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Sclerosis, Ulcers •‘Mask-Like’ Face •Dyspigmentation •Calcinosis Cutis

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Sclerosis, Ulcers •‘Mask-Like’ Face •Dyspigmentation •Calcinosis Cutis Skin Manifestations of Systemic Disease Dr Binita Guha-Niyogi ST6 Dermatology Email: [email protected] Aims • To provide an overview of the dermatological manifestations associated with common systemic diseases • To address some of competences outlined in the curriculum • The trainee should be able to: • Assess the patient • Produce a valid differential diagnosis • Investigate appropriately • Consider when a biopsy is appropriate • Formulate and implement a management plan for the acute period of care Question 1 35 year old lady with a rash over her knees and elbows for a few years. Improves with sunlight and worse with stress. 1. What is the diagnosis? 2. List 2 nail signs associated with this condition? 3. Give 2 medical conditions associated with this diagnosis? Psoriasis • Well demarcated erythematous, scaly plaques • Abnormal T cell activation, increased epidermal turnover, genetic (HLA) Triggers • Trauma • Infection (Strep) • Stress • Medications • Lithium, B-blockers, Antimalarial, ACEi, NSAIDS, Withdrawal of PO Steroids, G-CSF, INF Psoriasis Nail signs Treatment • Pitting • Topicals • Onycholysis • Steroids, Vit D3 analogue, • Discolouration - Leukonychia calcineurin inhibitors • Subungal hyperkeratosis • Oil Spot • Systemics • Splinter haemorrhages • Acitretin, MTX, Ciclosporin Medical Associations • Phototherapy • Arthritis • Biologics • IBD • Obesity • Cardiovascular disease, HTN, Dyslipidaemia Question 2 35 year old lady who is currently investigated for joint pains, develops a photosensitive facial rash. 1. What is the likely diagnosis? 2. Name a blood test that you would like to perform. Systemic lupus erythematosus (SLE) Multiorgan (4/11) •Malar erythema, DLE, oral ulcers •Photosensitivity •Haematological disorder •Nephropathy •Arthritis •Serositis •Neurologic disorder •ANA, Immunologic (dsDNA ,anti- Sm) Other bloods: FBC, C3,C4 •Other skin features: Alopecia, Raynaud’s, Livedo reticularis, Acrocyanosis, Urticarial vasculitis SLE Drug Induced SLE Treatment • Hydralazine, Procainamide, •Top Steroid/calcineurin INH, Quinidine, PUVA, inhibitor Minocycline, D- •Antimalarials – HCQ Penicillamine •Steroid Sparing Agent •Sun protection Differentials for facial rash: • Skin biopsy •DLE – Lymphocytic infiltrate •Rosacea – Dermal mucin •Seborrhoeic dermatitis •Dermatomyositis Question 3 79 year old lady complains of tightening of her fingers and facial skin for some years 1. What is the diagnosis? 2. Give two symptoms would you enquire about? Systemic Sclerosis • Scleroderma is a group of AI disorders: Morphoea, Systemic Sclerosis, CREST • AI inflammatory condition • Characterised by inflammation, fibrosis and vasculopathy • Underlying mechanisms are complex and largely unknown • >Women (30-50yrs) • African-Americans (early onset/ diffuse) Systemic Sclerosis Skin •Pruritus •Oedema of digits ->sclerosis, ulcers •‘Mask-like’ Face •Dyspigmentation •Calcinosis cutis Vascular • Raynaud’s Phenomenon Other •GI symptoms (reflux, dysphagia), •SOB (ILD, pulm HTN) •Cardiac •Renal •Synovitis (Symmetrical), migratory polyarthritis Systemic Sclerosis Investigations Treatment • Bloods: • Raynauds: • ANA • Avoid cold temperatures, Nifedipine, Low dose aspirin, • Anti-SCL-70 PGE1 • Anti-Fibrillarin • Ulcers: Bosentan • Anti-centromere • Prostacyclin • Anti-RNA Polymerase • Immunosuppressant • D-penicillamine • Skin Biopsy • ACEi • Collagen deposition • Loss of s/c fat CREST • Limited form of • Anticentromere Abs sclerosis • Rarely progresses to SSc • Calcinosis • Raynaud’s Phenomenon • Better prognosis than • E oesphageal SSc involvement • S clerodactyly • T elangiectasia Question 4 22 year old woman presents with a sore throat and rash to her legs 1. What is this eruption? 2. Give two possible causes for this eruption? Erythema Nodosum • >Women • Tender red s/c nodules may ’bruise-like’ • >Symmetrical Pre-tibial areas • +/- fever, arthralgia, malaise Causes • Infection- viral, strep, salmonella, campylobacter, TB, leprosy, fungi • Drugs – OCP, sulphonamides, penicillin,NSAIDs • Inflammatory – Crohn’s > UC, Behcet’s • Sarcoidosis - (Good prognostic sign) • Malignancy – lymphoma • Pregnancy • Idiopathic (30%) Erythema Nodosum Investigations Treatment • Skin biopsy: Septal • Often self limiting panniculitis • If mild subsides 3-6 weeks • Throat swabs • FBC, CRP, ESR, ASOT • Treat underlying cause • Bed rest • CXR • NSAIDs • Doxycycline • Quantiferon • Prednisolone Question 5 40 year old woman presents with a gradual history of skin changes over her lower legs. She is otherwise well. 1. What is the diagnosis? 2. Give one investigation would you like to perform? Necrobiosis lipoidica • Well defined yellow/red- • Check Blood glucose brown atrophic/indurated • Skin biopsy: waxy plaques – Histiocytes encircle necrobiotic collagen in dermis +/-sclerosis, granulomatous inflammation • Surface telangiectasia +/- ulceration Treatment • Pretibial areas • Potent topical steroid: Dermovate + occlusion • 30-40% have DM • Others • IL Steroid, Aspirin • Only 0.03-3% with DM present +Dipyridamole, Nicatinamide, with NLD Ciclosporin, Biologics, PUVA, PDT Skin Manifestations in Diabetes • Acanthosis Nigricans • Scleroedema of • Bullous diabeticorum Buschke • Diabetic dermopathy • Eruptive xanthomas • Disseminated GA • Ulcers • Diabetic neuropathy Question 6 40 year old man complains of increased appetite and skin changes over lower legs 1. What is the diagnosis? 2. Where else would you like to examine? Give 2 other sites. Pretibial Myxoedema (Grave’s Disease) • Elevated lesions on the skin • Skin is shiny with ‘orange peel’ appearance Other sites to examine • Eyes - exopthalmos • Thyroid - enlargement, nodules • Nails - Thyroid acropachy onycholysis • Hair - diffuse thinning • Tremor Thyroid Disease Hyperthyroid Hypothyroid • Velvety smooth skin • Coarse dry skin • Hyperpigmentation • Boggy, oedematous • Pretibial myxoedema • Dull, brittle hair • Fine hair • Alopecia lateral 1/3 • Mild but diffuse alopecia eyebrows (madarosis) • Koilonychia • Onycholysis • Onycholysis Question 7 30 year old male with ulcerative colitis develops a painful ulcer on his lower legs within the last few weeks 1. What is the diagnosis? 2. Give one condition which can be associated with this type of ulcer. Pyoderma Gangrenosum • Starts as papule/nodule • Rapidly expanding ulceration with undermined border and violet/bluish edge Associations IBD RA , Ankylosing spondylitis Haematological malignancy Hepatitis, PBC Neoplasia Post-trauma Pyoderma Gangrenosum Diagnosis • Clinical features • +ve pathergy test • Skin biopsy: Neutrophilic inflammatory infiltrate -> Necrotic • May have +ve pANCA • Swabs Treatment • Treat infection • Top Dermovate • PO Doxy • Dressings +/- compression • If larger: Po Steroids +/- steroid sparing agent Question 8 An 80 year old man presented with chronic ulcers over the lower legs. 1. What is the cause of her leg ulcers? 2. How would you manage these ulcers? Venous Insufficiency • Itchy red, blistered, crusted plaques, dry, fissured • Orange-brown macular pigmentation (Haemosiderin) • Atrophie blanche • White irregular scars surrounded by red spots • “Champagne bottle” • Lipodermatosclerosis Venous Insufficiency Risk Factors Treatment • Varicose veins/DVT • Elevation • Hx of cellulitis • Treat 2ry infection • Chronic swelling • Topical Steroids/Emollients aggravated by hot • Potassium permangenate weather and prolonged • Compression (Need ABPIs) standing • ?Need to treat veins • Venous leg ulcers Complications: • Infection, Secondary eczema, Contact allergy Question 9 A 65 year old woman presented with lethargy, pyrexia and a palpable rash. 1. Describe this rash? 2. Give a possible underlying cause. Vasculitis • Palpable Purpura, papules, vesicles, macules -> Necrotic, ulcers, oedema • > Dependent sites • +/- fever, arthralgia, myalgia, weight loss Causes •Infection •Drugs-Penicillin, NSAIDs, COX2-inhibitors, ACEi, Allopurinol, furosemide •Inflammatory – IBD, AI •Malignancy •50% Idiopathic Vasculitis • Prognosis depends on Treatment systemic involvement • Remove trigger • Vasculitis screen • Supportive therapy • ANA, ANCA, Ig and electrophoresis, • 90% Spontaneously resolve complement, haematinics, • Top Dermovate Cryoglobulins, FBC, U&Es, • Prednisolone LFTs • Immunosuppressant • Urine Dip/PCR • BP • Skin biopsy Vasculitis HSP Granulomatosis with polyangiitis • Palpable Purpura: Extensors • Mucocutaneous: Ulcers, red friable • Associations: ginigva, mimick PG, purpura – Arthritis, Haematuria, Colicky Abdominal Pain, +/- GI Bleeding • Lungs and kidneys and vomiting, Nephritis Churg-Strauss Cryoglobulinaemia • Palpable purpura, s/c nodules, livedo • Purpura, Raynauds, Arterial reticularis, urticaria thrombosis • Asthma, GI tract, peripheral • Associations: nerves+/- heart • Lymphproliferative disrorders, RF activity, HCV, AI connective tissue disease, Arthralgias, Polyarteritis Nodosa glomerulonephrotis, peripheral • Punched out ulcer, livedo reticularis, neuropathy s/c nodules, acral gangrene, • Associations: IBD, SLE,HBV/Strep Question 10 50 year old woman is under the respiratory team with SOB, joint pains and general fatigue. A rash is noted: 1. What is the likely diagnosis? 2. What tests would you like to perform? Lupus Pernio - Sarcoidosis • Chronic multisystem granulomatous disease • 30-40% present with skin findings Specific • Skin coloured-red/brown annular papules/plaques • Lupus pernio • S/C nodules • Infiltration of old scars Non-Specific • EN, EM, nummular eczema, calcinosis cutis, pruritus, Hypopigmentation, scarring alopecia,
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