common conditions in dermatology which v encounter in a casualty

1. DR Y SRI HARSHA

2. Common dermatological conditions which we
encounter in a casualty setting are:
1. STEVENS-JOHNSON SYNDROME
2. TOXIC EPIDERMAL NECROLYSIS
3. ERYTHEMA MULTIFORME
4. ACUTE URTICARIA
5. ACUTE ANGIOEDEMA
6. ERYTHRODERMA
7. DRUG ERUPTIONS
8. LEPRA REACTIONS
9. PEMPHIGUS
10.STAPHYLOCOCCAL SCALDED SKIN SYNDROME

3. Synonyms: TEN – Lyell’s syndrome
Defn: rare, acute, and life threatening mucocutaneous diseases
that occur as a consequence of extensive keratinocyte cell
death.
Differentiation of SJS from TEN
1. SJS – detachment < 10% of BSA, widespread erythematous
or purpuric macules or flat atypical targets.
2. Overlap SJS/TEN – detachment between 10% and 30% of
BSA, widespread purpuric macules or flat atypical targets.
3. TEN – detachment > 30% of BSA, widespread purpuric
macules or flat atypical targets

6. The factors implicated in the development of the rash are :
1. Drugs ( in more than 95% of the cases)
2. Infections
3. Immunization

7. The current model for the pathogenesis of SJS/TEN is as follows:
1. upon exposure to certain types of drugs, an individual with
particular predisposing factors mounts a specific immune
reaction to the drug or one of its metabolites.
2. As a result of an interplay of cell types and cytokines that
remains to be totally defined, there is a strong expression of the
cytolytic molecule FasL on keratinocytes as well as granulysin
secretion from CTLs, NK cells and NKT cells.
3. This leads to FasL- and granulysin-mediated apoptosis of
keratinocytes and subsequent epidermal necrosis and
detachment

8. Clinical features:- usually the rash appears 1- 3 weeks after the
intake of the drug
1.
Skin:
preceded by prodromal symptoms( fever, headache, myalgia)
Starts as an erythematous macule  dusky/ purpuric macule (irregularly
shaped , discrete in the beginning &
Sites: starts over the
later coalesce with on another)
face, upper parts of the
(ATYPICAL TARGET LESION)
trunk and proximal parts of
the extremities and rapidly
spreads to the rest of the
body
macules  flaccid blisters
( NIKOLSKY’S SIGN IS POSITIVE )
Necrotic epidermis comes off leaving large areas of red exudative dermis
exposed

9. 2. Mucous membranes: Buccal , ocular and genital mucosa are involved
usually more than 1 site is effected in SJS/TEN
Can be seen as Erythema over the mucosa, small vesicles and painful erosions
over the mucosa
Often more than 80 % of the patients have conjuctival involvement ( anterior
uveitis , synechiae , corneal ulcerations )
• Patients may complain of photophobia and burning micturition
3. Other systems :
• Respiratory system: breathlessness, hypoxia, hemoptysis
• Gastro intestinal tract : diarrhea, malena, esophageal necrosis
• Renal system: proteinuria, hematuria, azotemia

10. SJS
Epidermal detachment in SJS

12. Detachment of large sheets of epidermis in TEN

13. Hemorrhagic crusts with oral mucosal
involvement
Genital mucosal Involvement

14. •
•
•
•
The diagnosis of SJS/TEN is primarily based on the
appearance of the skin lesions and their typical
symmetrical distribution, especially if known risk
factors are present.
The following investigations can be done in all patients
Complete blood count ( normochromic & normocytic
anemia & lymphopenia)
Serum electrolytes
Complete urine examination (hematuria & proteinuria
are indicative of renal involvement)
Blood sugars

15. • Liver function tests (SGOT , SGPT are slightly elevated
in half of the patients ; or due it frank hepatitis caused
by drugs, sepsis or shock)
• Renal function tests ( elevated due to dehydration)
• Chest x ray
• HIV
• Skin Biopsy ( to confirm the diagnosis)
• Early lesion shows suprabasal layer apoptotic
keratinocytes.
• Later lesion shows full-thickness epidermal necrosis
and separation of epidermis from dermis.

16. Apoptotic keratinocytes are present individually and in clusters within the
epidermis. Subtle vacuolar changes along the basal layer are accompanied by
minimal inflammation, with scattered lymphocytes within the epidermis

17. Erythema multiforme major
Staphylococcal scalded skin syndrome
Acute generalised exanthematous pustulosis
Generalised bullous fixed drug eruption
Burns
Graft-versus-host disease
Pemphigus

19. 1.
2.
3.
Promptly discontinue any and all possible offending drugs
Admit the patient in an ICU or burns ward
Assess the condition of the patient to determine the prognosis based on the
SCORTEN score

20. Correct the fluid & electrolyte imbalance:
• Fluid loss and electrolyte imbalance should be closely
monitored and corrected
• Peripheral line is more recommendable than
central, which has a higher chance of infection, but
good peripheral venous access is difficult to find
• The fluid requirement is calculated based on the
parklands formula & 3/4ths of this total amount is
given to a pt with TEN
Parkland’ formula = 4 ml/kg body wt × % BSA involved
according to the rule of nine
• All lines should be checked for signs of infection daily
and changed two times a week with tips of lines and
catheters sent for culture

21. •
•
•
•
Nutritional support:
Patient should be allowed to eat a soft, easily
chewable diet if oral feeding is feasible
If oral feeding is not possible, then nasogastric
tube feeding or parenteral feeding can be given
Early and continuous feeding decreases the risk
of stress ulcers, reduces bacterial
dislocation, enterogenic infection
Temperature control:
Should be maintained at 30-32 c to avoid heat
loss

22. Wound care :
• Detached or detachable epidermis should be left in
position as a biological dressing & only the denuded
skin be covered with a dressing
• Condy’s compresses or petrolatum impregnated gauzes
can be used
• An air fluid bed / water bed may be used to make the
patient comfortable

23. •
•
•
•
•
Ophthalmic care :
2 hourly instillation of eye drops ( saline or antibiotic
drops )
Apply ointments at night
Developing synechiae are disrupted by a blunt
instrument
ANTIBIOTICS:
Are indicated if there is widespread involvement

24. Complications
1. Fluid and electrolyte imbalance
2. Infections ( due to loss of skin barrier)
3. Skin: scarring and dyspigmentation
4. Eye : adhesions  blindness
5. Acute renal failure
Mortality rates:
1. SJS : <5%
2. TEN : 30%

25. •
1.
•
•
SPECIFIC TREATMENTS:
CORTICOSTEROIDS:
Role of corticosteroids is controversial
Systemic corticosteroids may delay wound
healing, increase the risk of infection, mask early signs
of sepsis, and may precipitate gastrointestinal bleeding
• But if the involved area is more than 20% of BSA , then
the advantages of treatment outweighs its drawbacks
• Give oral prednisolone (1-2 mg/kg/day) or parenteral
steroids ( dexamethasone 8-16 mg daily or
hydrocortisone) can be started within the first 72 hours
in a patient with limited skin surface involvement to
prevent wide spread diffusion, and continue for 3-5
days followed by rapid tapering

26. 2. CYCLOSPORINE:
• It acts by inhibiting the activated T
lymphocytes, macrophages & also interferes with the
metabolism of TNF-α and possesses anti apoptotic
properties
• Cyclosporine interrupts the disease progression &
decreases the time taken for complete reepithelization
• Dose is 3-5 mg/kg/day oral or IV upto 2 weeks followed
by weaning over another 2 weeks
• Side effects are hypertension, renal toxicity ( but these
effects are not seen with short duration of treatment)
• Watch out septic complications and severe leukopenia

27. 3. INTRAVENOUS IMMUNOGLOBULINS:
• Can be considered if pt is seen within 48-72 hrs
of bulla onset & in cases with active progressing
lesions even after 72 hrs
• Total dose is 2 gr/kg , which is given as 0.4
g/kg/day for 5 consecutive days
• Adverse effects are risk of
thromboembolism, hemolysis, vasomotor
symptoms & anaphylactic reactions
• But the major limiting factor is its high cost

28. Definition: An acute, self limited skin disease characterised by
abrupt onset of symmetrical fixed red papules, some of which
evolve into typical/ atypical papular target lesions
Epidemiology: more commonly seen in young adults , with more
male preponderance , is very uncommon in childhood
Etiopathogenesis: current understanding suggest that EM is
most likely , & in most patients, a mucocutaneous manifestation
of a distinct skin directed immune reaction that occurs in the
setting of an infection in certain predisposed individuals
HSV is the most common associated infectious agent

30. Skin lesions:
The characteristic lesion of EMF is the typical TARGET/IRS lesion
• < 3cm in diameter, regular round shape and a well defined
border
• Has 3 distinct zones
1. Central dusky zone
2. Surrounding paler zone
3. Peripheral erythematous halo
Only limited no of fully developed typical target lesions are seen in
the patient
Also ATYPICAL target lesions may also occur or may be the primary
lesion
1. Round , edematous , palpable & reminiscent of EMF
2. Has only 2 zones & a poorly defined border
Similar lesions can also be seen in SJS/TEN, but they are flat
(macular) target lesions

31. Typical TARGET lesions with 3 zones in EMF

32. TARGET lesions
Koebnerization in EMF

33. Sites : commonly on the extremities ( dorsal aspects of
hands, forearm, palms , neck, trunk), face
Less commonly over the legs
Lesions tend to be grouped.
Kobner’s phenomenon is seen
• Mucosal lesions: are limited/ absent in EMF minor, present in
EMF major
Vesicobullous lesions painful erosions
Seen over the buccal mucosa, lips, ocular & anogenital mucosa
• Systemic symptoms: are limited/ absent in EMF minor, present
in EMF major
Fever , arthralgia, pulmonary involvement

34. Natural history of the disease:
Usually appears within 24 hours
Within 3 days
Fully developed and pt complains of pruritus and
burning senssation
Heals without any sequelae
Ocular sequelae may occur if not properly taken care of
May result in post inflammatory hypo/hyperpigmentation

36. Urticaria
Fixed drug eruptions
Sub acute lupus erythematousus
Erythema annulare centrifugum
vasculitis

37. • Early lesions:
1. Interface dermatitis showing a lymphocytic infiltrate along the
dermoepidermal junction associated with hydropic &
dyskeratosis of basal keratinocytes
2. Sparse to moderate lymphocytic infiltrate around the
superficial plexuses
• Late lesions:
1. Partial to full thickness epidermal necrosis, intraepidermal
vesiculation or sub epidermal blisters
2. Dermal inflammatory infiltrate is characterised by lymphocytes
& macrophages and occasional eosinophils

38. Early lesion- focal sites of apoptosis of keratinocytes with an interface dermatitis and
vacuolar degeneration of the basal layer (insert). A perivascular lymphocytic
infiltrate is also present

39. •
•
1.
2.
3.
•
•
•
Treat the identified precipitating factors
EMF minor – symptomatic treatment is sufficient
Topical antiseptics for eroded skin lesions
Antiseptic/anti histamine rinses & local anesthetic lesions for
oral lesions
Topical ophthalmic preparations
In HSV associated EMF, supressive therapy can be given for a
period of 6 months in the form of acyclovir ( 10 mg/kg/day) or
valacyclovir ( 500-1000 mg/day) or famciclovir ( 250 mg BD)to
prevent recurrences
In severe forms of EMF with functional impairment, systemic
corticosteroids (prednisone: 0.5-1 mg/kg/day) can be given
Severe cases of EMF are to be hospitalised and managed in an
ICU or burns ward similar to SJS/TEN

41. Definition: Urticaria is characterized by
recurrent, transient, cutaneous swelling & erythema due to fluid
transfer from the vasculature to the dermis
Typical lesion seen in urticaria is WHEAL
Urticaria can be :
1. Acute ( < 6 weeks duration)
2. Chronic ( > 6 weeks duration )

42. Urticaria results from the release of
histamine, Bradykinin, leukotriene C4, PG
D2, and other vasoactive substances from mast
cells and basophils in the dermis
These substances cause extravasation of fluid
into the dermis leading to urticarial lesion

46. Defn: Localized, sudden, transient, and often
recurrent swelling of the skin, subcutaneous
tissues, or sub mucosa that usually resolves
within 72 hours, occurring for <6 weeks, is
referred to as acute angioedema
It is associated with urticaria in 50% of the cases
It results from interstitial edema from mediators
affecting capillary and venule permeability.

47. Normal person
After developing angioedema

48. Angioedema of the eyelids
Angioedema of the upeer lip

49. Clinical features: acute pale or pink subcutaneous swelling
, usually of the face( lips, eyelids, ears, nose) & less often of the
extremities and Genitilia
Skin becomes warm, swollen & tender
Frequently a burning sensation is present, but pruritus is
typically uncommon
The areas of involvement are frequently unilateral or
asymmetric
Involvement of larynx, epiglottis and their surrounding tissue
may impair swallowing and lead to upper airway obstruction(
stridor )
Edema of the intestinal wall leads to abdominal
pain, nausea, vomiting & diarrhea( commonly in drug induced
AE)
Attacks of angioedema may last a few days and usually resolve
spontaneously

50. Complications of angioedema
Lifethreatening
airway
blockage
Anaphylaxis

51. A comprehensive and detailed history(History should detail
exposure to drugs, foods, physical triggers, infection (especially
viral hepatitis), occupational exposures, and insect stings or
bites.
Laboratory investigations that may be done are:
1. A complete blood count with differential counts, ESR
2. Urinalysis
3. liver function tests
4. antinuclear antibody test, anti thyroglobulin and
antimicrosomal antibodies
5. stool for ova and parasites (in patients with marked
eosinophilia),
6. skin testing (for acute urticaria/angioedema with possible food
or venom triggers),
7. skin biopsy with immunofluorescence

54. Treatment depends upon the severity of the condition
In many cases the swelling is self-limiting and resolves
spontaneously after a few hours or days
The goals of emergency treatment of angioedema are
to prevent spontaneous eruption, to maintain a patent
airway if eruption does occur, and to stop progression
of disease
In cases with laryngeal involvement, a definitive
airway, such as an endotracheal tube or
nasopharyngeal airway, should be established. If the
airway cannot be effectively secured with an
endotracheal tube, a surgical airway is
indicated, usually in the form of an emergency
cricothyrotomy

56. H1 anti
histamines
H1 AND H2 anti
histamines
doxepin
IV
epinephri
ne
(1:1000)
• Diphenhydramine,cetirizine,loratadine,fexofen
adine
• Ranitidine, cimetidine
• A tricyclic anti depressant
• Has both H1 and H2 activity
• Sedation limits its use
• Used in pts who demonstrate upper airway
obstruction, respiratory failure or shock

57. Endotracheal intubation
Cricothyrotomy

58. Prevention
Avoid irritating the
affected area.
Stay away from known
allergens.
Never take medications
that are not prescribed

59. Presence of erythema and scaling involving
more than 90% of skin surface (can be caused
by any inflammatory skin condition)
Primary: erythema (often initially on trunk)
extends within few days to weeks to involve
whole skin surface. Followed by scaling
Secondary: generalisation of a preceding
localized skin disease (e.g. psoriasis, atopic
eczema)

60. ERYTHRODERMA
Pre existing dermatoses
1. ECZEMAS( atopic,
seborrheic, ABCD,
phytophoto)
2.PSORIASIS
3. PEMPHIGUS
FOLLIACEUS
4. PITYRIASIS RUBRA
PILARIS
5. ICTHYOSIFORM
ERYTHRODERMA
DRUGS
arsenic, barbiturates,
captopril, chloroquine,
dapsone, gold, penicillin,
rifampicin, isotretinoin,
rifampicin, sulfadiazine,
tetracyclines, thalidomide
MISCELLANEOUS
Sarcoidosis, Haileyhailey's disease,
dermatomyositis, lupus
erythematosus, GVHD
MALIGNANCIES
1. cutaneous T cell
lymphoma
2. hodgkin's disease
3. other
lymphomas ,
leukemias
INFECTIONS
1. generalised
dermatophytosis
2. HIV infection
3. Norweigian Scabies

61. Pathogenesis is still unclear
it is believed that the condition is secondary to an intricate interaction of
cytokines and cellular adhesion molecules, including interleukins-1, -2, and 8, intercellular adhesion molecule-1 (ICAM-1), and tumor necrosis factor
(TNF).
These interactions result in a dramatic increase in the epidermal turnover
rate
1. Increased no of germinative cells & their absolute mitotic rate
2. Shortened transit time of the cells through the epidermis
An increased loss of epidermal
material, together with a significant loss of protein and
folate.

62. Erythroderma developing in primary eczema or
associated with a lymphoma is often of sudden onset
Initially erythema is localised in the pruritic patches
before spreading to involve the other body surface
area
Within a few days
Scaling occurs
• Size: fine/large & lamellar ( larger scales in chronic
disease, smaller scales in acute disease)
• Color: white- brown
Skin becomes bright red, hot & dry (and the patient
may complain of irritation & pruritus and report a
feeling of tightness which is characteristic )

63. Hair loss ( in 25-30% cases due to scalp involvment, loss
of body hair)
Nail changes are common
dystrophic, ridged, or thickened or may be
shed off
Palms, soles – fissured
Variable lymph node enlargement (dermatopathic
lymphadenopathy)
Exudation may be seen (if skin is secondarily infected)
The periorbital skin is inflamed and
oedematous, resulting in ectropion, with consequent
epiphora.

64. The general picture is modified according to the nature of any
underlying disease, and the patient’s age and general physical
condition.
1. ECZEMATOUS DERMATOSIS:
occurs most frequently in the sixth and seventh decades.
However, atopic erythroderma may occur at any age
Exacerbation of existing lesions usually precedes the
generalization, which follows the usual pattern.
2. PSORIASIS:
In erythrodermic psoriasis, the clinical picture may be highly
desquamative but when the erythroderma is fully developed
,the specific features of psoriasis are often lost.
Emotional stress, intercurrent illness and phototherapy over
dosage may also precipitate erythroderma.

65. 3. DRUGS:
The eruption may start as a generalized eczema, or
scarlatiniform or morbilliform erythema, often accompanied by
some irritation, which increases steadily in severity.
This group has the best prognosis of all the causes of
erythroderma , often resolving in 2–6 weeks
The cutaneous manifestations of drug hypersensitivity may be
accompanied by involvement of other organs (ex: DRESS)
Multisystem involvement may include
fever, eosinophilia, lymphadenopathy, hepatitis, renal
dysfunction and pneumonitis.

66. 4. Malignancies ( lymphoma, leukemia )
The clinical picture follows the pattern already
described, but the accentuation of certain features
should arouse suspicion that a lymphoma is
associated, even if repeated investigations over a
period of months fail to provide convincing
confirmatory evidence.
Pruritus is often very severe
The infiltration of the skin may be so severe that the
patient’s features are deformed
Enlargement of the lymph nodes may be
considerable, even if they are histologically not
involved by the lymphoma.

67. 5. Pemphigus Foliaceus:
Moist adherent scaling preceded by crusting on
the face and upper trunk & followed by erythema
is typical
• 6. Pityriasis Rubra Pilaris:
Keratotic follicular plugs on the backs of the
fingers, and on the knees and elbows are
characteristic
• 7. Erythroderma in neonates:
Congenital icthyosiform erythroderma, colloidion
baby and widespread candidiasis

68. •
increased blood flow to the skin  hypothermia, high output cardiac failure
Compensated hyper metabolism , increased
metabolic rate
• Loss of exfoliated scales may reach upto 9 gr/m2 of BSA
hypoalbuminemia
edema
• Altered immune response ( increase in gamma globulins)

69. A detailed history of the sequence of events leading to
the development of erythroderma/exfoliative
dermatitis is a prerequisite in all patients.
A thorough clinical examination is required in order to
diagnose the etiology of exfoliative dermatitis and to
allow appropriate urgent symptomatic treatment.
Histopathology is paramount and is rewarding in over
50% of cases
Fine needle aspiration cytology (FNAC) may be vital to
distinguish between dermatopathic lymphadenopathy
and malignant lymphadenopathy
Order the necessary investigations depending on the
suspected cause

72. Secondary infection
Dehydration
Electrolyte imbalance
Temperature dysregulation
high-output cardiac failure
Post inflammatory hypopigmentation or hyper
pigmentation

73. Erythroderma in sezary’s syndrome
A widespread drug rash going to
erythroderma

74. Erythroderma due to Pityriasis rubra pilaris
Erythroderma due to Pemphigus foliaceus

75. Erythrodermic atopic dermatitis
Erythrodermic psoriasis

76. •
•
•
•
Accounts for 5% of admissions to an input
dermatology service
Risk groups:
More common in women
Increased chances with increasing age & no of
drugs taken by the patient
More in immunosuppressed individuals ( HIV )
Patients with history of atopy

77. • Immunological mechanisms:
1. IgE dependent reactions: urticaria, angioedema
2. Cytotoxic drug induced reactions: petechiae secondary to drug induced
thrombocytopenia
3. Immune complex dependent: vasculitis, serum sickness
4. Delayed type/ cell mediated drug reaction: exanthematous , fixed &
lichenoid drug eruptions, SJS, TEN
• Non immunological mechanisms:
1. Overdose
2. Pharmacological side effects
3. Cumulative toxicity
4. Delayed toxicity
5. Drug-drug interactions
6. Altered metabolism

79. Synonyms: morbilliform drug eruptions
Most common type of drug eruptions effecting the skin
Drugs advocated: aminopenicillins, sulfonamides, cephalosporins, anti
convulsants
Entire pathophysiology is unclear & is probably thought to be immunogenic
Viral infections increase the incidence of exanthematous drug eruptions
The eruption begins 7-14 days after taking the new medications & can even
occur a few days after the drug has been discontinued
It starts as an erythematous macule which is slightly palpable
Sites: starts on the trunk & upper extremities and progressively becomes
confluent
It is a polymorphic rash with urticarial lesions on limbs, confluent areas on
thorax, purpuric lesions on ankles & feet
Mucous membranes are spared
Pruritus, low grade fever are often present
It usually resolves after 1-2 weeks without any complication or sequelae
DD : viral exanthems, TSS, Scarlet fever , Kawasaki's disease

80. EXANTHEMATOUS DRUG RASH

81. Drugs advocated are:
sulfonamides, NSAIDS, barbiturates, tetracyclines, carbamazepine
Onset of the eruption is within 1-2 weeks if exposed for the first time and
within 24 hrs on later re exposure to same drug
Upon readministering the causative drug lesions occur at exactly the same
sites
Lesions: one/few, round, sharply demarcated erythematous plaques are seen
with dusky, violaceous hue/ central blister/detached epidermis
Sites : lips, face, hands, feet, Genitilia but can occur anywhere on the body
Progress of the lesions: fades over several days, often leaving a residual post
inflammatory brown pigmentation
Histopathology: superficial & deep interstitial & perivascular infiltrate within
the dermis, composed of lymphocytes, eosinophils and sometimes
neutrophils
DD: single lesion- insect bite reaction
multiple lesions – EMF, SJS

82. FIXED DRUG ERUPTIONS

83. Synonyms: pustular drug rash, toxic pustuloderma
An acute febrile drug eruption characterised by numerous small, primarily
non follicular sterile pustules, arising within large areas of erythema
HLA –B5, HLA-DR11, HLA-DR3 have been found more frequently in patients
with AGEP
Drugs advocated: beta lactam antibiotics, macrolides, calcium channel
blockers, anti malarials
Onset of rash is within 2 days and lasts for 1-2 weeks followed by superficial
desquamation
Lesions: numerous, small, non follicular, sterile pustules arise within large
areas of edematous erythema
The lesions are associated with pruritus/ burning / both sensations
Site: first appears over the face / intertriginous region and later disseminates
Other findings are : edema of hands & face, purpura, vesicles, bullae, EMF
like lesions
Laboratory findings: leukocytosis with increased neutrophil count, mildmoderate eosinophilia, hypocalcemia
DD:- acute pustular psoriasis of von-zumbusch type, exanthematous drug
eruptions, DRESS

84. ACUTE GENERALISED EXANTHEMATOUS PUSTULOSIS

85. Synonyms: drug hypersensitivity syndrome
An adverse, idiosyncratic drug reaction characterised by a triad of fever, skin
eruption, and internal organ involvement
Drugs advocated are carbamazepine, phenytoin, sulfonamides, allopurinol
The rash starts 1-6 weeks after the intake of the drugs
It starts with a high fever accompanied by pharyngitis, cervical
lymphadenopathy
Later or simultaneously , skin eruption starts in the form of a macular
erythema on the face , upper trunk, & upper extremities; to a dusky reddish
& confluent papular rash that is pruritic and is associated with facial edema
It can progress into exfoliative dermatitis
Mucous membrane involvement is rare
Various internal organs can be involved like the liver, kidneys and
hematological system
Laboratory findings: atypical lymphocytosis, neutrophilia, hemolytic
anemia, elevated transamines, ALP
DD: serum sickness like reaction, SJS, TEN

86. DRESS

87. Stop the suspected offending drug
Low to mid potency topical steroids and bland
emollients can help relieve itching
Oral histamines, oral corticosteroids may be
needed in severe cases

88. Defn: An autoimmune, intraepithelial, blistering
disease affecting the skin and mucous membranes
Epidemiology: commonly seen in middle aged people (
3-89 years)
Winter exacerbations may be seen
• Pathogenesis: It is associated with binding of IgG
autoantibodies to keratinocyte desmosomes and to
desmosome free areas of the keratinocyte cell
membrane which results in a loss of cell to cell
adhesion (acantholysis) leading to an intraepidermal
cleft giving rise to blisters

89. Types of pemphigus we commonly encounter are:
1. Pemphigus vulgaris
2. Pemphigus vegetans
3. Pemphigus foliaceous
4. Pemphigus erythematosus

90. •
•
Most commonly encountered variety
Skin: localised/ generalised vesicles & bullae on apparently normal/
erythematous skin
Initial tense & clear bullae
2-3 days
Flaccid & turbid bullae
NIKOLSKY’S SIGN – POSITIVE
BULLA SPREAD SIGN - POSITIVE
Rupture easily to leave behind painful areas of oozing &
denuded skin which shows little tendency to heal
Lesions often become crusted along with painful erosions
Heals with hypo/hyperpigmentation

91. PEMPHIGUS VULGARIS WITH EROSIONS

92. •
•
•
•
•
Oral involvement: seen in all pts with p.vulgaris
Buccal, palatine & gingival mucosa are commonly
effected
Few/many
, small/large, superficial, painful, persistent, irregularly
shaped erosions, ulcers or collapsed bullae covered
with greyish necrotic slough and having little/ no
inflammation
The lesions do not resolve spontaneously & continue
to extend peripherally
Due to extensive oral lesions, there is decreased food
intake leading to weight loss , hypoproteinemia
There may be super imposed candida infection

93. PEMPHIGUS VULGARIS WITH ORAL EROSIONS

94. Other mucosal surfaces may be involved
including:
1. Conjuctival ( mucopurulent conjunctivitis &
non specific conjuctival hyperemia)
2. Esophagus ( odynophagia / dysphagia)
3. Labia , vagina, penis
4. Urethral, anal , nasal mucosa
• Nail changes seen are paronychia, nail plate
defects

95. 1.
2.
3.
4.
To confirm the diagnosis of the pemphigus :
Tzanck smear : shows acantholytic cells
Histopathology from the edge of a blister: shows an intra
epidermal blister with a cleft at suprabasal level, multiple
acantholytic cells in the blister cavity, basal keratinocytes gives
a tombstone appearance
Direct immunofluorescence on normal appearing skin : shows
the deposition of immunoglobulin G (IgG) & C3 at the surface
of the keratinocytes
Indirect immunofluorescence using the patient’s serum if DIF is
positive : demonstrates the presence of circulating IgG
autoantibodies that bind to the epidermis

96. ACANTHOLYTIC CELLS

97. Suprabasilar acantholysis with a few acantholytic cells in the blister cavity.
Attachment of the basal cells to the basement membrane via hemidesmosomes leads
to the “tombstones” appearance.

98. Direct immunofluorescence showing intercellular immunoglobulin G throughout the
epidermis of a patient with pemphigus vulgaris.

99. Bullous pemphigoid
Dermatitis herpetiformis
Erythema multiforme
Epidermolysis bullosa acquisita
Bullous impetigo

100. Most common causes of death in pemphigus
are SEPTICEMIA & PULMONARY EMBOLISM
BAD PROGNOSIS
• elderly patients
• patients with P.vulgaris
• Patients on prolonged steroid
therapy
GOOD PROGNOSIS
• patients with minimal disease for
longer duration
• Patients with cutaneous lesion only

101. Assess the general condition of the patient and the
extent & severity of the disease
Admit the patient
Maintenance of fluid, electrolyte balance
Proper nutrition in terms of high calorie and high
protein diet which is well tolerated by the patient
Control of secondary infections ( by means of
antibiotics )
Proper & regular dressing of raw areas till
reepithelization occurs

102. Topical therapy:
• Patients with mild pemphigus can be managed
by topical therapy alone
• Topical therapy is useful in treating localised
relapses after successful control of the disease
• Agents used are topical clobetasol, tacrolimus
• Intralesional corticosteroids:
Triamcinolone ( 5-10 mg/ml for cutaneous lesions
10-20 mg/ml for mucosal lesions)
0.05-0.1 ml/site every 1-2 wks till healing occurs

103. •
•
•
•
Systemic corticosteroids: prednisolone
Main stay of treatment in pemphigus
Mild – moderate cases : 60-80 mg/day
Severe cases: 80-120 mg/day
Dose may be increased by 50% once in every 47 days till lesions heal
Tapering of the dose by 50% once every 2 weeks
is started only after 80-90% of lesions have
healed

104. • Oral corticosteroids alone may not be effective
• So concomitant immunosuppressives / other
adjuvants are advocated if
1. If there are relative contraindications to use of
corticosteroids
2. If serious effects due to corticosteroids develop
3. If a reduction in the dose is not possible
because of repeated exacerbations of disease
activity
4. If high dose of steroids are used to achieve
remission

105. Anti metabolites:
Azathioprine ( 2-4 mg/kg/day)
Cyclophosphamide ( 1-1.5mg/kg/day)
Mycophenolate mofetil ( 2gr/day)
• DEXAMETHASONE-CYCLOPHOSPHAMIDE PULSE THERAPY:
• Involves the intravenous administration of 100 mg of
dexamethasone with 500 mg of cyclophosphamide in 500 ml of
5% dextrose over 1-2 hours on day 1, followed by daily
administration of 100 mg of dexamethasone for the next 2 days.
• These pulses are repeated every 4 weeks
• On the balance days, 50 mg of cyclophosphamide is
administered orally every day
• Major advantages seen with this therapy are quick healing of
lesions, absence of side effects of corticosteroids

106. Other agents used are :
1. Tetracycline (2gr/day)
2. Nicotinamide ( 1.5gr/day)
3. Dapsone (100-300 mg /day)
4. Cyclosporine (3-6 mg/kg/day)
5. Rituximab (375 mg/m2 IV once weekly for 4
consecutive weeks)
6. Intravenous immunoglobulin (2gr/kg/cycle
divided over 3-5 days, repeated every 3-4
weeks for a minimum of 3 cycles)
7. Plasmapheresis
8. Adsorption of pathogenic antibodies

107. LEPRA REACTIONS
 Occur during the course of leprosy
 They are sudden exacerbations in the clinical state of a patient with
pauci/multi bacillary leprosy
 They are due to immunological changes following effective treatment
and decrease in the bacillary load
 Other provoking factors are intercurrent infections

108. Lepra reactions are of two types:
1. Type 1 reactions:
• A type IV hypersensitivity reaction
• It is seen in the entire spectrum of leprosy except lepromatous type
• It manifests as a local aggravation of an existing lesion which become
more erythematous and indurated with increased loss of sensation.
• The lesions may be painful with paraesthesis.
• The nerve may become extremely painful and tender
• . It can be an UPGRADING reaction (occurs when the patient is
on treatment due to increased CMI) or DOWNGRADING reaction
(occurs in patients who are not on treatment & is due to
decreased CMI)

109. Diagnosis of type 1 reaction:
• Usually it is diagnosed clinically
• Naaf’s & his team has proposed a criteria system for diagnosing
the condition
1. Major: pre existing &/ new skin lesions become inflamed, red
and swollen
2. Minor:
• One or more nerves become tender and may be swollen
• Crops of new ( painless ) lesions occur
• Sudden edema of face and extremities
• Recent loss of sensation in hands and feet or signs of recent
nerve damage in an area supplied by a particular nerve
Presence of one major criterion or at least two minor criterion
(without signs of ENL) for the diagnosis of type 1 reaction

111. Type 1 reaction

112. 2. Type 2 Reactions:
• also known as erythema nodosum leprosum (ENL).
• It is mediated by Humoral hypersensitivity
• Usually occurs later during the course of treatment for
lepromatous and borderline lepromatous leprosy
• manifests as sudden eruption of
tender, erythematous, evanescent nodules, appearing suddenly
on any part of the body which may ulcerate
• Other features like joint pain, fever, malaise, tender enlarged
nerves, lymphadenitis, epididymo-orchitis, iridocyclitis, edema
of the extremities or face may be present

113. Diagnosis of ENL:
• Classical ENL with different morphological
patterns with or without constitutional features
and associated manifestations are diagnostic
• Clinical tests:
1. Ryrie’s test
2. Elli’s test

114. Type 2 reaction/ ENL

115. 1.
2.
•
1.
2.
•
1.
•
Type 1 Reaction:
start MB-MDT / continue using it if already started
Specific treatment has to be initiated depending on
the severity of the reaction
Mild reaction:
Daily aspirin/ paracetamol for few weeks
Reassurance to the patient
Severe reaction & Acute neuritis:
Oral corticosteroids (prednisolone) is the drug of
choice
Has to be administered in very high initial doses (12mg/kg/day) , over a longer period of time with slow
tapering to reduce the chance of recurrence of
reactions

116. DOSE
DURATION
40 mg
1st and 2nd week
30 mg
3rd and 4th week
20 mg
5th and 6th week
15 mg
7th and 8th week
10 mg
9th and 10th week
5 mg
11th and 12th week
• Other immunosuppressives which are useful while
tapering the dose of steroids are:
1. Methotrexate
2. Cyclosporine
3. Azathioprine

117. •
•
•
•
•
Neuritis :
Keep the affected nerves in resting position with
appropriate splinting & padding
After the active phase is over, passive and active
exercises should be initiated
Oil massage, short wave diathermy
(SWD), ultrasonic therapy ( UST )
NSAID’S can be given to relieve the pain
Surgical decompression

118. 1.
2.
3.
•
•
Assess the severity of the reaction ( mild, moderate, severe)
Mild- pt has few ENL lesions & no signs of involvement of
organs other than the skin
Moderate- pt has mild fever (< 100 f) & mild to moderate crops
of ENL. There is leukocytosis with involvement of some other
organs except nerves, eyes or testes
Severe- pt has high fever, pustular/ ulcerative lesions of
ENL, tender & enlarged lymph nodes, neuritis
, orchitis, arthritis, iridocyclitis, persistent albuminuria with
RBC’S in urine
Treat the precipitating factors like sore throat, herpes simplex
infection, intestinal parasites, filaria, malaria and psychological
stress
Continue the MBMDT/ start it if not started earlier along with
specific treatment for type 2 reactions

119. Specific management :
1. Mild:
• Analgesics and anti inflammatory drugs like aspirin ( 600 mg 6
hrly after food) , NSAID’S
2. Moderate:
• Chloroquine: an anti inflammatory drug ,
Dose : 250 mg TID for a week, then tapered to 250 mg BD for a
week , then 250 mg OD daily, further tapering to 250mg A/D may
be sufficient to maintain control
• Sodium antimony gluconate ( stibophen ): inhibits complement
activation
Dose : available as a 10% aqueous solution , initial dose of 1.5 ml
IM, if no adverse effects give 3ml IM after 2 days , followed by 3-5
ml IM every A/D
• Colchicine: acts by inhibiting neutrophil chemotaxis
Dose: 0.5 mg TID orally with due tapering

120. Severe reaction:
First attack of severe ENL
1. 1st option: prednisolone
A short 6-8 week course of oral prednisolone (0.5-1 mg/kg) till
clinical improvement and then taper every week by 5-10 mg over 68 wks
2. 2nd option: prednisolone and clofazamine
Prednisolone as dose mentioned above
Clofazamine 300 mg od for 1 month, 200 mg od for 3-6 months, 100
mg od for as long as symptoms remain
3. 3rd option : thalidomide
100 mg 4 times a day for 3-7 days or till reaction in under control
100 mg morning plus 200 mg evening for 4 weeks
200 mg evening od for 4 weeks
100 mg evening od for 4 weeks
50 mg daily evening or 100 mg every A/D evening for 8-12 weeks

121. Treatment of recurrent or chronic ENL
1. 1st option: clofazimine plus prednisolone
Clofazimine :300 mg daily for 3 months followed by 200 mg daily
for 3 months followed by 100 mg daily as long as symptoms persist
Prednisolone : 30 mg daily for 2 weeks followed by 25 mg daily for 2
weeks, 20 mg daily for 2 weeks, 15 mg daily for 2 weeks, 10 mg
daily for 2 weeks, 5 mg daily for 2 weeks and then stop
2 2nd option : thalidomide
200 mg bd for 3-7 days followed by
100 mg morning plus 200 mg evening for 4 weeks
200 mg evening od for 4 weeks
100 mg evening od for 4 weeks
100 mg evening A/D for 8-12 weeks or more

122. Staphylococcal scalded skin syndrome
STAPHYLOCOCCAL scalded skin syndrome (SSSS) is the
term used to define a potentially lifethreatening, blistering skin disease caused by
exfoliative toxins (ETs) of certain strains of
Staphylococcus aureus .
Synonyms:- Ritter’s disease, Pemphigus neonatorum
Epidemiology: Is primarily a disease of infancy and early childhood(
less than 6 years), but occasionally seen in adults with
chronic renal insufficiency and those who are
immunocomprimised
A male-to-female predominance of SSSS has been
documented (2:1 in sporadic cases; 4:1 in epidemics).

123. SSSS is caused by an exfoliative/epidermolytic
toxin(ET) produced by staphylococcus aureus phage
group 2 strains(types 3A,3C,55,71)
ET produced by the bacteria has two antigenically
different forms(ET-A,ET-B), both are capable of causing
the disease
ET-A.B are serine proteases with a high specificity for
human desmoglein 1(Dsg 1)
They bind to Dsg 1 directly, causing a confirmational
change in the toxin which cleaves the extracellular
domain of Dsg1.
They act at the granular layer of epidermis to cause a
split and sterile bullae

124. The onset of SSSS may either be acute with fever and
rash or be preceded by a prodrome of
malaise, irritability, and cutaneous tenderness, often
accompanied by purulent rhinorrhea, conjunctivitis, or
otitis media.
• The typical rash presents as a faint, orange–
red, macular exanthem, localized initially on the head
(Figure 11.3A ) and spreading within a few hours to the
remainder of the body, with peculiar periorificial and
flexural accentuation
• Later on the skin develops a wrinkled appearance
owing to the formation of flaccid bullae within the
epidermis

125. • NIKOLSKY’S SIGN is positive
• In 1-2 days, the bullae are sloughed, leaving
behind moist skin and areas of varnish like crust
• Patients may show “sad man” facies, perioral
crusting and radial fissuring with mild facial
edema
• Scaling and desquamation continue for the next
3-5 days with re-epithelialization in 10-14 days
• Lesions heal without any scarring

126. Extensive sloughing of the skin closely
resembling that of vast scalding

127. The diagnosis of SSSS is mainly clinical and should be taken into
consideration in any child who develops a generalized, tender
erythema, most prominent on periorificial (in particular
perinostril and periocular) areas, associated with Nikolsky sign.
Suspected SSSS is supported by the confirmation of
staphylococcal infection in different sites
(conjunctiva, nasopharynx, ear).
INVESTIGATIONS
• Blood culture- negative
• Culture from conjunctiva, nasopharynx,or pyogenic foci- positive
• Leucocyte count may be elevated or normal
• Slide latex agglutination, double immuno diffusion, ELISA are
used to confirm the diagnosis

128. DIFFERENTIAL DIAGNOSIS
•
•
•
•
TEN
Common in adults, but
uncommon in children
Widespread skin
involvment with
extensive mucosal
involvment
TEN shows
subepidermal
splitting, with fullthickness necrosis of
the epidermis
Tzanck smear taken
from a fresh bulla shows
necrotic keratinocytes
with inflammatory cells
in TEN
LEINER DISEASE
the erythrodermic
form of seborrhoeic
dermatitis in
newborns, lacks
blisters or erosion, but
yellowish scales are present
all over the body.
KAWASAKI DISEASE
Prolonged fever, heart
involvement, and generalized
lymphadenopathy

129. • A sharply demarcated
zone of clevage at or
below level of stratum
granulosum
• No inflammatory cells in
the bullae
• Upper dermis lacks an
inflammatory infiltrate

130. Patients with SSSS require hospitalization
because, besides the appropriate systemic antibiotic
therapy, intensive general supportive measures are
needed.
The mainstay of treatment is to eradicate
staphylococci from the focus of infection, which in
most cases requires intravenous (IV) antistaphylococcal
antibiotics (e.g., methicillin, flucloxacillin).
Subsequently, parenteral therapy may be replaced with
1-week oral treatment with a -lactamase–resistant
antibiotic (e.g., dicloxacillin, cloxacillin, cephalexin).

131. Second-line therapies include IV macrolide
(erythromycin or clarithromycin) or vancomycin.
IV replacement of fluids and lacking electrolytes is
recommended for prompt recovery
oral fluid intake and careful monitoring of urinary
Moist denuded areas should be lubricated with a bland
emollient to decrease pruritus and tenderness
Identification and treatment of s. aureus carriers