Chapter 7 Erythema & Urticaria

Question 1

Drugs associated with flushing

Answer 1

Niacin
CCB 
Cyclosporine 
Chemo agents
Vancomycin 
Bromocriptine 
IV contrast 
Sildenafil
High dose methylpred
MAO with SSRI

Question 2

Differentiate EM minor and major

Answer 2

EM minor - herpes simplex associated

EM major - more intense lesions, fever, arthralgia; mycoplasma infection

Question 3

Clinical lesion of EM minor

Answer 3

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

Question 4

Area of predilection for EM major

Answer 4

Extremities and face
Oral mucosa and lips
Genital
Ocular

(SJS distinguished by presence of purpura or bullae in macular lesions of trunk)

Question 5

Treatment for oral EM

Answer 5

Swish and spit

Lidocaine, dyphen,kaolin

Question 6

Gyrate erythemas often represent cutaneous manefestation of

Answer 6

Infection
Malignancy
Drug rxn

Question 7

Most common gyrate erythema

Answer 7

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

Question 8

Most common associated malignancy in erythema gyratum repens

Answer 8

Lung cancer

Question 9

Etiology, histology, tx of wells syndrome

Answer 9

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

Question 10

Reactive neutrophilic dermatoses tend to follow stimuli such as

Answer 10

URIs
IBD
hematologic diseases

Question 11

Primary skin lesion of sweet syndrome

Answer 11

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

Question 12

Presentation of pregnancy associated sweet syndrome

Answer 12

First or 2nd trimester
Head neck trunk less on upper ex
Resolve spontaneously or clear with topical or systemic ccs

Question 13

2 major criteria for dx of sweet syndrome

Answer 13

Red edematous plaques

Biopsy showing neutrophils karyorrhexis and marked papillary dermal edema

Question 14

Treatment of sweet syndrome

Answer 14

1mg/kg/day of oral prednisone

Question 15

Presentation of classic pyoderma gangrenosum

Answer 15

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

Question 16

Pustular PG consists of pustules that do not progress to ulcers, found most often in what disease

Answer 16

IBD

Question 17

Characteristics of bullous and vegetative PG

Answer 17

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

Question 18

Treatment of PG

Answer 18

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

Question 19

Most common inherited autoinflammatory syndrome and characteristics

Answer 19

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

Question 20

Characteristics of PAPA syndrome

Answer 20

Pyogenic sterile arthritis, PG, acne

AD, Mutation in PSTPIP1 and CD2BP1 genes - involved in cytoskeletal organization, interacts with pyrin

Question 21

Most common autoinflammatory syndromes in adulthood

Answer 21

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

Question 22

Hyper IgD syndrome mutation

Answer 22

MVK gene

AR, morbilliform urticarial eruptions, oral and genital ulcers may occur

Question 23

Characteristics of CANDLE

Answer 23

Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temp
Mutation in PSMB8 gene
Biopsy - atypical cells of myelocytic lineage in dermis

Question 24

Characteristics of Blau syndrome

Answer 24

AD, arthritis uveitis granulomatous inflammation camptodactyly
Mutations in NOD2 gene
Predisposes to crohn’s and early onset sarcoidosis

Question 25

Characteristics of urticaria

Answer 25

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

Question 26

Most common form of physical/inducible urticaria

Answer 26

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

Question 27

Primary effector cell in urticaria

Answer 27

Mast cell

Capillary permeability results from increased antihistamine from mast cells situated around capillaries

Question 28

Histopath in acute urticaria

Answer 28

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

Question 29

Tx for severe urticaria

Answer 29

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

Question 30

Tx of chronic urticaria

Answer 30

If after 2 weeks it persists -> increase dose to 4x standard dose, 2 pills in morning and evening

Question 31

Prognosis of chronic urticaria

Answer 31

20-50% of patients continue to have chronic spontaneous urticaria after 5yrs

Question 32

Characteristics of quincke edema

Answer 32

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