The Skin In Systemic Disease

Question 1

Lupus subtypes and symptoms?

Answer 1

Systemic lupus erythematosus - chillblains, photodistributed rash, palpable purpura, livedo reticularis, subacute (cutaneous) lupus erythematosus (CPPL)
Discoid - scarring, subacute (cutaneous) lupus erythematosus (SS)

Question 2

Mucocutaneous lupus erythematosus?

Answer 2

Cutaneous lupus - acute or chronic
Oral ulcers
Alopecia

Question 3

Haematological systemic lupus erythematosus?

Answer 3

Haemolytic anaemia
Thrombocytopenia
Leukopenia

Question 4

Immunological systemic lupus erythematosus?

Answer 4

ANA
AntidsDNA antibody
Anti-Sm
Antiphospholipid
Low complement
Direct Coomb’s test

Question 5

Neonatal Lupus tests?

Answer 5

Ro positive
ECG - fetal heartblock

Question 6

Dermatomyositis signs?

Answer 6

GReatnesS HelPs

Gottron’s papules
Ragged cuticles
Shawl sign
Heliotrope rash
Photosensitive erythema

proximal extensor inflammatory myopathy

Question 7

Anti Jo-1 subtype?

Answer 7

Fever, myositis, gottron’s papules

Question 8

Anti SRP subtype?

Answer 8

Necrotising myopathy

Question 9

Anti Mi-2 subtype?

Answer 9

Mild muscle disease

Question 10

Anti-p155 subtype?

Answer 10

Malignancy (in adults)

Question 11

Anti-p140 subtype?

Answer 11

Juvenile, associated with calcinosis

Question 12

Anti-SAE subtype?

Answer 12

Amyopathic

Question 13

Anti-MDA5 subtype?

Answer 13

ILD, digital ulcers, ischaemia

Question 14

Dermatomyositis tests?

Answer 14

ANA, CK, LFT (often ALT raised), EMG
Biopsy, screening for internal malignancy

Question 15

Vasculitis subtypes?

Answer 15

IgA vasculitis (Henoch-Schonlein purpura)
Small vessel
Small + medium vessel
Medium vessel
Large vessel

Question 16

IgA vasculitis?

Answer 16

Shown on biopsy for immunoflorescence (peri vascular IgA)
Abdo pain, bleeding, Arthralgia, arthritis (IgA associated)
Glomerulonephritis may develop later

Henoch-Schonlein purpura

Question 17

Small vessel manifestations?

Answer 17

Purpura

Question 18

Medium vessel manifestations?

Answer 18

Retiform purpura
Ulcers
Digital necrosis
Subcutaneous nodules along blood vessels

RUDS

Question 19

Sarcoidosis signs?

Answer 19

SURE (Let’s Play)

Scar sarcoid
Ulcerative
Red brown violaceous papules
Erythema nodosum
Lupus Pernio

Histology - non-caseating epithelioid Granulomas
Commonly affects lungs

Question 20

DRESS scoring criteria?

Answer 20

Fever (over 38.5)
Lymphadenopathy (over 2 sites, over 1cm)
Circulating atypical lymphocytes
Peripheral hyper eosinophilia (over 0.7)
Internal organ involvement (liver, kidney, cardiac)
Negative ANA, hep, chlamydia, mycoplasma
Skin involvement (>50% NSA, cutaneous eruption suggestive of DRESS, biopsy suggestive of DRESS)

Question 21

DRESS stands for?

Answer 21

Drug Reaction with Eosinophilia and Systemic Symptoms

Question 22

Internal organ involvement in DRESS?

Answer 22

Liver
Kidneys
Heart
Brain
Thyroid
Lungs

Question 23

DRESS onset, cause of death, triggers?

Answer 23

2-6 weeks after drug exposure
Liver most common organ involved, most common cause of death
Common triggers - sulfonamides, anti-epileptics, ABx, ibuprofen

Question 24

DRESS rash morphologies?

Answer 24

WHUME

Widespread erythema
Head/neck oedema
Urtricated papillae exanthem
Maculopapular eruption
Erythema multiforme-like

Question 25

DRESS treatment?

Answer 25

Withdrawal of culprit Corticosteroids

Question 26

Graft versus Host Disease signs?

Answer 26

*FAD* Face involvement Acral involvement Diarrhoea

Question 27

GvHD? What, who and where?

Answer 27

Multi-organ disease Affects 10-80% of allogenic haematopoietic stem cell transplants Mainly affects - skin, liver, GI tract

Question 28

GvHD pathogenesis?

Answer 28

Donor derived T lymphocyte activity against antigens in an immunocompromised recipient

Question 29

Pruritus causes?

Answer 29

Lymphoma, polycythemia Ureaemia, cholestasis Iron deficiency or overload HIV/hepatitis a/b/c Cancer Drugs Psychogenic Pruritus of old age

Question 30

Pruritus meaning?

Answer 30

Itching without rash suggestive of internal cause

Question 31

Pruritus investigations?

Answer 31

FBC, LDH Renal profile Liver function test Ferritin XR chest HIV/Hep A/b/c

Question 32

Pruritus sign?

Answer 32

Nodular Prurigo *NP*

Question 33

Carcinoid syndrome meaning, cause and symptoms?

Answer 33

Signifies metastases of malignant carcinoid tumour 5-HT secretion Hypotension Diarrhoea Bronchospasm Flushing *HD BF*

Question 34

Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis symptoms?

Answer 34

Prodromal - flu like sx Blisters merge - sheets of skin detachment ‘like wet wallpaper’ Abrupt onset of lesions on trunk>face/limbs Macules, blisters, erythema - atypical targetoid Extensive thickness mucocuteneous necrosis <2-3 days *Pro BAME*

Question 35

SJS vs TEN?

Answer 35

SJS - BSA detachment <10%, mortality <10% SJS TEN - BSA detachment 10-30% TEN - BSA detachment >30%, mortality >30%

Question 36

SJS/TEN causes and differentials?

Answer 36

Cell-mediated cytotoxic reaction against epidermal cells Drugs cause >80% of cases May be started up to 3 weeks prior to onset of rash DDx- staphylococcal scalded skin syndrome, thermal burns, cutaneous GvHD

Question 37

SCORTEN?

Answer 37

Score to assess severity of SJS/TEN Criteria - age >40, initial % epidermal detachment, serum urea + glucose + bicarbonate, presence of malignancy

Question 38

Erythroderma?

Answer 38

Generalised erythema affecting >90% BSA

Question 39

Erythroderma systemic manifestations?

Answer 39

*PT DLR* Peripheral oedema Tachycardia Disturbance in thermoregulation Loss of fluids and proteins Risk of sepsis

Question 40

Erythroderma etiologies?

Answer 40

Drug reactions Cutaneous T-cell lymphoma (Sézary syndrome) Psoriasis Atopic eczema Idiopathic

Question 41

Erythroderma management?

Answer 41

Treat underlying (psoriasis, drug) Hospitalisation if unwell Restore fluid and electrolyte imbalance Emollients +/- topical steroids +/- antibiotics

Question 42

Which condition can cause Erythroderma?

Answer 42

Sézary syndrome