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