Panniculitis Flashcards

1
Q

Classification of septal panniculitis

A

Septal Panniculitis Nearly Made Grandma Require New Elbows

  • superficial migratory thrombophlebitis
  • PAN
  • NLD
  • Deep morphea
  • Deep GA
  • Rheumatoid nodule
  • Necrobiotic xanthogranuloma
  • EN
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2
Q

Classificaiton of lobular panniculitis

A

Lobular Balls Lie Calcified + Cold Laying Like Dried Peas And Injured Fingers Sound So Traumatic - SSSo Good Fat X

  • leprosum
  • bazin
  • lipodermatosclerosis
  • calciphylaxis
  • cold panniculitis
  • LE
  • Lymphoma / cytophagic histiocytic
  • DM
  • Pancreatic
  • Alpha1 antitrypsin
  • Infectious
  • Factitious
  • Sweet’s
  • Sarcoid
  • Trauma
  • Subcut fat necrosis of newborn
  • Sclerema neonatorum
  • Steroid induced
  • Gouty
  • Fungal
  • XRT
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3
Q

Which panniculitis can ulcerate?

A

NICE PLANT

  • neutrophilic (autoimmune, Sweet’s)
  • infective (Bazin)
  • calciphylaxis
  • Cytophagic histiocytic panniculitis
  • PAN
  • lymphoma
  • alpha-1 antitrypsin
  • NLD
  • Trauma, factitious
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4
Q

What are the two lobular panniculitides with vasculitis

A

Erythema nodosum leprosum

Erythema induratum of Bazin

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5
Q

What is erythema nodosum leprosum?

A

Type II reaction in lepromatous/ borderline lepromatous leprosy
Immune complex vasculitis

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6
Q

What is erythema induratum of Bazin

A

Tuberculid reaction

Possible immune complex mediated vasculitis vs Type IV hypersensitivity

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7
Q

What are the triggers of nodular vasculitis of Whitfield

A

Infective - Hep C, nocardia, fungal, viral, protozoal
Drugs - propylthiouracil
Malignancy - paraneoplastic, leukemia
Autoimmune

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8
Q

DDx for lipodermatosclerosis

A

Acute - cellulitis, other panniculitides, pretibial myxoedema
Chronic - morphea, eosinophilic fasciitis, scleroderma, scleromyxoedema, GVHD, nephrogenic systemic fibrosis, EN, acrodermatitis chronica atrophicans

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9
Q

Associations with calciphylaxis

A

Uraemic, non-uraemic

  • end stage renalfailure
  • high calcium, phosphate
  • dialysis
  • hyper-parathyroid
  • Diabetes
  • HTN
  • obesity
  • warfarin
  • corticosteroids
  • high vitamin D
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10
Q

Clinical features of calciphylaxis

A

Pain out of proportion
Usually lower limbs - fatty areas, or abdomen (lots of pictures of ankles though)
Livedo, retiform purpura, bleeding, haemorrhagic bullae, stellate necrosis

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11
Q

Management of calciphylaxis

A
Admit to hospital (almost 'emergency' due to high mortality)
Multidisciplinary - derm, renal, nursing/wound care, surgeons, pain team
Analgesia
Low calcium diasylate
Calcium/phosphate binders
Cinacalcet
Low calcium diet
Anticoag if warfarin not a trigger
Parathyroidectomy
Sodium thiosulphate 
Debridement if necrotic/infected
Ab's if infected
Wound care - dressings etc
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12
Q

Management of lipodermatosclerosis

A

Leg elevation, compression
Lose weight, exercise
Reported - pentoxyfilline, HCQ, anabolic steroids
Refer to vascular surgeons

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13
Q

Histology of lipodermatosclerosis

A
Lobular panniculitis w/o vasculitis
Lipomembranous change, thickening/ fibrosis of septa
Prominent vessels (venous stasis)
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14
Q

DDx of lupus panniculitis

A

SC panniculitis-like TCL
dermatomyositis panniculitis
cold panniculitis

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15
Q

Cold panniculitis - who gets it?

A

Infants - extra brown fat more susceptible to cold

Adults - obese, horse-riders

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16
Q

Histology of cold panniculitis

A

Lobular panniculitis, absence of needle-clefts distinguishes from SC fat necrosis of the newborn + sclerema neonatorum

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17
Q

Lupus panniculitis risk of SLE

18
Q

Histology of lupus panniculitis

A

lobular panniculitis
Hyaline necrosis is characteristic
Lymphocytic infiltrate
Histo DDx - SC panniculitis-like TCL

19
Q

Clinical features of lupus panniculitis

A

Affects face, proximal arms

Can be associated DLE-changes overlying - pigment, telangiectasis, follicular plugging, atrophy, scarring, tethering)

20
Q

How to distinguish lupus panniculitis from SC panniculitis-like TCL?

A

Lupus panniculitis

  • more commonly ulcerate
  • more proximal sites
  • other signs of lupus
  • histology - may be overlying DLE changes + absence of rimming of lymphocytes, also IHC differs)
  • if perform TCR gene rearrangement - polyclonal
21
Q

Histo + IHC for SC panniculitis-like TCL?

A

Histo - may be limited pleomorphism, rimming of lymphocytes around the fat cells is a clue + infiltration between collagen bundles + around adnexa; may be atypical lymphocytes
if cytophagic panniculitis present - beanbag cells (macrophages containing fragmented cells/ debris)
CD2+ CD8+ CD4- CD30-
alpha-beta pattern (gamma-delta is aggressive + not considered in this classificaition anymore)

22
Q

Features of dermatomyositis panniculitis

A

Less common in DM than panniculitis in LE
Also resolves w atrophy
similar histo to LE panniculitis - lobular w/o vasculitis, lymphocytes, hyaline sclerosis

23
Q

What is schmid triad?

A

In pancreatic panniculitis - presence of EAP!! eosinophilia, arthritis + panniculitis
– may indicate pancreatic Ca

24
Q

Pathophysiology of pancreatic panniculitis

A

Release of lipase/ amylase /tryptase –> digests lobular fat –> necrosis

Associated with pancreatitis (acute or chronic), pancreatic malignancy, post procedural (transplant, ERCP)

25
Clinical features of pancreatic panniculitis
Tender erythematous nodules over ankles/ shins which ulcerate --> ooze oily brown sterile excudate
26
Genetics of alpha-1 antitrypsin deficiency
``` autosomal co-dominant SERPINA1 (PI) gene 120 allelic variants PIZZ = v slow PISS = slow PIMM = medium ```
27
Pathophysiology of alpha-1 antitrypsin deficiency
alpha-1 antitrypsin inhibits trypsin + complement etc - deficiency leads to increased enzymatic tissue destruction --> liver, pancreas, lungs, skin, kidneys
28
Clinical features of alpha-1 antitrypsin deficiency + Mx
more proximal than pancreatic panniculitis --- trunk, shoulders/ hips Can be fever cellulitis apearance --> ulceration --> oily exudate Pathergy MX - tetracycline, dapsone, pred, HCQ, liver t/p
29
Infectious panniculitis aetiology, features
Due to bacteria (strep, staph, pseudomonas, klebsiella...) or fungal Mostly immunocompromised patients May present as sporotrichoid spread - most commonly feet/ legs
30
What is factitious panniculitis?
External injury - injection (fillers, chemotherapy drugs... anything), mechanical trauma, chemical trauma, thermal injury Causes vasoconstriction, ischemia, inflammation
31
What are the causes of neutrophilic lobular panniculitis?
``` SC sweet's Behcet's RA BADAS Also - alpha-1 antitrypsin, pancreatic, factitious ```
32
What is the other name for SC sarcoid?
Darier Roussy
33
Ix in SC sarcoid?
Bx - deep incisional to confirm + tissue culture (exc TB) + Quant Gold (exc TB) FBC, ELFTs, ESR, CRP, ACE, 24H urinary Calcium, serum clacium ANA, ENA, dsDNA, serum EPP Ig (bad prognosis if elevated) Eye check, heart - TnI, ECG, lungs - CXR, RFTs, renal - urine PrCr Pre-treatment Ix
34
Mx options in sarcoid
Topicals Systemic - Sarcoid Has Many Typical Treatments, I May Also Try Steroid, HCQ, Mtx, Tetracycline, TNFi, isotretinoin, MMF, Aza, thalidomide
35
SC fat necrosis of the newborn - features
Typically well baby Triggered by cold, trauma, ischemia (eg prolonged birth, hypoxia, seizures, cardiac surgery) Histo - refractile, radially arranged needle-shaped clefts (as for sclerema neonatorum + steroid-induced) Self-limited, symmetrical over shoulders + buttocks Risk of hypercalcemia (treat w frusemide, low calcium feeds)
36
Sclerema neonatorum - features
Unwell baby - preterm, septic, congenital heart disease, disseminated infection) Generalised woody indurated skin High mortality (75%)
37
Criteria for metabolic syndrome?
Don't Have That Hot Pie for Breakfast - T2DM (+ 2 of) - HTN - Triglyceridemia - low HDL - Proteinuria - BMI > 30
38
What are the effects of obesity on the skin?
- sweat/friction - intertrigo, inflammation - infection - candida, dermatophyte, erythrasma, cellulitis, furunculosis, onychomycosis, necrotising fasciitis - circulatory changes - venous stasis, lymphoedema, lipodermatosclerosis, ulcers, elephantiasis nostra verrucosa - elevated androgens, insulin, GF - poor healing - irritant dermatitis - striae - keratoderma - acanthosis nigricans - skin tags - KP - cellulite obesity lymphoedematous mucinosis - worsening of HS, psoriasis, rosacea, eczema, acne etc
39
What are the monogenic obesity syndromes?
``` Prader Willi POMC defieincy McCune Albright Albright hereditary osteodystrophy Carney complex Fragile X ```
40
What are the genetic associations/causes for multiple lipomas?
``` Familial multiple lipomatosis Madelung's (benign symmetrical lipomatosis) Dercum disease NF-1 Gardner Cowden Proteus CLOVES MEN-1 ```