History
A 55-year-old woman presents to the dermatology clinic with a long history of a rash that occurred during the spring and summer months over her face and scalp. Over the past few years the affected area became more extensive, the skin became scarred and she noticed her hair did not grow back. She wears a hair weave to cover the area. More recently, however, she has developed new lesions over her cheeks and is worried about scarring. She is otherwise well.
Examination
There are large areas of scarring with associated hypo- and hyperpigmentation. She has alopecia predominantly over her fronto-temporal scalp (Fig. 37.1). Over the face and vertex of the scalp there are indurated erythematous plaques with overlying scale and follicular plugging. Her ears are similarly affected. Full skin examination was otherwise normal as were her nails.
Questions
• What is the diagnosis?
• What other investigations might you arrange?
Medicine
The scalp shows areas of alopecia with skin atrophy and scarring in the affected areas.
These changes are typical of discoid lupus erythematosus (DLE), which is a chronic, persistent, often indolent disorder seen mainly in women. DLE is characterized by fixed, indurated, erythematous plaques with overlying scale, predominantly over the face and scalp. The plaques in DLE are often annular or polycyclic and well demarcated. As the lesions expand, central regression occurs and, unlike in subacute cutaneous lupus erythematosus (SCLE), atrophy and eventually significant scarring results. ‘Burned out’ lesions become pink or hypopigmented but in Asian and African Caribbean patients the skin may also show hyperpigmentation. Permanently scarring alopecia is often a com-mon feature which can be very disfiguring. The external auditory canals are frequently involved as in this patient.
A skin biopsy in DLE revealed hyperkeratosis, epidermal atrophy, follicular plugging and degeneration of the basal cell layer. A perifollicular lymphocytic inflammatory infiltrate was seen. A positive immunofluorescence was seen in more than 85 per cent of new lesions.
Patients should be screened for systemic lupus erythematosus (SLE) with a full blood count, liver and kidney function tests, clotting time and auto-antibody titre. If the ini-tial work-up does not reveal systemic involvement the risk of developing SLE is only 1–5 per cent. Lesions of DLE are, however, not uncommon in patients with established SLE and can be found in up to 25 per cent of patients during the course of their disease. Complete remission occurs in 40 per cent of all patients. DLE without intervention can persist for many years and lead to extensive scarring. Early diagnosis and treatment are therefore essential to prevent further scarring. The mainstay of treatment is with photoprotection (SPF > 30). Potent topical corticosteroids can be very effective for active lesions. Antimalarials, gold and other immunosuppressants may be required.
KEY POINTS
• Without treatment discoid lupus erythematosus (DLE) can result in permanent scarring and disfiguring alopecia on the scalp.
• Screening for systemic disease should be undertaken. which occurs in 1–5 per cent of patients with DLE.
• Rigorous photoprotection is advised to try to prevent further DLE lesions appearing.
need an explanation for this answer? contact us directly to get an explanation for this answer