Reactive Inflammatory Erythemas

Question 1

Associated diseases of erythema gyratum repens

Answer 1

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

Question 2

Non-malignant associations of erythema gyratum repens

Answer 2

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

Question 3

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

Answer 3

Lupus
Immunobullous disorder
MF
Erythrokeratoderma variabilis 
Urticarial vasculitis
Neutrophilic dermatosis

Question 4

Histo features of erythema gyratum repens

Answer 4

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

Question 5

Clinical features of erythema gyratum repens

Answer 5

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

Question 6

DDx of erythema gyratum repens

Answer 6

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

Question 7

Baseline investigations for erythema gyratum repens

Answer 7

General screen -
FBC
Biochem
ANA

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

Question 8

Treatment ladder for erythema gyratum repens

Answer 8

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)

Question 9

Major criteria for rheumatic fever (caused by strep infection)

Answer 9

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

Question 10

Minor criteria for rheumatic fever (caused by strep infection)

Answer 10

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

Question 11

Subcutaneous nodules in rheumatic fever

Answer 11

Over bones/tendons
Painless
Firm

Question 12

Associated diseases of erythema marginatum

Answer 12

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

Question 13

Causative organisms of erythema marginatum

Answer 13

Beta haemolytic group A strep (often pharyngeal infection)

Psittacosis

Question 14

Histo features of erythema marginatum

Answer 14

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

Question 15

Site of rash of erythema marginatum

Answer 15

Trunk
Inner upper arms, thighs
Spares face

Question 16

Clinical features of erythema marginatum

Answer 16

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

Question 17

DDx of erythema marginatum

Answer 17

Annular erythema
Urticaria
Toxic erythema
EM

Question 18

Baseline investigation of individual presenting with erythema marginatum

Answer 18

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

Question 19

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

Answer 19

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

Question 20

Causes of necrolytic migratory erythema

Answer 20

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

Question 21

Consider necrolytic migratory erythema in the triad of -

Answer 21

Unusual dermatosis
Recent onset diabetes
Weight loss

Question 22

Necrolytic migratory erythema presentation

Answer 22

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

Question 23

Necrolytic acral erythema is a variant of necrolytic migratory erythema

Answer 23

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

Question 24

DDx of necrolytic migratory erythema

Answer 24

Eczema
Impetiginised eczema
Psoriasis 
Annular pustular psoriasis
Subcorneal pustular dermatosis
Pemphigus foliaceous

Question 25

Baseline investigations for necrolytic migratory erythema

Answer 25

``` 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 ```

Question 26

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

Answer 26

In part due to delay in reaching diagnosis

Question 27

Treatment ladder for necrolytic migratory erythema

Answer 27

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 ```

Question 28

EM triggers

Answer 28

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 ```

Question 29

EM clinical features

Answer 29

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

Question 30

EM target lesions Histo

Answer 30

``` 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 ```

Question 31

DDx of EM

Answer 31

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

Question 32

EM management

Answer 32

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 ```