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Dermpath-India

Pathology of Bullous Pemphigoid

Dr Sampurna Roy MD               

 

                                                                                                                      

 

 

Bullous Pemphigoid was first described in 1953, is a chronic subepidermal blistering disease.

Age:

Most commonly occurs in elderly persons (in the seventh or eighth decade of life) but occasionally it is seen in young adults and even in children.

Site: 

Lower part of abdomen, inner part of thighs, flexor surface of the arm and leg , axillae,  groin, and palmar and plantar surfaces;  oral lesions have also been reported ;  Other mucosal surfaces are usually not involved.

Clinical presentation:

Multiple tense bulla on normal or erythematous skin of variable size.

May present as erythematous macules, verrucous and vegetating lesions, urticarial plaques and crusted erosions, or annular lesions.

Cause of the disease:  Caused by autoantibody-mediated disruption of adhesion between basal keratocytes and the basement membrane, i.e., antibodies there bind the antigens of bullous pemphigoid. 

Antibodies against two hemidesmosomal components, BP antigen 2 (180 KDa) and BP antigen 1 (230 KDa), have been detected in sera of  the patients.

Circulating antibodies against the BP180 component are sufficient to produce a subepidermal blister.

Triggering factors: 

Trauma ;  burns ;  ultraviolet irradiation, shortly after vaccination;  drugs;  penicillin derivatives ; antibiotics ; ibuprofen and other non-steroidal anti-inflammatory and antipsychotic drugs.

May occur in association with other diseases:   Example:  Rheumatoid arthritis, lichen planus,  psoriasis,  systemic lupus erythematosus, diabetes mellitus,  primary biliary cirrhosis , ulcerative colitis. etc.

Microscopic features:

 
Diagnostic clue:  

A subepidermal blister containing numerous eosinophils ;  

Scattering of eosinophils, mostly as solitary units in the epidermis and across a broad front of it is a clue to the urticarial stage of bullous pemphigoid/herpes gestationis ; 

"Flame figures" (also present in response to the assault of an arthropod.

 

Early (urticarial)-  

Perivascular and interstitial infiltrate of lymphocytes and eosinophils ;  

Eosinophils scattered throughout the upper part of the reticular dermis, in the edematous papillary dermis, and along the dermoepidermal junction; 

Eosinophils may be present  in tiny collections together with spongiosis

Fully developed (vesicular) - 

Perivascular and interstitial infiltrate of lymphocytes and many eosinophils ; 

Numerous eosinophils throughout the edematous upper dermis; 

Subepidermal blister within which numerous eosinophils usually are present ;

Dermal papillae are often preserved ;

Spongiosis is often present at the sides of the blister.

Late (bullous) - 

All the changes seen in developed fully lesions ; 

Sometimes there is formation of spongiotic vesicles together with edema of the papillary dermis. This feature is so prominent at times that it forms subepidermal vesiculation ;

Re-epithelialization from infundibular and eccrine ductal epithelium is noted in some lesions

Rarely other histological changes may be present - 

Cell-poor type ; 

An intraepidermal blister occurs in conjunction with a subepidermal blister ; 

Presence of an infiltrate of lymphocytes and plasma cells ;

Presence of predominantly neutrophils.

Direct immunofluorescence reveals deposits in linear array predominantly of IgG and C3 along the basement membrane zone of lesions, demonstrated best in perilesional skin.

Differential diagnosis of Bullous Pemphigoid:

1. Urticarial lesions of pemphigus vulgaris

2. Blisters of herpes gestationis are indistinguishable from those of cell-rich bullous pemphigoid.

3. Dermatitis Herpetiformis :  Collections of neutrophils at the tip of dermal papillae and in subepidermal spaces. Nuclear "dust" of neutrophils and bands of neutrophils are noted 

4. Cicatricial pemphigoid .

5. Incontinentia pigmenti in the vesicular stage. 

6. Urticaria - No blisters are present consists of lymphocytes, neutrophils, and eosinophils around venules in the reticular dermis.

7. Insect Bite - a wedge-shaped infiltrate of lymphocytes and eosinophils around venules of the reticular dermis and eosinophils scattered interstitially.   

8. Pruritic urticarial papules and plaques of pregnancy. 

 

Further reading:

Bullous pemphigoid: etiology, pathogenesis, and inducing factors: facts and controversies.

Update on the pathogenesis of bullous pemphigoid: An autoantibody-mediated blistering disease targeting collagen XVII.

Diagnosis and Classification of Pemphigus and bullous pemphigoid.

Drug-induced pemphigoid: a review of the literature.

Specific causes of death in patients with bullous pemphigoid as measured by death certificate data: a retrospective cohort study.

Bullous pemphigoid.

Bullous pemphigoid: a cutaneous disorder to be known in aging persons.

Esophagitis dissecans superficialis presenting with massive haematemesis in a patient with bullous pemphigoid.

Bullous pemphigoid initially localized around the surgical wound of an arthroprothesis for coxarthrosis.

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

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