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 Various V i forms: f – Enzymatic, crystalline, suppurative, hyalinizing, microcystic – LIPOPHAGIC – most common, least specific – Fat often involved secondarily in another primary process Vasculitis Deep fungal R di ti Radiation Trauma metastatic – Considerable C id bl overlap!! l !! Stages g of lesion – Neutrophils first…. – Lymphocytes with giant cells and lipid lipid--containing macrophages – Fibrosis Adequate biopsy is the key Panniculitis Septal panniculitis (Venous disorders) – Erythema nodosum – Necrobiosis lipoidica p – Scleroderma Lobular panniculitis (Arterial supply disruption) – – – – – – – – 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 Case 129 Lupus profundus ½ of cases have epidermal and dermal changes of LE – ?Which are….. Lobular panniculitis with prominent lymphocytic infiltrate – Characteristic feature: paraseptal lymphoid follicles, sometimes with germinal center Uncommon in other panniculitides Late lesions with dystrophic calcification Cas130 Paraffinoma aka…Sclerosing k S l i lipogranuloma li l 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 Case 131 Lobular panniculitis, c/w Erythema i d induratum 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. Nodular vasculitis/Erythema induratum – Originally 1 entity, then 2 (Bazin, Whitfield), now one again? – Appears to be a disease of diverse etiologies Lobular or septolobular panniculitis… with: – Granulomatous inflammation – usually poorly developed, ALSO with neutrophils, neutrophils lymphocytes, lymphocytes and some plasma cells – Vasculitis – Necrosis – Septal fibrosis Case 132 Lipodystrophy Lipoatrophy p p y – Primary, idiopathic = lipodystrophy – Secondary Follows other panniculitides (lupus, morphea, …)) Looks the same histologically In established cases – atrophy of fat withOUT inflammation – Involutional changes… small lipocytes and intervening hyaline or myxoid connective tissue Early or “active” lesions may have lobular panniculitis w/ foam cells, small fat cysts Case 133 Pancreatic panniculitis Lobular panniculitis Enzymatic fat necrosis – Liquefaction Li f ti with ith breakdown b kd off fat f t cells ll Ghost Ghost--like outline of fat cells remaining – At margins of necrotic fat: Variable neutrophils, nuclear dusting, fine basophilic calcium deposits, hemorrhage Case 134 Septal panniculitis, c/w Erythema Nodosum d Septal panniculitis – Mostly lymphocytes, variable numbers of giant cells – Center of lobule spared Early lesions – more neutrophils Later lesions – more fibrosis, septal thickening Case 135 Calcifying panniculitis 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: Intravascular calcium deposits within small and mediummedium-sized vessels Also within extravascular soft tissues and viscera May have endovascular fibroblasticfibroblastic-intimal proliferation, proliferation luminal thrombosis, thrombosis or calcific obliteration of vessels “Clean” necrosis – Relatively scant inflammation “Calcifying y g panniculitis” p – A number of other conditions may imitate calciphylaxis histopathologically – diagnosis requires careful integration of clinical and pathologic information Peripheral vascular disease Atheroembolus syndrome Septic embolism Dermatomyositis Mixed CTD Antiphospholipid antibody syndrome DIC Pyoderma gangrenosum Lipodermatosclerosis …. Case 136 Subcutaneous fat necrosis of the newborn b Normal epidermis and dermis with an underlying lobular panniculitis – Mixed inflammatoryy infiltrate Many fat cells retain their outline – Narrow clefts radiate from periphery of cell Contain doubly refractile needleneedle-like crystals Same histology as Sclerema neonatorum – Except, SN has less inflammation Case 137 Lipodermatosclerosis Synonyms: – Membranous lipodystrophy – Sclerosing g Panniculitis Histology: – Stasis changes at top – Septal fibrosis – THICK septa – Fatty microcysts with “membranocystic change” Feathery cuticle inside cyst – “arabesque pattern” Case 138 Granulomatous panniculitis – AFB, GMS, Gram all negative – Secondary to: Trauma Infection …..?