Neutrophilic dermatoses Dan Flashcards

1
Q

What are the causes of Pyoderma gangrenosum?

A

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)

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2
Q

What are the diagnostic criteria for Pyoderma gangrenosum?

A

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

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3
Q

List the Sweet’s disease associations

A

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

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4
Q

What are the diagnostic criteria for Sweet’s disease?

A

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

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5
Q

What are the extracutaneous features of Sweet’s disease?

A

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

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6
Q

What is SAPHO syndrome? What are its associations?

A
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

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7
Q

what is MAGIC syndrome?

A

Mouth And Genital ulcers and Inflamed Cartilage

Overlap of Behcets and SAPHO syndrome

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8
Q

What is the triad of Behcets Disease?

A
autoimmune complex vasculitis
Triad - RIG
Recurrent oral ulcers (in 99% of pts)
Iridocyclitis
Genital ulcers
Remember No see, no pee, no spicy curry
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9
Q

What are the features of Behcet’s?

A
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/Thrombophelbitis/Tiredness
s

Mucocutaneous features – UPSET
Ulceration (aphthous/genital)
Pustules after skin trauma (Pathergy test)
Scratching leaves lines (dermatographism)
Erythema nodosum, erythematous pustule lesions
Thrombophlebitis

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10
Q

what is the Rx for Behcet’s?

A

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
e.g. steroids, AZA, CsA etc
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11
Q

What is PAPA syndrome?

A

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
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12
Q

What is PASH syndrome?

A

Pyoderma gangrenosum
Acne
Suppurative Hidradenitis (HS)

very rare recently described condition - gene unknown

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13
Q

What is PAPASH syndrome?

A

Pyogenic Arthritis
Pyoderma gangrenosum
Acne
Suppurative Hidradenitis (HS)

PSTPIP1 gene mutation

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14
Q

What are the subtypes of Pyoderma Gangrenosum?

A
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
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15
Q

Diagnostic criteria for Behcets?

A
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)
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