Sarcoidosis & Granulomatous Flashcards

Question

what are the classical and atypical types of cutaneous sarcoidosis?

Answer 1

``` Major skin types; Papular (very common) Plaque (very common) Nodular Maculopapular Annular (uncommon) Other classical mucocutaneous types; Lupus pernio (common) Scar sarcoid (common): Koebner phenomenon Subcutaneous (Darier-Roussy) (common) Angiolupoid form Mucosal involvement Nail involvement (affects one 1:400) Atypical forms Alopecia of scalp Atrophic forms Erythrodermic sarcoidosis (extremely rare) Hypopigmentation (esp afro-caribbeans) Ichthyosiform sarcoidosis Lichenoid sarcoidosis PMLE type Unilateral oedema of lower leg Miscellaneous Other; Lofgren’s syndrome: Heerfordt’s syndrome Mikulicz’s syndrome Reactive; EN Sweets ```

Answer 2

Infections – prone to stubborn wart virus infections Immunologically mediated conditions – autoimmune thyroid disease, CTD, lupus, chronic urticaria Effects of infiltration – pituitary or thyroid gland causing endocrine disease Vasculitis Malignancy – increased risk of lymphoproliferative disease esp lymphoma also leukaemia, cervix, lung, testis, uterus, thyroid, myloma NLD and GA

Answer 3

Most important are; Lungs, skin, eyes, heart, CNS and calcium General symptoms: Weight loss, fever, fatigue Lungs: Asymptomatic or dyspnoea, cough, wheeze, chest pain, tightness (4 stages on CXR) Eyes: anterior and posterior uveitis (anterior uveitis in 70%=most common eye feature), optic neuritis (medical emergency), dry eyes, diplopia, eyelid granulomas - Classically ‘Millet-seed’ nodules on conjunctivae Heart: often asymptomatic, sudden death, arrhythmias, heart failure, pericardial effusions, syncope CNS: cranial nerve neuropathy is most common feature (frequently facial CN7 lower MN lesion i.e. Bell’s palsy), seizures Calcium derangemnt is due to excess active vit D production; hypercalcaemia, hypercalciuria and nephrolithiasis MSS: polymyositis and myopathy occur rarely Other organs: liver, spleen, kidneys (nephrolithiasis), upper respiratory tract Lymphadenopathy and haem derangements can occur Joints: acute sarcoid arthritis often associated with EN (ankles and knees)

Answer 4

may improve during pregnancy and relapse after

Answer 5

T >60% of patients with sarcoidosis, granulomatous changes resolve without residua within 2-5 yrs 10-30% cases are chronic or progress to fibrosis

Answer 6

Acute (favourable): EN, papular, scar, | Chronic and systemic disease: plaque, lupus pernio, ulcerative

Answer 7

F usually associated with ‘benign’ transient disease that does not require treatment with clear (stage 0) CXR and >80% complete resolution within 2 yrs

Answer 8

Biopsy - usually done before proceeding to further work up FBC – thrombocytopenia, lymphopenia, pancytopenia ELFTs – elevated LFTs/bili (hepatic sarcoidosis) serum and 24hr urinary calcium serum ACE Qgold (TB in DD or as trigger) PA CXR RFTs w/DLCO ECG +/- Holter +/- Echo Refer to respiratory - all confirmed/likely cases Referral to ophthalmologist - all confirmed/likely cases Refer to cardiologist - all confirmed/likely cases Refer to neurologist - all confirmed/likely cases Also; Consider screening for infectious triggers; HSV, chlamydia, EBV, CMV, Mycoplasma, Mycobacteria Consider screening for associations; AI screen, Vasculitis screen, Malignancy screen, TFTs, prolactin, visual fields

Answer 9

4 active stages; 0) 5-10% have normal CXR 1) bilateral hilar or mediastinal lymphadenopathy 2) 1+ fine ‘fluffy’ or coarse parenchymal lung infiltrates 3) parenchymal lung infiltrates w/out hilar adenopathy 4) lung fibrosis and pulmonary insufficiency – other features variable

Answer 10

Epidermis normal Non-caseating well-demarcated, ‘naked’ granulomas in dermis or subcutis Inclusion bodies in giant cells of sarcoid include; Asteroid bodies (also in foreign body granulomas) and Schaumann (conchoids) bodies (dystrophic calcification) No mucin Always examine under polarised light for birefringent material that may mimic sarcoidosis. But FBs may be found and does not exclude sarcoid

Answer 11

Potent TCS under occlusion ILCS; 5-10mg/ml Oral corticosteroids for rapidly progressive or topically unresponsive followed by steroid-sparing agent; 1 mg/kg daily (maximum 60 mg) for up to 3 months, and then tapered if improvement or a stable level is reached, to a maintenance dose of 5–10 mg on alternate days for several months Hydroxychloroquine Methotrexate AZA then MMF used 2nd or 3rd line in Australia after MTX Others; - Minocycline/Doxycycline - Thalidomide - Isotretinoin - Anti-TNF alpha (infliximab, adalimumab) - CO2 laser for lupus pernio - UVA1 for forehead plaque sarcoid or PUVA - PDT - Excision of stubborn small areas

Answer 12

Rw at least every 6/12 if active disease If stable disease off treatment rw annually If resolved and off Rx rw annually for 3 years in case of relapse Evaluation at every visit • Medical history: skin lesions, wt loss, fever, fatigue, dyspnea, shortness of breath, cough, ocular problems, upper airway complaints, symptoms of nephrolithiasis, palpitations, syncope, symptoms of CCF, and neurologic complaints • Examination: lung, cardiac, abdomen and skin, palpate of LNs and neurologic examination Tests ordered annually • Ophthalmologic examination: slit-lamp and fundoscopic eye examination • Electrocardiogram (controversial) • Chest radiograph • Pulmonary function tests • FBC, ELFT, calcium

Sarcoidosis & Granulomatous Flashcards

(36 cards)