Clinical History:

This 43-year-old woman noticed the insidious onset of a red rash that involved the cheeks of her face and bridge of her nose. Otherwise, she was in good health. You perform a physical examination and find that the examination is entirely normal, except for the rash. You refer the patient to a dermatologist who performs a biopsy of the affected skin. Half the biopsy is sent for routine light microscopy and half for immunofluorescence microscopy.

Image 1.1:

The patient's rash is shown here. This is a typical malar "butterfly" type rash seen with lupus.

Image 1.2:

The light microscopic findings (H&E stain) in affected skin are shown here. What kind of inflammation is present? There are mostly mononuclear cells (lymphocytes and macrophages) at the dermal-epidermal junction and in the dermis in perivascular regions and around skin appendages.

Image 1.3:

This is a higher power view of the skin. Note the RBC's. The RBC's have spilled from blood vessels damaged by the inflammation. The epidermis shows focal liquefactive necrosis at the dermal-epidermal junction. This is characteristic of SLE but not diagnostic.

Image 1.4:

A high power view of the immunofluorescence pattern with anti-IgG is shown here. Where in the skin is the staining most prominent? Staining is most prominent at the dermal-epidermal junction.

Questions:

1. What is the diagnosis?

This is discoid lupus erythematosus (DLE).

2. Should an antinuclear antibody test be performed on this patient?

Yes. 35% of the time the ANA will be positive with DLE. Perhaps 10% of persons with DLE will eventually go on to SLE during their lifetime, and most of these will come from the group with a positive ANA.

3. How likely is it that this patient will have systemic symptoms from her disease?

If the ANA is negative, it is very unlikely that the patient will develop systemic symptoms.

4. What is your interpretation of the light and immunofluorescent microscopic findings (what is her disease process)? How specific are these findings?

The light microscopic findings are: liquefaction necrosis at the dermal-epidermal junction of the skin along with dermal perivascular inflammation. These findings are quite specific for lupus. The immunofluorescence findings of immunoglobulin and complement components at the dermal-epidermal junction is quite specific for lupus when it has a band-like configuration as in this case. If normal sun-exposed skin does not show immunofluorescent staining, a diagnosis of DLE is secure. SLE would have immunofluorescent staining even in normal sun-exposed skin.