bp case presentation
TRANSCRIPT
This is Complete B******!
Dr Ben CM Lee MB BS MRCP(UK)SAS Doctor in General Medicine with a special interest in Clinical
Dermatology
NC• 87 year old lady • Referred by GP directly to ENT with Haemoptysis
Initial complaints• Upon arrival: SoB, audible wheeze, evidence of blood stained sputum,
bilateral leg swelling• Examined and given the hospitality of nasendoscopy by the ENT team• FAO: SpR Dermatology ’87 female, breathless, haemoptysis, shallow
ulcers in soft palate and nasal pharynx; had biopsy but now has developed a rash – please review’
First impression is everything• o/e elderly lady, frail, hyperexpanded chest/ribcage, talking in full
sentences but clearly in resp distress, RR 32/min Sat 73% on air, bibasal coarse crepitus with bronchial breathing and widespread polyphonic wheeze
Blood stained mucus on tissuesJVP 6cm sitting uprightb/l ankle oedemaNot paleIn hospital gown*
• HPC (from patient): 6/52 of progressive SoB, orthopnoea, 10/7 of productive coughs and extremely breathless in the night; finds blood in sputum 2/7 very alarming
• No wt loss; had gained wt, rather• Ex smoker of 30/day for 50 years and quit just before admission• Normally independent• PMH:
• T2DM on Metformin, SU• HTN on Ramipril 10mg• CKD stage IIIa• IHD with angina, takes Aspirin 75mg OD, omeprazole 20mg• COPD• NKDA
more info • U+E: Ur 20*(12) Cr 230 (198) electrolytes balanced Na130 K+4.0• LFT ALP 138 ALT normal Alb 28• ABG: pH 7.32 PaO2 7.64 on air PaCO2 6.8 HCO 18.2 lactate 1.2 • FBC: Hb10.8 Plt 240 WCC 8.9 78% neutrophils
• c/o: SoB and sore throat
Immediate management plan• ABC: treat decompensated CCF
• Cautious use of diuretics, daily weight, fluid balance chart, encourage oral hydration, chase old notes and echo reports, chase nasendoscopy report and biopsy result
• D: BM 4.8 alert, PERLA
E for Exposure• X1 isolated haemorrhagic ulcer to the left of
soft palate; a degree of stomatitis; dentures in place. Appearance of buccal mucosa unremarkable
• Skin check reveals several tense blisters ranging from the size of 3cm to 6cm located in b/l groins, left shin; x2 non tender erythematous nodules over the right shoulder
• Eyes, ears and nose are reported as normal
• Breathing Difficulty: • clinically decompensated CCF/Fluid overload; but IVI 6-8 hrly N Saline (?chest
sepsis) for the first 24 hours of admission under surgeon• Low albumin• Raised ALP probably secondary to recent Rx by GP for ?chest infection:
amoxicillin 500mg TDS • Onset of rash at the same time as commencement of Abx• Raised Ur ++ but Cr at baseline ?UGIB.
• No melaena on PR• No haematemesis; patient brings up phlegm that is blood-stained
Contents• Differentials• Investigations and Management• The Diagnosis• Questions
Any Thought?
Subepidermal Immunobullous
DiseasesBullous Pemphigoid and others:Mucous membrane pemphigoid
Pemphigoid gestationisEpidermolysis bullosa acquisita
Linear IgA diseaseDermatitis herpetiformis
Differentials
Pemphigus vs Pemphigoid
• Bullous Pemphigoid• Elderly, primarily trunk limbs and flexures, mucosal involvement common, but minor• Tense blisters, developed from urticated plaques
• Mucous membrane pemphigoid• Middle-old age, infrequent cutaneous distribution, major and severe mucosal involvement*• Erosion, blisters, gingivitis, scarring, tense blisters
• Pemphigoid gestationis• Pregnant women, umbilicus, generalised, with minor mucosal involvement• Tense blisters as above
• Linear IgA disease• Chronic bullous disease of child hood vs adult LAD, trunk and limbs more common, also perineum,
face, mucosal involvement common but minor• Annular lesions, tense blisters, papulovesicles
• Epidermolysis bullosa acquisita• Adult and children, generalised, mucosal involvement for some• Tense blisters as above
• Dermatitis herpetiformis
Bullous Pemphigoid
Mucous membrane pemphigoid
http://burketsoralmedicine.blogspot.co.uk/2012/11/mucous-membrane-pemphigoid-cicatricial.html
Pemphigoid gestationis
Linear IgA disease: chronic bullous dx of childhood & adult IgA dx
EBA: epidermolysis bullosa acquisita
Dermatitis herpetiformis
Subepidermal Immunobullous Diseases
DH LAD
MMP
PG
EBA
BP
Immunopathology• BP: IgG, few IgA IMF = Linear pattern vs BMZ• MMP: IgG and IgA IMF= Linear pattern vs BMZ• Pemphigoid gestationis: IgG IMF = linear pattern vs BMZ• Linear IgA disease: IgA IMF = Linear pattern vs BMZ• EBA: IgG IMF = Linear pattern vs BMZ• DH: IgA IMF = Granular pattern dermal papillae
Basement Membrane Zone (BMZ)BP230 antigen: BP, MMP, PG, LAD
BP180 antigen: BP, MMP, PG, LAD
Laminin 5: MMP
Anchoring fibrils/collagen VII: MMP, EBA, LAD
BP: Aetiology• Onset usually >60 years of age (a mean of 80 years[Langan SM et al])• Idiopathic; autoimmune• No well defined causal agents; only a number of agents implicated in
younger patients with an association with the development of BP:• Furosemide• Spironolactone• Sulphasalazine• Penicillins• Penicillamine• B blockers• enaxoparin
Pathogenesis• IgG autoantibodies vs hemidesmosome-associated proteins• Autoantibody deposition + binding with C3 • Directed at BP180 and BP230 in BMZ• At least partially cell mediated: neutrophils, eosinophils, and
macrophages
Histopathology
Intact and viable epidermis
Dermal inflammatory infiltrate
Direct Immunofluorescence
Direct immunofluorescence study performed on a perilesional* skin biopsy specimen from a patient with bullous pemphigoid detects a linear band of immunoglobulin G deposit along the dermoepidermal junction. (courtesy of Medscape.org)
Investigation and Immediate RxSkin BiopsyIndirect ImmunofluorescenceSkin swabsAspirate fluids from any large uncomfortable blistersEmollients and skin care vs infectionUltra-potent topical corticosteroidSystemic steroid up to 60mg pred ODBone protection*Immunosuppressive meds if disease proves difficult to control
Progress
Summary• Immunobullous diseases: intraepidermal vs subepidermal• Various clinical entities of subepidermal dx differ in:
• Abg classes & subclasses• Target antigens• Associated reaction by complements into the inflammatory cascade• Perhaps associated conditions, not fully understood to date
• Early referral to Dermatologists (aka the skin experts)• Most patients Px BP are geriatric; a world of comorbidities and polypharmacies• If in doubt, take a biopsy• Barrier protection: emollient, emollients and more emollients.
The Rants‘There is no such thing as chronic cellulitis or bilateral cellulitis, I cure each and every one by elevating the leg higher than the heart for 5 minutes. The rubor, dolor, calor and tumor of cellulitis does not fade with elevation; the rubor, dolor, calor and tumor of stasis, which is the cause of ALL 'bilateral' and/or 'chronic' cellulitis, does fade with elevation. Lets pretend you are now in college.’ Infectious disease compendium http://pusware.com/testpus/disease_Cellulitis.html
Coming soon…
