Pathology of Dermatitis Herpetiformis

Dr Sampurna Roy MD                      2022

Dermatitis herpetiformis is a subepidermal autoimmune disease characterized by skin and intestinal lesions consistent with celiac disease.

Age: Symptoms typically start at age 30 to 40, but can happen at any age.

Site:

It is characterized by urticarial plaques and blisters on the elbows, buttocks, and knees. Other sites may also be involved.

Dermatopathology Quiz Case 37

Microscopic features: 

Early lesions:  

Collections of neutrophils and varying number of eosinophils.

These are located at the tips of edematous dermal papillae (papillary microabscesses).

In lesions of 36-48 hours duration:

There is increase in the number of eosinophils.

There may be fragmentation of neutrophils.

In older lesions:  

There is evidence of subepidermal vesicle formation.

Vesicles contain neutrophils, eosinophils and fibrin.

Occasional shadow epidermal cells may be present.

The histological distinction between dermatitis herpetiformis and linear IgA  bullous dermatosis is almost impossible.

The diagnosis is based on granular IgA deposits in the dermal papillae in a perilesional skin biopsy analysed by immunofluorescence microscopy.

Review: dermatitis herpetiformis.

Recent advances in dermatitis herpetiformis.

Newly described clinical and immunopathological feature of dermatitis herpetiformis.

Clinical and immunopathological features of 159 patients with dermatitis herpetiformis: an Italian experience.

Dermatitis herpetiformis, the celiac disease of the skin!

Dermatitis Herpetiformis: Clinical Presentations Are Independent of Manifestations of Celiac Disease.

Selected skin diseases with systemic involvement.

Dermatitis herpetiformis: from the genetics to the development of skin lesions.

The imbalance between metalloproteinases and their tissue inhibitors is involved in the pathogenesis of dermatitis herpetiformis.

Subepidermal blistering disorders: a clinical and histopathologic review.