Sarcoidosis & Granulomatous Flashcards
What is Lofgren’s syndrome?
Acute self-limiting form of sarcoidosis Loaf around all day and you FAILE: Fever Arthralgia + Migratory polyarthritis Iritis Lymphadenopathy - bilateral hilar on CXR Erythema nodosum NSAIDs 90% resolve in 2 years dont confuse with Loefflers syndrome
What is Mikulicz’s syndrome?
Milk - think of glands Bilateral swelling of parotid, salivary and lacrimal glands Associations; Sjogrens SLE Leukaemia Hodgkins Syphylis Sarcoidosis Tuberculoisis
What is Heerfordt’s syndrome?
Rare manifestation of sarcoidosis; Hear me PUFF Parotid swelling Uveitis Fevers Facial nerve palsies - sometimes
T/F
subcut GA needs aggressive Rx
F
dont treat it
resolves spontaneously
Treatment ladder for GA?
Do nothing 50% resolve in 2 years - esp if localised dont treat kids - resolve dont treat subcut gA - resolves Potent TCS and/or TCNI ILCS second line for localised LN2 cryo - 3rd line for localised Many 4th line options; PDT UVA1 Immiquimod PDL laser IFN injection scarification or excision
If generalised or severe; UVA1 first line Isotretinoin Dapsone topical tacrolimus PDT PUVA good for generalized GA – oral or topical psoralens NbUVb less good for generalized GA Many other 3rd line options inc; Hydroxychloroquine Nicotininamide CsA TNF blockers
Investigations in GA?
Fasting glucose and urine dip for sugar
TFTs and thyroid autoantibodies
FBC, HIV test and Hep C if generalised
Consider RF or antiCCP if rheum nodule differential
T/F
NLD seen in 3% of diabetics
F
0.3%
NLD treatment ladder?
Avoid trauma as can cause ulceration
Stop smoking
If diabetic optimize control
Urgent biopsy if clinical suspicion of SCC
Potent TCS under occlusion changed weekly esp for early lesions - 1st line
ILCS - after TCS
Then TOP NLD - 2nd line Rxs;
T - Tacrolimus ointment
OP - Oral Prednisolone short course (5 weeks) may halt progression
N - High dose Nicotinamide (500mg TDS) (some evidence) – starts as 500mg OD for 1wk, then BD for 1 wk then TDS – 2nd line
L - Light = topical PUVA
Dye - Clofazimine 200mg OD – 2nd line
T/F
Aspirin or assasantin/dipyridamole work for some people with NLD
F
shown to be ineffective
What are later options for NLD Rx?
Topical;
- Tretinoin
- G-CSF
- bovine collagen
- Perilesional heparin injection
Systemic;
- Chloroquine, hydroxychloroquine (some evidence)
- CsA (some evidence)
- Etanercept (some evidence)
- Infliximab (some evidence)
- Pentoxyfiline, stanozolol, inositol niacinate, nicofuranose, ticlopidine hydrochloride
- Thalidomide
- Fibrinolytic agents (streptokinase, tPA)
- Tretinoin
- Prostaglandin
- MMF, CsA
- IVIg
Physical and surgical;
- UVA1
- PDT
- Hyperbaric oxygen
- Split thickness autografting and immunomodulatory therapy
- Pulsed dye laser – improved erythema and telys but skin breakdown risk
- Simple excision and grafting (SSG) if ulcerated
- Grafting with bioengineered dermal tissue
T/F
optimizing diabetic control improves NLD
F
No link between diabetic control and developing or curing NLD
But diabetics with NLD have inc risk of retinopathy and nephropathy so should optimize to reduce these
Histo of actinic granuloma?
In mid and upper dermis there is an infiltrate of lymphocytes, histiocytes and foreign body type multi-nucleated giant cells
Dermal elastosis and Elastophagocytosis – elastin fibres in and around giant cells stain w/ EVG
Asteroid bodies in the granulomas
often solar elastosis
• No telys (cf NLD)
• No altered collagen (necrobiosis) or mucin
T/F
LN2 cryo works well for actinic granuloma
F
cryo not effective
No good treatment exists
Treatment ladder for actinic granuloma
sun protect and TIPP TCS ILCS PUVA Plaquenil/antimalarials
what conditions get rheumatoid nodules?
7-20% of patients with RA 75% with Feltys syndrome 5% patients with SLE rare assocs; ankylosing spondylitis granuloma annulare chronic active hepatitis infrequently in healthy children and adults
