Chapter 7 Erythema & Urticaria Flashcards
Drugs associated with flushing
Niacin CCB Cyclosporine Chemo agents Vancomycin Bromocriptine IV contrast Sildenafil High dose methylpred MAO with SSRI
Differentiate EM minor and major
EM minor - herpes simplex associated
EM major - more intense lesions, fever, arthralgia; mycoplasma infection
Clinical lesion of EM minor
Begin as sharply marginated erythematous papules over 24-48h, target/iris lesion (central dusky purpura, elevated edematous pale ring, surrounding macular erythema)
Palms and soles, symmetrically and acrally, initially on dorsal hands
Dorsal feet, extensor limbs, elbows, knees, palms, and soles
Area of predilection for EM major
Extremities and face
Oral mucosa and lips
Genital
Ocular
(SJS distinguished by presence of purpura or bullae in macular lesions of trunk)
Treatment for oral EM
Swish and spit
Lidocaine, dyphen,kaolin
Gyrate erythemas often represent cutaneous manefestation of
Infection
Malignancy
Drug rxn
Most common gyrate erythema
Erythema annulare centrifugum
- trailing scale at inner border of annular erythema
- trunk and proximal extremities
- majority idiopathic, some associated with dermatophytosis
Ddx: granuloma annulare, secondary syphilis, tinea, scle, sarcoidosis, hansen’s, erythema marginatum/migrans, annular urticaria, mycoses fungoides
Most common associated malignancy in erythema gyratum repens
Lung cancer
Etiology, histology, tx of wells syndrome
Or eosinophilic cellulitis
Most cases represent arthropod rxn, associated with onchoceriasis, intestinal parasites, varicella, mumps, immunization, anti TNF alpha agents, myeloprolif dse, atopic diathesis, angioimmunoblastic LAD,IBD, hyperEos syndrome, churg strauss, fungal
Tx: topical/IL ccs, oral antihistamines, tacrolimus ointment, minocycline, UVB,PUVA,dapsone,low dose prednisone
Reactive neutrophilic dermatoses tend to follow stimuli such as
URIs
IBD
hematologic diseases
Primary skin lesion of sweet syndrome
Or acute febrile neutrophilic dermatosis
Sharply marginated,rapidly extending,tender,erythematous,violaceous, painful elevated aque 2-10cm in diameter
Face neck upper trunk extremities
Lasts 3-6wks then resolves
Histo hallmark: nodular diffuse dermal infiltrate of neutro with karyorrhexis and massive papillary dermal edema
Presentation of pregnancy associated sweet syndrome
First or 2nd trimester
Head neck trunk less on upper ex
Resolve spontaneously or clear with topical or systemic ccs
2 major criteria for dx of sweet syndrome
Red edematous plaques
Biopsy showing neutrophils karyorrhexis and marked papillary dermal edema
Treatment of sweet syndrome
1mg/kg/day of oral prednisone
Presentation of classic pyoderma gangrenosum
Inflammatory pustule with surrounding halo that enlarges and begins to ulcerate
Fully developed lesions - painful ulcers with sharply marginated, undermined, blue to purple borders, heal with thin atrophic scars
Age 40-60
Lower ex and trunk
Pustular PG consists of pustules that do not progress to ulcers, found most often in what disease
IBD
Characteristics of bullous and vegetative PG
Bullous - superficial less destructive, overlap with “bullous sweet syndrome”, usually in leukemia and PV, not deep, less painful
Vegetative - least aggressive, chronic superficial cribriform ulcerations usually on trunk, enlarge slowly with elevated borders and clean bases, rarely painful, not associated with systemic disease
Treatment of PG
Systemic ccs 1mg/kg
If unresponsive to oral ccs -> pulse methylprednisone 1mg/kg 3-5days ff by 40-60 prednisone tapering as lesions heal
If still unresponsive -> cyclosporine 5mg/kg/day up to 10 or infliximab IV 5mg/kg at 0 2 6 weeks then q8weeks
Most common inherited autoinflammatory syndrome and characteristics
Familial mediterranean fever / FMF
AR, recurrent 12-72h fever, monoarthritis with overlying erysipelas like erythema
Peritonitis pleuritis vasculitis HSP may occur to those presented before teens
Mutation in MEFV gene producing pyrin
Colchicine as mainstay of tx -> rilonacept if resistant
Characteristics of PAPA syndrome
Pyogenic sterile arthritis, PG, acne
AD, Mutation in PSTPIP1 and CD2BP1 genes - involved in cytoskeletal organization, interacts with pyrin
Most common autoinflammatory syndromes in adulthood
TRAPS - TNF receptor assoc periodic syndrome, AD, longer attacks, lack of response to colchicine, mutation in TNFRSF1A, decreased serum soluble TNF receptor
DITRA - def of IL 36 rec anta, febrile eps 1-3w with periorbital edema, painful distally migrating erythematous urticarial like plaques
Tx NSAIDs, prednisones for acute, anti TNF rec anta or anakinra may prevent bouts
Hyper IgD syndrome mutation
MVK gene
AR, morbilliform urticarial eruptions, oral and genital ulcers may occur
Characteristics of CANDLE
Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temp
Mutation in PSMB8 gene
Biopsy - atypical cells of myelocytic lineage in dermis
Characteristics of Blau syndrome
AD, arthritis uveitis granulomatous inflammation camptodactyly
Mutations in NOD2 gene
Predisposes to crohn’s and early onset sarcoidosis
Characteristics of urticaria
Vascular rxn of skin, evanescent wheals, rarely lasts >12h with complete resolution within 6wks
Chronic urticaria >6wks
Caused by infections ingestants inhalants injections
Children - URIs, viral
Adults & children - drugs, foods
Most common form of physical/inducible urticaria
Dermatographism - localized edema/wheal with surrounding erythematous flare occuring seconds to min after being stroked
Cholinergic - produced by axn of Ach on mast cell; minute higly pruritic, punctuate wheals or papules 1-3mm in diameter surround by erythematous flare
Cold - exposure result to edema and whealing on exposed areas usually face and hands, occurs on rewarming
Primary effector cell in urticaria
Mast cell
Capillary permeability results from increased antihistamine from mast cells situated around capillaries
Histopath in acute urticaria
Mild dermal edema and margination of neutrophils within postcapillary venules
Tx: antihistamines as mainstay but if unresponsive change to 3 week tapered course of systemic ccs
Tx for severe urticaria
0.3mL dose of 1:1000 dilution of epi q10-20min as needed
Adjunct: IM 25-50mg hydroxyzine or diphenhydramine q6h and 250mg hydrocortisone or 50mg methylpred IV q6h for 2-4 doses
Tx of chronic urticaria
If after 2 weeks it persists -> increase dose to 4x standard dose, 2 pills in morning and evening
Prognosis of chronic urticaria
20-50% of patients continue to have chronic spontaneous urticaria after 5yrs
Characteristics of quincke edema
Or hereditary angioedema
Before age 20, q2wks lasting 2-5d
Minimal response to antihistamines epi or ccs
Mortality high often due to laryngeal edema
Triggered by minor trauma,surgery,sudden temp changes,sudden emotional stress
Types I II III
Tx for type I & II - ecallantide or icatibant; danazol for III
