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41 Cards in this Set

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MC locations for lupus panniculitis?

cheeks, shoulders, chest, upper back, outer thighs

MC locations for a1-antitrypsin deficiency panniculitis?

butt and hips

MC location for erythema nodosum?

anterior shins

MC location for erythema induratum?

calves

Location erythema nodosum vs erythema induratum?

EN is on the anterior shins


EI is on the calves

MC location of lipodermatosclerosis?

wine bottle!! medial shins

Septal panniculitis includes which entities?

EN, a1AT, morphea profundus

Causes of erythema nodosum?

SHOUT BCG!!!



Sarcoidosis/sulfa/strep, histo, OCP/preg, UC, TB, Bechets, Chrons, GI (yersenia)



NODOSUM


No cause is found in 30-50% of cases


Drug (OCP, sulfa, minocycline, PCN(


Oral contraceptives


Sarcoidosis (Lofgren's syndrome)


Ulcerative colitis


Malignancy/microbiology (URI, strep, tb, yersinia, mycoplamsa, HSV, HBV, lymphomas)

Lesions of EN re often bilateral and preceded by a prodrome of fever, malaise, leukocytosis and arthralgias. How long do they last?

they DO NOT ulcerate


they resolve spontaneously within 3-6 weeks


new lesions continue to develop over a period of weeks to months

What is a miescher granuloma?

pathognomonic for EN, collection o fhistiocytes in radial array around vessels

Treatment for EN?

They will resolve spontaneously in 3-6 weeks



But, bed rest, elevation NSAIDS, ASA (avoid if presents with IBD), SSKI, IL or oral steroids, colchicine, antimalarials

Dosing regimen and side effects of SSKI?

5 drops TID, increase by 1 drop per dose per day up to 30 drops TID, once controlled, taper over 2-3 weeks



SE:


acute--> nausea, bitter taset, salivation, urticaria, vasculitis, angioedema


chronic--> hypothryoidism > hyperthyroidism, enlargement of salivary and lacrimal glands, acneiform eruption, iododerma

Erythema nodosum migrans?

same as erythema nodosum, except UNILATERAL



less painful and more chronic than EN (course is months to years)



TX: SSKI

Alpha 1 antitrypsin deficiency, gene found on which chromosome? How does it present?

Chromosome 14, found in PIZZ individuals (PIMM individuals are normal)



Presents with multiple painful subQ nodules (fluctuant, ulcerate and drain) on butt and lower back, will also see LUNG issues and LIVER issues

Which panniculitic nodules can ulcerate and drain?

panniculitis of alpha 1 antitrypsin deficiency

Histopathologic progression of alpha-1 antitrypsin deficiency?

early septal panniculitis with neutrophilic infiltrate



later stages include septal and lobular infiltrate with lymphocytes, foam cells and fibrosis

Treatment of alpha-1 antitrypsin deficiency?

doxy 200mg BID x 3 months for mild disease



dapsone, colchicine, IV a-1



liver transplant is curative

Lobular panniculitis includes:



subcutaneous fat necrosis of the newborn


sclerema neonatorum


postersteroid panniculitis


pancreatic fat necrosis


sclerosing panniculitis


cold panniculitis


cytophagic histiocytic panniculitis

healthy, full term infant born via c section, at birth, notice firm subcutaneous nodules on cheeks, buttocks and back. Workup?

This is subcutaneous fat necrosis of the newborn



Baby is HEALHTY



Most times related to some sort of fetal distress: birth trauma, cord accidents, meconium aspiration, C section, placenta previa, neonatal asphyxiation, hypothermia



Get a calcium level! can be associated with hypercalcemia, so you need to get levels on these little guys for the next 4 months



this will spontaneously regress in a few months

Histology of subcutaneous fat necrosis of the newborn?

Needle shaped clefts arranged in radial fashion within adiposites
 
Crystals are formed from dissolved TG
 
Can have focal calcium deposition

Needle shaped clefts arranged in radial fashion within adiposites



Crystals are formed from dissolved TG



Can have focal calcium deposition

fat necrosis of the newborn- see needle shaped clefts



can have focal calcium deposition in SQFNNB, calcs are rare in sclerema neonatorum



path looks the same as sclerema neonatorm, so get the clinical!

Pull baby out, he's cold and hypoglycemic, put him in the NICU and within 3 days he starts developing symmetric, diffuse induration on the legs spreading upwards to the truck. Work up?

Thsi is sclerema neonatorum- presents in first week of life



Cold and hypoglycemia? sepsis. Other associated illnesses: sepsis, pneumonia, diarrhea, dehydration, intestinal obstruction or congenital heart disease



presents in first week of life, a/w high morbidity and mortality

What is post steroid panniculitis?

rare complication of rapid systemic steroid WITHDRAWAL in children



see firm, red nodules/plaques on cheeks, arms trunk within 1-40 days



path looks identical to SQFNNB



resolves spontaneously within weeks or when steroids are started again

Pancreatic panniculitis- lobular panniculitis with 'ghost cells' (saponification of fat)

49 year old male smoker comes in to the ER with diffuse jaundice, periumbilical abdominal pain, 20 pound weight loss over the past 2 months, fever and tender, red subcutaneous nodules on pretibial legs and buttocks. Derm is paged for the nodules. What are we worried about?

20# weight loss and fever, periumbilical pain, diffuse jaundice? Sounds like pancreatic cancer to me. Now has pancreatic panniculitis on legs and butt (a/w pancreatitis and carcinoma)



Thought to be d/t release of trypsin, lipase, amylase by disease pancreatitis --> trypsin damages the vasculature allowing lipase to enter fat and necrose it

Lipodermatosclerosis! aka sclerosing panniculitis.



occus in middle aged and older women with vascular insufficiency, mc on medial LE



venous stasis --> ischemia and infarction of subcutaneous fat

biopsy from a woman with 'bilateral cellulitis'.  What is this finding called?

biopsy from a woman with 'bilateral cellulitis'. What is this finding called?

Lady has venous stasis change and resulting sclerosing panniculitis (lipodermatosclerosis)



membranous fat necrosis with lipomembranous change (feathering)

This can be found in which conditions?

This can be found in which conditions?

LIPOMEMBRANOUS CHANGE



venous insufficiency


diabetes


erythema nodosum


nodular vasculitis


infective panniculitis


pancreatic panniculitis


sarcoidosis


morphea profundus

What is cold panniculitis?

Erythematous and indurated plaques and nodules found in infants and young children after exposure to cold or cold substances, secondary to higher saturated to unsaturated fatty acid ratio in infants



resolve in 1-3 weeks

Lupus erythematous panniculitis (lupus profundus) can be seen in either localized cutaneous or systemic lupus. Most common locations?

head, UPPER ARMS, buttocks, thigh



deep, firm, asymptomatic, single or multiple subQ nodules

SLE patient develops several deep, firm, asymptomatic subQ nodules on her cheeks bilaterally, what is this? What do we need to check for?

Lupus panniculitis (lupus profundus) most likely, BUT, need to rule out subcutaneous panniculitis like T cell lymphoma, so STAIN IT!



more indolent form is CD8+, BF1+ and should be classified as subcutaneous panniculitis like T cell lymphoma



more aggressive form is CD8+ and BF-

Cytophagic histiocytic panniculitis is a chronic recurrent disease of immune dysregulation. Develops in association with what infections and a/w which malignancies?

Infections: CMV, EBV, HIV, various bacterial



Malignancies: subcutaneous panniculitic T cell lymphoma, angiocentric T cell lymphoma

Histiocytes stuffed with lymphocytes, erythrocytes, platelets, neuts aka beanbag cells are pathomneumonic for...

cytophagic histiocytic panniculitis

Calciphylaxis has an uncertain pathogenesis, but is almost exclusively occurs in what population?

patients with ESRD, often undergoing dialysis --> abnormality of calcium/phosphate matabolism followed by calcification of the vasculature in subcutaneous fat

How does calciphylaxis present?

unilateral or bilateral pruritic and painful livedoid, violaceous plaques and nodules on legs



morbility and high mortality of up to 60%

Labs to check when you suspect calciphylaxis?

calcium phosphate product > 60-70mg/dL




strangely, most patients have normal calcium and phosphate

Treatment for calciphylaxis?

phosphate binders, cinacalet, IV sodium thiosulfate, parathyroidectomy

Type II reactions typically occur in lepromatous leprosy and can be subdivided into three clinical variants:


erythema nodosum leprosum



erythema polymorphous-like reaction



Lucio's phenomenon (mainly considered type II reaction, but may be classified as type III)



Attending to the clinical and histopathological manifestations, leprosy reactions may be separated in 2 or 3 different variants: reverse reaction (type I), erythema nodosum leprosum (type II), erythema polymorphous (type II) and Lucio's phenomenon, mainly considered a type II reaction, but sometimes designated type III.

treatment of erythema nodosum leprosum?

thalidomide

What is Lucio's Phenomenon?

Type II/III (depending on what you read) reaction to lepromatous leprosy, see crops of painful hermorrhagic infarcts forming crusted ulcerations

Type II/III (depending on what you read) reaction to lepromatous leprosy, see crops of painful hermorrhagic infarcts forming crusted ulcerations

type X hypersentisitivity?

type X hypersentisitivity?

Erythema induratum/nodular vasculitis- likely a type IV hypersensitivity cell mediated response




look for TB with PPD or quantiferon gold



Will see fat necrosis + vasculitis