Neutrophilic dermatoses Flashcards
What are the causes of Pyoderma gangrenosum?
Pie(pyoderma) CRUMBS By Valeries WET Car(CAH)
Crohn’s > UC
RA or seroneg inflammatory arthritis/ spondyloarthropathy
Unknown - Idiopathic in 50%
Monoclonal gammopathy esp IgA
Blood - other haematologic malignancy esp AML, CML, hairy cell leukaemia, myelodysplasia
Syndromes – PAPA, PASH, PAPASH
Behcets
Vasculitis – Wegeners, EED, Takayasu’s (WET)
Chronic Active Hepatitis (Hep B,C, α1-antitrypsin, Wilson’s, PBC)
What are the diagnostic criteria for Pyoderma gangrenosum?
Diagnostic Criteria (both major and two minor)
Major:
• Rapid (usually >1cm/day) progression of painful, necrolytic ulceration with irregular, undermined, violaceous border – preceeding papule, pustule, or bulla, and pain out of proportion to size of ulcer
• Exclusion of other causes of ulceration
Minor:
• History of pathergy or cribriform scarring
• Associated disease (Py CRUMBS)
• Histological findings excluding infection
• Rapid response to oral corticosteroids
List the Sweet’s disease associations
Sweet girls have MAD HIPS
Malignancy – solid (breast, bowel, GU)
Autoimmune – SLE, RA, Sjogren’s, IBD, other neutrophilic derms (Behcets, PG)
Drugs (esp women) - Sweet fruit BAT Pilfers Granny’s Minnows; Frusemide, Bactrim, AZA, Tretinoin, Pill (OCP), G-CSF, minocyline
Haem malignancy esp AML also myelodysplasia/ myeloproliferative disorders
Infection – esp strep /other URTI/LRTI, Gastro (salmonella, yersiniosis), atypical myco, HIV
Pregnancy
Sarcoidosis
What are the diagnostic criteria for Sweet’s disease?
Diagnostic Criteria (both major and two minor)
Major:
1. Abrupt onset of typical cutaneous lesions; -Erythematous to violaceous tender papules or nodules that coalesce to form irregular plaques
- Arms, face, neck
-Pseudo vesicular appearance later due to prominent dermal oedema
- Studded with tiny pustules
2. Histopathology consistent with Sweet’s
Minor:
1. An association (infection, vaccination, malignancy, autoimmune, drug, pregnancy)
2. Fever and Constitutional symptoms; arthralgias, myalgia
3. Abnormal bloods (leucocytosis, elevated ESR/CRP, neutrophilia)
4. Excellent response to systemic corticosteroids or KI
What are the extracutaneous features of Sweet’s disease?
Fever, myalgia, flu-like prodromal illness
Lung – bronchiolitis obliterans, effusion, alveolitis
Heart – aortitis with stenosis or dilatation, pericarditis, cardiomegaly, coronary artery occlusion
Bowel – colitis, visceral abscess
Liver – hepatomegaly, elevated transaminases
Kidney – glomerulonephiritis
Bone and joint – arthralgias, osteitis , chronic recurrent multifocal sterile osteomyelitis, SAPHO, sterile arthritis
Neurological – Neuro-Sweet’s syndrome (menigoencephalitis), headache, decreased LOC
Ocular – conjunctivitis, iritis
What is SAPHO syndrome? What are its associations?
Synovotis Acne - face and upper back Pustulosis - mainly palmoplantar Hyperostosis Osteitis
Describes skin conditions which may be associated with several MSS abnormalities Can be caused by Sweet’s disease
what is MAGIC syndrome?
Mouth And Genital ulcers and Inflamed Cartilage
Overlap of Behcets and SAPHO syndrome
What is the triad of Behcets Disease?
autoimmune complex vasculitis Triad - RIG Recurrent oral ulcers (in 99% of pts) Iridocyclitis Genital ulcers Remember No see, no pee, no spicy curry
What are the features of Behcet’s?
Behçet’s Disease remember - BOOGIE PANTS
Bowel and stomach inflammation
Oral ulcers
Ocular lesions
Genital ulcers
Immune complex vasculitis
Erythema nodosum, erythematous pustule lesions
Pump (heart) problems, and Pulmonary inflammation
Arthritis/Arthralgias
Neuro and psych problems - headache, psychosis etc
Thrombosis/Thrombophlebitis/Tiredness
Spectrum of Renal Involvement (minimal change to prolif GN to rap prog crescentic GN)
Mucocutaneous features – UPSET
Ulceration (aphthous/genital)
Pustules after skin trauma (Pathergy test)
Scratching leaves lines (dermatographism)
Erythema nodosum, erythematous pustule lesions
Thrombophlebitis
what is the Rx for Behcet’s?
Rx of ulcers
- general measures
- Tacrolimus/pimecrolimus or Potent TCS 1st line
tetracyclines, ILCS
Systemic Rx of mucocutaneous Behcets Topicals combined with; - Colchicine 0.6mg TDS - Oral zinc sulphate 100mg TDS Then can try; - Dapsone 100-200mg OD (as effective but more AEs/monitoring) - Thalidomide 100-300mg daily - MTX - Prednisolone taper for severe flare ups If recalcitrant; IFNα TNFα blocker - Infliximab 5mg/kg, Etanercept Isotretinoin AZA, CsA, cyclophosphamide, MMF If pt has systemic features - need Rx by rheum/neurology e.g. steroids, AZA, CsA etc
What is PAPA syndrome?
Pyogenic Arthritis
Pyoderma gangrenosum
Acne
Autosomal dominant PSTPIP1 mutation; arthritis onset in childhood, acne in teens Pg may or may not occur Papa is(15) dominant
What is PASH syndrome?
Pyoderma gangrenosum
Acne
Suppurative Hidradenitis (HS)
very rare recently described condition - gene unknown
What is PAPASH syndrome?
Pyogenic Arthritis
Pyoderma gangrenosum
Acne
Suppurative Hidradenitis (HS)
PSTPIP1 gene mutation
What are the subtypes of Pyoderma Gangrenosum?
Classical type + 4 major subtypes; Bullous Pustular Superficial (vegetative) Pyostomatitis vegetans esp seen in UC some include; Atypical-superficial form (overlaps neutrophilic derm of dorsal hands) has strong haem cancer association Peristomal PG esp seen in Crohn's
Diagnostic criteria for Behcets?
ICBD system – need 4 or more points; GOO2 CVSP Genital ulcers = 2 Oral ulcers = 2 Occular manifestations = 2 CNS manifestations = 1 Vascular manifestations = 1 Skin manifestations = 1 (positive pathergy test if performed = 1)
