Neutrophilic dermatoses Flashcards
What is the dermatological diagnosis?
Sweet’s syndrome
List 3 dermatological features of sweet’s syndrome
List 5 more common systemic manifestations of sweet’s syndrome
- Acute onset
- Erythematous, edematous papules and plaques that are tender but not pruritic
- Favours face, neck, upper trunk and upper extremities
- Fever
- Leukocytosis
- Arthralgias
- Arthritis (asymmetric, non-erosive, sterile) favouring knees + wrists
- Myalgias
- Ocular involvement (conjunctivitis, limbal nodules, episcleritis)
What are the common associations with sweet’s syndrome?
- Idiopathic (50%)
- Infections (25%)
- URTI (viral, Strep), GI (yersinosis), HIV or atypical mycobacteria
- Drugs (10%)
- G-CSF, retinoic acid, furosemide, minocycline
- Hematologic malignancies (10-20%)
- AML (also myelodysplasia and myeloproliferative disorders) - especially the vesiculobullous form, more widespread including oral cavity
- Inflammatory bowel disease
- Carcinomas
- Breast, Colon, GU
What is the diagnostic criteria for Sweet’s syndrome?
2 MAJOR and ≥2 MINOR
MAJOR
- Acute onset of typical cutaneous lesions
- Histopathology consistent with Sweet’s syndrome (neutrophilic infiltrate without vasculitis)
MINOR
- URTI or GI infection or accompanied by an associated malignancy or inflammatory disorder, drug exposure or pregnancy
- Fever >38˚C
- Leukocytosis with ANC predominance + elevated inflammatory markers
- Excellent response to corticosteroids
What are characteristic features of pyoderma gangrenosum?
List 3 types of pyoderma gangrenosum
- Recurrent, painful, rapidly-enlarging ulcer
- Commonly lower leg
- Gray-violent, undermined, necrotic border
- Peripheral rim of erythema
- Ulcerative PG
- begin as inflammatory papulopustule, rapidly expands to ulcer
- Pathergy (occur at site of trauma) 20-30%
- Lower extremities
- When healed, have a cribiform pattern
- Vesiculobullous (aka atypical or bullous PG)
- favours the dorsal hands, face and upper extremities
- More commonly seen with AML, myelodysplasia or myeloproliferative disorders (CML when drug-induced with G-CSF)
- Pustular
- more commonly seen with IBD
What is the dermatological diagnosis?
Ulcerative pyoderma gangrenosum
What are the histological findings in pyoderma gangrenosum?
- Sterile dermal neutrophilia
- +/- lymphocytic vasculitis
- +/- mixed inflammation
What investigations are recommended for pyoderma gangrenosum?
- Sterile biopsy of active skin lesion including fat
- GI studies
- Stool for FOBT and O&P
- Colonoscopy + biopsies
- LFTs
- +/- Hepatitis evaluation
- Hematologic studies
- CBC
- peripheral smear
- +/- bone marrow biopsy
- Serologies
- Serum protein electrophoresis
- Immunofixation electrophoresis
- ANA
- Antiphospholipid antibodies
- ANCA antibodies
- VDRL
- CXR
- Urinalysis
What plasma cell dyscrasias are associated with pyogenic gangrenosum?
IgA monoclonal gammopathy
What is the recommended treatment for pyoderma gangrenosum?
- Treat underlying disorder
- Mild/slowly progressive disease
- Ultrapotent topical CS
- Topical tacrolimus
- Minocycline
- Dapsone
- Clofazimine
- Systemic CS
- Prednisone 1g/kg/day
- Pulse CS
- 1 IV x 3-5 days
- Cyclosporine, TNF-alpha inhibitors
What is the triad associated with Behçet’s disease?
What is the diagnostic criteria?
What other features are associated with Behçet’s disease?
- Genital ulcers
- Recurrent painful oral ulcers
- Uveitis
MAJOR + ≥2 MINOR
MAJOR
- Recurrent oral ulceration (≥3 times in 12 months)
MINOR
- Recurrent genital ulceration
- Eye lesions (anterior or posterior uveitis, retinal vasculitis)
- leading cause of morbidity
- Cutaneous lesions (EN, papulopustular lesions or pseudofolliculitis, acneiform nodules)
- +Pathergy test (interpreted at 48h [20-22G needle, bevel up inserted 5mm into volar forearm, withdrawn with a twisting motion]; +ve if papule or pustule forms)
- 50% arthritis (knees, wrists and ankles)
- GI ulcerations
- Neurological changes (poor prognosis + late findings)
- Acute meningoencephalitis
- CN palsies
- Brain stem lesions
- Pyramidal or extrapyramidal signs
- Vascular
- aneurysms or occlusive arterial disease
- superficial or deep venous thrombosis
- Cardiopulmonary
- Coronary arteritis, valvular disease, myocarditis
- Recurrent ventricular arrhythmias
- Pulmonary artery aneurysms
- Renal
- Glomerulonephritis
What disease is most associated with erythema nodosum?
Sarcoidosis
