Neutrophilic Dermatoses Flashcards
What groups does Sweet’s syndrome most commonly occur in?
- Japanese
- female predominance of 4 to 1
- 30 - 60 years old but children and elderly can be affected.
- drug -induced Sweet’s syndrome occurs more frequently in women.
Wht are the clinical features of Sweet’s syndrome?
- tender, non-pruritic erythematous plaques or papules with significant edema.
May have a yellow targetoid appearance.
What ares does sweet’s syndrome favour?
- head and neck, upper extremities and dorsal aspects of the hands.
- oral lesions are uncommon except in patients with heamatological disorders. –> initially look ‘psuedopustular’ then ulcerated.
the eruption of Sweet’s syndrome resolves in 5-12 weeks but recurs.
what are some systemic manifestions of Sweet’s syndrome?
- Fever
- Leukocytosis
- arthralgias
- arthritis
- myalgias
- ocular involvement: conjunctivitis, episcleritis, iridocyclitis.
- neutrohilic alveoloitis
- sterile osteomyelitis
- renal involvement
What condititions are associated with Sweet’s syndrome?
- Infectsions: especially stretpococcus infections
- myelogenous leukemias
- solid tumours
- inflammatory bowel disease
- drgus: G-CSP, frusomide, hydralazine, minocycline, bactrim
What is seen histologically in Sweet’s syndrome?
EARLY:
- minial epidermal jchange
- odema
- lymphocytic infiltrate in early lesions
- neutrophils in the epidermis
CHARCTERISTIC:
- diffuse nodular and perivascular neutrophilic infiltrate without evidence of vasculitis.
what is the criteria for the diagnosis of Sweet syndrome?
- abrupt onset of typical cutaneous lesions
- histopathology consisten with sweets
Minor:
- preceded by infection/vaccination/malignancy/ inflammatory disorder, drug exposure or pregnancy.
- prescence of fever/ constitutional signs and smptoms
- leukocytosis
- good response to corticosteroids.
*1 major and 2 minor required.
What is the treatment for Sweets syndrome?
- oral prednisone 0.5- 1mg/kg/day for 4-6 weeks.
- super potent or intralesional corticosteroids
- topical calcineurin inhibitors
Alternatives include:
- potassium iodide 900 mg.day
- dapsone 100- 200 mg/day
- colchicine 1.5 mg/day
What are the four major clinical forms of pyoderma gangeronsum?
- ulcerative
- bullous
- pustular
- superficial granulomatous
What are the neutrophilic dermatoses associated with haematological malignancy?
- Sweet’s sundrome
- atypical pyoderma gangrenosum
- neutrophilic eccrine hidradenitis
What is the epidemiology of pyoderma gangrenosum?
- affects women between the ages of 20 -50 years of age.
- patients usually have an underlying inflammatory bowel disease, arthrits or meyloproliferative disorder.
What are the clinical features of pyoderma gangrenosum?
- usually occur on the lower area including the pretibial region.
- Lesions begin asa tender papulopustule with surrounding erythematlus or voiolaceous induration.
- ulcer has a purulent bas with undermined overhanding border.
- cribiform scarring
when does the vesiculobullous form of pyoderma gangrenosum occur?
with acute myelogenous leukemia, myelodysplasia and myeloproliferative disorders.
(Usually favours the face, the upper extremities and the dorsal hands).
Diseases associated with pyogenic granuloma.
- inflammatory bowel disease
arthritis (seronegative arthritis/ rheumatoid arthritis), heamatological disease (AML, CML, hairy cell leukemia).
monocolonal gamopathy seen in 15% of patients.
What is seen on histopathology in pyoderma gangrenosum?
- neutrophilic infiltrates often with leukocytoclasia (active untreated expanding lesions)
- there may be fibrosing inflammation at the edge of an ulcer.
