Granulomatous Disorders

Question 1

Sarcoidosis can be induced by IFN-a

Answer 1

True

Question 2

Sarcoidosis peaks at 20-40 years and again at 60y

Answer 2

True

Question 3

Sarcoidosis can relate to Hodgkin lymphoma

Answer 3

True

Question 4

Mycobacterial DNA/RNA is not usually found in sarcoidosis lesions

Answer 4

False 80%

Question 5

Pathology of sarcoidosis

Answer 5

Aggregates of epithelioid cells with sparse lymphocytic component “naked”
- in some cases, tuberculoid granuloma are observed
—> these are NON CASEATING granuloma with lymphoid cuff >25% granulomas diameter
—> caseous necrosis is typically absent
—> epidermis usually normal

Question 6

List types of sarcoidosis

Answer 6

1. Maculopapular
2. Plaque or nodules
3. Scar sarcoidosis
4. Lupus pernio
5. Subcutaneous sarcoidosis

Question 7

Maculopapular sarcoidosis has a poorer prognosis compared to other forms

Answer 7

False - more favourable

Question 8

Polarisable foreign bodies are often found in biopsies

Answer 8

True

Question 9

Nodular and plaque sarcoidosis is much rarer than maculopapular sarcoidosis

Answer 9

False - almost as common as maculopapular

Question 10

Nodular and plaque scarring do not scar

Answer 10

False - permanent scarring can occur when they resolve

Question 11

Nodular and plaque sarcoidosis activity persists for <18 months

Answer 11

False >2years

Question 12

Sarcoidosis Nodular and plaque type can be associated with sarcoidosis of the upper resp tract and bony cysts

Answer 12

False not associated unlike lupus pernio

Question 13

Lupus pernio is very painful and tends to ulcerate

Answer 13

False - not painful and does not ulcerate

Question 14

Lupus pernio is more mutilating than lupus vulgaris

Answer 14

False - less mutilating

Question 15

In a minority of cases, lupus pernio is associated with sarcoidosis of the upper resp tract, especially with involvement of nasal rims

Answer 15

False - majority >1/2 have involvement eg. Paratracheal granulomas and infiltration of vocal cords
- also associatd with pulmonary fibrosis and chronic uveitis

Question 16

Lupus pernio is associated with bony cysts affecting terminal phalanges —-> when this occurs, nails are usually dystrophic

Answer 16

True

Question 17

Scar sarcoidosis — tattoo sarcoidosis is considered a variant

Answer 17

True

Question 18

Scar sarcoidosis can occur decades after tattoo

Answer 18

True

Question 19

Scar sarcoidosis is often located on the knees

Answer 19

True

Question 20

Most cases of subcutaneous sarcoidosis are in males

Answer 20

False - white women in 5th to 6th decades

Question 21

Subcutaneous sarcoidosis may form indurated linear bands from elbow to hand

Answer 21

True

Question 22

Sarcoid dactylitis occurs with SC sarcoidosis

Answer 22

True

Question 23

Scarring alopecia secondary to sarcoidosis is often in Caucasian women

Answer 23

False - African women

Question 24

Nail changes in sarcoidosis

Answer 24

Splinter haemorrhages, thinning, pitting, thickening, longitudinal ridging, onycholysis, paronychia, pterygium, subungal HK, TRACHYONYCHIA, red /brown nail bed discolouration all reported in sarcoidosis

Question 25

What are ocular findings in sarcoidosis

Answer 25

Anterior/posterior uveitis, chorioretinitis, periblepharitis, papilloedema, retinal haemorrhage

Question 26

What are chest radiograph stages of sarcoidosis

Answer 26

Stage 0 - normal CXR Stage 1 - bilateral hilar LN without pulmonary involvement Stage 2 - bilateral hilar LN with pulmonary involvement Stage 3 - pulmonary involvement without bilateral hilar involvement

Question 27

Haemoptysis is a common feature of sarcoidosis

Answer 27

False - rare

Question 28

Extracutaneous features of sarcoidosis

Answer 28

PERL <3 MPN Pulmonary Eye Reticuloendothelial system (lymphadenopathy, spleen 5-10% , BM inv rare)’ Liver (hepatomegaly, periportal areas affected, severe chronic cholesterol syndrome, portal hypertension and Budd-Chirac rare) Heart (arrhythmias, heart block , sudden death, cor pulmonale) MSK (transient or chronic poly arthralgias common, myalgias rare) Parotid (enlargement and xerostomia) Neurological

Question 29

What is Heerfordt syndrome

Answer 29

Parotid gland enlargement, with cranial nerve palsy, uveitis and fever

Question 30

What are nonspecific lesions of sarcoidosis

Answer 30

``` Erythema nodosum Sweet syndrome Cutaneous calcinosis Prurigo Digital clubbing (poor prognostic sign) PG associated ```

Question 31

Sarcoidosis pulmonary function test results

Answer 31

Abnormal RFTs in 20% in patients with mild disease, Obstructive and restrictive abnormalities - reduced DLCO and VC - as functional impairment occurs with decreased FVC and diffusing capacity

Question 32

Granuloma annulare subcutaneous form is usually in middle aged women

Answer 32

False , in children

Question 33

List CLINICAL subtypes of GA

Answer 33

Localised Generalised Perforating Subcutaneous

Question 34

List histological subtypes of GA

Answer 34

Necrobiotic palisading granulomas Interstitial Sarcoidal Subcutaneous

Question 35

What infections can trigger GA?

Answer 35

``` Scabies Hep B/C TB HPV VZV EBV Parvovirus B19 HIV Borrelia burgdorferi ```

Question 36

Traumatic triggers of GA

Answer 36

Vaccination, Tuberculin testing Arthropod bites Isomorphic response - saphenectomy, waxing, PUVA

Question 37

75% cases of GA are localised

Answer 37

True

Question 38

subcutaneous GA occurs on scalp and legs, particularly pretibial

Answer 38

True

Question 39

Subcutaneous GA involves the mucous membranes commonly

Answer 39

False - uncommon except in HIV patients

Question 40

GA most common locations?

Answer 40

``` 60% isolated to hands and arms 20% on legs and feet 7% upper and lower extremity 5% on trunk and other RARE facial lesions ```

Question 41

Actinic granuloma more common in males

Answer 41

False - females

Question 42

Actinic granuloma has more telangiectasia, alopecia and yellow discolouration than GA

Answer 42

False - absent

Question 43

Necrobiosis lipoidica more common in males

Answer 43

False - females

Question 44

Annular erythematous rim enhancing lesions with central atrophy and telangiectases accurately describes NL

Answer 44

True

Question 45

Comedo like lesions can be seen at periphery of NL

Answer 45

True

Question 46

IBD is not associated with NL

Answer 46

False , it is

Question 47

Palisaded neutrophilic and granulomatous dermatitis (PNGD), where annular plaques or linear cords favour trunk, axillae and medial thighs in patients with RA or seronegative arthritis

Answer 47

False, this is interstitial granulomatous dermatitis

Question 48

Palisaded neutrophilic and granulomatous dermatitis —> asymmetrical umbilicated papules over elbow and extensor surfaces

Answer 48

False symmetrical

Question 49

PNGD occurs in pt with RA, systemic LE, Wegener’s

Answer 49

True

Question 50

Cutaneous crohn’s - erythematous plaques, lymphoedema and knife-like ulcerations most commonly in the anogenital region

Answer 50

True

Question 51

There is consistent correlation between mucutaneous disease and degree of GI involvement

Answer 51

False

Question 52

Cutaneous involvement of crohn’s can be indicative of colonic rather than ileal involvement

Answer 52

True

Question 53

Treatment of cutaneous crohn’s includes metronidazole 250mg tds for 3 mo , pred, sulfasalazine, AZA, 6-MP

Answer 53

True