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Tuesday, January 06, 2009

persistent oral ulcers followed by multiple flaccid bullae on trunk Pemphigus vulgaris.

Question 60
A 40 year old female developed persistent oral ulcers followed by multiple flaccid bullae on trunk and extremities. Direct examination of a skin biopsy immunofluorescence showed intercellular igG deposits in the epidermis and suprabasal split with acantholytic cells. The probable diagnosis is
a.       Pemphigus vulgaris 
b.      Pemphigoid.
c.       Erythema multiforme.
d.      Dermatitis hepetiformis
Answer
a. Pemphigus vulgaris.
Reference
Harrison Table 58-1.  Immunologically Mediated Blistering Diseases
Quality
Spotter
Status
Repeat
QTDF
All books give this
Discussion
Pemphigus Vulgairs
1.      is an Auto Immune Mediated blistering skin disease(other immune mediated blistering skin diseases are Pemphigus Foliaceus, Bullous Pemphigoid, Cicatrical Pemphigoid, Dermatitis Herpetiformis, Epidermolysis Bullosa Acquista and Linera IgA disease)
2.      HLA-DR4 and DRW6
3.      Acantholyssis and Intraepidermal L\Blisters are haracteristic features
4.      Acantholytic cells are Round cells with hyperchromatic nucleus and Perinuclear Halo
5.      Nikolsky’s Sign is seen(Other diseases with Nikolsky’s Sign are Pemphigus, Pophyria, Steven Johnson’s Toxic Epidermal Necrolysis, Staphylococcal Scalded Skin Syndrome) Nikolsky’s Sign is negative in Pemphigoid
6.      Tzanck’s Test is used.
7.      Tzanck’s Cell is Keratinocyte
8.      IgG antibodies are seen
9.      Row of Tombstones appearance is present in Pemphigus Vulgaris

Explanation

1.         Pemphigus vulgaris presents with Acantholysis and Intraepidermal Bullae.
2.         Pemphigoid presents with subepidermal bullae IgG Antobodies are present against Basement Membrane and usually affect the elderly in 60 - 80 years and Mucosal Involvement is rare. There is no statistical association with internal malignancy and HLA and Direct Immunonoflourescence reveals IgG and C3 deposits .
3.         Erythema multiforme is usually due to Viral(Herpes) or other Bacteria and Fungus. Lesions come in crops and last for 2 ot3 weeks,and commonly affect the face, dorsal surface of hand and feet and ecxtensor sirface of forearm and legs, palms and soles. Target Lesions (Bullet's Eye lesions) are specific and they consist of 3 conventric zones of color changes and are mostly found acrally on the hand and feet in Erythema Multiformae Minor
4.         Dermatitis herpetiformis presents as Intensely priiritic, chronic, papulovesicular lesions that are symmetrically distributed over extensor surfaces. Associate with gluten sensitive Enteropathy. More than 90 % express HLA B8 HLA DRW3 and HLA DQW2. Subepidermal Blisters with Neutrophils ar seen and Granular IgA deposits are seen in the dermal papillae. The IgA antibodies are against Gliadin. Treatmetn is with Dapsone and if the patient cannot tolerate Dapsone, Sulphapyridine is substituted. Diet should exclude Gluten which is present in Barley, Rye and Wheat. Oats are tolerated and Gluten is not seen in Rice
Comments
Though Dermatology is a bigggggggg topic, the questions that are usually asked can be answered with minimal knowledge from SARP, PARAS and Sure Success in PG.
Tips
  • PemphiguS has Superficial (Intra epidermal) blisters
  • PemphigoiD has Deep (Sub Epidermal) blisters
  • And among Pemphigus
o       P.Erythematosus and P.Foliaceous show intraepidermal Acantholytic Split in Stratum Granulosum (Pnemonic EFG)and
o       P.Vulgaris and P.Vegetans show Intraepidermal Acantholytic split in between the Basal layer and the Prickle cell layer
  • The Nikolsky sign is positive in pemphigus and more rarely toxic epidermal necrolysis.
  • Pemphigus Vulgaris is due to autoimmunity directed against Cadherin
  • Pemphigus Foliaceus is due to autoimmunity directed against Desmoglein and this Drug induced Pemphigus is seen after administration of Penicillamine and Captopril
·         Pemphigus Vegitans is the least common
  • Let us see the differences between Pemphigus and Phempigoid
Features
Pemphigus
Pemphigoid
Age
40-60
60-80
HPE-Row of Tombstones
Present
Absent
Nikolsky’s Sign
Present
Absent
Bulla Location
Intraepidermal
Sub Epidermal
Bulla Features
Flacced
Tense
Mucosa (eg Oral )
Involved
Not involved
Acantholysis
Present
Absent

131 comments:

  1. Canker sores (aphthous stomatitis) are abnormalities of the mucous membranes of the mouth in the form of sores or white patches of yellowish. Treat ulcers better done quickly so as not to interfere with daily activities, such as when food or speak. The most common cause of canker sores is a lack of vitamin C, B12 or iron. To meet these elements can be done by increasing consumption of fruits like oranges and others. Then how do I treat thrush if already grown? Follow the explanation below

    COCONUT WATER
    Coconut water is useful to treat dehydration in addition, can also be used as a mouth ulcer drug. How to treat canker disease using coconut water is to drink

    Guava leaves
    How to treat cara mengobati sariawan ulcers by using guava is chewed, then gargle using clean water

    BANANA AND HONEY
    How to treat ulcers by using banana mixed with honey is to grind. Then campuranlah bananas and honey that has been smoothed smeared on canker sores

    GARLIC
    How to treat ulcers by using garlic is by attaching directly to the wound caused by canker sores

    Turmeric
    The trick treat thrush with turmeric is to soften and then paste it into the wounds caused by thrush

    Natural materials mentioned above can be used to cure or treat canker sores. But if before the sprue should we do prevention

    How to prevent ulcers is with sufficient vitamin C, B12 or iron from the food we eat. Expand eat fruits that contain vitamin C is recommended to prevent ulcers grow. Prevention is wiser than treating canker sores are already arising

    Canker sores (aphthous stomatitis) are abnormalities of the mucous membranes of the mouth in the form of sores or white patches of yellowish. Treat ulcers better done quickly so as not to interfere with daily activities, such as when food or speak. The most common cause of canker sores is a lack of vitamin C, B12 or iron. To meet these elements can be done by increasing consumption of fruits like oranges and others. Then how do I treat thrush if already grown? Follow the explanation below

    COCONUT WATER
    Coconut water is useful to treat dehydration in addition, can also be used as a mouth ulcer drug. How to treat canker disease using coconut water is to drink

    Guava leaves
    How to treat ulcers by using guava is chewed, then gargle using clean water

    BANANA AND HONEY
    How to treat ulcers by using banana mixed with honey is to grind. Then campuranlah bananas and honey that has been smoothed smeared on canker sores

    GARLIC
    How to treat ulcers by using garlic is by attaching directly to the wound caused by canker sores

    Turmeric
    The trick treat thrush with turmeric is to soften and then paste it into the wounds caused by thrush

    Natural materials mentioned above can be used to cure or treat canker sores. But if before the sprue should we do prevention

    How to prevent ulcers is with sufficient vitamin C, B12 or iron from the food we eat. Expand eat fruits that contain vitamin C is recommended to prevent ulcers grow. Prevention is wiser than treating canker sores are already arising

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