Erythroderma Flashcards
Clinical features
@ exfoliative dermatitis
Any inflammatory skin disease affecting > 90% of the body surface
INITIAL STAGE
Sudden onset if primary eczema or lymphoma
Patchy erythema which rapidly generalises/extends within 24-48 hrs
+/- fever, rigors, malaise, hypothermia
Scaling appears after 2-6 days —> in flexures first
Scales may be large of fine bran-like
Skin bright red, hit, dry, thickened
Skin feels tight
Feeling cold as erythema increases
SUBACUTE STAGE (if present for a few weeks)
Scalp, bidy hair may shed
Nails become ridged and thickened —> may shed
Periorbital skin imflamed, oedematous —> ectropion —> epiphora (watery eyes)
Variable degree of enlargement of LN in the absence of underlying malignant lymphoma —> DERMATOPHATHIC LN
- Usually slightly or moderately enlarged with rubbery consistency, but may be gross
CHRONIC STAGE
Pigmentary disturbances esp in dark skinned ppl —> patchy/widespread pigment loss
Causes
Eczema (most common)
Psoriasis
Lymphoma and leukaemias
Drug eruption
Ichthyosiform erythroderma
Pityriasis rubra pilaris
Papuloerythroderma of Ofuji
Pemphigus foliaceus
Pemphigoid
Lichen planus
Dermatomyositis
Lupus erythematosus
GVHD
Dermatophytosis
Crusted scabies
HIV seroconversion
Histology
Identifies cause in up to 50% esp if multiple skin bx taken
ACUTE STAGE Spongiosis Parakeratosis Dermal oedema Non-specioic dermal inflamm infiltrate
CHRONIC STAGE
+ Acanthosis
+ Elongation of rete ridges
IF DUE TO LYMPHOMA Derma; infiltrate becomes increasingly pelomorphic Band-like lymphoid infiltrate at DEJ Atypical cerebriform lymphocytes Pautrier microabscesses
IF DUE TO PSORIASIS
Papillomatosis
Clubbing of dermal papillae
IF DUE TO PEMPHIGUS FOLIACEUS
Superficial acantholysis
Clinical variants (underlying causes)
ECZEMATOUS DERMATOSES
Intense pruritus
Common causes -
- Generalisation of eczema in context of venous eczema in the elderly
- Atopic eczema at any age (some elderly patients have raised IgE, LDH, eosinophilia)
PSORIASIS May be highly desquamative Soecific features of psoriasis often lost when erythroderma fully developed Crops of miliary pustules may develop at intervals —> transition to pustular psoriasis may occurs esp if treated woth potent TCS or systemic steroids Predisposing factors - - Emotional stress - Intercurrent illness - Phototherapy
LYMPHOMA, LEUKAEMIA Causes - - CTCL/Sezary syndrome (commonest) - Hodgkin disease - Non-Hodgkin lymphoma - Leukaemia - Myelodysplasia
Clinical features -
- Often very severe pruritus
- Universal erythroderma
- Patient features deformed d/t severe infiltration of the skin
- Considerable LN
- May be HSM
Skin bx -
- May show only non-specific features initially
- May need to be repeated several times before infiltration with atypical lymphocytes become evident
- T-cell gene rearrangement to determine if clonality is present may be helpful
LN bx -
- May be diagnostic
- But often only shows reactive features i.e. dermatopathic LN
Bloods -
- WCC with diff and smear —> for abnormal cells
- Atypical lymphocytes with cerebriform nuclei (Sezary cells) often observed in erythroderma regardless of the cause —> when > 20% of circulating lymphocytes OR large Sezary cells even in small numbers —> diagnostic of Sezary syndrome
- Eosinophilia —> suggest Hodgkin disease
- T-cell gene rearrangement for clonality —> Sezary syndrome (repeat this if negative and CTCL still suspected)
DRUGS Causes - - Antiepiletics - phenytoin, carbamazepine - Antipsychotics - lithium - Cimetidine - Gold salts
Clinical features -
- May start as generalised eczema or morbiliform erythema
- May start in flexures first, or over whole skin
- Assoc irritation
- May have systemic invovlement i.e. DRESS
- Best prognosis out of all the causes of erythroderma —> resolves in 2-6 weeks
UNKNOWN ORIGIN
Dx of exclusion
‘Red man syndrome’
- Mainly elderly men
- Chronic course
- Partial and temporary remissions
- Marked PPK
- Dermatopathic LN
- Raised serum IgE
Other idiopathic causes -
- Atopic eczema of the elderly
- Drug eruption (cause not determined)
- Prelymphomatous eruptions
ICHTHYOSIFORM ERYTHRODERMA
Present from birth/early infancy
PRP
Childhood vs adulthood
Distinctive Follicular horny plugs knees and elbows, backs of fingers, toes
Islands of sparing in erythrodermic regions
Orangey discolouration palms and soles
PAPULOERYTHRODERMA OF OFUJI
Differ from ordinary erythroderma —> papulation prominent, Tends to spare face and flexures< Intense pruritus
Unclear if distinct disease or reaction pattern
? Paraneoplastic phenomenon
Elderly M
Brownish red flat topped papules that become confluent
Trunk, limbs
Spares face, flexures
‘Deck chair sign’ —> sparing of abdo flexures
Hyperkeratosis and fossuring of palms, soles
Benign LN
Associated diseases - T cell lymphoma B cell lymphoma Lung CA Gastric CA Hepatocellular CA Colon CA Prostate CA HIV
Bloods -
Peripheral eosinophilia
Raised IgE
Lymphocytopenia
Histology Non-specific Mild acanthosis Mild spongiosis Mild hyperkeratosis Focal parakeratosis Marked dermal perivascular lymphohistiocytic infiltrate Mild epidermotropism Prominent eosinophils Rarely plasma cells, multinucleate giant cells DIF neg
Mx -
- Emollients
- TCS
- Antihistamines
- Oral pred
- AZA
- CSA
- Acitretin
- PUVA
Retinoids and phototherapy preferred over immunosuppressants d/t possibpe pr0gression to CTCL + assoc woth malignancy
Prognosis -
Persists for many years
Refractory to Rx
PEMPHIGUS FOLIACEUS
Moist, crusted lesions Face, upper trunk Precedes erthroderma
Scaling evident, moist and adherent
Crops of fragile bullae —> rupture
LICHEN PLANUS Very rare Lichenoid reactions to gold, quinine etc As initial erythema and oedema subside —> individual violaceous papules revealed Buccal mucosa —> wickham striae
DERMATOPHYTOSIS
Chronic infection with i.e. T violaeceum
NORWEGIAN SCABIES
Heavily crusted hands, feet
Thickened nails
Accompanied by generalised erythema, scaling
Often mistaken for eryhrodermic psoriasis
Complications
HAEMODYNAEMIC DISTURBANCE
Increased blood flow through the skin —> High output cardiac failure esp in elderly
Increased skin perfusion —> Hypothermia
Fluid loss by transpiration —> dehydration
METABOLIC DISTURBANCE
Increased protein loss from exfoliated scale —> Hypoalbuminaemia
Hypoalbuminaemia —> Peripheral oedema
INFECTIONS
Cutaneous, subcutaneous
Pneumonia —> death
FROM SYSTEMIC TREATMENT
Course/prognosis
Potentially fatal
Particularly dangerous in elderly
Eczematous, psoriatic, or idiopathic causes may cintinue for months-years, often relapsing, remitting
Ix
Multiple skin bx (greatest diagnostic yield) —> characteristic histo features lacking
T-cell clonality in skin bx or blood (if lymphoma suspected)
Mx
Hospital/day admission often necessary esp in acute/fulminant cases
Withdraw all non-essential meds or switch meds if may be implicated as a cause
Temperature BP HR Fluid balance Correct fluid loss i.e. encourage fluid intale, IVF
Swab MCS (monitor for secondary infection) —> treat with ABs if needed
Albumin (Monitor protein balance)
U/Es (Monitor electrolyte balance)
Frequency greasy emollients to restore skin barrier function —> majority will improve over 1-2 weeks, pending Dx of underlying condition will be established)
Other topicals i.e. TCS used with caution as increased systemic absorption
Systemic Rx depending on underlying cause
Avoid systemic steroids d/t psoriasis as a possible cause —> to avoid pustular psoriasis
