Overview of Dermatologic Emergencies: Recognition and Management

 
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Course 394
 
Prof. Marwan Al Khawajah
College of Medicine
King Saud University.
 
Definition
Emergency is ---
Acute
Unexpected
Dangerous
Requires quick action.
 
Alarming Morphological patterns
.
 
Urticaria / Angioderma
Purpura / Echymosis
Bullae / Sloughing
Necrosis / Gangrene
Exfoliative Erythroderma Syndrome
Generalized/ widespread rashes in the acutely ill
 
febrile patient
 
Urticaria / Angioedema
Transient swellings and erythema due to
vasodilatation and fluid exudation. Manifest by
weals that develop rapidly and clear within
hours.
Can be life threatening esp. when associated
with angioedema of the larynx.
May take years to resolve.
 
 
5
 
 
6
 
Purpura
Bleeding into the skin (petechiae, purpura,
Echymoses)
Caused by pathology:
I - Inside blood vessel (disorders of coagulation
II - Of blood vessel walls (Vasculitides)
III – Outside blood vessels (affecting supporting
stroma eg: aging, drugs, Vit C deficiency,
amyloidosis)
 
 
8
 
 
9
 
 
10
 
 
11
 
 
12
 
 
13
 
Bullous Diseases
Blisters are circumscribed fluid filled skin
lesions.
Burns, bullous impetigo, herpes simplex and
zoster, severe contact dermatitis and insect bites
are common examples.
Skin diseases presenting mainly with blisters
are relatively rare but may be fatal
eg: autoimmune and mechanobullous diseases.
 
 
15
 
 
16
 
 
17
 
18
 
Erythema Multiforme (EM) –
Stevens Johnson Syndrome (SJS)
– Toxic Epidermal Necrolysis
(TEN) Spectrum
 
EM is a cutaneous reaction pattern to several
provoking stimuli including herpes simplex,
bacterial infection and drugs. May be
idiopathic.
 
The target (iris-like) lesions involve the hands
and feet and less frequently the elbows and
knees. There is now consensus that SJS and
TEN are different from EM
 
20
 
21
 
22
 
23
 
SJS and TEN are severe variants of an
identical pathologic process (apoptosis of
kerayinocytes induced by a cell-mediated
cytotoxic reaction: Haptens vs. Cytokines) and
differ only in the percentage of body surface
involved.
 
24
 
Both can start with macular and EM-like
lesions; however about 50% of TEN evolve
from diffuse erythema to necrosis and
epidermal detachment.
 
25
 
Rare and life threatening.
Most common in adults more than 40 years
Male = Female
Risk factors : SLE, HIV, HLA –B12
Polyetiologic: Drugs (sulfas, anticonvulsants,
allopurinol, NSAIDS, antibiotics), infections,
immunization, chemicals and idiopathic.
 
26
 
Usually start with prodromes: fever, malaise,
arthralgias 1-3 weeks after drug exposure and
1-3 days before mucocutaneous lesions. There
may be tenderness, itching, burning, pain or
parasthesia, photophobia, painful micturition,
impaired alimentation and anxiety.
 
27
 
Rash starts on face and extremities, may
generalize rapidely (few hours/days).
 
Scalp, palms, and soles may be spared
 
Mucous membranes invariably involved, 85%
have conjunctival lesions.
 
28
 
 
29
 
Evolve later to:
 
- Confluent erythematous macules with
crinkled surface
 
- Raised flaccid blisters
 
- Sheet like loss of epidermis
 
- Red, oozing dermis resembling second-
degree burn
 
30
 
 
31
 
32
 
33
 
34
 
Histopathology: Full thickness
necrosis of the epidermis and a
sparse lymphocytic infiltrate.
 
35
 
Recovery begins within days, completed in 3
weeks.
Pressure points and periorificial sites take
longer
Nails and eyelashes may be shed.
 
36
 
Systemic Involvement:
 
 
Respiratory, GIT, Renal, CV, Anaemia,
Lymphopenia, Neutropenia, Eosinophilia
 
37
 
Sequelae
:
 
 
Scarring, dyspigmentation, eruptive
melanocytic nevi, abnormal nails, phimosis,
vaginal synechiae, entropion, trichiasis, sicca
syndrome, keratitis and corneal scarring,
neovascularization, synblepharon, persistent
photophobia, blindness.
 
38
 
Mortality:
 
 30% for TEN
5 -10% for SJS
Due to sepsis, GI hemorrhage and fluid/
electrolyte imbalance.
Re exposure more rapid recurrence and more
severe.
 
39
 
Differential dx
:
 
Exanthematous drug eruption, phototoxic
eruptions, GVHD, Toxic shock syndrome,
burns, SSSS, generalized bullous fixed drug
eruption, exfoliative dermatitis.
 
40
 
Management
:
 
 
- Withdrawal of suspected drug(s)
 
- in ICU or burn unit
 
- IV fluids and electrolytes as for a third degree
 
   burn.
 
- Symptomatic treatment
 
- IV glucocorticoids/ immunoglobulins/
 
  pentoxifylline
 
- Treat eye lesions early (refer to ophth)
 
- No surgical debridement
 
41
 
Bad prognostic factors
 
Body surface area > 10%
Serum Urea >10mM
Age > 40 years
Heart rate >120
Serum glucose > 14mM
Serum Bicarbonate <20mM
Malignancy
 
42
 
EXFOLIATIVE ERYTHRODERMA SYNDROME (EES)
 
EES is a serious, at times life-threatening reaction
pattern of the skin characterized by:
 
 
- generalized and uniform redness
 
- scaling (branny/ lamellar)
 
- fever, malaise, shivers, pruritis, fatigue anorexia
and generalized lymphadenopathy
 
- loss of scalp and body hair, nail thickening and
onycholysis
 
43
 
 
44
 
45
 
46
 
Usually > 50 years
Male > Female
In children results from atopic dermatitis or
PRP
 
47
 
Etiology
:
 
- Pre existing dermatosis (psoriasis, 
  
50%
 
eczema, id rxn, PRP, Pf)
 
 
- Drugs (eg. Allopurinol, CCB, 
   
15%
 
carbamazepine, cimetidine, gold, lithium,
quinidine)
 
 
- Lymphoma, Leukemia 
    
10%
 
 
- Undetermined (history/histology) 
  
25%
 
48
 
 
Acute erythroderma is caused by drugs and is
potentially fatal
Erythroderma has profound effects on the entire
body. eg: poikilothermia, fluid and electrolyte
imbalance, high output cardiac failure,increased
basal metabolic rate,hypoproteinemia, anemia
due to reduced levels of iron, folic acid and
other vitamins, endocrine, hepatic and renal
complications, effects on hair and nails.
 
Clinical clues about etiology:
 
Acute : drugs
 
Areas of sparing : PRP
 
Massive hyperkeratosis and deep fissures of
palms/soles: Psoriasis., CTCL, PRP
 
Sparing of scalp hair : Psoriasis, Eczema
 
Variable erythema and scale thickness/ brownish
hue/ large lymphnodes: CTCL
 
50
 
Massive scaling of scalp with hair loss : CTCL,
PRP
 
Dusky Red : Psoriasis
 
Yellow/orange – red : PRP
 
Lichenification/erosions/excoriations : Eczema
 
Typical nail changes of psoriasis
 
Ectropion :CTCL, PRP
 
51
 
Management
 
Histopathology is not always helpful
History and physical examination for clues are
important
Chest X ray, immunoelectrophoresis, CT scans/
MRI and bone marrow aspiration
Lymphnode biopsy
Skin and blood bacterial cultures
 
- Treatment is supportive, including fluid
electrolytes and albumin restoration, parenteral
nutrition and temperature control.
-
Be aware of signs of sepsis, renal and cardiac
failure.
-
 Watch for deleterious adverse effects of
prolonged glucocoticoid therapy.
 
Topical
: Water baths, bland emollients ± topical steroids.
 
Beware of ↑ absorption of topically applied medications
eg: salicylism, methaemoglobinemia.
 
Be cautious of irritant topicals eg: dithranol, tar
 
Systemic
:
Oral glucocorticoids for remission induction but not for
maintenance.
 
Specific Systemic therapy for the underlying condition.
 
54
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In this course taught by Prof. Marwan Al Khawajah at King Saud University College of Medicine, the definition of emergencies, alarming morphological patterns, and specific conditions like urticaria, purpura, and bullous diseases are covered. Dermatologic emergencies are acute, unexpected, and dangerous conditions requiring quick action. Various skin manifestations are discussed, such as transient swellings in urticaria, bleeding into the skin in purpura, and blister formation in bullous diseases. Recognition and appropriate management of these conditions are crucial for optimal patient care.

  • Dermatologic Emergencies
  • Recognition
  • Management
  • Urticaria
  • Purpura

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  1. Dermatologic Emergencies Dermatologic Emergencies Course Course 394 394 Prof. Marwan Al Khawajah College of Medicine King Saud University.

  2. Definition Emergency is --- Acute Unexpected Dangerous Requires quick action.

  3. Alarming Morphological patterns. Urticaria / Angioderma Purpura / Echymosis Bullae / Sloughing Necrosis / Gangrene Exfoliative Erythroderma Syndrome Generalized/ widespread rashes in the acutely ill febrile patient

  4. Urticaria / Angioedema Transient swellings and erythema due to vasodilatation and fluid exudation. Manifest by weals that develop rapidly and clear within hours. Can be life threatening esp. when associated with angioedema of the larynx. May take years to resolve.

  5. 5

  6. 6

  7. Purpura Bleeding into the skin (petechiae, purpura, Echymoses) Caused by pathology: I - Inside blood vessel (disorders of coagulation II - Of blood vessel walls (Vasculitides) III Outside blood vessels (affecting supporting stroma eg: aging, drugs, Vit C deficiency, amyloidosis)

  8. 8

  9. 9

  10. 10

  11. 11

  12. 12

  13. 13

  14. Bullous Diseases Blisters are circumscribed fluid filled skin lesions. Burns, bullous impetigo, herpes simplex and zoster, severe contact dermatitis and insect bites are common examples. Skin diseases presenting mainly with blisters are relatively rare but may be fatal eg: autoimmune and mechanobullous diseases.

  15. 15

  16. 16

  17. 17

  18. 18

  19. Erythema Multiforme (EM) Stevens Johnson Syndrome (SJS) Toxic Epidermal Necrolysis (TEN) Spectrum EM is a cutaneous reaction pattern to several provoking stimuli including herpes simplex, bacterial infection and drugs. May be idiopathic.

  20. The target (iris-like) lesions involve the hands and feet and less frequently the elbows and knees. There is now consensus that SJS and TEN are different from EM 20

  21. 21

  22. 22

  23. 23

  24. SJS and TEN are severe variants of an identical pathologic process (apoptosis of kerayinocytes induced by a cell-mediated cytotoxic reaction: Haptens vs. Cytokines) and differ only in the percentage of body surface involved. 24

  25. Both can start with macular and EM-like lesions; however about 50% of TEN evolve from diffuse erythema to necrosis and epidermal detachment. 25

  26. Rare and life threatening. Most common in adults more than 40 years Male = Female Risk factors : SLE, HIV, HLA B12 Polyetiologic: Drugs (sulfas, anticonvulsants, allopurinol, NSAIDS, antibiotics), infections, immunization, chemicals and idiopathic. 26

  27. Usually start with prodromes: fever, malaise, arthralgias 1-3 weeks after drug exposure and 1-3 days before mucocutaneous lesions. There may be tenderness, itching, burning, pain or parasthesia, photophobia, painful micturition, impaired alimentation and anxiety. 27

  28. Rash starts on face and extremities, may generalize rapidely (few hours/days). Scalp, palms, and soles may be spared Mucous membranes invariably involved, 85% have conjunctival lesions. 28

  29. 29

  30. Evolve later to: - Confluent erythematous macules with crinkled surface - Raised flaccid blisters - Sheet like loss of epidermis - Red, oozing dermis resembling second- degree burn 30

  31. 31

  32. 32

  33. 33

  34. 34

  35. Histopathology: Full thickness necrosis of the epidermis and a sparse lymphocytic infiltrate. 35

  36. Recovery begins within days, completed in 3 weeks. Pressure points and periorificial sites take longer Nails and eyelashes may be shed. 36

  37. Systemic Involvement: Respiratory, GIT, Renal, CV, Anaemia, Lymphopenia, Neutropenia, Eosinophilia 37

  38. Sequelae: Scarring, dyspigmentation, eruptive melanocytic nevi, abnormal nails, phimosis, vaginal synechiae, entropion, trichiasis, sicca syndrome, keratitis and corneal scarring, neovascularization, synblepharon, persistent photophobia, blindness. 38

  39. Mortality: 30% for TEN 5 -10% for SJS Due to sepsis, GI hemorrhage and fluid/ electrolyte imbalance. Re exposure more rapid recurrence and more severe. 39

  40. Differential dx: Exanthematous drug eruption, phototoxic eruptions, GVHD, Toxic shock syndrome, burns, SSSS, generalized bullous fixed drug eruption, exfoliative dermatitis. 40

  41. Management: - Withdrawal of suspected drug(s) - in ICU or burn unit - IV fluids and electrolytes as for a third degree burn. - Symptomatic treatment - IV glucocorticoids/ immunoglobulins/ pentoxifylline - Treat eye lesions early (refer to ophth) - No surgical debridement 41

  42. Bad prognostic factors Body surface area > 10% Serum Urea >10mM Age > 40 years Heart rate >120 Serum glucose > 14mM Serum Bicarbonate <20mM Malignancy 42

  43. EXFOLIATIVE ERYTHRODERMA SYNDROME (EES) EES is a serious, at times life-threatening reaction pattern of the skin characterized by: - generalized and uniform redness - scaling (branny/ lamellar) - fever, malaise, shivers, pruritis, fatigue anorexia and generalized lymphadenopathy - loss of scalp and body hair, nail thickening and onycholysis 43

  44. 44

  45. 45

  46. 46

  47. Usually > 50 years Male > Female In children results from atopic dermatitis or PRP 47

  48. Etiology: - Pre existing dermatosis (psoriasis, eczema, id rxn, PRP, Pf) 50% - Drugs (eg. Allopurinol, CCB, carbamazepine, cimetidine, gold, lithium, quinidine) 15% - Lymphoma, Leukemia 10% - Undetermined (history/histology) 25% 48

  49. Acute erythroderma is caused by drugs and is potentially fatal Erythroderma has profound effects on the entire body. eg: poikilothermia, fluid and electrolyte imbalance, high output cardiac failure,increased basal metabolic rate,hypoproteinemia, anemia due to reduced levels of iron, folic acid and other vitamins, endocrine, hepatic and renal complications, effects on hair and nails.

  50. Clinical clues about etiology: Acute : drugs Areas of sparing : PRP Massive hyperkeratosis and deep fissures of palms/soles: Psoriasis., CTCL, PRP Sparing of scalp hair : Psoriasis, Eczema Variable erythema and scale thickness/ brownish hue/ large lymphnodes: CTCL 50

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