Skin in Systemic Disease 2 Flashcards
What is graft versus host disease (GvHD)?
Multiple-organ disease
How many people does graft versus host disease affect?
~10-80% of allogenic haematopoetic stem cell transplants (HSCT)
What is the pathogenesis of GvHD?
donor-derived T-lymphocyte activity against antigens in an immunocompromised recipient
What does GvHD mostly affect?
- Skin
- Liver
- GI tract
What are the two major forms of GvHD?
- Acute
- Chronic
What is itching without a rash suggestive of?
internal cause
What are the haematological causes of pruritus?
ymphoma, polycythemia
What are the other causes of pruritus?
- Uraemia
- Cholestasis
- Iron deficiency or iron overload
- HIV / Hepatitis A / B / C
- Cancer
- Drugs (NB opiates / opioids)
- Psychogenic
- Pruritus of old age
What are the investigations for pruritus?
- FBC, LDH
- Renal profile
- Liver function tests
4, Ferritin - XR Chest
- HIV / Hepatitis A / B /C
What is systemic amyloidosis?
- Manifestation of underlying plasma cell dyscrasia
* Fibrils composed of AL protein (immunoglobulin light chains, usually λ chains)
What are the presenting symptoms of systemic amyloidosis?
- Weight loss
- Fatigue
- Paraesthesias
- Dyspnoea
- Syncopal attacks (orthostatic hypotension)
What are the investigations for systemic amyloidosis?
abdominal fat / rectal mucosa biopsy – SAP scintigraphy
What is the treatment for systemic amyloidosis?
- melphalan
- autologous peripheral blood stem cell transplant
- lenalidomide
- bortezomib
What are the cutaneous features of systemic amyloidosis?
- [Clinically evident] Skin involvement in ~ 25%.
- Petechiae, purpura and ecchymoses - due to infiltration of vessel walls
- Periorbital purpura ( ‘Raccoon sign’) precipitated by: coughing, Valsalva manoeuvre or pinching (pinch purpura)
- Waxy, translucent or purpuric papules, nodules
- Face, neck, and scalp, anogenital region, digits
What is scurvy?
Vitamin C (ascorbic acid) deficiency
What are the signs of scurvy?
- Spongy gingivae with bleeding and erosion
- Petechiae, ecchymoses, follicular hyperkeratosis
- Corkscrew hairs with perifollicular haemorrhage
What is kwashiorkor?
protein deficiency
What are the systemic features of kwashiorkor?
- Hepatomegaly
- Bacterial / fungal infections
- Diarrhoea
- Loss of muscle mass
- Oedema
- Failure to thrive
What are the skin signs of kwashiorkor?
- Superficial dequamation large areas of erosion
- Sparse, dry hair
- Soft, thin nails
- Cheilitis
Why is zinc important?
- Important role in 200 enzymes – regulation of lipid, protein, nucleic acid synthesis
- Roles in wound healing, antioxidant
What is the deficiency in zinc?
- genetic (SLC39A4) or acquired
- Triad of
- Dermatitis
- Diarrhoea
- Depression
Where are most of the cutaneous manifestations of zinc deficiency?
perioral, acral and perineal sites
What are the cutaneous manifestations of zinc deficiency?
- Erythema
- Scale-crusts
- Erosions
- Alopecia
- Stomatitis
- Conjunctivitis
What is a vitamin B3 (niacin) deficiency?
•Required for most cellular processes
•Deficiency:
- Dermatitis | Diarrhoea | Dementia | Death
What are the cutaneous manifestations of Vitamin B3 deficiency?
- Photodistributed erythema
2, ‘Casal’s necklace’ - Painful fissures of the palms and soles
- Peri-anal and perioral inflammation and erosions
- Cheilitis and glossitis
- Vaginitis with erosions
What does carcinoid syndrome signify?
metastases of a malignant carcinoid tumour
What happens in carcinoid syndrome?
•5-HT secretion •Flushing in 25% of cases •Other symptoms: - Diarrhoea - Bronchospasm - Hypotension
What is Steve Johnson Syndrome (SJS)?
- Prodromal: flu-like sx
- Abrupt onset of lesions on trunk > face/limbs
- Macules, blisters, erythema – atypical targetoid
- Blisters merge – sheets of skin detachment
- Nikolsky +ve
- Extensive full thickness mucocutaneous necrosis <2-3 days
What is nikolsky?
exfoliation of epidermis on slight skin ru
What is the different between SJS and Toxic epidermal necrolysis (TEN)?
same condition = just difference in severity
What happens in SJS and TEN?
- Cell-mediated cytotoxic reaction against epidermal cells
- Drugs cause >80% of cases
- May be started up to 3 weeks prior to onset of rash
What is scorten in SJS and TEN?
–score used to help assess severity
-Criteria: age >40, HR, initial % epidermal detachment, serum urea + glucose + bicarbonate, presence of malignancy
What are the complications of SJS and TEN?
- Death - Overall mortality 30%
- Blindness, dehydration, hypothermia/hyperthermia, renal tubular necrosis, eroded GI tract, interstitial pneumonitis, neutropaenia, liver and heart failure
What is erythroderma?
•Generalized erythema affecting >90% BSA
What are the systemic manifestations that reflect impairment in skin function in erythroderma?
- Peripheral edema
- Tachycardia
- Loss of fluid and proteins
- Disturbances in thermoregulation
- Risk of sepsis
What are the etiologies of erythroderma?
- Drug reactions
- . Cutaneous T-cell lymphoma – Sézary syndrome
- Psoriasis
- Atopic eczema
- Idiopathic (25-30%)
What is the management of erythroderma?
- Underlying cause (e.g. treat psoriasis, withdraw drug if drug cause, etc)
- Hospitalisation if systemically unwell
- Restore fluid and electrolyte balance, circulatory status and manage body temperature.
- Emollients to support skin barrier
•+/- Topical steroids
•+/- Antibiotics
What are cutaneous signs of systemic disease?
- Excoriations / prurigo
- Xerosis
- Half and half nails
- calciphylaxis
- nephrogenic systemic fibrosis
- perforating disorder
- viral warts (immunosuppressed)
- conjunctival pallor
What are the signs of CKD?
- Anaemia – mucosal pallor, hair thinning
- Excoriations, prurigo
- Calciphylaxis
- Half and half nails
What are the signs of CKD related to primary disease?
- ANCA-associated vasculitis
- Systemic Lupus Erythematosus
What are the signs of CKD related to immunosuppression?
- Viral warts
- Skin cancer
What are the cutaneous signs of chronic liver disease?
- Excoriations, prurigo
- Jaundice
- Muehrcke’s lines of nails
- Terry’s nails
- Palmar erythema
- Spider telangiectasia
- Clubbing
What is necrobiosis lipoidica?
- 20-65% of cases occur in setting of Diabetes Mellitus
* Plaques with red-brown raised edge with yellow-brown atrophic centre
What is the treatment with necrobiosis lipoidica?
topical / intralesional steroids
what are other cutaneous manifestations of diabetes?
- Terry’s nails
- Xerosis
- Granuloma annulare
- Xanthelesma and xanthomata
- Skin infections
- Neuropathic ulcers
- Acanthosis nigricans
What are the cutaneous signs of hyperlipidemia?
eruptive xanthoma
What endocrinological disorder is hyperpigmentaion a sign of?
addisions disease
What endocrinological disorder is acne a sign of?
- acromegaly
- cushing’ syndrome
- Polycystic ovarian syndrome
What are variable non-specific manifestations in seroconversion in HIV?
- Morbilliform rash
- Urticaria
- Erythema multiforme
- Oral / genital ulceration
- NB Low threshold for testing
What are other signs of HIV?
- Persistent or atypical manifestations or common infections
- Opportunistic infections
- Severe manifestations of common dermatoses (e.g. psoriasis, seborrheic dermatitis)
- Itch
- Suggestive dermatoses e.g. eosinophilic folliculiti
What is hidradenitis suppuritiva?
Inflamed nodes, sterile abscess, sinus tracts, fistulae and hypertrophic scars
Where is hidradentis suppuritiva?
Favours intertriginous zones: especially axillary, anogenital and inframammary area
What is pyoderma gangrenosum?
- Pustule on an erythematous base –ulcerates and extends with necrotic undermined border
- Painful
What is pyoderma gangrenosum assoicated with?
inflammatory bowel disease, leukemia, seronegative arthritis in 50-70% of cases
What cutaneous disease are associated with inflammatory bowel disease?
- Pyoderma gangrenosum
- Orofacial granulomatosis
- Panniculitis (erythema nodosum)
- Aphthous ulceration
- Association with psoriasis, pemphigoid
What is the cutaneous manifestation of celiac disease?
Dermatitis herpetiformis
What are cutaneous signs of an internal malignancy?
Cutaneous metastases
What malignancy reflects an internal malignancy?
Extramammary Paget’s disease
What genetic conditions predispose to internal cancer and skin lesions?
- Hereditary leiomyomatosis and renal cell cancer
- Peutz–Jeghers syndrome
What skin disease is associated with malignancy?
- Sweet’s syndrome
- Dermatomyositis
- Erythema gyratum repens
- Pyoderma gangrenosum
- Paraneoplastic pemphigus
What are non-specific skin disease?
- Pruritus
- Vasculitis
- Urticaria
