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Erythema and Urticaria August 31, 2004 |
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Flushing Transient, diffuse redness of face/neck/trunk
Niacin, Calcium Channel Blockers, 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 |
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Erythema Palmare Hypothenar erythema
Elevated Estrogen
Cirrhosis
Metastatic Liver CA
Pregnancy |
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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 with eosinophils and neutrophils |
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Erythema Multiforme Minor AKA Herpes Simplex-Associated EM (HAEM)
Minor is typically associated with orolabial HSV
Major (SJS) is associated with sulfonamides, NSAIDS, antibiotics, allopurinol, Mycoplasma pneumoniae, anticonvulsants, and radiation
Cause not identified in 20% of cases
Self-limited, recurrent, young adults, spring/fall
Mild or no prodrome lasting 1-4 weeks
Lesions evolve over 24-48 hours
“Target” or “iris” lesions are diagnostic |
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Erythema Multiforme |
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1) Central dusky purpuric area 2) Elevated edematous pale ring 3) Surrounding macular erythema |
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EM: Vacuolar interface with “tagging” of lymphocytes along DEJ with necrotic and apoptotic keratinocytes Cytoid Bodies |
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Erythema Multiforme Minor Locations: dorsal feet, limbs, elbows, knees, palms and soles
EMM appears 1-3 weeks after the herpes lesion
Tx: Self-limited, supportive care
If HSV: antivirals improve/steroid worsen; sunblock
If SJS or TEN, stop suspect medications, admit to burn unit, IVIG, steroids, etc.
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Oral Erythema Multiforme
Usually limited to orolabial involvement
Concomitant skin involvement in 25%
Tongue, gingiva and buccal mucosa are the most severly affected
Erosions +/- pseudomembrane
Important to r/o Candida, because topical antifungal therapy leads to improvement in 40% of cases in which Candida is found, otherwise prednisone |
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Oral Erythema Multiforme |
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Erythema Annulare Centrifugum Most common gyrate erythema
Unknown pathogenesis
Polycyclic, trailing scale at inner border
Eccentric growth 2-3mm per day
Asymptomatic but chronic, recurrent
Evaluate for Tinea
Recommended: Good H&P, CBC, LFT’s, UA and CXR to r/o internal cancer
Responsive to topical steroids |
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Erythema Annulare Centrifugum |
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EAC: “coat in sleeve” = lymphos tightly associated with vessels |
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Erythema Gyratum Repens Rare
Undulating bands of slightly elevated wavy erythema over the entire body
“Wood grain” with “trailing scale”
Severe pruritis; eosinophilia often found
80% underlying malignancy, MC lung CA
Rash may precede CA by 9 months
Remove CA, rash resolves |
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Erythema Gyratum Repens “WOOD GRAIN” APPEARANCE |
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Annular Erythema of Infancy Rare
Lesions are transitory, last 36-48 hours
Onset: 6 months, resolves by 11 months without treatment |
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Necrolytic Migratory Erythema AKA Glucagonoma Syndrome
Associated with amino precursor and uptake decarboxylation (APUD) tumor of the pancreas
Increased glucagon, decreased zinc
Pancreas scan may be normal
Location: 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
Tx: removal of tumor |
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Necrolytic Migratory Erythema |
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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. |
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Erythema Brucellum Vets and Cow tenders
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 in lesions, suggesting a sensitization phenomenon |
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Recurrent Granulomatous Dermatitis with Eosinophilia 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 |
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Recurrent Granulomatous Dermatits with Eosinophilia AKA Eosinophilic Cellulitis, Well’s Syndrome |
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Dermal eos and histiocytes surrounding central masses of brightly pink collagen that has lost its fibrillar appearance and is more amorphous “FLAME FIGURES” |
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Erythema Nodosum Young adult women
Crops of bilateral deep tender nodules, pretibial
Overlying skin shiny, red
Acute onset with arthralgia, malaise, edema
In 2-3 days, lesions flatten and have a bruised appearance; may last days or weeks |
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Erythema Nodosum in Sarcoid MC nonspecific cutaneous finding in sarcoidosis
Young females
Anterior shins
Good prognosis
Lofgren’s Syndrome = EN with fever, arthralgias, hilar adenopathy, & fatigue |
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Erythema Nodosum Reactive Process
Strep, Yersinia, Salmonella, Shigella, Coccidiomycosis, Histoplasmosis, Sporotrichosis, Blastomycosis, Toxoplasmosis, TB, Sarcoidosis, Hematologic Malignancies, Pregnancy, Oral contraceptives
HISTO: Septal panniculitis |
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Sweet’s Syndrome |
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MAY BURN, BUT DO NOT ITCH
PATHERGY |
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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
The overlap between sweets and pyoderma gangrenosum well documented |
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Marshall’s Syndrome Skin lesions that resemble Sweet’s but is followed by Cutis Laxa changes
Primarily, affects children
Small red papules expand to urticarial targetoid plaques with hypopigmented centers
Eosinophilic infiltrate may be seen
Biopsies demonstrate loss of elastin |
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Marked diminution of elastic fibers in lower dermis (Verhoeff-van Gieson stain) |
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Pyoderma Gangrenosum Pathergy, as in Sweet’s Syndrome
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 associations: leukemia, myeloma, polycythemia vera, Hep C, SLE, HIV, pregnancy, Takayasu’s arteritis |
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Pyoderma Gangrenosum ulcers with distinct rolled edges, sharply marginated, undermined blue to purple borders |
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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 Wegener’s granulomatosis |
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TX: Pyoderma Gangrenosum Excise colon segment for IBS, UC, Crohn’s
Rule out/treat malignancy or infection
Steroids: topical, IL or oral depending on severity and aggressiveness
Topical 4% cromolyn or tacrolimus
Hyperbaric oxygen leads to rapid pain relief
Cyclosporine, Sulfasalazine, Dapsone, Clofazimine, Azathioprine, Mycophenolate, IVIG, Plasma exchange |
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History is Key
Illness: e.g., fever, sore throat, cough, rhinorrhea, vomiting, diarrhea, headache -- INFECTIOUS: STREP, HEP C, H. PYLORI
Medications: 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: e.g., 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 |
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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 |
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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 |
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H&E: collagen bundles separated by edema, perivascular infiltrate |
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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 |
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Urticaria Evaluation Dental and sinus x-rays can be of benefit
Order laboratory tests based only on symptoms and signs from H&P including:
TSH, LFTs, Hepatitis panel, ANA, CBC
Eosinophilia: search for parasites
Food skin tests
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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 |
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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 |
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Anaphylaxis Acute, life threatening
urticaria/angioedema 90%, SOB 60%
Onset: peak severity within 5-30 minutes
MC causes of serious anaphylactic reactions are: antibiotics, especially PCNs, NSAIDS, radiographic contrast dyes
2nd MC cause – hymenoptera, shellfish |
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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, IVFs |
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Hereditary Angioedema 2nd to 4th decade, +Family history, Autosomal Dominant
May occur q 2 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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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 are from laryngeal edema
Tx of choice: fresh frozen plasma, stanazol, tranexamic acid |
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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 is best screening test, it will be low in both of the above cases |
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HA - Treatment 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 -- useful for acute or chronic HA |
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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 |
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Schnitzler’s Syndrome Chronic non-pruritic urticaria
Fever of unknown origin
Disabling bone pain
Hyperostosis
Increased ESR
Macroglobulinemia (IgM Kappa)
Tx: Oral Steroids |
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Physical Urticarias 20% of all urticarias
Dermatographism
Cholinergic/Adrenergic
Cold/Heat
Solar
Pressure
Exercise-induced
Aquagenic
Vibratory angioedema |
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Dermatographism |
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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 |
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Cholinergic Urticaria |
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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 |
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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 |
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Cold Urticaria and Angioedema MC face/hands, occurs with rewarming
25% patients are atopic
Tx: PERIACTIN 4mg TID
Desensitize: repeated colder exposures
Test: Ice cube in saran wrap x 5-20 min.
Associations: Cryoglobulins, Myeloma, Syphillis, Hepatitis, Mononucleosis
Familial variant – Bx: LCV; Tx: Stanazol |
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Heat Urticaria occurs within 5 minutes of exposure
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. |
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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; repetitive phototherapy |
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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 |
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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 |
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Vibratory Angioedema Autosomal Dominant or acquired
Usually occupational in origin
Plasma histamine levels elevated during attacks
Provocation test: Laboratory vortex vibration applied for 5 minutes
Tx: OAH |
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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 |
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