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
What is the histologic difference between PAN and erythema induratum/nodular vasculitis?
Main difference between this and PAN is that PAN doesn’t affect the fat lobules themselves
What is the treatment of erythema induratum/nodular vasculitis?
Treat TB if present, otherwise care needed is supportive + various systemic tx like CS, NSAIDs, TCN, or SSKI
What is the pathogenesis of pancreatic panniculitis?
A/w pancreatic disorders (pancreatitis/carcinoma, and pseudocysts)
- Occurs due to hydrolysis of fat by lipase, amylase, and trypsin (increased serum levels of any (or all) of these three enzymes and detectable
Clinical features of pancreatic panniculitis?
Subcutaneous nodules of legs (most common sites )
- May occur prior to known pancreatic issues
- May have systemic symptoms
What is Schmid’s triad?
Nodular lesions + polyarthritis + eosinophilia in the setting of pancreatic panniculitis –> a/w poor prognosis
What is the histology of pancreatic panniculitis?
Mixed panniculitis w/ “ghost cells” (anucleate necrotis adipocytes) formation and fat necrosis w/ saponification by calcium salts –> basophilic color of damaged fat lobules
Treatment for pancreatic panniculitis?
Treat the underlying pancreatic disorder
What is the pathogenesis of lipodermatosclerosis?
Due to a combination of venous insufficiency and fibrinolytic abnormalities –> increased capillary permeability –> fibrin cuffs form around vessels –> decreased O2 exchange/tissue anoxia –> cystic fat necrosis with or without dermal stasis changes
What are the clinical features of lipodermatosclerosis?
Acute = rubor, dolor, and color, can look like b/l cellulitis
Chronic= well-defined induration, hyperpigmentation “inverted wine bottle look from sclerosis)
- Medial lower leg, superior to malleolus is the most commonplace
What is the histopathology for lipodermatosclerosis?
Septal thickening, fibrosis, cystic fat necrosis / surrounding lipophages, mild non-specific inflammation, lipomembranous change, and you can see stasis changes (angioplasia, inflammation, and fibrosis)
What is the treatment for lipodermatosclerosis?
- Leg compression and elevation
- Systemic options: danazol, oxandrolone, and pentoxifylline
What are the panniculitis due to external factors?
Cold panniculitis or physical trauma/foreign material
What is the clinical presentation of cold panniculitis?
acute, firm, painful, cool (in temperature), erythematous plaques/nodules that develop 1-3 days post-cold exposure; most commonly affects areas of prominent fat distribution (central cheek, thighs, and back)
What is popsicle panniculitis?
Seen in infants on the cheeks mostly (dude to higher saturated to unsaturated fatty acid ratio in that area)
What is equestrian panniculitis?
Young women equaestions on the thighs form cold exposure
What are some risk factors for cold panniculitis in neonates?
Head or whole-body hypothermia for hypoxic-ischemic encephalopathy and use of ice therapy for supraventricular tachycardia
What is the histology of cold panniculitis?
Lobular panniculitis + typical pernio changes (superficial and deep perivascular and peri-eccrine lymphohistiocytic infiltrate w/ papillary dermal edema)
What is the prognosis of cold panniculitis?
Resolves over several weeks; lipoatrophy may develop in affected areas
What is sclerosing lipogranuloma?
Male genitalia (penis) due to injection of oil-based materials for augmentation
What is the histology of physical trauama/foreign material?
Vacuolated spaces, foreign material (Swiss cheese look in sclerosing lipogranuloma) and evidence of needle stick
