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Erythema and Urticaria August 12, 2003. Flushing  Transient diffuse redness of face & neck  Niacin, CCBs, cyclosporine, chemotx, vancomycin, bromocriptine,

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Presentation on theme: "Erythema and Urticaria August 12, 2003. Flushing  Transient diffuse redness of face & neck  Niacin, CCBs, cyclosporine, chemotx, vancomycin, bromocriptine,"— Presentation transcript:

1 Erythema and Urticaria August 12, 2003

2 Flushing  Transient diffuse redness of face & neck  Niacin, CCBs, cyclosporine, chemotx, vancomycin, bromocriptine, contrast dye, tamoxifen, leuprolide acetate, high dose methylprednisolone  Capsaicin (red pepper), sodium nitrate, sulfites, alcohol, food poisoning (ciguatera, scrombroid)  Carcinoid, Mastocytosis, Pheochromocytoma  Menopause, oophorectomy.

3 Erythema Palmare Elevated Estrogen Cirrhosis Liver CA metastatic Pregnancy

4 Erythema Toxicum Neonatorum Occurs in most healthy full term newborns, usually on 2 nd - 3 rd day. Multiple papules that rapidly evolve into pustules with an erythematous base Lesions may become confluent, especially on the face No fever, gone by 10 th day Ddx Miliaria, Herpes, Bacterial folliculitis, scabies Pustule smear revealing eosinophils is diagnostic. Bx shows follliculitis of eos and neuts

5 Erythema 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/fall  Mild or no prodrome x 1-4 weeks  Lesions evolve over 24-48 hours  “Target” or “iris” lesions are diagnostic

6 Erythema Multiforme

7 1) Central dusky purpuric area 2) Elevated edematous pale ring 3) Surrounding macular erythema

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

9 Erythema Multiforme Minor  Usually associated with orolabial HSV  Antivirals improve it and steroids worsen it  Appear 1-3 weeks after the herpes lesion  Sometimes EMM comes without herpes  Sometimes herpes comes without EMM

10 Oral Erythema Multiforme  Oral only in 45%, lip & oral 30%  Oral specialists usually handle this  Tongue, gingiva and buccal mucosa are the most severly affected.  Erosions +/- a pseudomembrane  Important to r/o Candida, because topical antifungal therapy leads to improvement in 40% of cases in which Candida is found, otherwise prednisone.

11 Oral Erythema Multiforme

12 EM 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.

13 Erythema Annulare Centrifugum

14 EAC: “coat in sleeve”

15 Erythema Annulare Centrifugum  Most common gyrate erythema  Polycyclic, trailing scale at inner border  Eccentric growth 2-3mm per day  Asymptomatic but chronic, recurrent  Look for tineas, rarely CA  Good H&P, CBC, LFT’s, UA and CXR

16 Erythema Gyratum Repens “WOOD GRAIN” APPEARANCE

17 Erythema Gyratum Repens  Rare  Undulating bands of slightly elevated wavy erythema over the entire body  “Wood grain” with “trailing scale”  Severe pruritis, eosinophilia  80% underlying malignancy, MC lung CA  Rash may precede CA by 9 months.  Remove CA, rash resolves.

18 Annular Erythema of Infancy  Rare  Onset: 6 months, resolves by 11 months  Lesions are transitory, last 36-48 hours  No treatment necessary

19 Necrolytic Migratory Erythema

20 NME 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.

21 Necrolytic Migratory Erythema  Aka Glucagonoma Syndrome  Amino Precursor and uptake decarboxylation (APUD) cell tumor of the pancreas  Elevated serum Glucagon, low Zinc  Pancreas scan may be normal  Distribution: periorificial, flexural, acral.  Papulovesicular lesions coalesce, form pustules then erode. Active erythematous gyrate or circinate borders with central confluence  Patients present ILL, with hyperglycemia, weight loss, diarrhea, anemia, atrophic glossitis, angular cheilitis

22 Erythema 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 papules  Resolves without tx in 2 weeks.  Brucella organisms not identified, this is a sensitization phenomenon.

23 Recurrent Granulomatous Dermatits with Eosinophilia  Aka Eosinophilic Cellulitis, Well’s Synd.

24 Dermal eos and histiocytes surrounding central masses of brightly pink collagen that has lost its fibrillar appearance and is more amporphous “FLAME FIGURES”

25 Well’s Syndrome  Clinical hybrid between cellulitis and urticaria.  Recurrent  Reaction 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

26 Erythema Nodosum  Young adult women  Crops of bilateral deep tender nodules, pretibial  Overlying skin shiny, red.  Onset acute with arthralgia, malaise, edema  2-3 days lesions flatten and have a bruised appearance, may last days or weeks

27 Erythema Nodosum in Sarcoid  MC nonspecific cutaneous finding in sarcoidosis  Young females  Anterior shins  Good prognosis  Lofgren’s Syndrome = fever, arthralgias, hilar adenopathy, fatigue, EN

28 Erythema Nodosum  Reactive Process  Strep, Yersinia, Salmonella, Shigella, Coccidiomycosis, Histoplasmosis, Sporotrichosis, Blastomycosis, Toxoplasmosis, TB, Sarcoidosis, Hematologic Malignancies, Pregnancy, Oral contraceptives  HISTO: Septal panniculitis

29 Sweet’s Syndrome


31  Sweet'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 disease  11% hematologic disease (including leukemia)  16% immunologic disease (rheumatoid arthritis, inflammatory bowel disease)  2% pregnancy  TX systemic corticosteroids  Overlap between sweets and pyoderma gangrenosum well documented.

32 Marshall’s Syndrome  Skin lesions that resemble Sweet’s but is followed by Cutis Laxa changes  Children  Small red papules expand to urticarial targetoid plaques with hypopigmented centers.  Eosinophilic infiltrated may be seen  Biopsies demonstrate loss of elastin

33 Marked diminution of elastic fibers in lower dermis (Verhoeff-van Gieson stain).

34 Pyoderma Gangrenosum ulcers with distinct rolled edges, sharply marginated, undermined blue to purple borders

35 Pyoderma Gangrenosum  Pathergy (Sweet’s too)  Heal with atrophic scars  Extremely painful  50% of pts have associated disease  MC: Crohn’s and Ulcerative Colitis  1/3 of PG patients have arthritis  Other associated: Leukemia, Myeloma, Polycythemia vera, Hep C, SLE, HIV, pregnancy, Takayasu’s arteritis

36 Pyoderma 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

37 TX: Pyoderma Gangrenosum  Excise colon segment for IBS, UC, Crohns  Rule out/treat malignancy or infection  Steroids: topical, IL or oral depending on severity and aggressiveness  Topical 4% cromolyn or tacrolimus  Hyperbaric oxygen- rapid pain relief  Cyclosporine, Sulfasalazine, Dapsone, Clofazimine, Azathioprine, Mycophenolate, IVIG, Plasma exchange

38 Urticaria

39 History: Use a questionnaire!  Recent illness (eg, fever, sore throat, cough, rhinorrhea, vomiting, diarrhea, headache) INFECTIOUS: STREP, HEP C, H. PYLORI  Medication 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 clothes  Exposure to new pets (dander), dust, mold, chemicals, or plants  Pregnancy (PUPPP)  Contact with nickel (eg, jewelry, jean stud buttons), rubber (eg, gloves, elastic bands), latex, industrial chemicals, and nail polish  Sun exposure or cold exposure, exercise

40 Urticaria 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

41 Chronic Urticaria  1/3 of these patients have circulating functional histamine-releasing autoantibodies that bind to the high-affinity IgE receptor producing mast cell-specific histamine releasing activity  Fc epsilon RI  Get a good drug history: ACEIs, NSAIDS, Antibiotics

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

43 Urticaria & Angioedema Ddx:  Clinical diagnosis  Ddx: Urticarial Vasculitis, Bullous Pemphigoid, GA, Sarcoidosis, CTCL  Most of the diseases listed above have lesions that last longer than 24 hours.  Biopsy urticarial lesions that last > 24 hours.

44 Urticaria Evaluation  Good H&P is most cost effective  Dental and sinus x rays can be of benefit  Order laboratory tests based only on symptoms and signs from H&P including:  Thyroid, LFTs, Hepatitis panel, ANA, CBC.  Eosinophilia -> search for parasites  Food skin tests.

45 Urticaria 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 Tagamet  Cool bathing  Pramoxine, Sarna  Oral steroids rarely helpful

46 Urticaria Treatment  Foods to avoid: Fish and shellfish  Pork  Garlic, onions  Mushrooms  Tomatoes, melons, strawberries, citrus fruits, pickles and relishes  Nuts, peanuts, cheese  Remove suspected food x 3 weeks then resume

47 Anaphylaxis  Acute, life threatening  urticaria/angioedema 90%, SOB 60%  Onset: peak severity within 5-30 minutes  MC causes of serious anaphylactic reactions are: Anitbiotics, especially PCNs, NSAIDS, Radiographic contrast dyes  2 nd MC cause – hymenoptera, shellfish

48 Anaphylaxis  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 fatalities  TX: 0.3 - 0.5mL dose of 1:1000 dilution of epinephrine SQ q 10-20 minutes  IV Solumedrol 50mg q6h x 2-4 doses  Benadryl, aminophyliine, neb. Metaproterenol, O2, glucagon, intubation, IV fluids.

49 Angioedema

50 Hereditary Angioedema  2 nd to 4 th decade, + Family history, AD  May occur q2 weeks, lasting 2 to 5 days  Eyelid and lip involvement NOT SEEN.  Face, hands, arms, legs, genitals buttocks, stomach, intestines, bladder affected.  N/V, Colic, may mimic Appendicitis  Triggers: minor trauma, surgery, sudden changes in temperature or sudden emotional stress  Presence of urticaria rules out HA

51 Hereditary Angioedema  aka Quincke’s Edema  NO PRURITIS OR URTICARIA, + PAIN  Low C4, C1, C1q, C2 levels  Low or dysfunctional plasma C1 esterase inhibitor protein.  25% of deaths from laryngeal edema  Tx of choice: fresh frozen plasma, stanazol, tranexamic acid

52 Type I and Type II HA  Type I – LOW serum levels of NORMAL C1 esterase inhibitor protein  Type II – NORMAL levels of DYSFUNCTIONAL C1 esterase inhibitor protein.  C4 best screening test, it will be low in both of the above cases.

53 HA - Treatment  25% of deaths due to HA are the result of laryngeal edema  TOC for acute HA is fresh frozen plasma  Stanazol 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.

54 Acquired Angioedema  Symptoms same as HA, but NO family hx.  Aka Caldwell’s Syndrome  Occurs at night, pt wakes up with it.  Acute evanescent circumscribed edema  Affects most distensible tissues: eyelids, lips, earlobes, genitalia, mouth, tongue, larynx.  Swelling is subcutaneous, not dermal.  Overlying skin is not affected.

55 Schnitzler’s Syndrome  Chronic non-pruritic urticaria  Fever of unknown origin  Disabling bone pain  Hyperostosis  Increased SED rate  Macroglobulinemia (IgM Kappa)  Tx: Oral Steroids

56 Physical Urticarias  20% of all urticarias  Dermatographism  Cholinergic/Adrenergic  Cold/Heat  Solar  Pressure  Exercise induced  Aquagenic  Vibratory Angioedema

57 Dermatographism

58 Dermatographism  Sharply localized wheal and flare seconds to minutes after stroking skin  2% to 5% of the population  Associated with penicillin induced urticaria, Pepcid (famotidine), hypothyroidism, hyperthyroidism, infectious disease, diabetes mellitus, onset of menopause  Tx: OAH

59 Cholinergic Urticaria

60 Cholinergic Urticaria Cholinergic Urticaria  Acetylcholine induced  Tiny punctate extremely pruritic wheals or papules 1-3mm in diameter surrounded by erythema  MC trunk and face, spares palms & soles  Triggers: exercise, heat,  Tx: Cold shower, OAH high dose  Provoke: Methacholine skin test, heat

61 Adrenergic Urticaria  Norepinephrine induced  Small 1-5mm papules, +/- pale halo  10-15 minutes after emotional upset, coffee or chocolate  Serum adrenalin elevated, histamine nl.  Tx: Propranolol 10mg QID  Provoke: 3 to 10 nanograms noradrenalin intradermally

62 Cold Urticaria and Angioedema  MC Face/hands, occurs with rewarming  25% Patients atopic  Tx: PERIACTIN 4mg TID  Desensitize: repeated colder exposures.  Test: Ice cube in saran wrap x 5-20 min.  Assoc: Cryoglobulins, Myeloma, Syphillis, Hepatitis, Mononucleosis  Familial variant – Bx LCV, Tx Stanazol

63 Heat Urticaria  5 minutes  Heat > 109.4 farenheit (43 C)  Burns, stings, red, swollen, indurated  May become generalized with cramps, weakness, flushing, salivation and collapse  Tx: heat desensitization  Provoke heated cylinder 122 F x 30 min.

64 Solar Urticaria  Classified by the wavelength of light causing it.  Visible light may cause it so sunscreens may be of little help.  Sun Avoidance.  OAH  PUVA, Repetetive phototherapy.

65 Pressure Urticaria  3-12 hours after local pressure has been applied.  MC feet/walking and buttocks/sitting  Arthralgias, fever, chills, leukocytosis can occur  Tx: ORAL STEROIDS HELPFUL, ANTIHISTAMINES NO HELP!  Provoke: 15 lb. weight x 20 minutes

66 Exercise Induced Urticaria  Not related to body temperature  Wheals are larger than those seen in cholinergic urticaria  Starts after 5-30 minutes of exercise  Patients often atopic  Avoid celery and gliadin or other food allergy  Tx: OAH

67 Vibratory Angioedema  AD or acquired  Usually occupational in origin  Plasma histamine levels elevated during attacks  Provocation test: Laboratory vortex vibration applied for 5 minutes  Tx: OAH

68 Aquagenic Urticaria  Water, seawater, tears, sweat, saliva at any temperature may provoke  Immediately or within minutes and clear within 30-60 seconds.  Wheezing, dysphagia, SOB may accompany  Water soluble antigens the etiology?  Tx: Petrolatum, OAH, PUVA.

69  The End

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