Chapter 73 - Panniculitis

Question 1

TABLE 73-1
Summary of Different Types of Panniculitis

PANNICULITIS

AGE GROUPS
Young women, 2nd to 4th decades

Answer 1

Erythema nodosum

Question 2

TABLE 73-1
Summary of Different Types of Panniculitis

PANNICULITIS

AGE GROUPS
Young/middle-aged women

Answer 2

Erythema induratum

Question 3

TABLE 73-1
Summary of Different Types of Panniculitis

PANNICULITIS

AGE GROUPS
Overweight women older than 40 years

Answer 3

Lipodermatosclerosis

Question 4

TABLE 73-1
Summary of Different Types of Panniculitis

PANNICULITIS

AGE GROUPS
Most common in 30-60 years of age groups

Answer 4

α1-Antitrypsin panniculitis

Question 5

TABLE 73-1
Summary of Different Types of Panniculitis

PANNICULITIS

Most common phenotype of α1-Antitrypsin panniculitis

Answer 5

MM phenotype
(normal AAT)

Question 6

TABLE 73-1
Summary of Different Types of Panniculitis

PANNICULITIS

Phenotype of α1-Antitrypsin panniculitis associated with 10%-15% of normal levels; associated with >60% of cases

Answer 6

ZZ phenotype

Question 7

TABLE 73-1
Summary of Different Types of Panniculitis

PANNICULITIS

AGE GROUPS
Occurs in 2%-3% of patients with pancreatic disorders

Answer 7

Pancreatic panniculitis

Question 8

TABLE 73-1
Summary of Different Types of Panniculitis

PANNICULITIS

AGE GROUPS
Females more frequently affected than males (4:1)
30-60 years old, also in childhood

Answer 8

Lupus panniculitis

May occur before or after diagnosis of lupus erythematosus or discoid lupus erythematosus

Question 9

TABLE 73-1
Summary of Different Types of Panniculitis

PANNICULITIS

AGE GROUPS
Very rare

Answer 9

Panniculitis with dermatomyositis

Question 10

TABLE 73-1
Summary of Different Types of Panniculitis

PANNICULITIS

AGE GROUPS
Rare; seen in adults, adolescents, and children

Answer 10

Cytophagic histiocytic panniculitis

Question 11

TABLE 73-1
Summary of Different Types of Panniculitis

PANNICULITIS

AGE GROUPS
Rare, first few weeks of life

Answer 11

Subcutaneous fat necrosis of the newborn

Question 12

TABLE 73-1
Summary of Different Types of Panniculitis

PANNICULITIS

AGE GROUPS
Scrotal cold panniculitis in 9–14-year-old males

Answer 12

Cold panniculitis
(Haxthausen disease)

Question 13

TABLE 73-1
Summary of Different Types of Panniculitis

PANNICULITIS

ASSOCIATED FACTORS
Venous insufficiency, obesity
Infectious etiology: MTB, hepatitides B, C

Answer 13

Erythema induratum

Question 13

TABLE 73-1
Summary of Different Types of Panniculitis

PANNICULITIS

ASSOCIATED FACTORS
Infections (commonly streptococcal), medications, malignancies (leukemias, lymphomas)

Answer 13

Erythema nodosum

Question 14

TABLE 73-1
Summary of Different Types of Panniculitis

PANNICULITIS

ASSOCIATED FACTORS
Venous insufficiency
Obesity
Systemic sclerosis
Pulmonary infarction
Hypertension

Answer 14

Lipodermatosclerosis

Question 15

TABLE 73-1
Summary of Different Types of Panniculitis

PANNICULITIS

ASSOCIATED FACTORS
Wide variety of bacteria, fungi, parasites, viruses
Either primarily inoculated, or hematogenous
Staphylococcus aureus panniculitis with juvenile diabetes
Panniculitis of mycetoma, chromoblastomycosis, sporotrichosis

Answer 15

Infectious panniculitis

Question 16

TABLE 73-1
Summary of Different Types of Panniculitis

PANNICULITIS

ASSOCIATED FACTORS
Pulmonary and hepatic disease (emphysema in COPD, cirrhosis, hepatocellular CA)

Answer 16

α1-Antitrypsin panniculitis

Pulmonary and hepatic disease (emphysema in COPD, cirrhosis, hepatocellular CA), highest risk ZZ phenotype
Panniculitis uncommon in AAT deficiency

Question 17

TABLE 73-1
Summary of Different Types of Panniculitis

PANNICULITIS

ASSOCIATED FACTORS
Pancreatitis, pancreatic CA

Answer 17

Pancreatic panniculitis

Question 18

TABLE 73-1
Summary of Different Types of Panniculitis

PANNICULITIS

ASSOCIATED FACTORS
Hemophagocytic Lymphohistiocytosis (HLH)
Macrophage Activation Syndrome (MAS)

Answer 18

Cytophagic histiocytic panniculitis

Question 18

TABLE 73-1
Summary of Different Types of Panniculitis

PANNICULITIS

ASSOCIATED FACTORS
Systemic lupus erythematosus
Sjogren’s Syndrome
Rheumatoid arthritis

Answer 18

Lupus panniculitis

Question 19

TABLE 73-1
Summary of Different Types of Panniculitis

PANNICULITIS

ASSOCIATED FACTORS
Dermatomyositis

Answer 19

Panniculitis with dermatomyositis

Question 20

TABLE 73-1
Summary of Different Types of Panniculitis

PANNICULITIS

ASSOCIATED FACTORS
History of perinatal complications (meconium aspiration, hypothermia, hypoxemia, gestational diabetes)

Answer 20

Subcutaneous fat necrosis of the newborn

Question 21

TABLE 73-1
Summary of Different Types of Panniculitis

PANNICULITIS

ASSOCIATED FACTORS
Exposure to cold weather, popsicles, ice packs, swimming

Answer 21

Cold panniculitis
(Haxthausen disease)

Question 22

TABLE 73-1
Summary of Different Types of Panniculitis

PANNICULITIS

ASSOCIATED FACTORS
Associated with personality aberrations

Answer 22

Factitial panniculitis

Question 23

TABLE 73-1 Summary of Different Types of Panniculitis PANNICULITIS CLINICAL COURSE AND MANIFESTATIONS - Acute onset; symmetric, tender, nodules & plaques - Affecting anterior lower extremities - Fever, fatigue, arthralgias, arthritis, headache, no ulceration, no atrophy, no scarring

Answer 23

Erythema nodosum

Question 24

TABLE 73-1 Summary of Different Types of Panniculitis PANNICULITIS CLINICAL COURSE AND MANIFESTATIONS - Erythematous SQ nodules on lower extremities - May affect calves, anterolateral leg - Tenderness, ulceration, scarring - Protracted course, with recurrent episodes - 3-6 weeks duration

Answer 24

Erythema induratum

Question 25

TABLE 73-1 Summary of Different Types of Panniculitis PANNICULITIS CLINICAL COURSE AND MANIFESTATIONS - Indurated, woody plaques on lower extremities, with acute & chronic changes (most commonly, anteromedial calf area) - Intense pain is the most frequent symptom - Stage: -- Acute inflammatory -- Chronic fibrosis -- Inverted champagne bottle

Answer 25

Lipodermatosclerosis

Question 26

TABLE 73-1 Summary of Different Types of Panniculitis PANNICULITIS CLINICAL COURSE AND MANIFESTATIONS - Erythematous plaques, nodules, abscess with purulent discharge (fluctuant/abscess-type lesions) - Most commonly on legs and feet - Upper extremities, trunk, face may be involved

Answer 26

Infectious panniculitis

Question 27

TABLE 73-1 Summary of Different Types of Panniculitis PANNICULITIS CLINICAL COURSE AND MANIFESTATIONS - Painful erythematous nodules and plaques - Cellulitis, fluctuant abscess type - Most common site: lower trunk, also buttocks, proximal extremities - May be life-threatening

Answer 27

α1-Antitrypsin panniculitis

Question 28

TABLE 73-1 Summary of Different Types of Panniculitis PANNICULITIS CLINICAL COURSE AND MANIFESTATIONS - Erythematous nodules with spontaneous ulceration - Lower extremities, especially periarticular areas (knee, ankle) - Crops of lesions; red-brown - Atrophic, hyperpigmented scarring - Oily abscesses

Answer 28

Pancreatic panniculitis

Question 29

TABLE 73-1 Summary of Different Types of Panniculitis PANNICULITIS CLINICAL COURSE AND MANIFESTATIONS - UPPER ARMS (lateral), shoulders, face, scalp, hips, buttocks, breasts - Rare on lower extremities - Deep, tender SQ nodules; no surface changes - Resolves with depressed lipoatrophic areas - Chronic, with yearly/periodic flares - Duration: average of 6 years

Answer 29

Lupus panniculitis

Question 30

TABLE 73-1 Summary of Different Types of Panniculitis PANNICULITIS CLINICAL COURSE AND MANIFESTATIONS - Erythematous nodules and plaques, affecting arms, buttocks, thighs, abdomen

Answer 30

Panniculitis with dermatomyositis

Question 31

TABLE 73-1 Summary of Different Types of Panniculitis PANNICULITIS CLINICAL COURSE AND MANIFESTATIONS - SQ erythematous to violaceous plaques on extremities, trunk - Fulminant cases may have: ulceration, fever, hepatosplenomegaly, hemocytophagocytosis in BM, LN, liver, CNS - May be acute and intermittent, or have a rapidly fatal course

Answer 31

Cytophagic histiocytic panniculitis

Question 32

TABLE 73-1 Summary of Different Types of Panniculitis PANNICULITIS CLINICAL COURSE AND MANIFESTATIONS - Erythematous to violaceous, firm nodules or plaques affecting buttocks, back, shoulders, cheeks, thighs - Anterior trunk spared - Oily/chalky white material - Late hypercalcemia (monitor for 6 months)

Answer 32

Subcutaneous fat necrosis of the newborn

Question 33

TABLE 73-1 Summary of Different Types of Panniculitis PANNICULITIS CLINICAL COURSE AND MANIFESTATIONS - Indurate, erythematous plaques or nodules at sites of cold exposure; affects face, thighs, scrotal fat of prepubertal boys - Resolution within 3 months

Answer 33

Cold panniculitis (Haxthausen disease)

Question 34

TABLE 73-1 Summary of Different Types of Panniculitis PANNICULITIS CLINICAL COURSE AND MANIFESTATIONS - SQ implantation (medications, cosmetic fillers, oils, human waste)

Answer 34

Factitial panniculitis

Question 35

TABLE 73-1 Summary of Different Types of Panniculitis PANNICULITIS HISTOPATHOLOGY - Septal Panniculitis - No vasculitis - Neutrophils (early), Meischer granulomas (late)

Answer 35

Erythema nodosum

Question 36

TABLE 73-1 Summary of Different Types of Panniculitis PANNICULITIS HISTOPATHOLOGY - Lobular, often with vasculitis (90%) - Early central necrosis of adipocytes; neutrophilic infiltrate - In older lesions, epithelioid histiocytes, multinucleated giant cells

Answer 36

Erythema induratum

Question 37

TABLE 73-1 Summary of Different Types of Panniculitis PANNICULITIS HISTOPATHOLOGY - Stasis changes - Lobular panniculitis - No vasculitis - Ischemic necrosis at center - Thickened, fibrotic septa, atrophy of subcutaneous fat - Membranocystic changes

Answer 37

Lipodermatosclerosis

Question 38

TABLE 73-1 Summary of Different Types of Panniculitis PANNICULITIS HISTOPATHOLOGY - Mostly lobular panniculitis, but with a mixed pattern - Pattern dependent on whether inoculation related or hematogenous - Neutrophilic infiltrate

Answer 38

Infectious panniculitis

Question 39

TABLE 73-1 Summary of Different Types of Panniculitis PANNICULITIS HISTOPATHOLOGY - Early necrosis of SQ fat - Splaying of neutrophils between collagen bundles is characteristic - Liquefactive necrosis

Answer 39

α1-Antitrypsin panniculitis

Question 40

TABLE 73-1 Summary of Different Types of Panniculitis PANNICULITIS HISTOPATHOLOGY - Lobular panniculitis - No vasculitis - Intense necrosis at center of lobule - Ghost adipocytes - Saponification and calcification

Answer 40

Pancreatic panniculitis

Question 41

TABLE 73-1 Summary of Different Types of Panniculitis PANNICULITIS HISTOPATHOLOGY - Vacuolar alteration of basal cell layer, thickened basement membrane, mucin deposition, superficial and deep perivascular infiltrate - Mostly lobular panniculitis, with lymphoid follicles, variable hyaline fat necrosis, sclerotic collagen bundles, lymphocytic and plasma cell infiltrate

Answer 41

Lupus panniculitis

Question 42

TABLE 73-1 Summary of Different Types of Panniculitis PANNICULITIS HISTOPATHOLOGY - Mixed septal and lobular panniculitis, lymphocytic and plasma cell infiltrate, hyaline sclerosis of septal collagen; calcification, membranocystic changes in late stages

Answer 42

Panniculitis with dermatomyositis

Question 43

TABLE 73-1 Summary of Different Types of Panniculitis PANNICULITIS HISTOPATHOLOGY - Lobular panniculitis - No vasculitis - Bean bag cells: macrophages with intact or fragmented erythrocytes, leukocytes, or platelets - Necrosis

Answer 43

Cytophagic histiocytic panniculitis

Question 44

TABLE 73-1 Summary of Different Types of Panniculitis PANNICULITIS HISTOPATHOLOGY - Lobular panniculitis - No vasculitis - Needle-shaped clefts in radial array - Nodules and plaques, resolving spontaneously

Answer 44

Subcutaneous fat necrosis of the newborn

Question 45

TABLE 73-1 Summary of Different Types of Panniculitis PANNICULITIS HISTOPATHOLOGY - Lobular panniculitis - No vasculitis - Perivascular lymphohistiocytic infiltrate

Answer 45

Cold panniculitis (Haxthausen disease)

Question 46

TABLE 73-1 Summary of Different Types of Panniculitis PANNICULITIS HISTOPATHOLOGY - Lobular panniculitis - No vasculitis - Suppurative granuloma involving fat lobule - Refractile foreign material

Answer 46

Factitial panniculitis

Question 47

TABLE 73-1 Summary of Different Types of Panniculitis TREATMENT Erythema nodosum

Answer 47

- Bed rest, aspirin, NSAIDs - SSKI, 2-10 drops thrice daily - Colchicine - Corticosteroids (rarely indicated)

Question 48

TABLE 73-1 Summary of Different Types of Panniculitis TREATMENT Erythema induratum

Answer 48

- If MTB testing is positive, full course of multidrug therapy - Treat underlying cause - SSKI, NSAIDs, colchicine, antimalarials, corticosteroids, gold - Bed rest - Pentoxifylline, compression

Question 49

TABLE 73-1 Summary of Different Types of Panniculitis TREATMENT Lipodermatosclerosis

Answer 49

- Compression therapy is the major recommended treatment (30-40 mm Hg) - Stanozolol: to decrease pain, erythema, induration - Pentoxifylline, horse chestnut seed extract, oxerutins, flavonoid fraction - Weight loss

Question 50

TABLE 73-1 Summary of Different Types of Panniculitis TREATMENT Infectious panniculitis

Answer 50

Depends on the known organisms

Question 51

TABLE 73-1 Summary of Different Types of Panniculitis TREATMENT α1-Antitrypsin panniculitis

Answer 51

- MILD-MODERATE: dapsone, doxycycline - SEVERE: protein replacement therapy

Question 52

TABLE 73-1 Summary of Different Types of Panniculitis TREATMENT Pancreatic panniculitis

Answer 52

- Octreotide - Plasmapheresis

Question 53

TABLE 73-1 Summary of Different Types of Panniculitis TREATMENT Lupus panniculitis

Answer 53

- Hydroxychloroquine is first-line treatment - Quinacrine

Question 54

TABLE 73-1 Summary of Different Types of Panniculitis TREATMENT Panniculitis with dermatomyositis

Answer 54

Corticosteroids alone, or corticosteroids with methotrexate

Question 55

TABLE 73-1 Summary of Different Types of Panniculitis TREATMENT Cytophagic histiocytic panniculitis

Answer 55

- Corticosteroids - Cyclosporine - Multidisciplinary care in a hospital setting

Question 56

TABLE 73-1 Summary of Different Types of Panniculitis TREATMENT Subcutaneous fat necrosis of the newborn

Answer 56

- Spontaneous resolution - Monitor serum calcium for hypercalcemia - Systemic glucocorticoids may be considered; nephrocalcinosis

Question 57

TABLE 73-1 Summary of Different Types of Panniculitis TREATMENT Cold panniculitis (Haxthausen disease)

Answer 57

Spontaneous resolution

Question 58

TABLE 73-1 Summary of Different Types of Panniculitis TREATMENT Factitial panniculitis

Answer 58

Psychiatric treatment; Intralesional steroids, surgical excision

Question 59

TABLE 73-2 Differentiating Septal vs Lobular Panniculitis Origin of inflammation Interlobular septa

Answer 59

Septal Panniculitis

Question 60

TABLE 73-2 Differentiating Septal vs Lobular Panniculitis Origin of inflammation Primarily the lobule or blood vessel

Answer 60

Lobular Panniculitis

Question 61

TABLE 73-2 Differentiating Septal vs Lobular Panniculitis Appearance of lobule Lobule appears intact, individual adipocytes well defined

Answer 61

Septal Panniculitis

Question 62

TABLE 73-2 Differentiating Septal vs Lobular Panniculitis Appearance of lobule Lobule is necrotic, lipophages common; free liquid may accumulate in pools of varying shapes

Answer 62

Lobular Panniculitis

Question 63

TABLE 73-2 Differentiating Septal vs Lobular Panniculitis Necrosis Absent

Answer 63

Septal Panniculitis

Question 64

TABLE 73-2 Differentiating Septal vs Lobular Panniculitis Necrosis Common

Answer 64

Lobular Panniculitis

Question 65

TABLE 73-2 Differentiating Septal vs Lobular Panniculitis Inflammatory cell - Neutrophils (acute) - Mononuclear cells, multinucleated giant cells (chronic)

Answer 65

Septal Panniculitis

Question 66

TABLE 73-2 Differentiating Septal vs Lobular Panniculitis Inflammatory cell - Lymphoid infiltrates - Plasma cells are common

Answer 66

Lobular Panniculitis

Question 67

TABLE 73-2 Differentiating Septal vs Lobular Panniculitis Vasculitis Absent

Answer 67

Septal Panniculitis

Question 68

TABLE 73-2 Differentiating Septal vs Lobular Panniculitis Vasculitis May be present

Answer 68

Lobular Panniculitis

Question 69

TABLE 73-2 Differentiating Septal vs Lobular Panniculitis Characteristic findings Miescher granuloma

Answer 69

Septal Panniculitis

Question 70

TABLE 73-2 Differentiating Septal vs Lobular Panniculitis Characteristic findings None

Answer 70

Lobular Panniculitis

Question 71

TABLE 73-2 Differentiating Septal vs Lobular Panniculitis Type of panniculitis Erythema nodosum

Answer 71

Septal Panniculitis

Question 72

TABLE 73-2 Differentiating Septal vs Lobular Panniculitis Type of panniculitis Erythema induratum

Answer 72

Lobular Panniculitis

Question 73

TABLE 73-2 Differentiating Septal vs Lobular Panniculitis Type of panniculitis Lupus panniculitis

Answer 73

Lobular Panniculitis

Question 74

TABLE 73-2 Differentiating Septal vs Lobular Panniculitis Type of panniculitis Pancreatic panniculitis

Answer 74

Lobular Panniculitis

Question 75

TABLE 73-2 Differentiating Septal vs Lobular Panniculitis Type of panniculitis Lipodermatosclerosis

Answer 75

Lobular Panniculitis

Question 76

TABLE 73-2 Differentiating Septal vs Lobular Panniculitis Type of panniculitis α1-Antitrypsin panniculitis

Answer 76

Lobular Panniculitis