Cutaneous (discoid) lupus erythematosus
Cutaneous (discoid) lupus erythematosus (DLE) is an autoimmune disease believed to occur in patients who are genetically predisposed and in which antibodies are directed against components of the cell nuclei. It is more common in women, tends to be more severe in smokers and presents between the ages of 20-40 years. An initial rash of erythematous plaques or papules appears, mainly in sun-exposed areas, and these lesions may merge, become confluent, thicken and become scaly with a central area of hypopigmentation.
Cutaneous (Discoid) lupus erythematosus - scarring
Over time the skin lesions resolve, but they leave patches of white scarring. Treatment should be quickly instituted to reduce the effect of long-term problems. Any causative factors should be withdrawn. At the time of diagnosis, this patient was started on hydroxychloroquine but stopped the treatment within 10 days because of unpleasant side-effects. The rash settled following the use of a potent topical steroid followed by a milder one as the rash improved. The patient was left with permanent white scars.
Cutaneous (Discoid) lupus erythemAtosus - scarring alopecia
Lesions occur on the scalp and the scale can plug the hair follicles. This can lead to scarring and permanent alopecia. Midor high-potency topical corticosteroids can be tried initially. If sufficient response is not achieved after four weeks, intralesional triamcinolone can be tried. In more serious cases systemic hydroxyquinoline, mycophenolate, ciclosporin or isotretinoin can be used. Oral prednisolone may slow or stop activity but is not recommended for long-term use.
Subacute cutaneous lupus erythematosus
Subacute cutaneous lupus erythematosus presents with a dry, non-itchy rash on the upper back and chest with lesions that may take the form of annular or polycyclic rings, papulosquamous lesions or raised nodules. Unlike the rash of DLE this rash is not followed by scarring. It occurs on the upper back or chest and will usually follow exposure to the sun. It is commonly associated with cutaneous vasculitis. Treatment is with topical corticosteroids, hydroxyquinoline or cytotoxic drugs.
Cutaneous (discoid) lupus erythemAtosus - lips
Patients with DLE may develop lesions on the lips and the mucous membranes, causing mouth ulcers, occasionally affecting the nose and causing dry scaling of the lips. Such changes may predispose to squamous cell carcinoma. Mouth ulcers clear spontaneously but may be helped by antiseptic washes or steroid pastes or lozenges. A positive diagnosis of DLE can be made by skin biopsy in which direct immunofluorescence demonstrates deposition of antibody along the basement membrane.
Sun protection in lupus erythematosus
Exposure to the sun and UV light is a factor in the flare-up of all types of lupus erythematosus. The patient should be advised to avoid sun exposure. It is now recommended that if sun exposure is grossly restricted, and especially if there is a dietary deficiency in vitamin D, a supplement of vitamin D should be taken. The dose recommended is 10 microgram ergocalciferol or colecalciferol a day.
Systemic lupus erythematosus (SLE) is also an autoimmune condition more common in women and usually presents between the ages of 10-50 years. About 5% of patients with DLE have or may go on to develop SLE, in which the effects are not limited to the skin; involvement of other organs, such as the lungs, will have serious implications. Symptoms are variable but many complain of fever, joint pains and swelling, hair loss, mouth ulcers, lymphadenopathy and sensitivity to sunlight. A typical skin rash, known as a 'butterfly rash', spreads across the cheeks and bridge of the nose.
SLE affecting the ear lobe
This patient complained of swelling, inflammation and a violaceous coloration of the ear lobe. Investigations to confirm the diagnosis of SLE include ANA, anti-double strand DNA, antiphospholipid antibodies and anti-Smith antibodies. FBC may show leucopenia, anaemia or low platelets. A chest X-ray may show signs of pleuritis or pericarditis. A urine analysis for casts, blood or protein is indicated. Cytotoxic drugs may be used for patients who fail to respond. The 10-year survival rate for patients with SLE is 85%. Those with severe internal organ complications do less well.