Case presentation

1. ERYTHRODERMA

2. • DEFINITION
Universal erythema with constant scaling involving
more than 90% body surface area
HISTORY
Age
Sex
Occupation
Duration
c/o peeling of skin
Redness
Itching
Tightness

3. H/O PRESENTING ILLNESS
• Itching – severe in atopic eczema, sezary
syndrome
• Atopy
• Pre existing disease – psoriasis, eczema, lichen
planus, pemphigus foliaceous
• Photosensitivity
• Drug intake – mercury, arsenic, gold,
phenobarbitone, captropril, carbamezapine,
eptoin, allopurinol, antimalarial

4. • H/O external application
• Difficulty in breathing
• Palpitation
• Fever & chills
• Diarohea
• Hematamesis / malena
• Loss of appetite/ loss of wt
• Dysuria / scanty micturition
• Cough with expectoration /hemoptysis

5. • Pustules during course of disease
• Fluid filled blister
• Syncopal attack
• Blurring of vision
• Epiphora
• Phototherapy
• Organ and bone marrow transplantation
• H/O exposure
• Recurrence

6. PAST HISTORY
• Similar illness in the past – if so , duration &
treatment
GENERAL EXAMINATION
nourishment
anemia
lymphadenopathy – site
discrete or matted
consistency
tenderness
edema of dependent parts
jaundice

7. SYSTEMIC EXAMINATION
• CVS – BP, High output cardiac failure, wide
pulse pressure
• RS – hypostatic pneumonia
• ABDOMEN – hepatosplenomegaly
• PR/PV
• TEMPERATURE

8. DERMATOLOGICAL EXAMINATION
• Universal erythema within 24-48 hours
• Sensation of cold – erythema is more
• Constant scaling 2-6 days
• Flexures

15. SCALES – large & crusted –acute
small & dry – chronic
silvery – psoriasis
Greasy – seb derm
cornflake – pemphigus foliaceous
fish like – icthyosis
double edged – netherton syndrome
asbestos like – tinea amantiaecia
carpet tuk – DLE
Mica like – PLC
Peripheral collerate of scale – PR
Bran like – TV
Black – icthyosis nigra
fine scale – P. alba
sheets of scale - TEN

16. DISTRIBUTION
Scalp- diffuse non-scarring alopecia and scarring
alopecia
Body surface
Palms and soles (40%)- palmoplantar
keratoderma, PRP, Psoriasis, keratotic plaque –
crusted scabies, painful and fissured type in sezary
syndrome
Mucous membrane – drug induced, generalised
pustular psoriasis, LP, pemphigus foliaceous,
DRESS syndrome

17. • Thickening and edematous skin – cutaneous T
cell lymphoma
• Nose sparing sign – atopic eczema
• Head light sign – atopic eczema
• Deck chair sign – Ofuji’s erythroderma
• Lichenification – atopic eczema
• Seborrhic keratosis – pale in colour
• Multiple cutaneous and subcutaneous abcess

18. • EYE
• periorbital edema – drug induced
erythroderma, DRESS syndrome
• Ectropion , epiphora
• LYMPHNODE
• Dermatogenic lymphadenopathy/
lipomelanotic reticulosis
• Stage I – INCREASE in size of lymph follicle in
para cortex, filled with lipid and melanocytes,
no atypical cells

19. • STAGE II – partial obliteration of lymphnode
architecture, few atypical lymphocytes
• STAGE III- complete obliteration of
architecture with full of atypical cells

20. • HAIR-loss
• NAIL – Polishing
Subungual keratosis
Beau’s line
Onycholysis
• Pigmentary change
Pigmentation
Depigmented spots

21. CLASSIFICATION
• ACUTE & CHRONIC
• PRIMARY & SECONDARY
• Chronically relapsing (Wilson–Brocq),
Chronically persisting (Hebra), and
Selflimiting epidemic (Savill)
• IN CHILDREN AND ADULT

22. INFANTILE ERYTHRODERMA
• NON BULLOUS CONGENITAL ERYTHRODERMA
• BULLOUS CONGENITAL ICTHYOSIFORM ERYTHRODERMA
• LAMELLAR ICTHYOSIS
• NETHERTON SYNDROME
• TRICHOTHIODYSTROPHY
• NEUTRAL LIPID STORAGE DISEASE
• CONDRADI’S SYNDROME
• SJOGREN LARSON SYNDROME
• KID SYNDROME
• INFANTILE ATOPIC ECZEMA
• INFANTILE SEBORRHIC DERMATITIS
• DIFFUSE CUTANEOUS MASTOCYTOSIS
• OMEN SYNDROME
• CONGENITAL CUTANEOUS CANDIDIASIS
• GRAFT VERSUS HOST DISEASE
• HOLOCARBOXYLASE & BIOTIN DEFICIENCY

23. • IN ADULTS:
• Eczema of various type
• Psoriasis
• Drugs
• common:-lymphomas{non-Hodgkins and
Hodgkins}
• Leukemias

24. RARE CAUSES:
• Pemphigus foliaceous
• LP
• Dermatophytosis
• Crusted scabies
VERY OCCASIONAL CAUSES:
• Hailey-hailey disease
• Toxic shock syndrome
• Sarcoidosis
• Pemphigoid
• SLE
• Histoplasmosis
• Angioimmunoblastic lymphadenopathy

25. INVESTIGATION
BEDSIDE:
• Scraping
Dermatophytes
Acarus scabei
• Tzanck smear
Tzanck cells in pemphigus foliaceous
• Peripheral smear
Sezary cell
Immature neutrophils

26. INVESTIGATION TO ARRIVE AT
ETIOLOGICAL DIAGNOSIS
• Blood hemogram
Eosinophilia
• Peripheral smear
Sezary cells
Leukemic cells
• Hb decreased
• Skin Scraping
Dermatophytes
Acarus scabei
• Tzanck smear
Acantholytic cells
Nasal and skin swab

27. INVESTIGATION
SKIN BIOPSY
• Acute and chronic Dts:Eczema
• Acantholysis:P.foliaceus
• Parakeratosis & KP : PRP
• Epidermolytic HK : Bullous CIE
• Psoriasiform : Psoriasis
• Epidermotrophism
• Pautrier’s microabcess
• Sezary cells
LYMPH NODE BIOPSY
• Dermatopathic lymphadenopathy
• Lymphoma
T CELL receptor analysis of lymphnode

28. INVESTIGATION FOR SYSTEMIC
INVOVEMENT
URINE
• Albumin,Sugar & Deposits
BLOOD CHEMISTRY:
• Sugar, protein, calcium, uric acid
• LFT, RFT, Sr.electrolyte
• Skiagram
• T CELL receptor analysis in blood
Blood culture
Screening for connective tissue disorder
Ig E estimation
Lactate dehydrogenase examination
USG
C.T.Scan

29. PSORIATIC ERYTHRODERMA
• Stable plaque psoriasis – itching is less severe,
few areas of normal skin can be seen,
prognosis is good
• Unstable psoriasis – entire body surface
involved, itching is more severe, prognosis is
bad
• Psoriatic features – lost
• Multiple pustules
• Confirmed by nail changes & repeated biopsy

30. • PRECIPITATING FACTORS
• Sudden withdrawal of potent topical steroid &
systemic steroid, cyclosporine, efalizumab,
methotrexate,
• Emotional stress
• Intercurrent infection
• phototherapy
• hypocalcaemia
• Vigrous treatment with coal tar and diathronol

31. PRP
• Occurs in children and adult.
• Lesions are salmon to orange red colour
• Large scales
• Perifollicular papule over knee , elbow ,
dorsum of hand,
• Nappes clares – islands of uninvolved skin
within erythroderma
• PRP sandal

32. ATOPIC ECZEMA
• Sudden onset
• Exacerbation of existing skin lesions
• Intense pruritis
• starts over flexural area
• Lichenification
• Prurigo like lesions
• Eosinophilia
• Increased Ig E & LDH level
• Eczema with dissemination – ingestion of drugs
• External contact with drug, bacterial
hypersensitivity, conditioned hyperirritability

33. CUTANEOUS T CELL LYMPHOMA
• Sezary syndrome, erythrodermic mycosis fungoides,
hodgkin lymphoma, leukemia
• Pruritis is very sever
• Erythroderma is universal
• Diffuse infilteration of skin
• Lichenification
• Enlargement of lymphnodes
• Hepatosplenomegaly
• Atypical lymphocytes
• Eosinophilia
• Diffuse alopecia
• Leonine facies
• Painful and fissured keratoderma

34. Sezary syndrome
• Triad of erythroderma , circulating malignant T
lymphocytes, generalised lymphadenopathy
• Criteria for Sezary syndrome
• Sezary cell counts of more than 1000cells/cu mm
• CD4:CD8 ratio >10 – flow cytometry
• Evidence of T cell clones in the blood, determined
by PCR

35. Drug induced erythroderma
• Acute onset
• Mucus membrane involvement
• Palms and soles involvement
• Purpura and eccyhmoses around ankle
• Over the flexures
• Has generalized eczema – topical preparation
• Scarlatiform of morbiliform with sys drugs
• Resolves within 2-6 weeks

36. PARANEOPLASTIC ERYTHRODERMA
• Lymphoproliferative and solid organ
malignancy
• Erythroderma occurs later with fine scales
• Erythema with browinish hue-
melanoerythroderma

37. Due to bullous dermatosis
• Pemphigus foliaceous, paraneoplastic
pemphigus, bullous pemphigoid
• Bulla , erosion followed by cornflake like scale
• Erythroderma is preceded by localised lesion
over face and upper trunk

38. IDIOPATHIC ERYTHRODERMA
• 1/3 of erythroderma
• No underlying disease
• Elderly
• Chronic relapsing pruritic eruption
• Dermatopathic lymphadenopathy
• Palmoplantar keratoderma – redman
syndrome

39. METABOLIC COMPLICATIONS
THERMOREGULATION
• Poikilothermic
• Intraepidermal occlusion of sweat
• Water loss
• Heat loss
• Lack of dilatation or constriction of blood vessels
• Concealed pyrexia
Shivering
Indicates infection
GIT
• Malabsorption
• Dermatogenic enteropathy

40. CVS – High output cardiac failure
Acute tubular necrosis with acute renal failure
Septecemia
Pulmonary complication
MISCELLANEOUS
• Protein loss
• Electrolyte imbalance
• Gynaecomastia due to hyperestrogenism
• Depression
• Thrombophlebitis
• Cutaneous and subcutaneous abcess

41. TREATMENT
GENERAL
• Rest, maintain constant temp
• High protein diet
• Local emmolients
SPECIFIC
• Systemic steroids-oral/parentral
• Suitable antibiotic
• Anti-histamines
TREATMENT OF METABOLIC COMPLICATION & ALTERATION:
As and when they arise
• Treatment should be directed towards the cause

42. • Systemic steroids- to tide over metabolic crisis
• Low dose methotrexate
• Systemic bexarotene – sezary syndrome
• Cyclosporine – Ofuji’s papulo erythroderma

43. CAUSE OF DEATH IN ERYTHRODERMA
• High output failure
• Acute renal failure
• Septicemia
• Pulmonary complication