Lupus Erythematosus

Etiology: Heterogeneous autoimmune disease resulting from the interplay of genetic, environmental, and hormonal factors. F > M.

History & Physical: Spectrum of disease varies from limited cutaneous involvement to severe systemic disease.

Systemic Lupus Erythematosus (SLE): 1997 Update of the 1982 ACR Revised Criteria

Management

LE-specific skin manifestations are divided into 3 categories (other less common forms exist as well).

Clinical

Comment

Acute cutaneous LE

Classic "butterfly" malar rash.

Often association with anti-dsDNA Ab and lupus nephritis.

Evaluate for evidence of systemic disease.

Hydroxychloroquine.

Systemic steroids (0.5 mg-1 mg/kg/d) + steroid-sparing agents (azathioprine, methotrexate, mycophe-nolate mophetil).

Subacute cutaneous LE

Two subtypes: Annular or papu-losquamous presentation.

Often associated with anti-Ro Ab.

Sun protection. Corticosteroids (topical, intralesional) and hydroxychloroquine.

Chronic cutaneous/discoid LE

Most often in head/neck area: Atrophic inflammatory plaques.

Can lead to scarring alopecia.

5%-10% will get systemic disease Tx: Photoprotection, topical or intralesional steroids, hydroxychloro-quine

Other important variants of lupus: Drug-induced lupus, lupus profundus, neonatal lupus.

Investigations: Diagnosis requires clinicopathologic correlation;

often multiple skin biopsies needed before diagnosis made. DDx: Eczema, neurodermatitis, parapsoriasis.

Mycosis fungoides (MF)

The most common clinicopathologic subtype of primary cutaneous T-cell lymphoma (CTCL).

Management

Choice of therapy and management setting depends on stage of disease: "stage-directed therapy"

Overview of Mycosis Fungoides Therapies

Skin-directed Therapies

Systemic Therapies

Topical therapies Steroids (mid to strong potency) Topical chemotherapy

■ Nitrogen mustard

■ Carmustine (BCNU) Bexarotene 1% gel Imiquimod 5% cream Phototherapy: PUVA,

BB/NB-UVB, UVA-1 Radiation therapy Local x-ray therapy Electron Beam Therapy

Biological/immune therapies Bexarotene, Acitretin Denileukin

Diftitox Interferon

Extracorporeal photopheresis (ECP) Monoclonal antibodies Cytokine therapy Chemotherapy Methotrexate, gemcitabine

Pentostatin and purine analogues Combination chemotherapy Bone marrow/stem cell transplantation

Melanoma

Images Lupus Profundus

Etiology: Melanocyte-derived skin cancer. May arise within a previously existing nevus or dysplastic nevus, but ~70% arise de novo.

History:

Risk factors

■ Fair complexion: Red/blonde hair, blue/green eyes, tendency to freckle and burn.

■ Sun exposure, particularly blistering sunburns during childhood.

■ Personal or family history of melanoma; Genes involved in some cases: CDKN2A, BRAF.

■ Giant congenital melanocytic nevi or multiple dysplastic nevi.

Physical: Pigmented macule or plaque with some or all of the following features (ABCDE of melanoma): Asymmetry, Borders (irregular), Color variegation, Diameter (>6 mm), Evolution (lesion change by history).

Classically divided into subtypes based on clinical and histopathologic features:

1. Superficial spreading malignant melanoma—60%—70% of melanomas.

2. Nodular melanoma.

3. Acral-lentiginous melanoma — Most common form in blacks, Asians, and Hispanics, mostly on volar skin of the palms or soles and the nailbeds.

4. Lentigo maligna melanoma — Develops from a lentigo maligna, usually on the face of elderly, slow-growing.

5. Amelanotic melanoma — Pink-red.

6. Rare variants.

Most common sites of local and/or regional metastases— Draining lymph node basins and the skin between the primary site and the lymph nodes; most common sites of systemic metastases—Lung, liver, brain (#1 cause of death), bone, and gastrointestinal tract.

Investigations: Dermoscopy (ABCD rule or 7-point checklist; requires expertise); excisional biopsy if melanoma is suspected.

■ Most important prognostic indicator is maximal thickness of tumor invasion on biopsy (Breslow depth in mm).

DDx: BCC, blue or dysplastic nevus, SK.

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