Reactive Inflammatory Erythemas Flashcards

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1
Q

Associated diseases of erythema gyratum repens

A

Malignancy (paraneoplastic phenomenon, precede diagnosis and may not be diagnosed at presentation of the eruption or post-date) - lung most common, oesophagus, breast, stomach, kidney etc

Absence of underlying malignancy but assoc with other conditions -
Inflammatory - PRP, psoriasis, Ichthyosis
Infections - mycobacterial
Connective tissue disease - lupus, sjogren syndrome, scleroderma, RA)
Hypereosinophilic syndrome
Drug induced

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2
Q

Non-malignant associations of erythema gyratum repens

A

Inflammatory - PRP, psoriasis, Ichthyosis
Infections - mycobacterial
Connective tissue disease - lupus, sjogren syndrome, scleroderma, RA)
Hypereosinophilic syndrome
Drug induced

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3
Q

Erythema gyratum repens-like eruption may be a manifestation of the following

A
Lupus
Immunobullous disorder
MF
Erythrokeratoderma variabilis 
Urticarial vasculitis
Neutrophilic dermatosis
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4
Q

Histo features of erythema gyratum repens

A

Not diagnostic
Superficial and occasionally deep perivascular lymphohistiocytic infiltrate
Hyperkeratosis, parakeratosis, acanthosis,
Granular IgG and C3 at BMZ involved and uninvolved skin

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5
Q

Clinical features of erythema gyratum repens

A

Wood-grain appearing concentric annular eruption (series of concentric figurate bands from sequential daily eruptions, migration of leading edge by 1cm every day)
Scaling at the trailing edge
Sometimes ichthyosis and bullae appear within the erythema
Prominent itch
Hyperkeratosis palms

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6
Q

DDx of erythema gyratum repens

A
EAC (each lesion distinct ring or arc with variable but usually without prominent scaling)
EM
Necrolytic migratory erythema
SCLE
Tinea corporis 
Erythrokeratoderma variabilis
Annular psoriasis
PRP
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7
Q

Baseline investigations for erythema gyratum repens

A

General screen -
FBC
Biochem
ANA

Malignancy screen - 
CXR (lung CA most common)
CT CAP
Breast mammogram
Cervical smear 
PSA
Endoscopy, colonoscopy (if indicated)
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8
Q

Treatment ladder for erythema gyratum repens

A

1st line -
Identify underlying cause

2nd line -
Treat underlying cause (treatment of cancer may result in clearance of the eruption, and tumour recurrence/mets can precipitate recurrence)

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9
Q

Major criteria for rheumatic fever (caused by strep infection)

A
Erythema marginatum (10%)
Subcutaneous nodules
Carditis
Migratory polyarthritis 
Chorea
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10
Q

Minor criteria for rheumatic fever (caused by strep infection)

A
Fever
Arthralgia
Previous rheumatic fever/rheumatic heart disease
Raised ESR/CRP/WCC
Prolonged PR interval on ECG
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11
Q

Subcutaneous nodules in rheumatic fever

A

Over bones/tendons
Painless
Firm

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12
Q

Associated diseases of erythema marginatum

A

Rheumatic fever
Psittacosis
Angioedema (C1 inhibitor deficiency, acquired or hereditary)

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13
Q

Causative organisms of erythema marginatum

A

Beta haemolytic group A strep (often pharyngeal infection)

Psittacosis

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14
Q

Histo features of erythema marginatum

A

Non-specific
Perivascular infiltrate of neuts and lymphocytes/histiocytes
Leukocytoclasia/nuclear debris
No vasculitis

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15
Q

Site of rash of erythema marginatum

A

Trunk
Inner upper arms, thighs
Spares face

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16
Q

Clinical features of erythema marginatum

A
Not itchy
Not painful
Appears in crops lasting hours to days
Blanches on pressure
Transient - resolving and reappearing 
Migrate from one part of body to another
Erythematous urticated macula/papule spread peripherally, merge to produce serpiginous polycyclic annular eruption
Rapid spread
17
Q

DDx of erythema marginatum

A

Annular erythema
Urticaria
Toxic erythema
EM

18
Q

Baseline investigation of individual presenting with erythema marginatum

A
Skin biopsy
ECG and ECHO
FBC
U/E
LFT
ESR
CRP
Ferritin
Blood cultures
ASOT
Throat swab MCS
19
Q

Treatment ladder for erythema marginatum (when assoc with rheumatic fever)

A

1st line -
NSAID/aspirin - for severely painful polyarthritis
Penicillin/erythromycin - for strep infection

20
Q

Causes of necrolytic migratory erythema

A

Pancreatic islet cell tumour (anatomical predilection towards tail of pancreas)

Glucagonoma - assoc with MEN, bronchial CA, nasopharyngeal CA

Pseudoglucagonoma syndrome (absence of glucagonoma-secreting tumour) - assoc with pancreatic insufficiency, bronco pancreatitis, coeliac disease, GI malabsorption syndrome, IBD, alcoholic liver disease, cirrhosis, non-pancreatic malignancies, myelodysplastic syndrome

21
Q

Consider necrolytic migratory erythema in the triad of -

A

Unusual dermatosis
Recent onset diabetes
Weight loss

22
Q

Necrolytic migratory erythema presentation

A

Lower abdomen, groin, genitalia, buttocks
Macular erythema evolve to centrifugally extending annular eruption with crusted edge which can be blistered/eroded
Eczematous/psoriasiform features can be seen
Central areas heal over 1-2 weeks
Leaves PIH
Itchy, tender, painful
Fluctuating, cyclical pattern
Angular cheilitis
Painful beefy coloured glossitis
Neuro/pschy disturbances - dementia, psychosis, agitation, delusions, ataxia, hyperreflexia
Thromboembolic complications i.e. PE, DVT, dilated cardiomyopathy

23
Q

Necrolytic acral erythema is a variant of necrolytic migratory erythema

A
Well-demarcated dusky discolouration with peripheral blisters progressing to erythrokeratoderma-like
Hands, feet
Can affect forearms, knees, lower legs
Necrolytic process on histo
Hep C positive
Normal glucagon
24
Q

DDx of necrolytic migratory erythema

A
Eczema
Impetiginised eczema
Psoriasis 
Annular pustular psoriasis
Subcorneal pustular dermatosis
Pemphigus foliaceous
25
Q

Baseline investigations for necrolytic migratory erythema

A
Skin biopsy from edge of early lesions (may need serial biopsies)
FBC
Biochem and glucose
Serum glucagon
Abdo USS
CT CAP
PET CT
Single photon emission CT (SPECT)
Coeliac axis angiography
Somatostatin receptor imaging using octreotide
26
Q

50% of patients with necrolytic migratory erythema secondary to glucagonoma will have metastatic disease at time of diagnosis

A

In part due to delay in reaching diagnosis

27
Q

Treatment ladder for necrolytic migratory erythema

A

First line -
Surgery (curative rarely obtained due to mets at presentation)

Second line -
Octreotide (somatostatin analogue) inhibit release of glucagon - good symptom control but does not influence tumour growth

Third line - 
Chemo
Embolisation of liver mets
Everolimus 
Sunitinib
28
Q

EM triggers

A

None

Viral infection - 
Facial/genital HSV
Mycoplasma (esp if conjunctival, corneal involvement occurs)
HIV
EBV (infectious mononucleosis)
CMV 
Hep B
Varicella
Mumps
Adenovirus
Milkers nodules, Orf
Psittacosis

Bacterial -
Rickettsiae

Fungal -
Histoplasmosis

Drug reactions (controversial, confirmation necessitates re-exposure which may carry unacceptable risk and not usually carried out) -
vaccinations
NSAIDS
Paracetamol
ABs - penicillins, cepaholosporins, sulphonamides, co-trimoxazole, sulfones, tetracyclines
Anticonvulsants - carbamazepine, lamotrigine, barbiturates
CNS - lithium, phenothiazines
Anti-HTN - frusemide, thiazides
Antifungal - griseofulvin, Terbinafine

Contact reaction -
Rubber gloves
Nickel
Balsam of Peru

Other misc - 
Pregnancy, premenstrual, autoimmune progesterone dermatitis
Sarcoidosis
Polymorphous light eruption 
Lupus (Rowell syndrome)
PAN
Malignancy
29
Q

EM clinical features

A

EM MINOR, PAPULAR/SIMPLEX FORM
80% of cases
Dusky Macular, papular, urticarial lesions
Target lesion/classic iris - 3 zones (central area dusky erythema/purpura, middle paler zone of oedema, outer ring of erythema with well defined edge
Atypical target lesions —> only have 2 zones
Koebner phenomenon
Photoaggravation

Sites -
Distal extremities
Palms/hands often selectively involved
Trunk
+/- oral mucosa w/ assoc erosions, bullae
+/- genital mucosa w/ assoc erosions, bullae

Successive crops over few days
Fade in 1-2 weeks
Sometimes leaves dusky discolouration

LOCALISED VESICULOBULLOUS FORM
Red macules/plaques
Central bulla
Marginal ring of vesicles (herpes iris of Bateman)

Sites -
Acral
Mucous membranes often involved

EM MAJOR
More extensive target lesions + mucous membrane involvement
Sudden onset
Prodromal systemic illness within 2 weeks before eruption

ATYPICAL EM
Clinically atypical
Histology characteristic
EM-like lesions in AGEP
Persistent EM I.e. large lesions enlarge slowly over several weeks/months
Blaschkoid EM

ROWELL SYNDROME
LE (positive ANA, anti-Ro/La, Rh factor) assoc with EM-like skin lesions

30
Q

EM target lesions Histo

A
Vacuolar degeneration of the basal layer
Necrotic apoptotic cells
Epidermal necrosis
Epidermotropism of lymphocytes
Subepidermal blister 
Pigment incontinence w/ melanophages
RBC extravasation (rare)
No vasculitis
Histo DDx -
Fixed drug eruption
GVHD
Photodermatitis
Severe vasculitis
31
Q

DDx of EM

A

Other drug eruptions I.e. Vancomycin induced linear IgA disease
LE with EM-like lesions (Rowell syndrome)
Autoimmune bullous disorders I.e. Pemphigoid
Toxic erythema of unknown cause
Urticarial vasculitis (can resemble atypical EM with atypical targets)
Kawasaki disease

32
Q

EM management

A

Limited papular/localised vesiculobullous forms —> Symptomatic treatment

Ocular involvement —> early referral to ophthal

Severe/widespread —> similar to TEN -
HDU/burns unit
Pred 30-60mg/d decreasing over 1-4 weeks

Recurrent EM triggered by HSV -
antiviral Rx at first sign of recurrent herpes +/- consider long term prophylaxis I.e. aciclovir 200mg TDS —> EM tends to relapse when ceased
Thalidomide
Dapsone 
AZA
MMF
CSA