Approach to a patient with vesicobullous lesions
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Approach to a patient with vesicobullous lesions
- 1. Approach to a patient with vesiculobullous lesions Dr. Anshul Agrawal Dr. Farhana Taj
- 2. Approach to child with vesiculobullous lesions
- 3. • Clinical assessment of a child • Diagnostic Alogorithm • Causes • Clinical approach –Morphology of the lesions /Distribution • Laboratory Diagnosis
- 4. Clinical assessment Intraepidermal blister Subepidermal blister Thin, flaccid blisters which rupture easily Erosion, scaling or crusting Pigmentation and scarring are not common unless secondary infection occurs Tense bullae Base Devoid of any pigmentation Other Factor : Scaling, Crusting , Pustules Scarring Erosions and ulcers Haemorrhagic, petechiae photosensitivity
- 7. • Other key factors: (i) the morphology of lesions (flaccid versus tense blisters) (ii) the presence of secondary changes (iii) the pattern of eruption (e.g. annular, serpiginous or grouped).
- 8. Blistering in the neonate Non-infectious transient conditions: • Erythema toxicum neonatorum • Milaria crystallina • Acropustulosis of infancy • Sucking blisters • Transient neonatal pustular dermatoses
- 9. Infections • Viral-Herpes (herpes simplex, varicella zoster), cytomegalovirus • Fungal-Candidiasis • Bacterial-Gram-positive organisms -Treponema pallidum,Staphylococcus(impetigo neonatorum, SSSS) ,Streptococcus(particularly group B β-haemolytic),Bacillus anthracis, Gram-negative organisms -Chlamydia trachomatis,E.coli, H. influenzae,Klebsiella pneumoniae,Pseudomonas aeruginosa
- 10. • Genodermatoses EB junctional dystrophic Incontinentia pigmenti Bullous CIE • Autoantibody-mediated disorders Pemphigus vulgaris Pemphigus foliaceus
- 11. • Langerhan cell histicytosis • Mastocytosis • Hyper IgE Syndrome • Congenital erythropoitic porphyria • Hailey–Hailey disease
- 12. Neonate Vesicles Systemically well Localized Generalized Dew drop vesicle Vesicopustules Erythematous macules,Pustules Milaria crystallina Collaratte of scales Pruritis+ pustules TNPM ETN Palms and soles---Acropustulosis of infancy
- 15. Neonate --------->>Vesiculopustules Systemically unwell Vesicles,Pustules,Fine scaling Vesicles on an erythematous base Congenital candidiasis Cicatracial skin lesions Scars,microcephaly, No Chorioretinitis Hemorrhagic Limb Hypoplasis No Or necrotic Chorioretinitis CNS,Mucocutaneous Vesicles involvement pneumonia Intrauterine herpes Fetal varicella syn. simplex Neonatal Herpes Neonatal varicella simplex
- 19. Neonate --------->>Vesiculopustules Systemically unwell Painful vesicle Linera pattern nodule,ulcered With hemorrhagic bulla umblicated Incontinentia pigmenti Punched out necrotic ulcer Congenital self healing no Hangerhan cell Petechiae with purple black Histiocytosis Ecthyma gangrenosum Seizures and hydrocephalus Purpura fulminans
- 23. Neonate------->> Bulla, Erosions Localized Systemic association No Systemic association Photosensitivity NO face,napkin area Periumbilical and Congenital Palms and soles diaper area Fingers and wrists erythropoetic porphyria Hepatomegaly Congenital syphilis Bullous impetigo Sucking blisters
- 25. Bullous impetigo
- 26. Neonate------->> Bulla, Erosions Generalized Fragile bullae,skin tenderness, Septicimia,meningitis, Periorificial accentuation SSSS Honey colored crusting Group B streptococcus
- 27. SSSS
- 28. Neonate------->> Bulla, Erosions Generalized Mucosal erosions No No No scarring Scarring Infiltration Erythroderma milia +diarrhea ,flushing Nail dystrophy Maternal Bullous mastocytosis Bullous diease Epidermolysis bullosa CIBE
- 29. Recessively inherited dystrophic epidermolysis bullosa, oral cavity blistering and scarring
- 30. Mastocytosis with blister formation
- 32. Blistering in Child/Infant • Infections: 1. Viral : HS,HZ,Hand, foot and mouth disease 2. Bacterial:bullous impetigo 3. Infestations:scabies 4. Fungal:bullous Tinea 5. Envenomation: Bites, Sting
- 33. • Dermatitis • Physical agents: Burns,Friction ,Radiation • Inflammatory conditions: FDE,miliaria crystallina • Autoimmune Blistering Disorder: Pemphigus vulgaris,Pemphigus foliaceus , Bullous pemphigoid ,DH,CBDC
- 34. • Genodermatoses-EB Simplex,Hailey–Hailey disease (benign familial pemphigus) • Photosensitivity disorders - Porphyria cutanea tarda Porphyria variegata Congenital erythropoietic porphyria Heriditary coproporphyria
- 35. Vesicobullous lesion in an infant/child Localized Vesicles on an erythematous base Grouped Painful with lesion over hand Foot and oral cavity HS Hand ,foot and mouth disease HZ
- 38. Vesicobullous lesion in an infant/child Localized Papulovesicular Itching Pain Burrows Exposed areas Scabies insect bite reaction ORF Cow pox
- 39. Vesicullobullous Periorifacial Photosensitivity Localized Recurrent ( Palms & Sole) (Penis/scrotum) Vesicles/Bulla Atrophy Erosions Milia/scar EBS(Weber-Cockayne) FDR Acrodermatitis PORPHYRIA( Neurovisceral Symptoms) Enteropathica No YES No Late Onset Early Onset PCT Variegate Porphyria Mutilating Hereditary Coproporphyria Scarring Hepato(EPP)
- 40. Generalized • Systemically unwell Systemically well • Infections Drug Induced Nikolsky Sign • Varicella TEN Yes No • Meningo- SJS Pemphigus coccemia Oral lesions Tense Flaccid No Yes Bulla Vesiculo- Pustule Head & Neck Seborrheic Annular Hailey-Hailey Endemic PF IgA Pemphigus PF
- 41. Tense Bulla With Oral Lesions Yes No Grouped Lesion With- Subepidermal Genetic Blistering Itching Autoimmune Dermatitis Herpetiform Trauma prone site Grouped Lesion With Scarring EBS( Koebner) (Dowling Meara) Flexures Annular Scarring(Milia) Palms/sole Buttock/periorofacial Elbow/knee/dorsum BP CBDC of Hand & Feet(EBA)
- 42. Approach to adult with vesiculobullous lesions
- 43. Introduction • Vesicle-discrete,scattered, grouped,linera • Directly from macule, papule , pustule bullae • Bullae- flaccid or tense • Painful or pruritic • Hemorrhagic—pemphigus,drugs,EBA,LABD
- 44. Diagnostic outline Distribution Lesion morphology Systemic association Autoimmune or genetic infections drugs, systemic diseases, metabolic , nutritional
- 45. Causes • Infections • Viral-HS,Hz,ORF,milkers nodules,cowpox • Bacterial: gram positive, staph , strepto, gram negative-pseudomonas meningococcal, disseminated gonococcal infection • Fungal: vesiculobullous tinea infection
- 46. • Autoimmune blistering disorders: • Genetic blistering:EBS(weber cockayne type) • Hailey hailey disease • Connective tissue disorders :BLE,Bullous morphea • Vasculitis : cutaneous small vessel,Urticarial vasculitis, • Papulosquamous disorders: lichen planus pemphigoides,pitryaisis lichenoides et varioliformis acuta
- 47. • Neutrophilic dermatoses: pyoderma gangrenosum • Nutritional deficiency : zinc ,niacin • Photosensitive disorder:Ple,Phototoxic, photoallergic, phytophoto dermatitis, • Dermatitis • Phytodermatitis • Vesiculobullous hand eczema
- 48. • Metabolic disorders: sys. Amyloidosis ,porphyrias,bullous diabeticorum, • bullous drug eruptions • FDR,SJS,TEN,EM,drug induced autoimmune disorders,pseudo porphyria’s • Sign of internal malignancy: paraneoplastic syn, necrolytic migratory erythema • Graft v/s host disease
- 49. • Others: bullous IBR, edema blisters, coma blisters post burn or frictional blisters
- 50. Approach in an adult with vesiculopustules Grouped on erythematous base multinucleated giant cells HS,HZ gram +ve bacteria Vesicles +honey colored crusts non bullous impetigo TS-mng Papules, pustules vesicles crust Varicella -ve Palms and soles PLEVA Painful papulovesicles with ulceration Fingers,forearm face ORF/cow pox
- 51. Papulovesicles with itching Papulovesicles, bullae Photodistributed Linear exposed sites PLE, or H/o drug or chemical Phytodermatitis Photoallergy (poison IVY)
- 52. Flaccid vesicles with Vesicles, pustules,erosions Pustules Flexural areas Eczematous areas, TZ- acntholytic cells widespread fever Hailey hailey diease H/O skin disease Kaposi’s ,varicelliform eruption,eczema herpeticum
- 53. Localized vesicobullous leions Photodistributed Vesicle Tense bullae Sunburn,erythema,edema Bulla with scarring with hyperpigmentation Diarrhea milia dementia urine porphyrin pellagra PCT Pseudoporphyria phototoxic dermatitis
- 54. Erythema swelling Palpable purpura Tenderness, blisters Legs Dependent areas H/P: vasculitis Erysipelas cellulitis CSVV
- 55. Deep seated vesicles Present over friction sites Itching Palms ,fingers, sole Friction blisters EBS spongiosis Bullous diabeticorum Vesicullobulous Palmoplantar eczema
- 56. Macules , plaques, bullae Bulla with central clearing H/O recurrent lesions Healing with hyperpigmentaion face,intertriginous, arms Gram stain +ve Genital , periural FDR Bullous impetigo
- 57. Generalized vesiculobullous lesions Mucosal lesions nikolsky sign +ve TZ: acantholytic cells H/O drug intake Intraepidermal blistering SJS,TEN (Pemphigus)
- 58. Tense blisters, scarring with mucosal erosions Nikolsky –ve Elbows, knees, widespread blisters Grouped annular lesions hands and feet +ve ANA IF: linear IgA EBA BSLE LABD
- 59. Grouped papulovesicles Tense blisters,grouped Ithcing Umbilicus pregnancy DH Pemphigoid gestationalis
- 60. Purpura,necrosis,hemmrhagic bulla DIC Gangrene and meningitis Infarct leions eschar Joint pain erythema Purpura fulminans ecthyma gangrenosum Disseminated Gonococcal infection
- 61. Bulla with LP LP like lesions suddun bulla involved + uninvolved skin DIF –ve IF +ve Bullous LP Lichen planus pemphigoides
- 62. Macules, erythroderma,bulla Vesicobullloous lesions With diarrhea, H/O H/O internal malignancy Blood tranfusions other systemic disease Organ transplantation Acute GVHD Neutrophilic dermatoses Paraneoplastic syndrome
- 63. • Lab diagnosis : • Tzanck smear • Histopathology • Confirmatory : • Immunofluroscence • Antigen mapping • Electron microscopy
- 64. • Tzanck smear : 1. Infections: Herpes simplex----multinucleated giant cells Bullous impetigo /SSSS-------Acantholytic cells 2. Non infectious conditions Acantholytic cells----Pemphigus ,hailey-hailey disease Eosinophils ------ETN,Arthropod bites Neutrophils------Acropustulosis of infancy Histiocytosis with reniform nucleus----congenital self healing LCH
- 65. • Histopathology: • LEVEL OF BLISTES INTRAEPIDERMAL: 1. Subcorneal+Acanthosis—PF,PE,IgA pemphigus—eosinophilks SSSS,BI—neutrophils 2. Spinous layer----viral—Balloning degenration 3. Suprabasal layer—pemphigus vulgaris—eosinophilc spongiosis Dilipidated brick wall---hailey hailey disease 4. Basal Layer with full thickness epidermal necrosis--TEN
- 66. • SUBEPIDERMAL: • Minimal Inflammatory infiltrate 1. PCT: extension of dermal papillae from floor into cavity of bulla 2. EBA,EB • Predominantly neutrophilic infiltrate: 1. DH-Neutrohils in dermal papillae 2. CBDC—mixed eiosinophils and neutrophils 3. Scarring—EBA • Predominant Eosinophilic infiltrate: BP
- 67. • Immunofluorescence: • DIF: 1. IgA deposition in ICS only –PV,PF,PNP 2. IgA deposition in ICS and BMZ—PE 3. IgG deposition • BMZ and vessel wall—IgG and other immunoreactants(IgM1,A1,C3) in homogeneous pattern – PCT,EPP • BMZ deposition alone IgG in linear, wavy,tubular—BP IgG,C3,IgA--EBA
- 68. 4. IgA deposition BMZ • IgA,C3—granular,speckeled,fibrillar dermal papillae—DH • Linear , continuous ,homogeneous -CBDC
- 69. • INDIRECT IMMUNOFLUROSCENCE- 1. Anti ICS antibody— IgG class—PV,PF,PNP IgA class– IgA pemphigus 2. Anti BMZ antibodies: IgG class—BP,EBA IgA class-- CBDC
- 70. • ELECTRON MICROSCOPY FOR GENETIC BLISTERING DISORDERS 1. EBS----intraepidermal clumping of keratin filaments 2. JEB---- Lamina lucida abnormal, appearance of hemidesmosomes 3. DEB--- Below lamina densa, decreased or absent anchoring fibrils
- 71. • IMMUNOHITOCHEMISTRY AND ANTIGEN MAPPING: EBS—all AB stain base of blister EB(J)—Collagen XVII is expressed on the roof of split skin whereas other antibodies are seen on the floor of the blister. EB(D)– all antibodies—roof of spilt
- 72. Thank you