neb.

metaproterenol, O2, glucagon, intubation, metaproterenol, O2, glucagon, intubation, IVFs IVFs

AngioedemaAngioedema

Hereditary AngioedemaHereditary Angioedema 22ndnd to 4 to 4thth decade, +Family history, Autosomal decade, +Family history, Autosomal

DominantDominant May occur q 2 weeks, lasting 2 to 5 daysMay occur q 2 weeks, lasting 2 to 5 days Eyelid and lip involvement NOT SEENEyelid and lip involvement NOT SEEN Face, hands, arms, legs, genitals buttocks, Face, hands, arms, legs, genitals buttocks,

stomach, intestines, bladder affectedstomach, intestines, bladder affected N/V, colic, may mimic appendicitisN/V, colic, may mimic appendicitis Triggers: minor trauma, surgery, sudden Triggers: minor trauma, surgery, sudden

changes in temperature or sudden emotional changes in temperature or sudden emotional stressstress

Presence of urticaria rules out HA Presence of urticaria rules out HA

Hereditary AngioedemaHereditary Angioedema

AKA Quincke’s EdemaAKA Quincke’s Edema NO PRURITIS OR URTICARIA, +PAINNO PRURITIS OR URTICARIA, +PAIN Low C4, C1, C1q, C2 levelsLow C4, C1, C1q, C2 levels Low or dysfunctional plasma C1 esterase Low or dysfunctional plasma C1 esterase

inhibitor proteininhibitor protein 25% of deaths are from laryngeal edema25% of deaths are from laryngeal edema Tx of choice: fresh frozen plasma, Tx of choice: fresh frozen plasma,

stanazol, tranexamic acidstanazol, tranexamic acid

Type I and Type II HAType I and Type II HA

Type I – LOW serum levels of NORMAL Type I – LOW serum levels of NORMAL C1 esterase inhibitor proteinC1 esterase inhibitor protein

Type II – NORMAL levels of Type II – NORMAL levels of DYSFUNCTIONAL C1 esterase inhibitor DYSFUNCTIONAL C1 esterase inhibitor proteinprotein

C4 is best screening test, it will be low in C4 is best screening test, it will be low in both of the above casesboth of the above cases

HA - TreatmentHA - Treatment

TOC for acute HA is fresh frozen plasmaTOC for acute HA is fresh frozen plasma Stanazol useful for short-term prophylaxis Stanazol useful for short-term prophylaxis

in patients undergoing dental surgery, in patients undergoing dental surgery, endoscopic surgery or intubationendoscopic surgery or intubation

Tranexamic acid in low doses has few side Tranexamic acid in low doses has few side effects -- useful for acute or chronic HAeffects -- useful for acute or chronic HA

Acquired AngioedemaAcquired Angioedema Symptoms same as HA, but NO family HxSymptoms same as HA, but NO family Hx AKA Caldwell’s SyndromeAKA Caldwell’s Syndrome Occurs at night, pt wakes up with itOccurs at night, pt wakes up with it Acute evanescent circumscribed edemaAcute evanescent circumscribed edema Affects most distensible tissues: eyelids, Affects most distensible tissues: eyelids,

lips, earlobes, genitalia, mouth, tongue, lips, earlobes, genitalia, mouth, tongue, larynxlarynx

Swelling is subcutaneous, not dermalSwelling is subcutaneous, not dermal Overlying skin is not affectedOverlying skin is not affected

Schnitzler’s SyndromeSchnitzler’s Syndrome

Chronic non-pruritic urticariaChronic non-pruritic urticaria Fever of unknown originFever of unknown origin Disabling bone painDisabling bone pain HyperostosisHyperostosis Increased ESRIncreased ESR Macroglobulinemia (IgM Kappa) Macroglobulinemia (IgM Kappa) Tx: Oral SteroidsTx: Oral Steroids

Physical UrticariasPhysical Urticarias 20% of all urticarias20% of all urticarias DermatographismDermatographism Cholinergic/AdrenergicCholinergic/Adrenergic Cold/HeatCold/Heat SolarSolar PressurePressure Exercise-inducedExercise-induced AquagenicAquagenic Vibratory angioedemaVibratory angioedema

DermatographismDermatographism

DermatographismDermatographism

Sharply localized wheal and flare seconds Sharply localized wheal and flare seconds to minutes after stroking skinto minutes after stroking skin

2% to 5% of the population2% to 5% of the population Associated with penicillin induced urticaria, Associated with penicillin induced urticaria,

Pepcid (famotidine), hypothyroidism, Pepcid (famotidine), hypothyroidism, hyperthyroidism, infectious disease, hyperthyroidism, infectious disease, diabetes mellitus, onset of menopausediabetes mellitus, onset of menopause

Tx: OAHTx: OAH

Cholinergic UrticariaCholinergic Urticaria

Cholinergic UrticariaCholinergic Urticaria

Acetylcholine inducedAcetylcholine induced Tiny punctate extremely pruritic wheals or Tiny punctate extremely pruritic wheals or

papules 1-3mm in diameter surrounded by papules 1-3mm in diameter surrounded by erythema erythema

MC trunk and face, spares palms & solesMC trunk and face, spares palms & soles Triggers: exercise, heatTriggers: exercise, heat Tx: Cold shower, OAH high doseTx: Cold shower, OAH high dose Provoke: Methacholine skin test, heatProvoke: Methacholine skin test, heat

Adrenergic UrticariaAdrenergic Urticaria

Norepinephrine inducedNorepinephrine induced Small, 1-5mm papules, +/- pale haloSmall, 1-5mm papules, +/- pale halo 10-15 minutes after emotional upset, 10-15 minutes after emotional upset,

coffee or chocolatecoffee or chocolate Serum adrenalin elevated, histamine NLSerum adrenalin elevated, histamine NL Tx: Propranolol 10mg QIDTx: Propranolol 10mg QID Provoke: 3 to 10 nanograms noradrenalin Provoke: 3 to 10 nanograms noradrenalin

intradermallyintradermally

Cold Urticaria and AngioedemaCold Urticaria and Angioedema

MC face/hands, occurs with rewarmingMC face/hands, occurs with rewarming 25% patients are atopic25% patients are atopic Tx: PERIACTIN 4mg TIDTx: PERIACTIN 4mg TID Desensitize: repeated colder exposuresDesensitize: repeated colder exposures Test: Ice cube in saran wrap x 5-20 min.Test: Ice cube in saran wrap x 5-20 min. Associations: Cryoglobulins, Myeloma, Associations: Cryoglobulins, Myeloma,

Syphillis, Hepatitis, MononucleosisSyphillis, Hepatitis, Mononucleosis Familial variant – Bx: LCV; Tx: StanazolFamilial variant – Bx: LCV; Tx: Stanazol

Heat UrticariaHeat Urticaria

occurs within 5 minutes of exposure occurs within 5 minutes of exposure Heat > 109.4 farenheit (43 C)Heat > 109.4 farenheit (43 C) Burns, stings, red, swollen, induratedBurns, stings, red, swollen, indurated May become generalized with cramps, May become generalized with cramps,

weakness, flushing, salivation and weakness, flushing, salivation and collapsecollapse

Tx: heat desensitizationTx: heat desensitization Provoke: heated cylinder 122 F x 30 min.Provoke: heated cylinder 122 F x 30 min.

Solar UrticariaSolar Urticaria

Classified by the wavelength of light Classified by the wavelength of light causing itcausing it

Visible light may cause it, so sunscreens Visible light may cause it, so sunscreens may be of little helpmay be of little help

Sun AvoidanceSun Avoidance OAHOAH PUVA; repetitive phototherapyPUVA; repetitive phototherapy

Pressure UrticariaPressure Urticaria

3-12 hours after local pressure has been 3-12 hours after local pressure has been appliedapplied

MC feet/walking and buttocks/sittingMC feet/walking and buttocks/sitting Arthralgias, fever, chills, leukocytosis can Arthralgias, fever, chills, leukocytosis can

occuroccur Tx: ORAL STEROIDS HELPFUL, Tx: ORAL STEROIDS HELPFUL,

ANTIHISTAMINES NO HELP!ANTIHISTAMINES NO HELP! Provoke: 15 lb. weight x 20 minutesProvoke: 15 lb. weight x 20 minutes

Exercise Induced UrticariaExercise Induced Urticaria

Not related to body temperatureNot related to body temperature Wheals are larger than those seen in Wheals are larger than those seen in

cholinergic urticariacholinergic urticaria Starts after 5-30 minutes of exerciseStarts after 5-30 minutes of exercise Patients often atopicPatients often atopic Avoid celery and gliadin or other food Avoid celery and gliadin or other food

allergyallergy Tx: OAH Tx: OAH

Vibratory AngioedemaVibratory Angioedema

Autosomal Dominant or acquiredAutosomal Dominant or acquired Usually occupational in origin Usually occupational in origin Plasma histamine levels elevated during Plasma histamine levels elevated during

attacksattacks Provocation test: Laboratory vortex Provocation test: Laboratory vortex

vibration applied for 5 minutesvibration applied for 5 minutes Tx: OAHTx: OAH

Aquagenic UrticariaAquagenic Urticaria

Water, seawater, tears, sweat, saliva at Water, seawater, tears, sweat, saliva at any temperature may provokeany temperature may provoke

Immediately or within minutes and clear Immediately or within minutes and clear within 30-60 secondswithin 30-60 seconds

Wheezing, dysphagia, SOB may Wheezing, dysphagia, SOB may accompanyaccompany

Water soluble antigens the etiology?Water soluble antigens the etiology? Tx: Petrolatum, OAH, PUVATx: Petrolatum, OAH, PUVA

The EndThe End