Pathology of Discoid Lupus Erythematosus

Custom Search

Pathology of Discoid Lupus

Erythematosus

"The lesion usually involves the face and has a "butterfly distribution" on the cheeks and bridge of the nose"

Lupus erythematosus is a chronic inflammatory autoimmune disease with a wide range of clinical manifestations ranging from an indolent chronic cutaneous form to a severe potentially life-threatening disease, systemic lupus erythematosus.

Discoid lupus erythematosus is a subphenotype of chronic cutaneous lupus erythematosus, characterised by scaly disk-shaped plaques which may be localised or widespread, occurring predominantly on sun-exposed skin.

Frequently the only manifestation is the "classic butterfly rash". This is a delicate erythema of the malar area of the face.

The circumscribed, raised, erythematous, scaly, lesions are present on the face, scalp and ears in 70% of patients.

Oral mucosa and hands are sometimes involved.

Eyelid involvement occurs in only 6% of patients, often associated with other cutaneous abnormalities.

Squamous cell carcinoma may occur rarely.

The clinical variants are as follows:

Hypertrophic ; Annular; Papulonodular ; Tumid ; Lymphocytic infiltration of the skin; Linear lesion.

Microscopic features:

- Lichenoid (Interface)Tissue Reaction Pattern - There is vacuolar change along the dermoepidermal junction together with scattered apoptotic keratinocytes.

- An inflammatory infiltrate is present both in the superficial and deep dermis. There is moderately dense or dense lymphocytic infiltrate.

- The inflammatory infiltrate is often peri-infundibular and perifollicular

- Epidermis is usually thinned and there is keratotic follicular plugging.

- Epidermal basement membrane is often thickened.

Lupus band test is performed on skin biopsy to confirm the diagnosis.

Direct immunofluorescence of the involved skin shows a broad band of immunoglobulin along the basement membrane.

IgG and IgM are present in 50-90% of cases. Complement 3 is present less frequently.

Dermatopathology Quiz Case 55