Panniculitis Flashcards
What is the most common panniculitis?
Erythema nodosum
What is the most common group of people with of erythema nodosum?
Women in the 2nd and 4th decades
What is the pathogenesis of erythema nodosum?
Th1 cytokine hypersensitivity reaction to various antigens
Most common etiology of erythema nodosum?
- Idiopathic
- Streptococcal infection (#1 identifiable cause), other infxn (Yersinia, Salmonella, Campylobacter), viral URI’s coccidioidomycosis
- Drugs: Estrogens/OCPs, sulfonamides, NSAIDs
- Inflammatory conditions: Sarcoidosis and IBD (Crohn’s> UC)
What drugs are associated with erythema nodosum?
Estrogens/OCPs, sulfonamides, NSAIDs
What is Lofgren’s syndrome?
Sarcoidosis w/ EN, hilar lymphadenopathy, fever, polyarthritis, and uveitis a/w good progress
What is the clinical presentation of erythema nodosum?
Acute, tender subcutaneous nodules on pretibial areas (most commonly) bilaterally with overlying erythema to bruises-like patches
Develop over 1-2 weeks, then resolve spontaneously
New lesions can pop up over 1-2 months
Can be a/w fever, arthralgias, malaise (may precede cutaneous findings)
What are the chronic types of erythema nodosum?
Subacute nodular migratory panniculitis/erythema nodosum migrans
Characteristics of chronic erythema nodosum?
Women usually, unilateral, migrating centrifugally expanding nodules (less ender than acute version)
- Usually idiopathic, but may be a/w streptococcus infxn
What is the treatment for chronic erythema nodosum?
Saturated solution of potassium iodide
What is the histology of erythema nodosum?
Septal panniculitis w/ thickening/fibrosis of septae
- neutrophils seen particularly in early lesions
- Miescher’s migrogranulomas: small histiocyte aggregates surrounding a central stellate cleft located in the subcutaneous fat septa
- +/- thrombophlebitis (more common in EN-like lesions seen Behcet’s disease
What is the treatment for erythema nodosum?
Lesions tend to resolve w/o scarring in a few days to weeks.
- Treating underlying medical issue if identified can be helpful
- Other tx: NSAIDs, elevation, SSKI, colchicine (especially if Behcet’s associated), systemic immunosuppressants
What does erythema nodosum suggest regarding infection with coccidioidomycosis and sarcoidosis?
Improved prognosis
What is the pathogenesis of alpha1-antitrypsin deficiency panniculitis?
Alpha1-antitrypsin (made in the liver, serine protease inhibitor) is low –> dysregulation of immune system w/ increase in neutrophils –> release of proteolytic enzymes –> fat necrosis
What are the different alleles seen in alpha1-antitrypsin deficiency and what are their significance?
M= medium (normal quantities of enzyme)
S = slow (moderately decreased quantities of enzyme)
Z = very slow (severely decreased quantities of enzyme)
So people w/ one copy of Z or S have mild/moderate dz; most severe form is seen with homozygous Z alleles
Clinical features of anti1 antitrypsin deficiency panniculitis?
Large erythematous to purpuric, tender nodules or plaques appear especially on lower trunk and proximal extremities(flanks, buttocks, and thighs) Oily discharge can be seen
- As opposed to EN heals w/ scarring and subcutaneous atrophy
- preceding trauma in 1/3
Systemic associations of anti1 antitrypsin deficiency panniculitis?
A/w chronic liver dz (cirrhosis) , emphysema, pancreatitis, membranoproliferative glomerulonephritis c-ANCA vasculitis, and angioedema
What is the histology of anti1 antitrypsin deficiency panniculitis?
Early: neutrophilic panniculitis followed by rapid necrosis and destruction of fat (liquefactive necrosis of fat w/ foamy macrophages and cystic spaces)
Chronic inflammation and hemorrhage may be present at the periphery of involved areas
What is the treatment of anti1 antitrypsin deficiency panniculitis?
Alpha1-antitrypsin replacement leads to rapid improvement, can also use doxy, colchicine, cyclophosphamide, dapsone, decreased alcohol consumption, plasma exchange, and liver transplant
What is the distinction between erythema induratum vs nodular vasculitis?
Classically the erythema induratum of Bezin referred to cases associated with TB and then the nodular vasculitis were those caused from other causes/idiopathic
What test should be performed on the tissue collected for erythema induratum in regards to TB?
PCR should be performed
What type of reaction is erythema induratum/nodular vasculitis?
Type IV hypersensitivity
Clinical features of erythema induratum/nodular vasculitis?
Tender, recurrent, red-purple nodules and plaques on calves most commonly
- may ulcerate and scar
What is the histology of erythema induratum/nodular vasculitis?
Lobular and septal panniculitis w/ neutrophils, lymphocytes, macrophages, giant cells + (this mix of cells is classic), mixed vessel vasculitis [connective tissue and septa]
- Main difference between this and PAN is that PAN doesn’t affect the fat lobules themselves
