Derm Dx: A confluent swollen itchy symmetrical rash on the cheeks and bridge of the nose

Acute cutaneous lupus erythematosus (ACLE) is a manifestation of systemic lupus erythematosus (SLE), a chronic autoimmune disorder of unknown origin that can also affect the joints, kidneys and other organs. SLE ranges from mild to severe and affects nine times...

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Acute cutaneous lupus erythematosus (ACLE) is a manifestation of systemic lupus erythematosus (SLE), a chronic autoimmune disorder of unknown origin that can also affect the joints, kidneys and other organs.

SLE ranges from mild to severe and affects nine times more women than men. Disease onset usually occurs between ages 10 and 50 years.

Pathogenic mechanisms for LE-specific skin disease poorly understood. Humoral autoimmune responses are thought to play a role, as well as environmental stimuli.

Etiologic factors include UV radiation, viral infections, drug and chemical exposure and cigarette smoking.

Signs & Symptoms

SLE symptoms vary from person to person and may affect one organ or body system and then spread to others, waxing and waning with the course of the underlying illness.

Acute cutaneous lupus erythematosus (ACLE) occurs in about 30% to 60% of people with SLE and typically affects the face in a malar or butterfly pattern on the cheeks and bridge of the nose that worsen with sun exposure.

The rash can also be more widespread with exanthematous eruptions focused over extensor aspects of the arms and hands.  ACLE can last anywhere from hours to days to weeks. 

Other symptoms include chest pain with deep breathing, fatigue, unexplained fever, general malaise, mouth sores, hair loss, light sensitivity and swollen lymph nodes.

Perivascular nail-fold erythema, pitting, horizontal and longitudinal ridging, leukonychia, onycholysis, dyschromia and nail bed atrophy have also been observed, as well as telangiectasia.

If the brain and nervous system are involved patients may experience headaches, mild cognitive impairment; numbness, tingling or pain in the arms and legs; personality changes; psychosis; increased risk for stroke; seizures and vision problems.

Diagnosis

At least four typical sings and symptoms must be present for clinicians to make an SLE diagnosis. Clinicians should perform both physical and neurologic exams in patients with suspected SLE.

Other tests that aid in SLE diagnosis include those that detect anti-nuclear antibody, anti-double strand DNA, antiphospholipid antibodies and anti-smith antibodies.

Histopathology may also be useful with the following characteristics common for LE-specific skin conditions: hyperkeratosis, epidermal atrophy, liquefactive/bacuolar basal cell degeneration, dermal-epidermal junction basement membrane thickening, dermal edema, mononuclear cell infiltration of the epidermis, dermal epidermal junction and dermis focused in a perivascular and peri-appendageal distribution.

Treatment

All patients with cutaneous LE should be advised on how to protect their skin from sunlight and artificial UV sources, as well as to avoid potentially photosensitizing drugs including hydrochlorothiazide, griseofulvin and piroxicam (Feldene, Pfizer).

Recommend that patients wear tightly woven clothing, broad brimmed hats and broad-spectrum water-resistant sunscreen with an  SPF ≥15 that contains UVA blockers when outdoors. UB blocking film can be applied to home and automobile windows, and acrylic diffusion shields can be placed over fluorescent lighting.

Systemic immunosuppressive treatment with medications including glucocorticoids, azathioprine and cyclophosphamide used to treat the underlying systemic LE usually helps resolve ACLE lesions, but should be avoided in patients whose LE is limited to the skin only. In these cases, local glucocorticoids such as cobetasol propionate 0.05% and betamethasone dipropionate 0.05% produce the greatest benefits.

The antimalaria drug hydroxychloroquine and low-dose corticosteroids have also been used to treat patients with arthritis and skin symptoms.

Cytoxic drugs are another option for patients who do not respond well to corticosteroids or who are unable to discontinue corticosteroids. Patients with severe or life-threatening symptoms involving the heart, lungs, kidney or central nervous system should be referred to an appropriate specialist.

References

1.     Freedberg IM, Isin AZ, Wolff K, et al. Fitzpatrick’s Dermatology in General Medicine (5^th ed.). 1999. New York: McGraw-Hill.

2.     National Institute of Arthritis and Muskoskeletal and Skin Diseases. Handout on Health: Systemic Lupus Erythematosus. NIH Publication No. 09-4178. Accessed online: June 16, 2011.