| What is Epidermolysis bullosa, and when does it typically onset? | 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 | 1.000 | https://www.icmr.gov.in/icmrobject/uploads/STWs/1725967366_immunobullous.pdf | • 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 common 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 | https://www.icmr.gov.in/icmrobject/uploads/STWs/1725967366_immunobullous.pdf | • 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 typical characteristics of the pemphigus group of autoimmune bullous diseases? | 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 | https://www.icmr.gov.in/icmrobject/uploads/STWs/1725967366_immunobullous.pdf | • 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 information about the onset and progression of autoimmune blistering diseases is considered important? | Age at onset and duration of blistering | 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 | 67 | summary | 0.970 | https://www.icmr.gov.in/icmrobject/uploads/STWs/1725967366_immunobullous.pdf | 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 associated with autoimmune blistering diseases on the skin? | Appearance of fluid-filled, 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 | 423 | summary | 0.970 | https://www.icmr.gov.in/icmrobject/uploads/STWs/1725967366_immunobullous.pdf | 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 question is asked to determine if mucosal surfaces are affected in autoimmune blistering diseases? | Are mucosae involved? | • Are the blisters flaccid or tense? • Are the erosions crusted? • Do the blisters contain clear or hemorrhagic fluid? • Are the blisters umbilicated? • Is the base of the blisters erythematous/ urticarial? • Are the blisters healing with or without scarring? • Are they healing leaving behind hyper/hypopigmentation? • What is the color of the crust? • Are mucosae involved? | 354 | definition | 0.940 | https://www.icmr.gov.in/icmrobject/uploads/STWs/1725967366_immunobullous.pdf | • Are the blisters flaccid or tense? • Are the erosions crusted? • Do the blisters contain clear or hemorrhagic fluid? • Are the blisters umbilicated? • Is the base of the blisters erythematous/ urticarial? • Are the blisters healing with or without scarring? • Are they healing leaving behind hyper/hypopigmentation? • What is the color of the crust? • Are mucosae involved? | 1 | page=1,block=14 | 0.700 | valid |
| What are some physical manifestations of Epidermolysis bullosa? | presence of scarring on limbs, acral areas, trunk and abnormality of the teeth or nails | • 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 | 203 | list | 0.820 | https://www.icmr.gov.in/icmrobject/uploads/STWs/1725967366_immunobullous.pdf | • 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 |
| What items are listed under “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 | 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 | https://www.icmr.gov.in/icmrobject/uploads/STWs/1725967366_immunobullous.pdf | • 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 medical history factors are relevant for autoimmune blistering diseases? | History of any recent drug intake • History of prior varicella/ chicken pox • History of similar illness in family | 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 | 109 | list | 0.820 | https://www.icmr.gov.in/icmrobject/uploads/STWs/1725967366_immunobullous.pdf | 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 related to discomfort are important to note in 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 | https://www.icmr.gov.in/icmrobject/uploads/STWs/1725967366_immunobullous.pdf | 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 |