DERM: Cutaneous manifestations of systemic disease Flashcards
what is Acanthosis nigracans?
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.
which type of people does Acanthosis nigracans commonly affect?
typically occur in people who are obese or have diabetes
what is Pyoderma gangrenosum?
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.
what is the cause of Pyoderma gangrenosum thought to be?
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.
what is Erythema nodosum?
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
Pyoderma gangrenosum has strong associations with which underlying disorders?
haematological malignancy
inflammatory bowel disease
rheumatoid
who does Erythema nodosum commonly affect?
young women
which part of the body does Erythema nodosum commonly affect?
lower legs
what is lupus erthematosus?
Autoantibodies drive inflammation in the skin +/- other organs
- Discoid (DLE)
- Sub-acute cutaneous (SACLE)
- Systemic (SLE)
what is SLE?
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.
which auto antibodies are associated with SLE?
SLE is associated with anti-nuclear antibodies (ANA). These are antibodies against normal proteins in the cell nucleus.
what is the treatment for SLE?
- NSAIDs
- Steroids (prednisolone)
- Hydroxychloroquine (first line for mild SLE)
- Suncream and sun avoidance for the photosensitive the malar rash
what is Discoid lupus erythematosus (DLE)?
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.
who does Discoid lupus erythematosus (DLE) affect?
- much more women than men
- between ages 20 - 40
- more common in smokers
- can be present in a small number of patients with SLE
what is the presentation of Discoid lupus erythematosus (DLE)?
- 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.
what parts of the skin does Discoid lupus erythematosus (DLE) commonly affect?
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.
which dermatological condition causes causing wart-like lesions and white scars, most often on the extensor aspects of the arms?
Discoid lupus erythematosus (DLE)
what investigations would you do to diagnose Discoid lupus erythematosus (DLE)?
- 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.
how do you manage Discoid lupus erythematosus (DLE)?
- 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).
why is Smoking cessation is highly recommended in patients with Discoid lupus erythematosus (DLE)?
- smoking is associated with more severe disease
- decreases the efficacy of antimalarials.
what are some complications of Discoid lupus erythematosus (DLE)?
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.
what are some risk factors for SLE?
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.
what is the presentation of SLE like?
- remitting-relapsing
- non-specific symptoms e.g. fatigue, malaise, fever, lymphadenopathy, weightloss
- arthralgia common
- secondary fibromyalgia common
- Raynaud’s phenomenon
- malar (butterfly) rash
- photosensitivity
what is systemic sclerosis (scleroderma)?
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.
what are the two types of systemic sclerosis (scleroderma)?
Limited cutaneous systemic sclerosis
Diffuse cutaneous systemic sclerosis
what are features of Limited cutaneous systemic sclerosis?
NB: used to be called CREST syndrome
C – Calcinosis R – Raynaud’s phenomenon E – oEsophageal dysmotility S – Sclerodactyly T – Telangiectasia
what are features of Diffuse cutaneous systemic sclerosis?
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.
what does scleroderma mean?
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.
what does sclerodactyly mean?
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.
what are some clinical features of systemic sclerosis (scleroderma)?
- scleroderma
- sclerodactyly
- telangiectasia
- Calcinosis
- Raynaud’s phenomenon
- oesophageal dysmotillity
- systemic and pulmonary hypertension
- pulmonary fibrosis
which auto antibodies are associated with systemic sclerosis (scleroderma)?
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.
how can you treat systemic sclerosis (scleroderma)?
- 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
what is morphoea?
localised scleroderma (patchers of hard skin)
- plaques are usually self-limiting and tend to fade
- often no treatment is needed
- more common in children.
what is lichen sclerosus?
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.
what is the presentation of lichen sclerosus?
The lesions are white thickened patches (porcelain-white papules and plaques) in genital region.
- itching common in women but not men.
- may be asymptomatic
how do you diagnose lichen sclerosus?
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.
what is the treatment for lichen sclerosus?
Use ultra-potent topical steroids (clobetasol propionate 0.05%) applied once-daily until remission, then gradually reduced.
