DERM: Cutaneous manifestations of systemic disease

Question 1

what is Acanthosis nigracans?

Answer 1

Acanthosis nigricans is a skin condition characterized by areas of dark, velvety discoloration in body folds and creases. The affected skin can become thickened. Most often, acanthosis nigricans affects your armpits, groin and neck.

• No specific treatment is available for acanthosis nigricans. Treatment of underlying conditions may restore some of the normal color and texture to affected areas of skin.

Question 2

which type of people does Acanthosis nigracans commonly affect?

Answer 2

typically occur in people who are obese or have diabetes

Question 3

what is Pyoderma gangrenosum?

Answer 3

Pyoderma gangrenosum (pie-o-DUR-muh gang-ruh-NO-sum) is a rare condition that causes large, painful sores (ulcers) to develop on your skin, most often on your legs.

Question 4

what is the cause of Pyoderma gangrenosum thought to be?

Answer 4

unknown, but it appears to be a disorder of the immune system. People who have certain underlying conditions, such as inflammatory bowel disease or arthritis, are at higher risk of pyoderma gangrenosum.

Question 5

what is Erythema nodosum?

Answer 5

swollen fat under the skin causing red bumps and patches. It usually goes away by itself but it can be a sign of something serious.

• looks like cellulitis in patches

Question 6

Pyoderma gangrenosum has strong associations with which underlying disorders?

Answer 6

haematological malignancy

inflammatory bowel disease

rheumatoid

Question 7

who does Erythema nodosum commonly affect?

Answer 7

young women

Question 8

which part of the body does Erythema nodosum commonly affect?

Answer 8

lower legs

Question 9

what is lupus erthematosus?

Answer 9

Autoantibodies drive inflammation in the skin +/- other organs

• Discoid (DLE)
• Sub-acute cutaneous (SACLE)
• Systemic (SLE)

Question 10

what is SLE?

Answer 10

Systemic lupus erythematosus (“lupus”) is an inflammatory autoimmune connective tissue disease. It is “systemic” because it affects multiple organs and systems and “erythematosus” refers to the typical red malar rash that occurs across the face.

Question 11

which auto antibodies are associated with SLE?

Answer 11

SLE is associated with anti-nuclear antibodies (ANA). These are antibodies against normal proteins in the cell nucleus.

Question 12

what is the treatment for SLE?

Answer 12

• NSAIDs
• Steroids (prednisolone)
• Hydroxychloroquine (first line for mild SLE)
• Suncream and sun avoidance for the photosensitive the malar rash

Question 13

what is Discoid lupus erythematosus (DLE)?

Answer 13

a chronic photosensitive skin eruption which can be either localised or widespread.

It is confined to the skin and does not cause any systemic symptoms. DLE can cause permanent scarring if treatment is inadequate.

Question 14

who does Discoid lupus erythematosus (DLE) affect?

Answer 14

• much more women than men
• between ages 20 - 40
• more common in smokers
• can be present in a small number of patients with SLE

Question 15

what is the presentation of Discoid lupus erythematosus (DLE)?

Answer 15

• red scaly patches which leave pigmentation, atrophy, white scars
• lesions may be asymptomatic or may present with mild pruritus or sometimes pain within the lesions.
• affects areas exposed to. sunlight
• may affect the lips and inside the mouth, causing ulcers and scaling.

Question 16

what parts of the skin does Discoid lupus erythematosus (DLE) commonly affect?

Answer 16

DLE mainly affects areas exposed to sunlight, such as the cheeks, nose, ears, upper back, neck and the backs of hands. It may rarely occur on the palms or soles.

Question 17

which dermatological condition causes causing wart-like lesions and white scars, most often on the extensor aspects of the arms?

Answer 17

Discoid lupus erythematosus (DLE)

Question 18

what investigations would you do to diagnose Discoid lupus erythematosus (DLE)?

Answer 18

• clinical features.
• biopsy
• Serology: approximately 20% of patients with DLE have a positive antinuclear antibody (ANA).
• There may be a low white cell count and raised ESR.

Question 19

how do you manage Discoid lupus erythematosus (DLE)?

Answer 19

• minimise sun exposure, high-SPF sunscreen every 2 hours.
• Smoking cessation.
• cosmetics can be used to hide lesions
• Corticosteroids may be used topically or intralesionally e.g. triamcinolone acetonide, (systemic don’t work)
• Other possible treatments include topical retinoids and immunosuppressive agents (eg, azathioprine or methotrexate).

Question 20

why is Smoking cessation is highly recommended in patients with Discoid lupus erythematosus (DLE)?

Answer 20

• smoking is associated with more severe disease

- decreases the efficacy of antimalarials.

Question 21

what are some complications of Discoid lupus erythematosus (DLE)?

Answer 21

A minority of patients with DLE (fewer than 5%) progress to SLE.
In paediatric patients, however, DLE carries a significant risk of progression to SLE but may predict a milder phenotype of systemic disease.

Malignant degeneration (basal cell carcinoma or squamous cell carcinoma) may occur but is uncommon

Dark skin may lose its inherent protection with depigmentation.

Question 22

what are some risk factors for SLE?

Answer 22

Certain human leukocyte antigen DRB1 types are more common in lupus patients - eg, DR3 and DR2.

Patients who have a defective C4 complement gene (C4A null allele) also develop a lupus-like illness.

Question 23

what is the presentation of SLE like?

Answer 23

• remitting-relapsing
• non-specific symptoms e.g. fatigue, malaise, fever, lymphadenopathy, weightloss
• arthralgia common
• secondary fibromyalgia common
• Raynaud’s phenomenon
• malar (butterfly) rash
• photosensitivity

Question 24

what is systemic sclerosis (scleroderma)?

Answer 24

Systemic sclerosis (SSc), also known as scleroderma, is a multi-system, autoimmune disease, characterised by functional and structural abnormalities of small blood vessels, fibrosis of skin and internal organs, and production of auto-antibodies.

Question 25

what are the two types of systemic sclerosis (scleroderma)?

Answer 25

Limited cutaneous systemic sclerosis

Diffuse cutaneous systemic sclerosis

Question 26

what are features of Limited cutaneous systemic sclerosis?

Answer 26

NB: used to be called CREST syndrome

C – Calcinosis
R – Raynaud’s phenomenon
E – oEsophageal dysmotility
S – Sclerodactyly
T – Telangiectasia

Question 27

what are features of Diffuse cutaneous systemic sclerosis?

Answer 27

includes CREST and:

Cardiovascular problems, particularly hypertension and coronary artery disease.

Lung problems, particularly pulmonary hypertension and pulmonary fibrosis.

Kidney problems, particularly glomerulonephritis and a condition called scleroderma renal crisis.

Question 28

what does scleroderma mean?

Answer 28

refers to hardening of the skin. This gives a the appearance of shiny, tight skin without the normal folds in the skin. These changes are most notable on the hands and face.

Question 29

what does sclerodactyly mean?

Answer 29

Sclerodactyly describes the skin changes in the hands. As the skin tightens around joints it restricts the range of motion in the joint and reduces the function of the joints. As the skin hardens and tightens further the fat pads on the fingers are lost. The skin can break and ulcerate.

Question 30

what are some clinical features of systemic sclerosis (scleroderma)?

Answer 30

• scleroderma
• sclerodactyly
• telangiectasia
• Calcinosis
• Raynaud’s phenomenon
• oesophageal dysmotillity
• systemic and pulmonary hypertension
• pulmonary fibrosis

Question 31

which auto antibodies are associated with systemic sclerosis (scleroderma)?

Answer 31

Antinuclear antibodies (ANA) are positive in most patients with systemic sclerosis. They are not specific to systemic sclerosis.

Anti-centromere antibodies are most associated with limited cutaneous systemic sclerosis.

Anti-Scl-70 antibodies are most associated with diffuse cutaneous systemic sclerosis. They are associated with more severe disease.

Question 32

how can you treat systemic sclerosis (scleroderma)?

Answer 32

• Nifedipine can be used to treat symptoms of Raynaud’s phenomenon
• Anti acid medications (e.g. PPIs) and pro-motility medications (e.g. metoclopramide) for gastrointestinal symptoms
• Analgesia for joint pain
• Antibiotics for skin infections
• Antihypertensives can be used to treat hypertension (usually ACE inhibitors)
• Treatment of pulmonary artery hypertension
• Supportive management of pulmonary fibrosis

Question 33

what is morphoea?

Answer 33

localised scleroderma (patchers of hard skin)

• plaques are usually self-limiting and tend to fade
• often no treatment is needed
• more common in children.

Question 34

what is lichen sclerosus?

Answer 34

Lichen sclerosus (LS) is a chronic inflammatory dermatosis which usually affects the skin of the anogenital region in women, and the glans penis and foreskin in men. It occurs less commonly in extragenital areas. It does not cause any systemic disease outside the skin.

Question 35

what is the presentation of lichen sclerosus?

Answer 35

The lesions are white thickened patches (porcelain-white papules and plaques) in genital region.

• itching common in women but not men.
• may be asymptomatic

Question 36

how do you diagnose lichen sclerosus?

Answer 36

Diagnosis usually made clinically in primary care

biopsy indicated when there is diagnostic uncertainty or suspected malignancy

swabs mot routinely required

Blood tests: current evidence and guidelines advise that an autoantibody screen to look for associated autoimmune disease is useful only if there are clinical features to suggest an autoimmune disorder.

Question 37

what is the treatment for lichen sclerosus?

Answer 37

Use ultra-potent topical steroids (clobetasol propionate 0.05%) applied once-daily until remission, then gradually reduced.