| What is Epidermolysis bullosa? | a hereditary blistering disease with onset in neonatal period or infancy and predominantly affecting pressure sites | • Bullous Impetigo, Varicella, Stevens Johnson Syndrome/TEN* • Epidermolysis bullosa, a hereditary blistering disease with onset in neonatal period or infancy and predominantly affecting pressure sites; presence of scarring on limbs, acral areas, trunk and abnormality of the teeth or nails • Consider Congenital syphilis in a neonate- get VDRL for mother and child *Refer to STW on Bacterial Infections; Varicella and Herpes Zoster and cADR Part B | 86 | definition | 0.980 | • Bullous Impetigo, Varicella, Stevens Johnson Syndrome/TEN* • Epidermolysis bullosa, a hereditary blistering disease with onset in neonatal period or infancy and predominantly affecting pressure sites; presence of scarring on limbs, acral areas, trunk and abnormality of the teeth or nails • Consider Congenital syphilis in a neonate- get VDRL for mother and child *Refer to STW on Bacterial Infections; Varicella and Herpes Zoster and cADR Part B | 1 | page=1,block=66 | 0.700 | valid |
| What are the characteristics of fluid-filled blisters on the skin? | itchy or painful blisters (either flaccid or tense) on skin, over a normal or erythematous base | WHEN TO SUSPECT? • Pemphigus vulgaris/ variants • Pemphigus foliaceus/ variants • Bullous pemphigoid • Pemphigoid gestationis • Mucous membrane pemphigoid • Linear IgA bullous diseases/ chronic bullous disease of childhood • Dermatitis herpetiformis • Epidermolysis bullosa acquisita • Bullous systemic lupus erythematosus CORRECT DIAGNOSIS; PREVENTION/ TREATMENT OF SEPSIS; AND REGULARITY OF TREATMENT BRINGS BEST RESULTS Appearance of fluid-filled, itchy or painful blisters (either flaccid or tense) on skin, over a normal or erythematous base Appearance of raw, erythematous erosions ± crusting on skin Appearance of erosions/ blisters inside oral cavity, eyes, nose and genitals | 451 | summary | 0.970 | WHEN TO SUSPECT? • Pemphigus vulgaris/ variants • Pemphigus foliaceus/ variants • Bullous pemphigoid • Pemphigoid gestationis • Mucous membrane pemphigoid • Linear IgA bullous diseases/ chronic bullous disease of childhood • Dermatitis herpetiformis • Epidermolysis bullosa acquisita • Bullous systemic lupus erythematosus CORRECT DIAGNOSIS; PREVENTION/ TREATMENT OF SEPSIS; AND REGULARITY OF TREATMENT BRINGS BEST RESULTS Appearance of fluid-filled, itchy or painful blisters (either flaccid or tense) on skin, over a normal or erythematous base Appearance of raw, erythematous erosions ± crusting |
| What are the common characteristics of autoimmune bullous diseases in the pemphigus group? | Flaccid blisters/ erosions ± crusting on skin ± mucosae | • Likely pemphigus group of autoimmune bullous diseases > Flaccid blisters/ erosions ± crusting on skin ± mucosae > Usually seen in adults; can rarely affect children • Likely sub-epidermal autoimmune bullous diseases > Tense, small to large blisters, containing clear or hemorrhagic fluid, on an itchy erythematous base, commonly healing with hypopigmentation ± scarring > Seen in children, adults and elderly (most common is bullous pemphigoid) • Get a Tzanck smear • Get a biopsy for histopathology from margin of a lesion • Get a peri-lesional biopsy for direct immunofluorescence, if facility is available PEMPHIGUS BULLOUS PEMPHIGOID CHRONIC BULLOUS DISEASE OF CHILDHOOD • Child < 5 years • Erosions with peripheral tense blisters • Urticarial base • Face/ peri-genital involvement RED FLAG SIGNS • Fever ± chills and rigors • Hypotension (indicating hypovolemia due to fluid loss or sepsis) • Altered sensorium (indicating dyselectrolytemia or sepsis) | 58 | summary | 0.970 | • Likely pemphigus group of autoimmune bullous diseases > Flaccid blisters/ erosions ± crusting on skin ± mucosae > Usually seen in adults; can rarely affect children • Likely sub-epidermal autoimmune bullous diseases > Tense, small to large blisters, containing clear or hemorrhagic fluid, on an itchy erythematous base, commonly healing with hypopigmentation ± scarring > Seen in children, adults and elderly (most common is bullous pemphigoid) • Get a Tzanck smear • Get a biopsy for histopathology from margin of a lesion • Get a peri-lesional biopsy for direct immunofluorescence, if facility |
| What are the features of sub-epidermal autoimmune bullous diseases? | Tense, small to large blisters, containing clear or hemorrhagic fluid, on an itchy erythematous base, commonly healing with hypopigmentation ± scarring | • Likely pemphigus group of autoimmune bullous diseases > Flaccid blisters/ erosions ± crusting on skin ± mucosae > Usually seen in adults; can rarely affect children • Likely sub-epidermal autoimmune bullous diseases > Tense, small to large blisters, containing clear or hemorrhagic fluid, on an itchy erythematous base, commonly healing with hypopigmentation ± scarring > Seen in children, adults and elderly (most common is bullous pemphigoid) • Get a Tzanck smear • Get a biopsy for histopathology from margin of a lesion • Get a peri-lesional biopsy for direct immunofluorescence, if facility is available PEMPHIGUS BULLOUS PEMPHIGOID CHRONIC BULLOUS DISEASE OF CHILDHOOD • Child < 5 years • Erosions with peripheral tense blisters • Urticarial base • Face/ peri-genital involvement RED FLAG SIGNS • Fever ± chills and rigors • Hypotension (indicating hypovolemia due to fluid loss or sepsis) • Altered sensorium (indicating dyselectrolytemia or sepsis) | 220 | summary | 0.970 | • Likely pemphigus group of autoimmune bullous diseases > Flaccid blisters/ erosions ± crusting on skin ± mucosae > Usually seen in adults; can rarely affect children • Likely sub-epidermal autoimmune bullous diseases > Tense, small to large blisters, containing clear or hemorrhagic fluid, on an itchy erythematous base, commonly healing with hypopigmentation ± scarring > Seen in children, adults and elderly (most common is bullous pemphigoid) • Get a Tzanck smear • Get a biopsy for histopathology from margin of a lesion • Get a peri-lesional biopsy for direct immunofluorescence, if facility |
| What can lead to the best results in managing autoimmune blistering diseases? | CORRECT DIAGNOSIS; PREVENTION/ TREATMENT OF SEPSIS; AND REGULARITY OF TREATMENT | WHEN TO SUSPECT? • Pemphigus vulgaris/ variants • Pemphigus foliaceus/ variants • Bullous pemphigoid • Pemphigoid gestationis • Mucous membrane pemphigoid • Linear IgA bullous diseases/ chronic bullous disease of childhood • Dermatitis herpetiformis • Epidermolysis bullosa acquisita • Bullous systemic lupus erythematosus CORRECT DIAGNOSIS; PREVENTION/ TREATMENT OF SEPSIS; AND REGULARITY OF TREATMENT BRINGS BEST RESULTS Appearance of fluid-filled, itchy or painful blisters (either flaccid or tense) on skin, over a normal or erythematous base Appearance of raw, erythematous erosions ± crusting on skin Appearance of erosions/ blisters inside oral cavity, eyes, nose and genitals | 323 | summary | 0.970 | WHEN TO SUSPECT? • Pemphigus vulgaris/ variants • Pemphigus foliaceus/ variants • Bullous pemphigoid • Pemphigoid gestationis • Mucous membrane pemphigoid • Linear IgA bullous diseases/ chronic bullous disease of childhood • Dermatitis herpetiformis • Epidermolysis bullosa acquisita • Bullous systemic lupus erythematosus CORRECT DIAGNOSIS; PREVENTION/ TREATMENT OF SEPSIS; AND REGULARITY OF TREATMENT BRINGS BEST RESULTS Appearance of fluid-filled, itchy or painful blisters (either flaccid or tense) on skin, over a normal or erythematous base Appearance of raw, erythematous erosions ± crusting |
| What can lead to the best outcomes in managing autoimmune blistering diseases? | CORRECT DIAGNOSIS; PREVENTION/ TREATMENT OF SEPSIS; AND REGULARITY OF TREATMENT BRINGS BEST RESULTS | WHEN TO SUSPECT? • Pemphigus vulgaris/ variants • Pemphigus foliaceus/ variants • Bullous pemphigoid • Pemphigoid gestationis • Mucous membrane pemphigoid • Linear IgA bullous diseases/ chronic bullous disease of childhood • Dermatitis herpetiformis • Epidermolysis bullosa acquisita • Bullous systemic lupus erythematosus CORRECT DIAGNOSIS; PREVENTION/ TREATMENT OF SEPSIS; AND REGULARITY OF TREATMENT BRINGS BEST RESULTS Appearance of fluid-filled, itchy or painful blisters (either flaccid or tense) on skin, over a normal or erythematous base Appearance of raw, erythematous erosions ± crusting on skin Appearance of erosions/ blisters inside oral cavity, eyes, nose and genitals | 323 | summary | 0.970 | WHEN TO SUSPECT? • Pemphigus vulgaris/ variants • Pemphigus foliaceus/ variants • Bullous pemphigoid • Pemphigoid gestationis • Mucous membrane pemphigoid • Linear IgA bullous diseases/ chronic bullous disease of childhood • Dermatitis herpetiformis • Epidermolysis bullosa acquisita • Bullous systemic lupus erythematosus CORRECT DIAGNOSIS; PREVENTION/ TREATMENT OF SEPSIS; AND REGULARITY OF TREATMENT BRINGS BEST RESULTS Appearance of fluid-filled, itchy or painful blisters (either flaccid or tense) on skin, over a normal or erythematous base Appearance of raw, erythematous erosions ± crusting |
| What is the focus of the ICMR Standard Treatment Workflow mentioned? | IMMUNOBULLOUS DERMATOSES | Dr Lokesh Sharma, ICMR ICMR Standard Treatment Workflow (STW) IMMUNOBULLOUS DERMATOSES Dermatology, IMMUNOBULLOUS DERMATOSES, ICMR, STW Adobe Illustrator 26. (Windows) ICMR Standard Treatment Workflow (STW) IMMUNOBULLOUS DERMATOSES | 62 | summary | 0.890 | Dr Lokesh Sharma, ICMR ICMR Standard Treatment Workflow (STW) IMMUNOBULLOUS DERMATOSES Dermatology, IMMUNOBULLOUS DERMATOSES, ICMR, STW Adobe Illustrator 26. | | pdf-properties | 0.700 | valid |
| What conditions are included under the category of complications with treatment management available? | • Bullous Impetigo, Varicella, Stevens Johnson Syndrome/TEN* • Epidermolysis bullosa, a hereditary blistering disease with onset in neonatal period or infancy and predominantly affecting pressure sites; presence of scarring on limbs, acral areas, trunk and abnormality of the teeth or nails • Consider Congenital syphilis in a neonate- get VDRL for mother and child *Refer to STW on Bacterial Infections; Varicella and Herpes Zoster and cADR Part B | AND MANAGEMENT OF COMPLICATIONS OF TREATMENT IS AVAILABLE)
• Bullous Impetigo, Varicella, Stevens Johnson Syndrome/TEN* • Epidermolysis bullosa, a hereditary blistering disease with onset in neonatal period or infancy and predominantly affecting pressure sites; presence of scarring on limbs, acral areas, trunk and abnormality of the teeth or nails • Consider Congenital syphilis in a neonate- get VDRL for mother and child *Refer to STW on Bacterial Infections; Varicella and Herpes Zoster and cADR Part B | 59 | list | 0.820 | • Bullous Impetigo, Varicella, Stevens Johnson Syndrome/TEN* • Epidermolysis bullosa, a hereditary blistering disease with onset in neonatal period or infancy and predominantly affecting pressure sites; presence of scarring on limbs, acral areas, trunk and abnormality of the teeth or nails • Consider Congenital syphilis in a neonate- get VDRL for mother and child *Refer to STW on Bacterial Infections; Varicella and Herpes Zoster and cADR Part B |
| What prior illnesses are relevant to autoimmune blistering diseases? | History of prior varicella/ chicken pox | July/ 2022 AUTOIMMUNE BLISTERING DISEASES ADDITIONAL INFORMATION • Age at onset and duration of blistering • History of any recent drug intake • History of prior varicella/ chicken pox • History of similar illness in family • History of itching, pain, burning • Predominant sites affected • Associated photosensitivity | 145 | list | 0.820 | July/ 2022 AUTOIMMUNE BLISTERING DISEASES ADDITIONAL INFORMATION • Age at onset and duration of blistering • History of any recent drug intake • History of prior varicella/ chicken pox • History of similar illness in family • History of itching, pain, burning • Predominant sites affected • Associated photosensitivity | 1 | page=1,block=10 | 0.700 | valid |
| What symptoms are commonly associated with autoimmune blistering diseases? | History of itching, pain, burning | July/ 2022 AUTOIMMUNE BLISTERING DISEASES ADDITIONAL INFORMATION • Age at onset and duration of blistering • History of any recent drug intake • History of prior varicella/ chicken pox • History of similar illness in family • History of itching, pain, burning • Predominant sites affected • Associated photosensitivity | 226 | list | 0.820 | July/ 2022 AUTOIMMUNE BLISTERING DISEASES ADDITIONAL INFORMATION • Age at onset and duration of blistering • History of any recent drug intake • History of prior varicella/ chicken pox • History of similar illness in family • History of itching, pain, burning • Predominant sites affected • Associated photosensitivity | 1 | page=1,block=10 | 0.700 | valid |