Rashes due to systemic diseases: Erythema nodosum, Pyoderma gangrenosum, Necrobiosis lipodica, Granuloma annulare, CLE, Erythema multiforme Flashcards

1
Q

What is erythema nodosum?

A

Acute erythematous nodular eruption from skin due to inflammation of subcutaneous fat

It is a skin condition characterized by painful, red nodules, primarily on the lower legs.

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2
Q

What type of hypersensitivity reaction is associated with erythema nodosum?

A

Delayed type 4 hypersensitivity reaction

This reaction is often linked to underlying systemic diseases.

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3
Q

What percentage of erythema nodosum cases have no identifiable cause?

A

60%

This indicates that in many cases, the underlying cause remains unknown.

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4
Q

Name three drug classes that can cause erythema nodosum.

A
  • NSAIDs
  • Bromides
  • Sulfonamides

Other drugs like iodides, penicillins, and salicylates can also be implicated.

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5
Q

List three systemic diseases associated with erythema nodosum.

A
  • SLE (Systemic Lupus Erythematosus)
  • Sarcoidosis
  • Crohn disease

Ulcerative colitis is also associated.

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6
Q

Which malignancies are linked to erythema nodosum?

A
  • Lymphoma
  • Renal cell carcinoma
  • Leukaemia

These cancers can contribute to the development of the condition.

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7
Q

Name two types of microbiological infections that can cause erythema nodosum.

A
  • TB (Tuberculosis)
  • Viruses

Other infections include leprosy, deep fungal infections, and group A strep.

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8
Q

Describe the appearance of erythema nodosum nodules.

A

Erythematous, non-scarring, oval nodules that begin as light red then become darker and bruised

They typically have a symmetrical distribution.

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9
Q

Where on the body are erythema nodosum nodules most commonly found?

A

On shins, knees, ankles

However, they can occur anywhere on the body.

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10
Q

What are some associated symptoms of erythema nodosum?

A
  • Fever
  • Malaise
  • Arthralgia

These symptoms often indicate an underlying illness.

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11
Q

What is the primary method of diagnosing erythema nodosum?

A

Clinical diagnosis

Investigations may include throat swabs and imaging tests.

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12
Q

Which test is used to identify streptococcal infection in erythema nodosum cases?

A

Streptolysin (ASO) titre

This test helps determine if a streptococcal infection is present.

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13
Q

What is the recommended initial management for erythema nodosum?

A

RICE and analgesia (e.g., NSAIDs)

RICE stands for Rest, Ice, Compression, Elevation.

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14
Q

True or False: Erythema nodosum usually resolves spontaneously within weeks to months.

A

True

Treatment should also focus on addressing the underlying cause.

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15
Q

What is pyoderma gangrenosum?

A

It is an ulcerative autoinflammatory disorder that is a form of neutrophilic dermatoses.

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16
Q

Who does pyoderma gangrenosum usually affect?

A

It usually affects adults, not children.

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17
Q

What are common associations in the pathophysiology of pyoderma gangrenosum?

A

It can be idiopathic but is usually associated with inflammatory disorders, haematological disorders, immunodeficiencies, drugs, and history of trauma or surgery.

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18
Q

What inflammatory disorders are associated with pyoderma gangrenosum?

A

Inflammatory bowel disease (IBD) and inflammatory arthritis.

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19
Q

What haematological disorders are associated with pyoderma gangrenosum?

A

Myeloproliferative disorders (commonly AML), myelodysplastic syndromes (MDS), and monoclonal gammopathy of undetermined significance (MGUS).

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20
Q

What immunodeficiency is associated with pyoderma gangrenosum?

A

HIV infection.

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21
Q

What drugs are associated with pyoderma gangrenosum?

A

Cocaine, isotretinoin, and propylthiouracil (for hypothyroidism).

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22
Q

What is the typical presentation of pyoderma gangrenosum?

A

Painful, rapidly growing, irregularly-shaped papules or pustules that develop into large purulent ulcers with a grey-purple border and surrounding erythematous rim.

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23
Q

Where are pyoderma gangrenosum lesions usually located?

A

They are usually located on the lower extremities.

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24
Q

What is the hallmark of healing in pyoderma gangrenosum?

A

Healing leaves hallmark cribriform scarring, which is a criss-cross pattern.

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25
Q

How are lesions classed in pyoderma gangrenosum?

A

Lesions are classed as pustular, vesiculobullous, ulcerative, or vegetative.

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26
Q

What are the Maverakis criteria for pyoderma gangrenosum?

A

It includes 1 major criterion (histopathology of ulcer edge shows neutrophil infiltrate) and several minor criteria.

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27
Q

What investigations are done for pyoderma gangrenosum?

A

Charcoal swab sample of ulcer to identify microorganisms, biopsy to rule out other ulcerative causes, and tests to screen for underlying disease.

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28
Q

What is the primary management for pyoderma gangrenosum?

A

Primarily treat the underlying cause, with dermovate or calcineurin inhibitor around ulcer edges and keep the wound dressed and clean.

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29
Q

What is the first-line treatment for pyoderma gangrenosum?

A

Systemic therapy with corticosteroids and/or immunosuppressants.

30
Q

What is the second-line treatment for pyoderma gangrenosum?

A

Infliximab.

31
Q

What is Necrobiosis lipoidica?

A

A chronic skin condition associated with diabetes, usually type 1.

More common in females than males and in young or middle-aged individuals.

32
Q

What is the pathophysiology of Necrobiosis lipoidica?

A

Usually associated with diabetes but can also be linked to other metabolic and autoimmune disorders, such as rheumatoid arthritis.

33
Q

What is the presentation of Necrobiosis lipoidica?

A

Initially presents as well-demarcated red-brown-yellow plaques that progress to yellow-brown, shiny, waxy lesions with telangiectasia.

Commonly distributed symmetrically on shins and pretibial skin.

34
Q

How long do lesions from Necrobiosis lipoidica last?

A

They can last for several months before resolution, healing slowly.

35
Q

What is the management for Necrobiosis lipoidica?

A

Treatment includes potent steroid cream or calcineurin inhibitors like Tacrolimus or cyclosporin, along with monitoring diabetic control.

36
Q

What is Granuloma annulare?

A

A benign skin condition characterized by papules that form a ring with a normal or sunken center.

37
Q

What is the presentation of Granuloma annulare?

A

Initially presents as a small ring of flesh-colored or pink papules that merge to form a large annular, non-scaly, shiny plaque with a paler, sunken center.

Commonly found on the lateral and dorsal surfaces of hands and feet.

38
Q

What are the main classifications of Granuloma annulare presentation?

A
  1. Localised: Usually confined to joints, more common in children and young adults, associated with Hashimoto’s thyroiditis.
  2. Generalised/disseminated: Widespread, symmetrical, and bilateral annular patches or papules that are pink or flesh-colored.
39
Q

What is Cutaneous Lupus Erythematosus (CLE)?

A

A form of Lupus Erythematosus that is confined to skin.

40
Q

What is the typical age of onset for CLE?

A

Typically in the 30-40s.

41
Q

Is CLE more common in males or females?

A

More common in females than males.

42
Q

What genetic predispositions are associated with CLE?

A

HLA-B88, TREX1, SAMHD1.

43
Q

What factors contribute to the pathophysiology of CLE?

A

Combination of genetic and environmental factors, with an autoimmune response to unknown antigen or UV sunlight.

44
Q

How does UV exposure affect CLE?

A

Antibodies and immune complexes infiltrate the dermoepidermal junction in response to UV exposure, causing tissue damage and inflammation.

45
Q

What drugs are associated with drug-induced CLE?

A

Smoking, PPIs, hydrochlorothiazide, procainamide, hydralazine, quinidine, isoniazid.

46
Q

Can CLE progress to SLE?

A

Yes, if the patient develops lung and renal involvement.

47
Q

What are the four subtypes of CLE?

A
  1. Acute CLE 2. Subacute CLE 3. Chronic CLE 4. Drug-induced CLE.
48
Q

What characterizes Acute CLE?

A

Malar rash in a butterfly pattern over the nose bridge and malar region, sparing the nasolabial folds, with transient lesions lasting several hours to days.

49
Q

What are the forms of Subacute CLE?

A

Annular form and Papulosquamous form.

50
Q

What is the appearance of the Annular form of Subacute CLE?

A

Pink/red annular, pruritic patches that coalesce into larger patches on sun-exposed areas.

51
Q

What is the appearance of the Papulosquamous form of Subacute CLE?

A

Small pink/red, pruritic patches with scaling in sun-exposed areas.

52
Q

What characterizes Chronic CLE?

A

Discoid lupus is the most common form, with disc-shaped or annular red/purple lesions that heal with scarring.

53
Q

What is the hallmark sign of Chronic CLE?

A

‘Carpet tack’ sign due to follicular plugging.

54
Q

What is the gold standard for investigating CLE?

A

Skin biopsy with immunofluorescence.

55
Q

What will a skin biopsy show in CLE?

A

Hallmark histopathological features of each subtype.

56
Q

What is the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) management for topicals?

A

Very potent corticosteroid creams or calcineurin inhibitors.

57
Q

What are the oral management options for Cutaneous Lupus Erythematosus?

A

Steroids, immunosuppressants such as Hydroxychloroquine, methotrexate, and MMF.

58
Q

What is erythema multiforme?

A

An immune-mediated, self-limiting mucocutaneous reaction to medications or infections.

59
Q

What is the epidemiology of erythema multiforme?

A

More common in males than females; most common in children, adolescents, and adults under 40 years old.

60
Q

What are some infective causes of erythema multiforme?

A

HSV, EBV, cytomegalovirus.

61
Q

What drugs are associated with erythema multiforme?

A

Anticonvulsants and NSAIDs.

62
Q

What is the typical presentation of erythema multiforme?

A

Well-demarcated, circular red/pink papules that enlarge into ringed-plaques (target lesions) with burning or itching sensation.

63
Q

What characterizes EM target/iris lesions?

A

They have 3 concentric zones: dark red centre, pink middle zone, red outer ring.

64
Q

Where is the symmetrical distribution of erythema multiforme typically found?

A

On distal extremities (palms and soles) and face, then diffuses proximally.

65
Q

Is mucosal involvement common in erythema multiforme?

A

It is uncommon but usually limited to oral mucosa, causing swollen lips and haemorrhagic crusting.

66
Q

Are prodromal symptoms common in erythema multiforme?

A

It is rare to have prodromal symptoms such as fever and malaise.

67
Q

What are the two classes of erythema multiforme?

A
  1. EM minor: without/with mild mucosal involvement and without systemic symptoms.
  2. EM major: with severe mucosal involvement and with systemic symptoms.
68
Q

How is erythema multiforme diagnosed?

A

Clinical diagnosis.

69
Q

What is the management for erythema multiforme?

A

Resolves spontaneously within 3-5 weeks; withdraw offending drug or treat infective cause.

70
Q

What is the management for mild cases of erythema multiforme?

A

Oral antihistamines and mildly-potent topical corticosteroids.

71
Q

What is the management for recurrent or severe cases of erythema multiforme with mucosal involvement?

A

Prednisolone, cyclosporine, or azathioprine.

72
Q

What is the management for herpes-associated erythema multiforme?

A

Continuous acyclovir.