Reactive Inflammatory Erythemas Flashcards
Associated diseases of erythema gyratum repens
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
Non-malignant associations of erythema gyratum repens
Inflammatory - PRP, psoriasis, Ichthyosis
Infections - mycobacterial
Connective tissue disease - lupus, sjogren syndrome, scleroderma, RA)
Hypereosinophilic syndrome
Drug induced
Erythema gyratum repens-like eruption may be a manifestation of the following
Lupus Immunobullous disorder MF Erythrokeratoderma variabilis Urticarial vasculitis Neutrophilic dermatosis
Histo features of erythema gyratum repens
Not diagnostic
Superficial and occasionally deep perivascular lymphohistiocytic infiltrate
Hyperkeratosis, parakeratosis, acanthosis,
Granular IgG and C3 at BMZ involved and uninvolved skin
Clinical features of erythema gyratum repens
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
DDx of erythema gyratum repens
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
Baseline investigations for erythema gyratum repens
General screen -
FBC
Biochem
ANA
Malignancy screen - CXR (lung CA most common) CT CAP Breast mammogram Cervical smear PSA Endoscopy, colonoscopy (if indicated)
Treatment ladder for erythema gyratum repens
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)
Major criteria for rheumatic fever (caused by strep infection)
Erythema marginatum (10%) Subcutaneous nodules Carditis Migratory polyarthritis Chorea
Minor criteria for rheumatic fever (caused by strep infection)
Fever Arthralgia Previous rheumatic fever/rheumatic heart disease Raised ESR/CRP/WCC Prolonged PR interval on ECG
Subcutaneous nodules in rheumatic fever
Over bones/tendons
Painless
Firm
Associated diseases of erythema marginatum
Rheumatic fever
Psittacosis
Angioedema (C1 inhibitor deficiency, acquired or hereditary)
Causative organisms of erythema marginatum
Beta haemolytic group A strep (often pharyngeal infection)
Psittacosis
Histo features of erythema marginatum
Non-specific
Perivascular infiltrate of neuts and lymphocytes/histiocytes
Leukocytoclasia/nuclear debris
No vasculitis
Site of rash of erythema marginatum
Trunk
Inner upper arms, thighs
Spares face
Clinical features of erythema marginatum
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
DDx of erythema marginatum
Annular erythema
Urticaria
Toxic erythema
EM
Baseline investigation of individual presenting with erythema marginatum
Skin biopsy ECG and ECHO FBC U/E LFT ESR CRP Ferritin Blood cultures ASOT Throat swab MCS
Treatment ladder for erythema marginatum (when assoc with rheumatic fever)
1st line -
NSAID/aspirin - for severely painful polyarthritis
Penicillin/erythromycin - for strep infection
Causes of necrolytic migratory erythema
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
Consider necrolytic migratory erythema in the triad of -
Unusual dermatosis
Recent onset diabetes
Weight loss
Necrolytic migratory erythema presentation
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
Necrolytic acral erythema is a variant of necrolytic migratory erythema
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
DDx of necrolytic migratory erythema
Eczema Impetiginised eczema Psoriasis Annular pustular psoriasis Subcorneal pustular dermatosis Pemphigus foliaceous
Baseline investigations for necrolytic migratory erythema
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
50% of patients with necrolytic migratory erythema secondary to glucagonoma will have metastatic disease at time of diagnosis
In part due to delay in reaching diagnosis
Treatment ladder for necrolytic migratory erythema
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
EM triggers
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
EM clinical features
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
EM target lesions Histo
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
DDx of EM
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
EM management
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
