Lupus erythematosus.
Lupus erythematous is chronic non- contagious inflammatory autoimmune disease with mucocutaneous manifestation, with or without systemic manifestation. Advanced stages, with systemic manifestation, involve the lungs, heart, kidney and Brain.
- Etiology: unknown etiology; autoimmune mechanism implicated in pathogenesis
- Predisposing factors: Genetic predisposition; complement defect; UV radiation; Transplacental transfer of maternal antibodies can lead to neonatal LE; smoking; pregnancy (more women are affected); photosensitivity.
Classification:
- A. Chronic cutaneous LE: chronic scaling erythematosquamous lesions primary in sun exposure skin. Types:
- Discoid LE - In this form, there is inflammation and scarring of the ears face and scalp, but the lesions can also occur on any body surface. These lesions develop as a red, inflamed patch with a scaling and crusty appearance. The center areas may appear lighter in color with a rim darker than the normal skin. When lesions occur in hairy areas such as the beard or scalp, permanent scarring and hair loss can occur. Oral mucosa is involved in 15-25% of cases (buccal mucosa, lower lip, gingiva, palate, tongue), associated with skin lesions. Oral lesions are characterized by well-defined central atrophic red plaque surrounded by sharp elevated border of irradiating white striae. Treatment aims to prevent new sores, limit scarring, and make the skin look healthier. It will usually consist of creams or ointments to be applied directly to affected areas of the skin; injections that contain prescription steroids into or around the lesions; antimalarial drugs, such as hydroxychloroquine, taken in tablet form.
- Lupus Tumidus is a chronic relapsing inflammatory disease of the skin. It occurs predominantly on sun exposed areas. Sometimes the skin lesions have an annular or semi-annular (“arciform”) appearance but there are no epidermal changes and therefore no scale, ulceration or crust formation. Every lesion heals without scarring or post-inflammatory hyper- or hypopigmentation and therefore LET does not result in chronic skin damage.
- Lupus Profundus (rare) Also called Lupus panniculitis. It most commonly presents in association with classic chronic cutaneous lesions of discoid lupus erythematosus. It is a chronic relapsing condition in which painful lesions may heal with significant lipoatrophy and scarring. It involves the subcutaneous tissues. Clinically appears as firm subcutaneous nodules of face, extremitites, trunk, breasts, buttocks. Treatment options include intralesional and systemic steroids, antimalarials, mycophenolate mofetil, dapsone(most effective), cyclosporine, and thalidomide.
- B. Subacute cutaneous LE – LE with widespread photosensitive skin disease, mild systemic involvement, characteristic antibody pattern. Has good prognosis.