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Erythema and Urticaria

Erythema and Urticaria. August 12, 2003. Flushing. Transient diffuse redness of face & neck Niacin, CCBs, cyclosporine, chemotx, vancomycin, bromocriptine, contrast dye, tamoxifen, leuprolide acetate, high dose methylprednisolone

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Erythema and Urticaria

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  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 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 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 area2) Elevated edematous pale ring3) Surrounding macular erythema

  8. EM: Vacuolar interface with “tagging” of lymphocytes along DEJ with necrotic and apoptotic keratinocytes 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. 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. NME path identical to Zinc Defic.

  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. 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 Erythema Nodosum

  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

  30. MAY BURN, BUT DO NOT ITCH PATHERGY

  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 • 2nd 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 • 2nd to 4th 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

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