Panniculitides



   
 
Table 3-22 Panniculitides
 DiseaseClinical FindingsPathologyAssociations
 
Erythema
nodosum (EN)


{Positive prognostic factor in sarcoidosis and coccidioidomycosis}

{Do not confuse with erythema nodosum leprosum (vasculitis)}
Delayed hypersensitivity response with painful, erythematous subcutaneous nodules commonly over pretibial areas → progress to bruise-like color, ± fever, arthralgias, malaise

Typically self-limited
Septal panniculitis; edematous widened septae with giant cells, neutrophils → later see lymphocytes, ± septal fibrosis, foamy histiocytes, Miescher’s microgranulomas (clusters of histiocytes surrounding clefts)
Idiopathic (30%), strep infection (also viral, deep fungal), sarcoidosis, drugs (OCP, sulfonamides), Behçet’s, malignancy

IBD
(Crohn’s > UC)
{Per Bolognia and Harrison}

Treatment (dependent on etiology): bed rest, NSAID, colchicine, potassium iodide (KI)
 
Subacute nodular migratory panniculitis (EN migrans)
Nodules that migrate or expand centrifugally, often unilateral; chronic course
Chronic septal panniculitis with greater septal thickening than EN
Most idiopathic, ± associated strep infection or thyroid disease

Treatment: KI
 
Morphea/scleroderma panniculitis
Deep indurated plaques typically over extremities
Septal panniculitis with mucin, thickened septae, lymphocytes and plasma cells
Treatment: see section on morphea
 
Erythema induratum (Bazin’s disease)
Erythematous plaques or nodules commonly over calves {Unlike shin location of EN}, ± ulceration, drainage
Lobular or mixed panniculitis: mixed infiltrate (histiocytes, lymphocytes, giant cells, plasma cells), vasculitis often in fat, ± caseation necrosis, fibrosis later
Associated with tuberculosis; if no TB association, termed ‘nodular vasculitis’

Treat underlying TB
 
α-1-antitrypsin deficiency panniculitis

{α 1-antitrypsin: major protease inhibitor in serum}
Erythematous, tender subcutaneous nodules that often ulcerate with oily discharge; commonly in lower trunk and extremities, ± fever, pleural effusion, pulmonary embolism
Lobular panniculitis with neutrophils and lymphocytes, liquefactive necrosis of fat (foamy macrophages, ± cystic spaces), ‘skip areas’ of normal fat next to necrotizing area
Deficiency of α 1AT

May have associated chronic liver disease, emphysema, pancreatitis, angioedema, glomerulonephritis
 
Pancreatic panniculitis (Pancreatic fat necrosis)
Subcutaneous nodules often on legs (± trunk, arms, scalp), ± oily discharge; ± fever, abdominal pain, arthralgias, ascites
Septal panniculitis → lobular or mixed with fat necrosis and ‘ghost-like’ lipocytes, basophilic calcium deposition in fat
Pancreatitis and pancreatic carcinoma

Amylase levels peak 2–3 days after eruption
 
Post-steroid panniculitis
Firm, red plaques on cheeks, trunk and arms, ± associated pruritus or tenderness
Predominantly lobular panniculitis, needle-shaped clefts in lipocytes or giant cells
Occurs after rapid withdrawal of systemic corticosteroids
 
Lupus panniculitis (Lupus profundus)
Tender, subcutaneous plaques and nodules on face, upper outer arms, trunk, shoulders and hips, ‘tethered’ depressed appearance; chronic, relapsing nature
Lobular panniculitis: hyaline necrosis of fat lobules, ± mucin, ‘lymphoid follicles’ (aggregates of lymphocytes), ± LE epidermal changes, ± vasculitis
Typically occurs antecedent to other manifestations of LE, closer relationship to CCLE than SLE

Treatment: antimalarials often
 
Cold panniculitis (Equestrian or popsicle panniculitis)
Erythematous, firm nodules or plaques typically over cheeks and chin (can be on outer thighs in equestrian panniculitis)
Lobular panniculitis with mixed infiltrate, perivascular dermal lymphocytic infiltrate with ↑↑ inflammation at dermal-subQ junction, + mucin, cystic spaces in fat
Usually infants and children exposed to cold (i.e. weather, cold food such as popsicle)

Self-limited
 
Sclerosing lipogranuloma (Paraffinoma)
Pain and erythema with induration, ± ulceration with oily discharge, often involving the penis or scrotum
Granulomatous lobular panniculitis with ↑ fibrosis, many round vacuoles of varying sizes in dermis and subcutis (‘Swiss-cheese’)
Usually due to self-injection of oily materials (paraffin or silicone)

Treatment: excision if small
 
Factitial panniculitis
Inflamed nodules (etiology hinted by distribution of lesions)
Central nidus of subcutaneous inflammation
Typically self-inflicted by psychiatric patients
 
Lipodermatosclerosis (Sclerosing panniculitis)
Wood-like induration, hyperpigmentation and erythema on lower legs bilaterally (‘inverted wine bottle’)
Thickening of dermis and septae, microcysts in lobules, cyst wall with PAS + cuticle-like eosinophilic membrane, pericapillary fibrin
Associated with chronic venous insufficiency

Treatment: compression therapy, pentoxifylline, stanozolol
 
Cytophagic histiocytic panniculitis
Subcutaneous nodules ± ulceration on trunk/extremities; fulminant systemic disease with fever, liver failure, DIC, pancytopyenia
Mixed panniculitis: macrophages (called ‘bean bag’ cells) contain erythrocytes, lymphocytes or karyorrhectic debris (cytophagocytosis)
Most cases associated with T cell lymphoma (specifically subcutaneous panniculitis- like T cell lymphoma)