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Pathology of Discoid Lupus

Erythematosus

"There is a butterfly on my nose"

                                      

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

Dermatopathology Quiz Case 55

Diagnosis: Discoid Lupus Erythematosus

 

                                                                                                                      

 

 

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:

      

 Microscopic images  of Discoid lupus erythematosus

- 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.

   

 Diagram showing some important features of Discoid lupus erythematosus

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.

 

Further reading:

Measuring disease activity and damage in discoid lupus erythematosus.

Two cases of refractory discoid lupus erythematosus successfully treated with topical tocoretinate.

Linear childhood discoid lupus erythematosus following the lines of Blaschko: successfully treated with topical tacrolimus.

Hypertrophic discoid lupus erythematosus.

Discoid lupus erythematosus of the eyelid.

Association of discoid lupus erythematosus with clinical manifestations and damage accrual in a multiethnic lupus cohort.

Discoid lupus erythematosus of the periorbita: clinical dilemmas, diagnostic delays.

Severe chronic blepharitis and scarring ectropion associated with discoid lupus erythematosus.

Comedonic lupus: a rare presentation of discoid lupus erythematosus.

Generalized discoid lupus erythematosus.

Discoid lupus erythematosus as it relates to cutaneous squamous cell carcinoma and to photosensitivity.

Squamous cell carcinoma arising in a recent plaque of discoid lupus erythematous, in a sun-protected area.

 

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

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