Granulomatous Disorders Flashcards
Sarcoidosis can be induced by IFN-a
True
Sarcoidosis peaks at 20-40 years and again at 60y
True
Sarcoidosis can relate to Hodgkin lymphoma
True
Mycobacterial DNA/RNA is not usually found in sarcoidosis lesions
False 80%
Pathology of sarcoidosis
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
List types of sarcoidosis
- Maculopapular
- Plaque or nodules
- Scar sarcoidosis
- Lupus pernio
- Subcutaneous sarcoidosis
Maculopapular sarcoidosis has a poorer prognosis compared to other forms
False - more favourable
Polarisable foreign bodies are often found in biopsies
True
Nodular and plaque sarcoidosis is much rarer than maculopapular sarcoidosis
False - almost as common as maculopapular
Nodular and plaque scarring do not scar
False - permanent scarring can occur when they resolve
Nodular and plaque sarcoidosis activity persists for <18 months
False >2years
Sarcoidosis Nodular and plaque type can be associated with sarcoidosis of the upper resp tract and bony cysts
False not associated unlike lupus pernio
Lupus pernio is very painful and tends to ulcerate
False - not painful and does not ulcerate
Lupus pernio is more mutilating than lupus vulgaris
False - less mutilating
In a minority of cases, lupus pernio is associated with sarcoidosis of the upper resp tract, especially with involvement of nasal rims
False - majority >1/2 have involvement eg. Paratracheal granulomas and infiltration of vocal cords
- also associatd with pulmonary fibrosis and chronic uveitis
Lupus pernio is associated with bony cysts affecting terminal phalanges —-> when this occurs, nails are usually dystrophic
True
Scar sarcoidosis — tattoo sarcoidosis is considered a variant
True
Scar sarcoidosis can occur decades after tattoo
True
Scar sarcoidosis is often located on the knees
True
Most cases of subcutaneous sarcoidosis are in males
False - white women in 5th to 6th decades
Subcutaneous sarcoidosis may form indurated linear bands from elbow to hand
True
Sarcoid dactylitis occurs with SC sarcoidosis
True
Scarring alopecia secondary to sarcoidosis is often in Caucasian women
False - African women
Nail changes in sarcoidosis
Splinter haemorrhages, thinning, pitting, thickening, longitudinal ridging, onycholysis, paronychia, pterygium, subungal HK, TRACHYONYCHIA, red /brown nail bed discolouration all reported in sarcoidosis
What are ocular findings in sarcoidosis
Anterior/posterior uveitis, chorioretinitis, periblepharitis, papilloedema, retinal haemorrhage
What are chest radiograph stages of sarcoidosis
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
Haemoptysis is a common feature of sarcoidosis
False - rare
Extracutaneous features of sarcoidosis
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
What is Heerfordt syndrome
Parotid gland enlargement, with cranial nerve palsy, uveitis and fever
What are nonspecific lesions of sarcoidosis
Erythema nodosum Sweet syndrome Cutaneous calcinosis Prurigo Digital clubbing (poor prognostic sign) PG associated
Sarcoidosis pulmonary function test results
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
Granuloma annulare subcutaneous form is usually in middle aged women
False , in children
List CLINICAL subtypes of GA
Localised
Generalised
Perforating
Subcutaneous
List histological subtypes of GA
Necrobiotic palisading granulomas
Interstitial
Sarcoidal
Subcutaneous
What infections can trigger GA?
Scabies Hep B/C TB HPV VZV EBV Parvovirus B19 HIV Borrelia burgdorferi
Traumatic triggers of GA
Vaccination,
Tuberculin testing
Arthropod bites
Isomorphic response - saphenectomy, waxing, PUVA
75% cases of GA are localised
True
subcutaneous GA occurs on scalp and legs, particularly pretibial
True
Subcutaneous GA involves the mucous membranes commonly
False - uncommon except in HIV patients
GA most common locations?
60% isolated to hands and arms 20% on legs and feet 7% upper and lower extremity 5% on trunk and other RARE facial lesions
Actinic granuloma more common in males
False - females
Actinic granuloma has more telangiectasia, alopecia and yellow discolouration than GA
False - absent
Necrobiosis lipoidica more common in males
False - females
Annular erythematous rim enhancing lesions with central atrophy and telangiectases accurately describes NL
True
Comedo like lesions can be seen at periphery of NL
True
IBD is not associated with NL
False , it is
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
False, this is interstitial granulomatous dermatitis
Palisaded neutrophilic and granulomatous dermatitis —> asymmetrical umbilicated papules over elbow and extensor surfaces
False symmetrical
PNGD occurs in pt with RA, systemic LE, Wegener’s
True
Cutaneous crohn’s - erythematous plaques, lymphoedema and knife-like ulcerations most commonly in the anogenital region
True
There is consistent correlation between mucutaneous disease and degree of GI involvement
False
Cutaneous involvement of crohn’s can be indicative of colonic rather than ileal involvement
True
Treatment of cutaneous crohn’s includes metronidazole 250mg tds for 3 mo , pred, sulfasalazine, AZA, 6-MP
True