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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_1093_Библиотеки_им_академика_М_И_Перельмана.pdf
ESSENTIAL NOTES FOR MEDICAL AND SURGICAL FINALS
7.10. Sjögren’s syndrome
An autoimmune disease causing lymphocytic infiltration of the exocrine glands
(predominantly lacrimal and salivary glands). Occurs either as a primary disorder
or with another connective tissue disease (secondary).
CLINICAL FEATURES
● Decreased secretion from exocrine glands:
◗ dry eyes (positive Schirmer’s test)
◗ dry mouth.
● Systemic features can occur: Raynaud’s phenomenon, fatigue, arthralgia/
arthritis, skin (e.g. purpura), peripheral neuropathy as well as respiratory, renal
and liver involvement.
Complications: increased incidence of lymphoma, particularly if anti-Ro or
●
anti-La positive.
ANTIBODIES
● ANA.
● Anti-Ro or anti-La (positive in ≈ 60%).
MANAGEMENT
● Treat symptomatically:
◗ dry eyes: artifi cial tears
◗ dry mouth: saliva substitutes
◗ systemic features: steroids/immunosuppresants.
7.11. Mixed connective tissue disease (MCTD)
There are patients who present with ‘overlap syndromes’ – features of different
connective tissue diseases. In MCTD there are features of SLE, systemic sclerosis
and polymyositis with positive anti-U1RNP antibodies:
Raynaud’s syndrome, swollen hands, sclerodactyly, arthritis, polymyositis and
●
pulmonary fi brosis.
7.12. Autoantibodies
Autoantibodies of clinical signifi cance
❍
Rheumatoid factor (RF): antibody against IgG – seen in RA, cryoglobulinaemia
❍
Anti-nuclear antibodies (ANA): seen in SLE, systemic sclerosis, Sjögren’s syndrome,
MCTD, polymyositis
❍
Anti-double stranded DNA: seen in SLE
❍
ENA (extractable nuclear antigens): anti-Ro, anti-La, anti-Sm, anti-centromere,
anti-Jo1, anti-Scl70 (topoisomerase), anti-U1RNP, anti-RNA polymerase
❍
Anti-neutrophil cytoplasmic antibodies (ANCA): p-ANCA (anti-MPO); c-ANCA (antiPR3) – seen in vasculitis
❍
Anti-phospholipid antibodies: anti-cardiolipin, anti-β
anti coagulant’
-glycoprotein I, positive ‘lupus
2
7.13. Vasculitis
A group of heterogeneous conditions that feature inflammation of the blood vessel
walls. Can be primary or can occur as part of another condition (secondary).
Clinical features: initial symptoms of a systemic vasculitis are often non-specifi c
– general malaise, fever, weight loss. Management: varies slightly for each type but
generally steroids +/– immunosuppresants (e.g. cyclophosphamide).
94
RHEUMATOLOGY
Secondary
Vasculitis secondary to connective tissue disorders (SLE, Sjögren’s, RA), infection,
drug-induced, para neoplastic.
Primary
Generally classified according to the size of the vessel predominantly affected,
although there is overlap.
Large vessel
● Giant cell arteritis (temporal arteritis)
Clinical features: headache with tenderness over temporal area; may have jaw
claudication; sudden visual loss can occur secondary to an anterior ischaemic
optic neuropathy. Management: needs urgent steroid treatment. Associated
with polymyalgia rheumatica: proximal limb/shoulder muscle pain and stiffness
(commonly morning) without weakness.
Takayasu’s arteritis
●
Clinical features: claudication in arms, absent pulses, bruits.
Medium vessel
● Polyarteritis nodosa
Can be associated with hepatitis B. Clinical features: microaneurysms with skin,
kidney and gut involvement; mononeuritis multiplex.
Kawasaki disease
●
Mainly affects children. Clinical features: skin involvement (palms/soles) with
coronary aneurysm formation and rarely myocardial infarcts.
Small vessel vasculitis
a) ANCA-associated – measure antibody titres (c-ANCA = PR3 titre, p-ANCA =
MPO titre)
Wegener’s granulomatosis:
●
c-ANCA positive in ≈ 90%; involvement of:
1) sinuses, ears, eyes; 2) lungs (nodules, lung haemorrhage); 3) renal
(glomerulonephritis).
Churg-Strauss syndrome:
●
p-ANCA positive in ≈ 70%; associated with asthma
and eosinophilia.
b) Not-ANCA associated
Henoch-Schönlein purpura:
●
mainly affects children and generally self-limiting;
palpable purpura over lower limbs/buttocks, arthralgia, abdominal pain and an
IgA glomerulonephritis with haematuria.
Cryoglobulinaemia:
●
can be associated with hepatitis C.
Behçet’s disease
Clinical features: orogenital ulceration, skin involvement including erythema
nodosum and pathergy reaction, uveitis, venous thromboses, arthritis, neurological
and GI involvement.
7.14. Collagen disorders
These heritable disorders of connective tissue (HDCT) are associated with joint
hypermobility which is scored with the Beighton score (out of 9).
Ability to put hands flat on the floor with knees straight (1).
●
● Hyperextension of the elbow beyond 90 degrees (1 for each side).
● Apposition of the thumb to the flexor aspect of the forearm (1 for each side).
● Hyperextension of the knee beyond 90 degrees (1 for each side).
95
ESSENTIAL NOTES FOR MEDICAL AND SURGICAL FINALS
● Passive dorsiflexion of the MCP joint to 90 degrees (1 for each side).
Marfan’s syndrome
Autosomal dominant disorder with mutations in the fi brillin-1 gene.
CLINICAL FEATURES
● Marfanoid habitus: tall stature with long limbs (arm-span to height ratio
>1.03), arachno dactyly (positive ‘wrist’ and ‘thumb’ signs), scoliosis, pectus
carinatum or excavatum.
Aortic dilatation: can lead to aortic regurgitation or dissection.
●
● High-arched palate.
● Lens dislocation.
● Joint hypermobility.
● Spontaneous pneumothoraces.
Osteogenesis imperfecta
A number of types which vary in severity.
CLINICAL FEATURES
● Bone fragility (‘brittle bones’) leading to multiple fractures.
● Blue sclerae.
● Short stature.
● Hearing problems.
● Joint hypermobility.
Ehlers-Danlos syndromes
A group of heterogeneous conditions (both clinically and genetically) with:
Skin hyperextensibility.
●
● Fragile skin, delayed wound healing, atrophic scarring.
● Easy bruising.
● Joint hypermobility.
(Benign) joint hypermobility syndrome
CLINICAL FEATURES
● Joint hypermobility.
● Arthralgia.
● Often have mild features of other syndromes (e.g. marfanoid habitus, skin
hyper extensibility).
96
8
Dermatology
8.1. Rashes
8.2. Skin infections
8.3. Eczema
8.4. Acne
8.5. Skin tumours
8.6. Psoriasis
8.7. Bullous disorders
8.8. Naevi
8.9. Erythroderma
8.10. Other skin conditions
8.11. Miscellaneous lesions
8.1. Rashes
When describing a rash it is important to think about the following:
Site: e.g. extensor surfaces (psoriasis), flexor surfaces (atopic eczema).
●
● Size and type:
◗ macule – flat area of change in skin colour
◗ patch – sometimes used to mean a macule greater than 1 cm
◗ papule – raised lesion less than 1 cm
◗ plaque – raised, flat-surfaced, disc-shaped lesion greater than 1 cm
◗ nodule – raised solid, palpable lesion greater than 1 cm
◗ vesicle – collection of fluid less than 1 cm
◗ bulla – collection of fluid greater than 1 cm
◗ pustule – collection of pus.
● Shape: e.g. round, linear or irregular.
● Sides (the border): well- or ill-defi ned.
● Surface: e.g. crust (dried exudate), lichenification (thickening of the skin).
8.2. Skin infections
Bacterial infections
● Impetigo: superficial skin infection caused by Staph. aureus or Strep. pyogenes.
Clinical features: multiple lesions that may be vesicles, bullae or pustules.
Normally a characteristic golden-coloured crust. Treatment: if localised,
topical antibiotics can be used but in most cases systemic antibiotics (e.g.
flucloxacillin) are required.
Cellulitis:
●
deep infection of the skin and subcutaneous tissues caused by Strep.
pyogenes or Staph. aureus. Clinical features: skin becomes hot, red, tender and
swollen. Edge is poorly defined. Patient may become systemically unwell with
97
ESSENTIAL NOTES FOR MEDICAL AND SURGICAL FINALS
fever. Treatment: if mild, oral antibiotics (e.g. penicillin V and fl ucloxacillin); if
more severe, IV flucloxacillin and benzylpenicillin.
Folliculitis:
●
infection of the hair follicles, normally with Staph. aureus.
Viral infections
● Herpes infections (simplex/zoster): see Infectious Disease section.
Human papilloma virus (HPV) infection.
●
CLINICAL FEATURES Causes warts, i.e. raised lesions usually 1–2 cm in diameter
– normally on hands or feet. Treatment: topical treatments (e.g. salicylic acid) or
cryotherapy if resistant.
Others:
●
Fungal infections – see Infectious Disease section
Infestations
molluscum contagiosum; hand, foot and mouth disease.
Includes scabies (caused by Sarcoptes scabiei). Clinical features: pruritus, papular
rash, burrows around hands/feet (often in finger web spaces). Treatment: permethrin
or malathion; if lice (various types), malathion.
8.3. Eczema
Eczema = dermatitis, an inflammatory skin condition.
CLINICAL FEATURES
● Itching.
● Redness.
● Scaling.
● Papulovesicular rash.
● If chronic, the skin becomes thickened (lichenifi cation).
● Can get secondary infection.
Atopic
Associated with other atopic diseases – asthma, hay fever. May appear in fi rst year
of life. Classically occurs in adulthood over flexures. Management: emollients and
topical steroids.
Seborrhoeic
Associated with Pitysporum fungal infection. Clinical features: affects scalp, face,
flexures. Often a scaly erythematous rash, particularly around nasolabial folds/
forehead. Treatment: topical ketoconazole (shampoo, cream) may be useful.
Contact
Delayed hypersensitivity to external allergens, e.g. nickel in jewellery. Can do patch
testing if cause unclear. Treatment: acutely with topical steroids; remove/avoid
allergen to prevent recurrence.
8.4. Acne vulgaris
Very common in adolescence with variable severity, although occasionally occurs
in infants or in 30s/40s (mainly women). Skin lesions include:
Comedones (‘whiteheads’ and ‘blackheads’).
●
● Papules, pustules, nodules.
● Later on there can be scarring.
98
DERMATOLOGY
TREATMENT
● Topical if mild disease: benzoyl peroxide, retinoic acid.
● Systemic: tetracycline or (in women) cyproterone acetate (Dianette) for
moderate disease; isotretinoin for more severe disease (give advice regarding
contraception and alcohol avoidance).
Rosacea
Often called ‘acne rosacea’ because lesions resemble acne (i.e. papules and pustules BUT
no comedones). Most common in middle-aged women, over cheeks, forehead, nose, chin.
Treatment: oral tetracyclines can be useful.
8.5. Skin tumours
Benign tumours
Seborrhoeic keratoses
Raised, flat, frequently pigmented lesions that typically develop on the trunk of
the elderly. Extremely common and often multiple. They are often said to seem as
if they are ‘stuck-on’. Management: generally do not require treatment but can use
cryotherapy.
Dermatofi broma
Small, asymptomatic firm papules, found mainly on the legs and more common
in women.
Solar (actinic) keratoses
Erythematous, scaly patches found on light-exposed skin and caused by sun damage.
Importantly, they are pre-malignant.
Malignant tumours
Basal cell carcinoma (BCC)
Occur on sun-exposed areas, particularly head and neck, in older people. Initially
a pearly-white nodule with telangiectatic vessels on the surface which bleed easily.
Later, expands outwards to leave a rolled edge with central ulceration (commonly
then known as a ‘rodent ulcer’). Locally invasive but rarely metastasises. Treatment:
surgical excision +/– local radiotherapy. If superficial, curettage or cryotherapy
may suffi ce.
Squamous cell carcinoma (SCC)
Occasionally remains confined to the epidermis, when it is known as SCC in situ
or Bowen’s disease. However, can be locally invasive and can metastasise to local
lymph nodes. Related to sun exposure (therefore occurs on sun-exposed regions)
but other factors are involved (e.g. smoking is associated with SCC of mouth/lips).
Commonly presents as an ulcerated nodule but can present in other forms, e.g. a
polypoid mass. Treatment: surgical excision +/– radiotherapy.
Malignant melanoma
Occurs in younger people than BCC or SCC but again related to sun exposure.
Three main types:
superfi cial spreading:
●
(most common) pigmented macule or patch; irregular
border and may have irregular pigmentation; may bleed or itch
nodular
●
● acral (occur on sole or palm).
99
ESSENTIAL NOTES FOR MEDICAL AND SURGICAL FINALS
Malignant melanoma may also occur within a lentigo maligna. Prognosis depends
on how deep the tumour is – the ‘Breslow thickness’ at biopsy. Treatment: surgical
excision. Radiotherapy and chemotherapy are of little value.
Keratoacanthoma
Rapidly growing lump (over a couple of months) with central keratin-filled crater.
Spontaneously resolves within 2–3 months leaving a scar. Recognition of this benign
lesion is important as it can clinically (and histologically) resemble a squamous cell
carcinoma, making it difficult to distinguish.
8.6. Psoriasis
Inflammatory disorder with hyperproliferation of epidermis. Occurs in ≈ 1% of the
population in the developed world, of which one-third will have a family history
(without any clear pattern of inheritance). Several clinical variants, e.g. classic
plaque, pustular, guttate, erythrodermic.
CLINICAL FEATURES OF CLASSIC PLAQUE PSORIASIS
● Commonly affects the extensor surfaces (particularly knees and elbows) and
the scalp.
Single or multiple plaques which are silvery-red and scaly.
●
● Koebner phenomenon: lesions occur at sites of trauma/scarring.
● Nails may be affected: pitting, onycholysis.
● Arthritis – see Rheumatology section.
MANAGEMENT
● Topical: e.g. coal tar, dithranol, calcipotriol (vitamin D analogue).
● Systemic: e.g. methotrexate, acitretin, ciclosporin.
● Phototherapy: PUVA with oral psoralen.
8.7. Bullous disorders
Causes of vesicles/bullae
❍
Infections (e.g. impetigo)
❍
Insect bites
❍
Drugs (e.g. barbiturates)
❍
Metabolic disease (e.g. porphyria cutanea tarda)
❍
Congenital disorders (e.g. epidermolysis bullosa)
❍
Inflammatory disease (e.g. pemphigus, pemphigoid)
Pemphigus vulgaris
Superficial (epidermal) bullae which break easily causing erosions – lesions can
be anywhere but commonly occur around the mouth. Positive Nikolsky sign
(superficial skin layers slide off when pressure applied to ‘normal’ epidermis).
Lesions can become secondarily infected. Treatment: high dose oral steroids
followed by steroid-sparing agents.
Bullous pemphigoid
More common than pemphigus, usually occurring in the over-60s. Bullae are
subepidermal, tense, and can occur anywhere but normally on the limbs (cf.
cicatricial pemphigoid which commonly affects the mouth). Treatment: steroids or
other immunosuppressive agents.
100
DERMATOLOGY
8.8. Naevi
Cutaneous hamartomas – commonest variety is the melanocytic naevus, i.e. collec tions of melano cytes; more commonly known as a ‘mole’. Can be congenital
or acquired (appear after birth). Pathologically, they begin as ‘junctional’ type
(melanocytes collect at the dermoepidermal junction), and progress through two
other stages (‘compound’, when cells migrate to the dermis and ‘intradermal’, when
all cells are in the dermis). Clinical features: pigmented initially but may become
pale later on when intradermal.
There is a small risk of malignant transformation (but note that the majority
of melanomas do not arise in pre-existing naevi). Treatment: removal indicated if
suspicion of malignant transformation, or for cosmetic reasons.
8.9. Erythroderma
Describes the situation where most of the skin becomes red.
CAUSES
● Psoriasis.
● Eczema.
● Mycosis fungoides/Sézary syndrome.
● Secondary to drugs.
COMPLICATIONS
● Abnormal control of temperature, e.g. hypothermia.
● Hypoalbuminaemia and peripheral oedema.
● Loss of water through the skin may lead to hypovolaemia.
● High-output cardiac failure.
● Sepsis.
Should be regarded as a medical emergency. Fluid resuscitation and treatment of the
underlying cause and complications are required.
8.10. Other skin conditions
Erythema multiforme
Ranges from mild rash with the classic ‘target’ lesions over palms/soles, through
to severe rash with inflammation also affecting mucous membranes, i.e. mouth,
genitals and conjunctivae (when it is known as Stevens-Johnson syndrome).
Triggered by infections (particularly herpes simplex), connective tissue disorders
and a number of different drugs.
Pityriasis rosea
Normally starts with a ‘herald’ patch followed by pinkish patches with a scale around
the edge, commonly over the trunk and said to be in the distribution of an inverted
Christmas tree. Lesions also occur proximally on the limbs. Self-limiting.
8.11. Miscellaneous lesions
Lipoma
Common, slow-growing benign tumour of mature fat cells. Can occur in any
location; usually found on the head and neck. Typically presents as a soft, fl uctuant,
lobulated mass which slips between the fingers (‘slippage sign’). Can be multiple and
may grow to a large size. Management: often excised for cosmesis or diagnosis.
101
ESSENTIAL NOTES FOR MEDICAL AND SURGICAL FINALS
Epidermoid cyst (‘sebaceous cyst’)
Common. Occurs due to a proliferation of epidermal cells within the dermis.
Typically, slow-growing, occuring on the face, trunk, neck and scalp; solitary, fi rm,
round and well-circumscribed, with a smooth surface and central punctum. Can be
complicated by recurrent infections requiring antibiotics +/– incision and drainage
acutely, followed by formal excision.
Dermoid cyst (Inclusion dermoid)
Uncommon. Painless, soft, smooth and slow growing. Can be classifi ed as:
Congenital: due to inclusion of the epidermis along lines of fusion of the skin
●
dermatomes during embryological development. Usually presents in the face,
neck or scalp at birth. Can produce symptoms due to external compression.
Acquired:
●
due to forced implantation of the epidermis into the dermis, e.g. in
gardeners.
MANAGEMENT Both types can be treated conservatively or surgically excised, if
symptomatic.
Keloid scar
Abnormal scar formation due to excessive collagen production and/or decreased
degradation. Scars typically extend beyond the wound margin. Usually affect
the earlobes, chin, neck, shoulder and chest. Incidence is greater amongst black
population (suggests racial predilection) and females with a peak age of presentation
between 10 to 30 years. Scars are associated with burns, surgery, tattoos, injections,
and bites, and tend to grow with time, puberty and pregnancy. Management: treat
conservatively, due to inevitable recurrence with excision.
Hypertrophic scar
Unlike keloid scars these are confined to the wound margin and usually regress
with time. They typically occur along flexor surfaces and skin creases and have no
racial, age or gender predeliction. Management: surgical excision carries a high risk
of recurrence, therefore treat conservatively.
Neurofi broma
Benign tumour of peripheral nerve elements. Lesions can be solitary or multiple
and are typically pedunculated and nodular. Association with café au lait spots (>6)
suggests Von Recklinghausen’s disease (Neurofibromatosis type I), an autosomal
dominant disorder associated with chromosome 17. Management: excision of
lesions is indicated if painful.
102
Infectious disease
9.1. Bacterial infections
9.2. Tuberculosis, leprosy and other mycobacterial infections
9.3. Antibiotics
9.4. Pyrexia of unknown origin
9.5. Fever in the returned traveller
9.6. Bacteraemia
9.7. Viral infections
9.8. HIV
9.9. Hepatitis viruses
9.10. Herpes viruses
9.11. Fungi
9.12. Protozoa
9.13. Helminths
9.14. Malaria
9.15. Other tropical infections
9.16. Sexually transmitted diseases
9.17. Intestinal infections
9.1. Bacterial infections
Gram-positive cocci
❍
Staphylococcus
❍
Streptococcus
Gram-positive rods
❍
Corynebacterium
❍
Listeria
❍
Bacillus
❍
Anaerobes, e.g. Clostridium, Nocardia, Actinomyces
Gram-negative cocci
❍
Neisseria
Gram-negative rods
❍
Enterobacteria (e.g. E. coli, Shigella, Salmonella, Yersinia, Klebsiella, Proteus)
❍
Parvobacteria (Brucella, Bordatella, Haemophilus)
❍
Vibrio, Campylobacter, Pseudomonas, Legionella
❍
Anaerobes, e.g. Bacteroides
Other types of bacteria (Spirochaetales, Rickettsiaceae, Chlamydiae, Mycoplasma)
9
103