Paediatrics, bullous pemphigoid: what it is, diagnosis and treatment

By Cristiano Antonino On Nov 8, 2022

A rare disease characterised by the appearance of blisters on the skin. It can benefit from immunosuppressive drugs and heals within a few years

This is a rare autoimmune disease characterised by the appearance of boils on the skin.

It also rarely involves the mucous membranes of the mouth and genital regions.

Bullous pemphigoid is particularly rare in children

It is mainly observed in infants, while it is more frequent in adults over 60 years of age.

Bullous pemphigoid is an autoimmune disease due to the formation of antibodies directed against certain skin proteins such as BPAg1 and BPAg2 called haemidesmosomes, which have the function of keeping cells attached to each other.

They are hypothesised as triggering factors:

Drugs such as furosemide, spironolactone, omeprazole, monoclonal antibodies, penicillin, antipsychotics;
Physical factors: trauma, radiotherapy for tumours, ultraviolet (UV) radiation;
Concomitant skin diseases such as psoriasis, Lichen planus and certain infections;
Systemic diseases such as diabetes, juvenile idiopathic arthritis, ulcerative colitis, multiple sclerosis.
Bullous pemphigoid is manifested by the appearance of bullae.
These are generally serous (transparent) surrounded by an erythematous (red) urticarial halo (pomphi: soft lesions raised on the skin surface).

The bullae are tense, do not break easily and evolve in successive waves.

They are localised on the trunk, the legs and in particular the knee crease.

In some cases they are also observed on the gingival mucous membranes, at the ano-genital region and more rarely on the conjunctiva.

Itching is intense.

Bullous pemphigoid (like pemphigus) is not contagious.

The diagnosis of bullous pemphigoid is based on the appearance of the lesions and the presence of itching

However, a biopsy of a small fragment of skin affected by the boils is required to confirm the diagnosis.

The fragment is sent to the laboratory where it is suitably treated, stained and observed under a microscope.

Using a special microscope, the autoantibodies attached to the membrane separating the epidermis from the dermis can be observed under immunofluorescence.

In some cases, it is necessary to contact the skin fragment in a test tube with serum taken from the same patient: the autoantibodies present in the blood stick to the membrane and can be observed by immunofluorescence.

Various other known diseases, manifested by the appearance of boils, must be excluded during the diagnostic course: pemphigus vulgaris, linear IgA disease, dermatitis herpetiformis, epidermolysis bullosa, drug reactions.

Bullous pemphigoid has a chronic-recurrent course and is relatively benign

There may be periods of disease flare-ups interspersed with periods of remission during which symptoms disappear.

The disease usually heals within 3-6 years. Treatment can put the disease into remission.

Treatment of bullous pemphigoid includes:

Local and oral corticosteroids;
Immunosuppressive drugs such as Methotrexate, Mycophenolate mofetil, Azathioprine and Cyclophosphamide to be combined with cortisone in order to reduce its dosage and side effects;
Rituximab and Omalizumab, monoclonal antibodies that act as immunosuppressants or inflammation inhibitors;
High-dose intravenous immunoglobulins acting as immunosuppressants.

Atopic Dermatitis: Treatment And Cure

Psoriasis, A Disease That Affects The Mind As Well As The Skin

Allergic Contact Dermatitis And Atopic Dermatitis: The Differences

Adverse Drug Reactions: What They Are And How To Manage Adverse Effects

Symptoms And Remedies Of Allergic Rhinitis

Allergic Conjunctivitis: Causes, Symptoms And Prevention

What Is And How To Read The Allergy Patch Test

Eczema Or Cold Dermatitis: Here’s What To Do

Psoriasis, An Ageless Skin Disease

Clinical Manifestations Of Atopic Dermatitis