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Proven Lupus Treatment By Dr Gary Levin

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SYSTEMIC LUPUS ERYTHEMATOSUS Clinical Presentation:

■ Chronic, systemic autoimmune disease with cutaneous, renal, joint involvement, and serositis.

■ Patients may also develop neurologic manifestations secondary to circulating anticardiolipin ("lupus anticoagulant") antibodies, which can precipitate thromboembolic events.

■ Typical cutaneous manifestations include: "butterfly" malar erythema; photosensitivity; erythematous, scaly plaques between joints on dorsal fingers and on sun-exposed skin; periungual telangiectases and Raynaud's phenomenon.

■ Less commonly, lesions may include purpura, bullae, ulcers, discoid lesions, scarring alopecia, and chilblains (perniosis).

Histology:

■ Vacuolar alteration of basal keratinocytes (Fig. 3A)

■ Perivascular lymphocytic infiltrate with obscuration of the dermoepidermal junction (Fig. 3B)

■ Altered basement membrane

■ Leukocytoclastic vasculitis may be present

Immunofluorescence: Direct Immunofluorescence:

■ Band-like staining pattern for IgG or IgM at dermoepider-mal junction of lesional and nonlesional, sun-exposed skin (the so-called "lupus band")

Pathophysiology:

■ Deregulated T lymphocytes activate B cells to produce pathogenic autoantibodies and cause immune complex deposition, which result in tissue damage and vascular injury

■ The etiology of this immune deregulation is unknown, but ultraviolet light (UVA and UVB), as well as genetic predisposition are thought to have some role in the pathogenesis of systemic lupus erythematosus.

■ Some drugs (e.g., procainamide, hydralazine, isoniazid, minocycline, and hydrochlorothiazide) may precipitate a lupus-like syndrome that slowly resolves with discontinuation of the drug.

References:

1. Clark WH, Reed RJ, Mihm MC. Lupus erythematosus. Histo-pathology of cutaneous lesions. Hum Pathol 1973; 4:157-163.

2. Al-Suwaid AR, Venkataram MN, Bhushnurmath SR. Cutaneous lupus erythematosus: comparison of direct immunofluorescence findings with histopathology. Int J Dermatol 1995; 34:480-482.

DERMATOMYOSITIS Clinical Presentation:

■ Systemic autoimmune disease characterized by dermatitis +/ — a nonsuppurative polymyositis. May occur in childhood or adulthood

■ Adult dermatomyositis is associated with an increased incidence of malignancy

■ Typical cutaneous findings include: violaceous, edematous patches on periorbital skin ("heliotrope" rash), erythema-tous, slightly scaly or poikilodermatous patches on shoulders ("shawl" sign), violaceous papules on bony prominences (Gottron's papules), periungual telangiectases, and photosensitivity

Histology:

■ Epidermal atrophy

■ Vacuolar alteration of basal keratinocytes (Fig. 3C)

■ Perivascular lymphocytic infiltrate with obscuration of the dermoepidermal junction (Fig. 3C)

■ Abundant mucin in the interstitial dermis (Fig. 3D)

Differential Diagnosis:

Dermatomyositis

Systemic Lupus Erythematosus

Negative lupus band test

Positive lupus band test

Pathophysiology:

■ Deregulated T lymphocytes activate B cells to produce pathogenic autoantibodies and cause immune complex deposition, which result in cutaneous and muscle damage, and microvascular injury

References:

1. Janis JF, Winklemann RK. Histopathology of the skin in derma-tomyositis. Arch Dermatol 1968; 97:640-650.

2. Magro CM, Crowson AN. The immunofluorescent profile of der-matomyositis: a comparative study with lupus erythematosus. J Cutan Pathol 1997; 24(9):543-552.

LICHEN SCLEROSUS AND ATROPHICUS Clinical Presentation:

■ Chronic inflammatory dermatosis, which typically presents as pruritic, ivory-to-white macules coalescing into patches with comedo-like plugs and a wrinkled surface occurring primarily on the anogenital region in middle-aged women.

■ Occurs rarely in men and children

■ Twenty percent of cases are extragenital

■ Genital lesions occasionally are associated with squa-mous cell carcinoma

Histology:

■ Vacuolar alteration of basal keratinocytes

■ Edema and/or sclerosis of the superficial dermis (Figs. 4A-C)

■ Patchy, lymphocytic infiltrate below the zone of altered collagen (Fig. 4D)

■ Thinned epidermis with effacement of the rete ridge pattern (epidermal atrophy)

■ Plugged follicular units

Differential Diagnosis:

Systemic Lupus

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