3Erythema Palmare Elevated Estrogen Cirrhosis Liver CA metastatic Pregnancy
4Erythema Toxicum Neonatorum Occurs in most healthy full term newborns, usually on 2nd - 3rd day.Multiple papules that rapidly evolve into pustules with an erythematous baseLesions may become confluent, especially on the faceNo fever, gone by 10th dayDdx Miliaria, Herpes, Bacterial folliculitis, scabiesPustule smear revealing eosinophils is diagnostic.Bx shows follliculitis of eos and neuts
5Erythema Multiforme Minor EM due to herpes virus (HAEM) +/- oral.SJS, TEN due to meds, mycoplasma, radiation.20% of cases cannot be classified.Self-l imited, recurrent, young adults, spring/fallMild or no prodrome x 1-4 weeksLesions evolve over hours“Target” or “iris” lesions are diagnostic
71) Central dusky purpuric area 2) Elevated edematous pale ring 3) Surrounding macular erythema
8EM: Vacuolar interface with “tagging” of lymphocytes along DEJ with necrotic and apoptotic keratinocytesCytoid Bodies
9Erythema Multiforme Minor Usually associated with orolabial HSVAntivirals improve it and steroids worsen itAppear 1-3 weeks after the herpes lesionSometimes EMM comes without herpesSometimes herpes comes without EMM
10Oral Erythema Multiforme Oral only in 45%, lip & oral 30%Oral specialists usually handle thisTongue, gingiva and buccal mucosa are the most severly affected.Erosions +/- a pseudomembraneImportant to r/o Candida, because topical antifungal therapy leads to improvement in 40% of cases in which Candida is found, otherwise prednisone.
12EM Treatment Depends on etiology. If HSV: Treat HSV, sunblock. If SJS or TEN, stop medications such as sulfonamides, antibiotics, NSAIDS, allopurinol, anticonvulsants. Look for history of mycoplasma or radiation therapy.SJS, TEN: Burn unit, IVIG, Steroids etc.
15Erythema Annulare Centrifugum Most common gyrate erythemaPolycyclic, trailing scale at inner borderEccentric growth 2-3mm per dayAsymptomatic but chronic, recurrentLook for tineas, rarely CAGood H&P, CBC, LFT’s, UA and CXR
17Erythema Gyratum Repens RareUndulating bands of slightly elevated wavy erythema over the entire body“Wood grain” with “trailing scale”Severe pruritis, eosinophilia80% underlying malignancy, MC lung CARash may precede CA by 9 months.Remove CA, rash resolves.
18Annular Erythema of Infancy RareOnset: 6 months, resolves by 11 monthsLesions are transitory, last hoursNo treatment necessary
20NME path identical to Zinc Defic. Acanthosis with upper epidermal necrolysis. There is a pallor of the keratinocytes in the granular layer due to intracellular edema, thus “loss” of the granular cell layer. Edema correlates with vesicles.
21Necrolytic Migratory Erythema Aka Glucagonoma SyndromeAmino Precursor and uptake decarboxylation (APUD) cell tumor of the pancreasElevated serum Glucagon, low ZincPancreas scan may be normalDistribution: periorificial, flexural, acral.Papulovesicular lesions coalesce, form pustules then erode. Active erythematous gyrate or circinate borders with central confluencePatients present ILL, with hyperglycemia, weight loss, diarrhea, anemia, atrophic glossitis, angular cheilitis
22Erythema Brucellum Veterinarians and Cattlemen Starts with itching and erythema of the upper extremities, sometimes face and neck, then skin thickens and erupts with conical follicular papulesResolves without tx in 2 weeks.Brucella organisms not identified, this is a sensitization phenomenon.
23Recurrent Granulomatous Dermatits with Eosinophilia Aka Eosinophilic Cellulitis, Well’s Synd.
24Dermal eos and histiocytes surrounding central masses of brightly pink collagen that has lost its fibrillar appearance and is more amporphous“FLAME FIGURES”
25Well’s Syndrome Clinical hybrid between cellulitis and urticaria. RecurrentReaction pattern to many possible things, including bites, onchocerciasis, parasites, varicella, mumps, tetanus immunization, drug reactions, myeloproliferative dz, atopic diathesis, hypereosinophilic synd. Fungal infection.TX: OAH, TCN, UVB, PUVA, Dapsone, Prednisone low dose
26Erythema Nodosum Young adult women Crops of bilateral deep tender nodules, pretibialOverlying skin shiny, red.Onset acute with arthralgia, malaise, edema2-3 days lesions flatten and have a bruised appearance, may last days or weeks
27Erythema Nodosum in Sarcoid MC nonspecific cutaneous finding in sarcoidosisYoung femalesAnterior shinsGood prognosisLofgren’s Syndrome = fever, arthralgias, hilar adenopathy, fatigue, EN
31Sweet's syndrome, or acute febrile neutrophilic dermatosis, is a condition characterized by the sudden onset of fever, leukocytosis, and tender, erythematous, well-demarcated papules and plaques.71% no known disease11% hematologic disease (including leukemia)16% immunologic disease (rheumatoid arthritis, inflammatory bowel disease)2% pregnancyTX systemic corticosteroidsOverlap between sweets and pyoderma gangrenosum well documented.
32Marshall’s SyndromeSkin lesions that resemble Sweet’s but is followed by Cutis Laxa changesChildrenSmall red papules expand to urticarial targetoid plaques with hypopigmented centers.Eosinophilic infiltrated may be seenBiopsies demonstrate loss of elastin
33Marked diminution of elastic fibers in lower dermis (Verhoeff-van Gieson stain).
34Pyoderma Gangrenosum ulcers with distinct rolled edges, sharply marginated, undermined blue to purple borders
35Pyoderma Gangrenosum Pathergy (Sweet’s too) Heal with atrophic scars Extremely painful50% of pts have associated diseaseMC: Crohn’s and Ulcerative Colitis1/3 of PG patients have arthritisOther associated: Leukemia, Myeloma, Polycythemia vera, Hep C, SLE, HIV, pregnancy, Takayasu’s arteritis
36Pyoderma Gangrenosum Histopathology is not helpful. Must rule out deep fungal, mycobacterial, gummatous syphillis, synergistic gangrene, amebiasis.Biopsy with special stains and cultures are very important.cANCA to rule out Wegner’s granulomatosis
37TX: Pyoderma Gangrenosum Excise colon segment for IBS, UC, CrohnsRule out/treat malignancy or infectionSteroids: topical, IL or oral depending on severity and aggressivenessTopical 4% cromolyn or tacrolimusHyperbaric oxygen- rapid pain reliefCyclosporine, Sulfasalazine, Dapsone, Clofazimine, Azathioprine, Mycophenolate, IVIG, Plasma exchange
39History: Use a questionnaire! Recent illness (eg, fever, sore throat, cough, rhinorrhea, vomiting, diarrhea, headache) INFECTIOUS: STREP, HEP C, H. PYLORIMedication use (especially ACE inhibitors, which result in angioedema, as well as anesthetics, penicillins, cephalosporins, sulfas, diuretics, aspirin, nonsteroidal anti-inflammatory drugs [NSAIDs], iodides, bromides, quinidine, chloroquine, vancomycin, isoniazid, antiepileptic agents)Travel (rule out amebiasis, malaria, helminthics)New foods (eg, shellfish, fish, eggs, cheese, chocolate, nuts, berries, tomatoes, alcohol)Perfumes, detergents, lotions, creams, or clothesExposure to new pets (dander), dust, mold, chemicals, or plantsPregnancy (PUPPP)Contact with nickel (eg, jewelry, jean stud buttons), rubber (eg, gloves, elastic bands), latex, industrial chemicals, and nail polishSun exposure or cold exposure, exercise
40Urticaria Pathogenesis Increased capillary permeability, which allows proteins and fluids to extravasate.Due to histamine release from mast cells degranulating, which in turn recruits eosinophils, neutrophils and basophils.Other triggers are leukotrienes (slow reacting substances of anaphylaxis), prostaglandins, proteases, bradykinins
41Chronic Urticaria1/3 of these patients have circulating functional histamine-releasing autoantibodies that bind to the high-affinity IgE receptor producing mast cell-specific histamine releasing activityFc epsilon RIGet a good drug history: ACEIs, NSAIDS, Antibiotics
42H&E: collagen bundles separated by edema, perivascular infiltrate
43Urticaria & Angioedema Ddx: Clinical diagnosisDdx: Urticarial Vasculitis, Bullous Pemphigoid, GA, Sarcoidosis, CTCLMost of the diseases listed above have lesions that last longer than 24 hours.Biopsy urticarial lesions that last > 24 hours.
44Urticaria Evaluation Good H&P is most cost effective Dental and sinus x rays can be of benefitOrder laboratory tests based only on symptoms and signs from H&P including:Thyroid, LFTs, Hepatitis panel, ANA, CBC.Eosinophilia -> search for parasitesFood skin tests.
45Urticaria Treatment TX: OAH, multiple if necessary Simons et al, randomized, double blind parallel series of 23 “refractory” urticarias found 58% of patients preferred H1+H2 combinations.Atarax + Tagamet much better than Zyrtec and TagametCool bathingPramoxine, SarnaOral steroids rarely helpful
46Urticaria Treatment Foods to avoid: Fish and shellfish Pork Garlic, onionsMushroomsTomatoes, melons, strawberries, citrus fruits, pickles and relishesNuts, peanuts, cheeseRemove suspected food x 3 weeks then resume
47Anaphylaxis Acute, life threatening urticaria/angioedema 90%, SOB 60% Onset: peak severity within 5-30 minutesMC causes of serious anaphylactic reactions are: Anitbiotics, especially PCNs, NSAIDS, Radiographic contrast dyes2nd MC cause – hymenoptera, shellfish
48Anaphylaxis Mortality rate less than 10% Still account for vast majority of fatal reactions, peak onset 5-30 minutes.One of every 2700 hospital patients.500 annual fatalitiesTX: mL dose of 1:1000 dilution of epinephrine SQ q minutesIV Solumedrol 50mg q6h x 2-4 dosesBenadryl, aminophyliine, neb. Metaproterenol, O2, glucagon, intubation, IV fluids.
50Hereditary Angioedema 2nd to 4th decade, + Family history, ADMay occur q2 weeks, lasting 2 to 5 daysEyelid and lip involvement NOT SEEN.Face, hands, arms, legs, genitals buttocks, stomach, intestines, bladder affected.N/V, Colic, may mimic AppendicitisTriggers: minor trauma, surgery, sudden changes in temperature or sudden emotional stressPresence of urticaria rules out HA
51Hereditary Angioedema aka Quincke’s EdemaNO PRURITIS OR URTICARIA, + PAINLow C4, C1, C1q, C2 levelsLow or dysfunctional plasma C1 esterase inhibitor protein.25% of deaths from laryngeal edemaTx of choice: fresh frozen plasma, stanazol, tranexamic acid
52Type I and Type II HAType I – LOW serum levels of NORMAL C1 esterase inhibitor proteinType II – NORMAL levels of DYSFUNCTIONAL C1 esterase inhibitor protein.C4 best screening test, it will be low in both of the above cases.
53HA - Treatment25% of deaths due to HA are the result of laryngeal edemaTOC for acute HA is fresh frozen plasmaStanazol useful for short-term prophylaxis in patients undergoing dental surgery, endoscopic surgery or intubation.Tranexamic acid in low doses has few side effects and useful for acute or chronic HA.
54Acquired Angioedema Symptoms same as HA, but NO family hx. Aka Caldwell’s SyndromeOccurs at night, pt wakes up with it.Acute evanescent circumscribed edemaAffects most distensible tissues: eyelids, lips, earlobes, genitalia, mouth, tongue, larynx.Swelling is subcutaneous, not dermal.Overlying skin is not affected.
55Schnitzler’s Syndrome Chronic non-pruritic urticariaFever of unknown originDisabling bone painHyperostosisIncreased SED rateMacroglobulinemia (IgM Kappa)Tx: Oral Steroids
56Physical Urticarias 20% of all urticarias Dermatographism Cholinergic/AdrenergicCold/HeatSolarPressureExercise inducedAquagenicVibratory Angioedema
58DermatographismSharply localized wheal and flare seconds to minutes after stroking skin2% to 5% of the populationAssociated with penicillin induced urticaria, Pepcid (famotidine), hypothyroidism, hyperthyroidism, infectious disease, diabetes mellitus, onset of menopauseTx: OAH
60Cholinergic Urticaria Acetylcholine inducedTiny punctate extremely pruritic wheals or papules 1-3mm in diameter surrounded by erythemaMC trunk and face, spares palms & solesTriggers: exercise, heat,Tx: Cold shower, OAH high doseProvoke: Methacholine skin test, heat
61Adrenergic Urticaria Norepinephrine induced Small 1-5mm papules, +/- pale halo10-15 minutes after emotional upset, coffee or chocolateSerum adrenalin elevated, histamine nl.Tx: Propranolol 10mg QIDProvoke: 3 to 10 nanograms noradrenalin intradermally
62Cold Urticaria and Angioedema MC Face/hands, occurs with rewarming25% Patients atopicTx: PERIACTIN 4mg TIDDesensitize: repeated colder exposures.Test: Ice cube in saran wrap x 5-20 min.Assoc: Cryoglobulins, Myeloma, Syphillis, Hepatitis, MononucleosisFamilial variant – Bx LCV, Tx Stanazol
63Heat Urticaria 5 minutes Heat > 109.4 farenheit (43 C) Burns, stings, red, swollen, induratedMay become generalized with cramps, weakness, flushing, salivation and collapseTx: heat desensitizationProvoke heated cylinder 122 F x 30 min.
64Solar Urticaria Classified by the wavelength of light causing it. Visible light may cause it so sunscreens may be of little help.Sun Avoidance.OAHPUVA, Repetetive phototherapy.
65Pressure Urticaria 3-12 hours after local pressure has been applied. MC feet/walking and buttocks/sittingArthralgias, fever, chills, leukocytosis can occurTx: ORAL STEROIDS HELPFUL, ANTIHISTAMINES NO HELP!Provoke: 15 lb. weight x 20 minutes
66Exercise Induced Urticaria Not related to body temperatureWheals are larger than those seen in cholinergic urticariaStarts after 5-30 minutes of exercisePatients often atopicAvoid celery and gliadin or other food allergyTx: OAH
67Vibratory Angioedema AD or acquired Usually occupational in origin Plasma histamine levels elevated during attacksProvocation test: Laboratory vortex vibration applied for 5 minutesTx: OAH
68Aquagenic UrticariaWater, seawater, tears, sweat, saliva at any temperature may provokeImmediately or within minutes and clear within seconds.Wheezing, dysphagia, SOB may accompanyWater soluble antigens the etiology?Tx: Petrolatum, OAH, PUVA.