Pannic litis Panniculitis

Transcription

Pannic litis Panniculitis
Panniculitis
Pannic litis
Nathan C. Walk, M.D.
Normal morphology of subcutaneous fat
„
Sub--Q fat has a limited repertoire of responses to
Sub
noxious stimuli
– Fat necrosis
ƒ Various
V i
forms:
f
– Enzymatic, crystalline, suppurative, hyalinizing, microcystic
– LIPOPHAGIC – most common, least specific
– Fat often involved secondarily in another primary process
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Vasculitis
Deep fungal
R di ti
Radiation
Trauma
metastatic
– Considerable
C
id bl overlap!!
l !!
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Stages
g of lesion
– Neutrophils first….
– Lymphocytes with giant cells and lipid
lipid--containing macrophages
– Fibrosis
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Adequate biopsy is the key
Panniculitis
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Septal panniculitis (Venous disorders)
– Erythema nodosum
– Necrobiosis lipoidica
p
– Scleroderma
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Lobular panniculitis (Arterial supply disruption)
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Subcutaneous fat necrosis of the newborn
Sclerema neonatorum
WeberWeber-Christian disease
Alpha 11-antitrypsin deficiency
Cytophagic histiocytic panniculitis
PanniculitisPanniculitis-like TT-cell lymphoma
Pancreatic panniculitis
Lupus panniculitis
Panniculitis associated with large vessel vasculitis
– Cutaneous polyarteritis
l
nodosa
d
– Superficial migratory thrombophlebitis
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Case 129
Lupus profundus
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½ of cases have epidermal and dermal changes of LE
– ?Which are…..
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Lobular panniculitis with prominent lymphocytic infiltrate
– Characteristic feature: paraseptal lymphoid follicles, sometimes with
germinal center
ƒ Uncommon in other panniculitides
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Late lesions with dystrophic calcification
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Cas130
Paraffinoma
aka…Sclerosing
k S l
i lipogranuloma
li
l
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Special form of facticial panniculitis resulting from injection of lipi
(often paraffin) into subcutaneous tissue
Well
e ccircumscribed
cu sc bed nodule
odu e
„ ? Swiss cheese appearance
„ Bands of hyaline fibrous tissue between fat cells and cystic spaces
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Case 131
Lobular panniculitis, c/w Erythema
i d
induratum
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In lobular panniculitides, inflammatory infiltrate is
presentt th
throughout
h t th
the llobule…BUT,
b l BUT there
th
is
i often
ft some
septal involvement as well.
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Nodular vasculitis/Erythema induratum
– Originally 1 entity, then 2 (Bazin, Whitfield), now one again?
– Appears to be a disease of diverse etiologies
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Lobular or septolobular panniculitis… with:
– Granulomatous inflammation – usually poorly developed, ALSO
with neutrophils,
neutrophils lymphocytes,
lymphocytes and some plasma cells
– Vasculitis
– Necrosis
– Septal fibrosis
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Case 132
Lipodystrophy
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Lipoatrophy
p
p y
– Primary, idiopathic = lipodystrophy
– Secondary
ƒ Follows other panniculitides (lupus, morphea, …))
ƒ Looks the same histologically
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In established cases – atrophy of fat withOUT
inflammation
– Involutional changes… small lipocytes and intervening
hyaline or myxoid connective tissue
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Early or “active” lesions may have lobular
panniculitis w/ foam cells, small fat cysts
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Case 133
Pancreatic panniculitis
Lobular panniculitis
„ Enzymatic fat necrosis
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– Liquefaction
Li
f ti with
ith breakdown
b kd
off fat
f t cells
ll
ƒ Ghost
Ghost--like outline of fat cells remaining
– At margins of necrotic fat:
ƒ Variable neutrophils, nuclear dusting, fine
basophilic calcium deposits, hemorrhage
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Case 134
Septal panniculitis, c/w Erythema
Nodosum
d
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Septal panniculitis
– Mostly lymphocytes, variable numbers of giant cells
– Center of lobule spared
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Early lesions – more neutrophils
Later lesions – more fibrosis, septal thickening
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Case 135
Calcifying panniculitis
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Calciphylaxis
– Rare,
R
life
lifelif -threatening
th t i syndrome
d
– Progressive microvascular and superficial soft tissue calcification
– Setting of secondary hyperparathyroidism and chronic renal failure
– Painful violaceous skin lesions that progress to nonhealing ulcers with
underlying tissue necrosis
– Many complications…mortality >60%
– Histology:
ƒ Intravascular calcium deposits within small and mediummedium-sized vessels
ƒ Also within extravascular soft tissues and viscera
ƒ May have endovascular fibroblasticfibroblastic-intimal proliferation,
proliferation luminal thrombosis,
thrombosis
or calcific obliteration of vessels
ƒ “Clean” necrosis
– Relatively scant inflammation
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“Calcifying
y g panniculitis”
p
– A number of other conditions may imitate calciphylaxis
histopathologically – diagnosis requires careful integration of
clinical and pathologic information
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Peripheral vascular disease
Atheroembolus syndrome
Septic embolism
Dermatomyositis
Mixed CTD
Antiphospholipid antibody syndrome
DIC
Pyoderma gangrenosum
Lipodermatosclerosis
….
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Case 136
Subcutaneous fat necrosis of the
newborn
b
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Normal epidermis and dermis with an
underlying lobular panniculitis
– Mixed inflammatoryy infiltrate
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Many fat cells retain their outline
– Narrow clefts radiate from periphery of cell
ƒ Contain doubly refractile needleneedle-like crystals
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Same histology as Sclerema neonatorum
– Except, SN has less inflammation
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Case 137
Lipodermatosclerosis
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Synonyms:
– Membranous lipodystrophy
– Sclerosing
g Panniculitis
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Histology:
– Stasis changes at top
– Septal fibrosis – THICK septa
– Fatty microcysts with “membranocystic change”
ƒ Feathery cuticle inside cyst – “arabesque pattern”
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Case 138
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Granulomatous panniculitis
– AFB, GMS, Gram all negative
– Secondary to:
ƒ Trauma
ƒ Infection
ƒ …..?