Skin Manifestations of Systemic Disease. Dr Binita Guha-Niyogi ST6 Dermatology

Transcription

1 Skin Manifestations of Systemic Disease Dr Binita Guha-Niyogi ST6 Dermatology

2 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

3 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?

4 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

5 Psoriasis Nail signs Pitting Onycholysis Discolouration - Leukonychia Subungal hyperkeratosis Oil Spot Splinter haemorrhages Medical Associations Arthritis IBD Obesity Cardiovascular disease, HTN, Dyslipidaemia Treatment Topicals Steroids, Vit D3 analogue, calcineurin inhibitors Systemics Acitretin, MTX, Ciclosporin Phototherapy Biologics

6 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.

7 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

8 SLE Drug Induced SLE Hydralazine, Procainamide, INH, Quinidine, PUVA, Minocycline, D- Penicillamine Skin biopsy Lymphocytic infiltrate Dermal mucin Treatment Top Steroid/calcineurin inhibitor Antimalarials HCQ Steroid Sparing Agent Sun protection Differentials for facial rash: DLE Rosacea Seborrhoeic dermatitis Dermatomyositis

9 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?

10 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)

11 Other GI symptoms (reflux, dysphagia), SOB (ILD, pulm HTN) Cardiac Renal Synovitis (Symmetrical), migratory polyarthritis Systemic Sclerosis Skin Pruritus Oedema of digits ->sclerosis, ulcers Mask-like Face Dyspigmentation Calcinosis cutis Vascular Raynaud s Phenomenon

12 Systemic Sclerosis Investigations Bloods: ANA Anti-SCL-70 Anti-Fibrillarin Anti-centromere Anti-RNA Polymerase Skin Biopsy Collagen deposition Loss of s/c fat Treatment Raynauds: Avoid cold temperatures, Nifedipine, Low dose aspirin, PGE1 Ulcers: Bosentan Prostacyclin Immunosuppressant D-penicillamine ACEi

13 CREST Limited form of sclerosis Calcinosis Raynaud s Phenomenon E oesphageal involvement S clerodactyly T elangiectasia Anticentromere Abs Rarely progresses to SSc Better prognosis than SSc

14 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?

15 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%)

16 Erythema Nodosum Investigations Skin biopsy: Septal panniculitis Treatment Often self limiting If mild subsides 3-6 weeks Throat swabs FBC, CRP, ESR, ASOT CXR Quantiferon Treat underlying cause Bed rest NSAIDs Doxycycline Prednisolone

17 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?

18 Necrobiosis lipoidica Well defined yellow/redbrown atrophic/indurated waxy plaques Surface telangiectasia +/- ulceration Pretibial areas 30-40% have DM Only % with DM present with NLD Check Blood glucose Skin biopsy: Histiocytes encircle necrobiotic collagen in dermis +/-sclerosis, granulomatous inflammation Treatment Potent topical steroid: Dermovate + occlusion Others IL Steroid, Aspirin +Dipyridamole, Nicatinamide, Ciclosporin, Biologics, PUVA, PDT

19 Skin Manifestations in Diabetes Acanthosis Nigricans Bullous diabeticorum Diabetic dermopathy Disseminated GA Scleroedema of Buschke Eruptive xanthomas Ulcers Diabetic neuropathy

20 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.

21 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

22 Thyroid Disease Hyperthyroid Velvety smooth skin Hyperpigmentation Pretibial myxoedema Fine hair Mild but diffuse alopecia Koilonychia Onycholysis Hypothyroid Coarse dry skin Boggy, oedematous Dull, brittle hair Alopecia lateral 1/3 eyebrows (madarosis) Onycholysis

23 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.

24 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

25 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

26 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?

27 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

28 Venous Insufficiency Risk Factors Varicose veins/dvt Hx of cellulitis Chronic swelling aggravated by hot weather and prolonged standing Venous leg ulcers Treatment Elevation Treat 2ry infection Topical Steroids/Emollients Potassium permangenate Compression (Need ABPIs)?Need to treat veins Complications: Infection, Secondary eczema, Contact allergy

29 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.

30 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

31 Vasculitis Prognosis depends on systemic involvement Vasculitis screen ANA, ANCA, Ig and electrophoresis, complement, haematinics, Cryoglobulins, FBC, U&Es, LFTs Urine Dip/PCR BP Skin biopsy Treatment Remove trigger Supportive therapy 90% Spontaneously resolve Top Dermovate Prednisolone Immunosuppressant

32 Vasculitis HSP Palpable Purpura: Extensors Associations: Arthritis, Haematuria, Colicky Abdominal Pain, +/- GI Bleeding and vomiting, Nephritis Cryoglobulinaemia Purpura, Raynauds, Arterial thrombosis Associations: Lymphproliferative disrorders, RF activity, HCV, AI connective tissue disease, Arthralgias, glomerulonephrotis, peripheral neuropathy Granulomatosis with polyangiitis Mucocutaneous: Ulcers, red friable ginigva, mimick PG, purpura Lungs and kidneys Churg-Strauss Palpable purpura, s/c nodules, livedo reticularis, urticaria Asthma, GI tract, peripheral nerves+/- heart Polyarteritis Nodosa Punched out ulcer, livedo reticularis, s/c nodules, acral gangrene, Associations: IBD, SLE,HBV/Strep

33 Question 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?

34 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, ulcers, ichthyosis

35 Sarcoidosis Can affect other organs: Eye 20-30% Liver 30-40% Heart 5-25% Nervous system 1-5% Musculoskeletal 2-38% Investigation Raised ACE, calcium, ESR Skin biopsy Non-caseating granulomas Asteroid bodies and Schaumann bodies in giant cells Lung function tests Exclude TB ECG CXR Treatment Top/IL/PO steroids HCQ MTX/steroid sparing agents

36 Question 11 A 60 year old woman presented with itching, burning rash on the face, recently had difficulty combing her hair and walking up the stairs 1. What is the cause of her symptoms? 2. What s associated with this condition?

37 Dermatomyositis Chronic Inflammatory dermatomyopathy Skin changes usually 2-3 months before muscle weakness (proximal symmetrical) +/- fever, malaise Can get cardiac and respiratory involvement 15 50% have an underlying malignancy Lung/GI >Men Ovaries/Breast > Women Poikiloderma Gottron s papules Samitz Sign Heliotrope Rash Calcinosis Cutis Gottron s sign Mechanic s Hands

38 Dermatomyositis Investigations Bloods: Myositis screen ANA (60%), CK (90%) aldolase, ESR, transaminases (AST), LDH Skin biopsy: Atrophic epidermis, mucin, lymphocytic infiltrate (Lupus picture), dermal sclerosis Muscle Biopsy, EMG, MRI Screen for malignancy Treatment PO Steroids +/- Steroid sparing agent HCQ and Sun protection Diltiazem/Colchicine Bed rest/physio Resolves in 1/5

39 Question year old lady with ESRF has regular dialysis, she s recently noticed tender lesions developing on her legs 1. What is the skin condition?

40 Calciphylaxis Rare >ESRF on HD Post renal Tx with elevated Ca-Phosphate product Systemic calcification of small/medium vessels Ischaemic necrosis of skin and soft tissue Skin Painful violaceous mottling (reticulated) >Lower limbs Purpura with central necrosis +/- bulla Necrosis and ulceration High mortality associated with gangrene and sepsis

41 Calciphylaxis Treatment Normalise Calcium and phosphate product (low calcium dialysis) Sodium thiosulfate (increase solubility of Calcium deposits) Bisphosphonates Calcimimetics Parathyroidectomy

42 End Stage Renal Disease Pruritus Uremic Frost Acquired perforating disorder Nephrogenic Systemic Fibrosis

43 Question 13 A 70 year old gentleman has been itchy for over a year. 1. What investigations would you like to do?

44 Pruritus Localised or Generalised Localised Skin (Primary rash) Eczema, seborrhoeic eczema, contact dermatitis Head lice, scabies, candida, tinea Lichen planus, Lichen sclerosus Bullous Pemphigoid Often present Excoriations Lichenification Lichen simplex Prurigo Nodularis Nerves Hypersensitive nerves (+/- reduced/absent sweating)

45 Pruritus Investigations Review medications Lymph nodes Bloods: FBC, U&Es, Extended LFTs TFTs, glucose?bbv screen Ig and electrophoresis Haematinics Skin autoantibodies Urine dip Treatment Depends on cause Skin Treat primary skin rash Cooling cream: Menthol in aqueous Emollients Antihistamines Topical steroids Amitryptylline/Gabapentin

46 Outcomes Recognise the dermatological manifestations associated with common systemic diseases How to assess the patient Consider differential diagnoses Investigate appropriately Known when a biopsy is appropriate Formulate and implement a management plan for acute period of care

47 Questions?