Inflammatory Dermatosis and Blistering Diseases--Westra

Question 1

Urticaria

Answer 1

Affects Dermis

Dermal edema

immunologic (IgE and histamine), mast cell–mediated condition that may be allergic or nonallergic.

Transient Wheals

Pruritic>(painful)

Ask about new exposures/medications

Question 2

Angioedema

Answer 2

severe Urticaria

Intense dermal and subcutaneous swelling

Burning/Painful>pruritic

Laryngeal Involvement = Emergency

Typically a medication reaction

Question 3

Urticaria and Angioedema–how would you begin to discover the cause?

Answer 3

Ask questions! Take a thorough history.

Medications, food, travel, physical stimuli

Question 4

pruritic

Answer 4

itching

Question 5

Urticaria and Angioedema: Immune causes

Answer 5

A. Type 1 IgE mediated

Foods: shellfish*, fish*, peanuts*, tree nuts*, eggs, milk, soy, wheat. (* seen in adults) (seen in kids)

Latex

Stinging insects including hymenoptera (bees, wasps, hornets), fire ants, bedbugs, fleas, mites.

Medications: penicillin, cephalosporin, sulfa
Aeroallergens: dust mites, pollens, molds, animal dander

B. Autoimmune disease: Hashimoto’s immune thyroiditis (production of anti-thyroid antibodies), SLE, vasculitis.
(there may be thyroid anti-autobodies in the absence of thyroid dysfunction)

C. Infection: viral (cytomegalovirus, Epstein-Barr, HIV, hepatitis A, B & C); parasitic, fungal or bacterial.

Question 6

Urticaria and Angioedema: Non-immune causes

Answer 6

A. Physical urticaria
B. Direct mast cell de-granulation
C. Foods containing high levels of histamine

Question 7

Small urticarial papules on red skin (axon reflex erythema) occurring on the neck within 30 minutes of vigorous exercise.

Answer 7

Cholinergic Uticaria

Question 8

as it appeared 5 minutes after stroking the skin with a wooden stick. The patient had experienced generalized pruritus for several months with no spontaneously occurring urticaria.

Answer 8

Dermographism

Question 9

Urticaria and Angioedema Laboratory Evaluation

Answer 9

Rule 1: don’t get carried away! Expensive lab evaluations usually have poor yields.

Punch biopsy to exclude vasculitis if lesion persists > 48 hrs or looks atypical

Question 10

Urticaria and Angioedema Tx

Answer 10

Avoid agitating cause!

First choice: Second generation, non-sedating H1-blockers

Second choice: (if symptoms not controlled, add) first generation, sedating H1 blockers

…

Last choice: add oral corticosteroid. Prednisone 40 mg →5 mg (course tapered over 10-14 days)

Question 11

Reaction pattern of blood vessels in the dermis

Answer 11

Erythema multiforme

erythematous iris-shaped papular and vesiculobullous lesions

an immune-mediated dermatologic disease of young adults and young children in which target lesions appear on the hands, feet, and the extensor surfaces of the limbs

Question 12

EM minor

Answer 12

erythema multiforme minor

mild form involving 1 mucosal site. major cause is herpes simplex infection with onset of EM rash at day 10

Question 13

EM major

Answer 13

erythema multiforme major

**severe with extensive skin and mucous membrane involvement. **

Stevens-Johnson Syndrome

Usually due to drugs (sulfa, PCN, dilantin) and after mycoplasma pneumoniae infection

Question 14

Stevens Johnson Syndrome

and

Toxic Epidermal Necrolysis

Answer 14

SJS: a hypersensitivity reaction commonly caused by drugs; infection can also be a trigger

Skin Tenderness and Erythema of Skin and Mucosa

Extensive Cutaneous and Mucosa Epidermal Necrosis and Sloughing

Potentially Life Threatening

Question 15

How do you differentiate between:

Erythema Multiforme

Stevens-Johnson Syndrome (SJS)

Toxic Epidermal Necrolysis

Answer 15

EM = 1 mucosal membrane

SJS = EM major (2+ mucosal membrane an <10% epidermal detachment)

TEN = maximal variant of SJS (2+ mucosal membrane and 30% epidermal detachment)

Question 16

Fixed Drug Eruption

Answer 16

a localized, sharply demarcated erythematous patch that can itch, burn or be asymptomatic

Question 17

Panniculitis

Answer 17

Major focus of inflammation: subcutaneous tissue

Described as lobular or septal depending on where disease process begins

Accurate diagnosis by skin biopsy

Question 18

Panniculitis:

Erythema Nodosum

Answer 18

Erythematous tender nodules

Typically ANTERIOR shins

Young women most common

Triggers
Infection (strep, TB, fungal)
Meds (OCP, Sulfa, NSAIDS)
Autoimmune (IBD, Sarcoid)

Treatment: rest, ice, pain control

Question 19

Panniculitis:

Erythema Induratum

Answer 19

Tender red nodules

Lobular panniculitis and vasculitis

Middle aged, usually female

Posterior legs>ant

Chronic, recurrent subcutaneous nodules and plaques with ulceration

Associated with TB

Question 20

6 Ps

Answer 20

LICHEN PLANUS (6 Ps)

1. Planar (flat topped)
2. Purple
3. Polygonal
4. Pruritic
5. Papules
6. Plaques

Erythematous to violaceous polygonal papules especially on the flexor areas such as wrists and ankles

Question 21

Subacute cutaneous lupus

Answer 21

A chronic multisystem autoimmune disease that predominantly affects the skin and joints, although any body system can be involved.

The most common presentation is a butterfly rash on the face, low-grade fever, and nondeforming arthritis

Question 22

Psoriasis (General)

Answer 22

common chronic skin disorder characterized by excessive proliferation of keratinocytes, resulting in the formation of thickened, scaly plaques, and inflammation, resulting in erythema and often pruritus

Risk factors: 30% genetic, stress, medications, infections, drugs (beta blockers, antimalarials)

Question 23

Post strptococcal infection

Answer 23

Psoriasis:

Guttate Type

Post strptococcal infection

children or young adults

eruptive trunkal dermatosis

Question 24

What is this?

Answer 24

Psoriasis: Pustular type

Generalized: Potentially life threating; Small pustules becoming generalized with fever

Localized: Hand and foot form involves the palms and soles. May be termed Pustular Psoriasis of Barber

Question 25

Hallmark of hives?

Answer 25

wheals

Question 26

**Butterfly rash** across the nose and the cheeks. Photosensitive pattern of erythema accross upper chest and extensor areas. Triggers: Sunlight and Medications (anti-seizure, antibiotics, BP Rx) Treat with NSAIDs, antimalarials, corticosteroids

Answer 26

Systemic Lupus Erythematosus (SLE)

Question 27

Hallmark is development of **Wickham’s Striae** Chronic, inflammatory, autoimmune disease Association with Hepatitis C (HCV) Location: wrists, shins, mucous membranes, Wickham’s stria (lacy, reticular, white lines)

Answer 27

lichen planus

Question 28

Group of vesicles on a red base which rapidly become purulent and crusted Two types

Answer 28

Herpes Simplex

Question 29

“dew drop on a rose petal” Incubation: average 14 days fever, chills, malaise, 2-3 days before onset of rash

Answer 29

**Varicella – Zoster** virus (Chickenpox)

Question 30

Primary infection usually occurs in childhood with lesions on the lips or face

Answer 30

HSV 1

Question 31

Disease of adults involving the genital area, primary infection extensive, painful vesiculations and necrosis Recurrent, lifelong disease with no cure

Answer 31

HSV 2

Question 32

Follows nerve root – **single dermatome**. Prodrome: pain along nerve root up to 5 days prior to **rash** Recurrence of varicella

Answer 32

Herpes Zoster (Shingles) Treatment options: Acyclovir, Prednisone, IV Acyclovir

Question 33

Bullae (bull’s eye filled with fluid), blisters occurring in the axillary, groin, fold areas most commonly. Superficial vesicles progress to rapidly enlarging, flaccid bullae with sharp margins and no surrounding erythema. When the bullae rupture, **yellow crusts with oozing result**.

Answer 33

Bullous Impetigo Caused by toxin producing Staph Treat with: Hygenic Measures Topical Antibiotics Oral Antibiotics

Question 34

Begins as a single red macule or papule that quickly becomes a vesicle. The vesicle ruptures easily to form an erosion, and the contents dry to form characteristic **honey-colored crusts** that may be pruritic.

Answer 34

Nonbullous Impetigo 70% of cases of impetigo are nonbullous

Question 35

Erythema, small pustules, fringe of white scale. Cutaneous and mucosal (thrush, vulval vaginitis)

Answer 35

Yeast-Candida Albicans

Question 36

Common in skin folds and on mucous membranes. Bright beefy red dermatitis surrounded by satellite micropustules or Infections can produce superficial blisters or pustules

Answer 36

Fungal Infections Candida Albicans Dermatophytes

Question 37

**Autoimmune** **Tense bullae** (grouping) on normal or erythematous skin Age**: 60-80 years** Diagnosis based on histological exam.

Answer 37

**Bullous pempigoid** Treat with: Prednisone is the cornerstone of treatment Topical cortisone for mild cases

Question 38

Autoimmune condition typified by clusters of erythematous papules, excoriations and vesicles that arise as a consequence of gluten sensitivity. Most patients are between 20 and 40 years of age, but the condition may occur at any age. Pruritic and distributed symmetrically along extensor surfaces

Answer 38

Dermatitis herpetiformis Associated with Celiac Disease

Question 39

Autoimmune disease that affects the skin and mucous membranes. The predominant skin lesions are **flaccid blisters.** The blisters are located on the head, trunk and intertriginous areas. **40 and 60** years of age. Associated with **Nikolsky sign** (top layers of the skin slip away from the lower layers when slightly rubbed and may create a blister) and has a mortality rate of 5 to 15 %

Answer 39

Pemphigus vulgaris

Question 40

**skin hyperpigmentation** and **urine discoloration** (increased uro and coproporphyrins in the urine) Blistering of the skin occurs on **sun-exposed areas**, especially the hands and forearms and occasionally face. Patients may also exhibit **hypertrichosis (growth of hair)** of the forehead and cheeks. Risk factors for the disease include Hepatits C, hemochromatosis and alcoholism.

Answer 40

Porphyria Cutanea Tarda Results from a deficiency in a heme-synthesizing enzyme.

Question 41

history findings and associated shoulder Disorders: Diabetes or thyroid disorders

Answer 41

Adhesive capsulitis