Medication Summary
Neonatal lupus erythematosus (NLE) does not require specific therapy. Some research suggests that infants from subsequent pregnancies are less likely to be affected by cardiac NLE if the mother is treated with prednisolone, dexamethasone, or betamethasone.
Strict photoprotection is an essential component of therapy for the treatment of cutaneous lupus erythematosus (LE) in children and adults, as well as NLE in infants. Sunscreens do not block all wavelengths of light, and spectrum coverage for various sunscreens differs; therefore, the preparation and characteristics of a specific sunscreen must be considered prior to delivering recommendations. In adult studies, the wavelengths of light responsible for induction of cutaneous LE are within the ultraviolet (UV)-B and UV-A range. Unfortunately, many sunscreens are labeled for UV-B protection only with the sun-protection factor (SPF). Only sunscreens labeled with the term “broad spectrum” provide UV-A and UV-B coverage.
Patients and parents should be advised to apply sunscreen well before exposure and to use a sunscreen with a high SPF, broad-spectrum (UV-A) coverage, and water resistance. Reapplication after several hours is necessary. Encourage behavior modification of UV avoidance. This is particularly important in infants with NLE as sunscreens are not typically recommended at this young age.
In children with cutaneous LE, topical and/or intralesional corticosteroids are often considered first-line therapy, and data support the use of topical calcineurin inhibitors as topical steroid-sparing agents in children as well. [42] For patients with cutaneous LE and/or systemic LE (SLE), antimalarial medications, such as hydroxychloroquine and chloroquine, are considered first-line systemic therapy, bearing in mind that any patient treated with antimalarials should have regular ophthalmologic examinations. [43] In patients refractory to topicals and antimalarial therapy, systemic corticosteroids or steroid-sparing agents, such as methotrexate, azathioprine, mycophenolate mofetil, cyclophosphamide, thalidomide, oral dapsone, and rituximab may be considered. [44, 45, 46, 47, 48, 49] In such cases, therapeutic decisions depend on disease severity and specific manifestations, such as renal involvement, along with thorough consideration of risk-profiles.
A general management plan of pregnancy in mothers with SLE includes treatment of disease flares using drugs that are effective but also safe for the fetus. [50] Such an approach may diminish or reduce the prevalence of complete heart block associated with NLE. Given that the use of hydroxychloroquine for anti-Ro/SSA positive mothers with SLE has been associated with a lower rate of NLE during pregnancy, this therapy is now frequently used in patients with SLE during pregnancy, particularly in mothers who have previously had an infant with NLE. Corticosteroids and some immunosuppressive drugs are sometimes used, but long-term outcome data in children exposed to immunosuppressive drugs in utero are lacking.
Topical corticosteroids
Class Summary
Topical corticosteroid agents are used to control cutaneous lesions. Select a specific agent based on the treatment site and type of lesion. Facial skin is more prone to atrophy than skin of the scalp or hands; therefore, use a weaker topical corticosteroid on the face. Thick lesions may require more potent agents. In addition, treat hair-bearing areas with a lotion, gel, or foam instead of a cream or ointment.
Hydrocortisone topical (Ala-Cort, Beta-HC, Westcort)
Topical hydrocortisones are lower potency topical steroids (0.5%, 1%, 2.5%) that are useful on the face and intertriginous areas. These agents have mineralocorticoid and glucocorticoid effects that result in anti-inflammatory activity.
Triamcinolone topical (Oralone, Zyloptic, Triderm, Kenalog)
Topical triamcinolone decreases inflammation by suppressing migration of polymorphonuclear lymphocytes (PMNs) and reversing capillary permeability. This agent is a moderate-potency topical steroid available in ointment (0.1%) and cream (0.1%, 0.5%) formulations.
Clobetasol propionate (Temovate, Blobex, Olux)
Clobetasol is a superpotent topical steroid that decreases inflammation by suppressing migration of polymorphonuclear lymphocytes (PMNs) and reversing capillary permeability.
Betamethasone dipropionate (Celestone, Luxiq, Diprolene)
Betamethasone is a superpotent topical steroid that decreases inflammation by suppressing migration of polymorphonuclear lymphocytes (PMNs) and reversing capillary permeability.
Immunomodulators
Class Summary
Patients with immune dysregulation and autoimmunity often benefit from immune modulation. Immunomodulators are useful in patients with skin disease that is unresponsive to topical agents and in patients with arthritis that does not respond to nonsteroidal anti-inflammatory drugs (NSAID)s. These drugs are not needed in neonatal lupus erythematosus but may be used in children with skin or joint disease of systemic lupus erythematosus (SLE).
Hydroxychloroquine (Plaquenil)
Hydroxychloroquine inhibits chemotaxis of eosinophils and locomotion of neutrophils as well as impairs complement-dependent antigen-antibody reactions.
Hydroxychloroquine sulfate 200 mg is equivalent to 155 mg hydroxychloroquine base and 250 mg chloroquine phosphate.
Corticosteroids
Class Summary
Corticosteroids are useful in adults or children with renal, central nervous system (CNS), or severe hematologic disease associated with systemic lupus erythematosus (SLE). These agents are rarely needed in neonatal lupus erythematosus, but they may be used in patients with severe hepatitis or thrombocytopenia.
Prednisone
Prednisone may decrease inflammation by reversing increased capillary permeability and suppressing polymorphonuclear lymphocyte (PMN) activity.
Non-steroid topical immunosuppressants
Class Summary
Nonsteroidal topical immunosuppressive agents may be useful in postpubertal children with cutaneous lupus erythematosus (CLE). Note that prolonged use of topical corticosteroids may produce significant skin adnexal atrophy. If needed, nonsteroidal topical immunosuppressive agents may be used intermittently and short term as second-line therapy.
Pimecrolimus (Elidel)
Pimecrolimus is a second-line agent for short-term and intermittent treatment in patients whose conditions are unresponsive to or who are intolerant of other treatments. This agent is a topical calcineurin inhibitor derived from ascomycin, a natural substance produced by the fungus Streptomyces hygroscopicus var ascomyceticus.
Pimecrolimus penetrates inflamed epidermis to inhibit T-cell activation by blocking transcription of proinflammatory cytokine genes such as interleukin (IL)-2, interferon gamma (T helper cell type 1 [Th1]), IL-4, and IL-10 (Th2-type). This agent also blocks the catalytic function of calcineurin and prevents the release of inflammatory cytokines and mediators from mast cells in vitro after stimulation by antigen/IgE (immunoglobulin E).
Pimecrolimus selectively inhibits production and release of inflammatory cytokines from activated T cells by binding to cytosolic immunophilin receptor macrophilin-12. The resulting complex inhibits phosphatase calcineurin, thus blocking T-cell activation and cytokine release. Cutaneous atrophy was not observed in clinical trials, which is a potential advantage over topical corticosteroids.
Tacrolimus (Protopic)
Tacrolimus is a second-line agent for short-term and intermittent treatment in patients whose condition is unresponsive to or who are intolerant of other treatments. This agent is a topical calcineurin inhibitor that penetrates the inflamed epidermis to inhibit T-cell activation by blocking transcription of proinflammatory cytokine genes such as interleukin (IL)-2, interferon gamma (T helper cell type 1 [Th1]), IL-4, and IL-10 (Th2-type).
Tacrolimus blocks the catalytic function of calcineurin and prevents the release of inflammatory cytokines and mediators from mast cells in vitro after stimulation by antigen/IgE (immunoglobulin E). This agent also inhibits transcription for genes that encode IL-3, IL-4, IL-5, granulocyte-macrophage colony-stimulating factor (GM-CSF), and tumor necrosis factor (TNF)-alpha, all of which are involved in the early stages of T-cell activation. Cutaneous atrophy was not observed in clinical trials, which is a potential advantage over topical corticosteroids.
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Neonatal lupus erythematosus.
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This child presented with petechial lesions, hepatosplenomegaly, and thrombocytopenia. Initially, he was thought to have histiocytosis (Letterer-Siwe disease); however, a skin biopsy revealed an interface dermatitis, and his mother had circulating autoantibodies.
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This child was one of a pair of fraternal twins. Her sibling was not affected, although the mother and both infants had similar autoantibodies in their circulations. Eventually, the lesions seen here resolved and healed without sequelae.
