Prognosis of Neonatal Childhood and Late Onset Lupus Erythematosus

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Neonatal LE (NLE) may occur in children of anti-Ro/SSA antibody-positive mothers owing to placental passage of these autoantibodies. The clinical characteristics of NLE include cutaneous lupus lesions, congenital heart block, cholestatic liver disease, and thrombocytopenia. Of these, cutaneous lupus and congenital complete heart block are the most common. The prevalence of NLE is not known but is probably at least 1 in 20,000 live births. The skin is affected in approximately half of the cases of

Table 14.3. Prognosis in rare variants of cutaneous lupus erythematosus

LE profundus/panniculitis

Chronic disease course with disfigurement

(Martens et al. 1999, Watanabe

and Tsuchida 1996)


Cutaneous variant without characteristics of

(Kuhn et al. 2000)



Skin is affected in 50%; skin lesions are

(Lee and Weston 1997,

SCLE-like; in most cases, resolution is by

Neimann et al. 2000)

age of 6 months

Childhood LE

(Lo et al. 1999, Wananukul et al. 1998)


Malar rash is frequently found in SLE,

10-year-survival of 65%


Lack of female predominance, low incidence

of photosensitivity, high rate of progression

to systemic disease

Late-onset LE

Reduction of female predominance, cuta-

(Domenech et al. 1992, Fomiga et al. 1999,

neous symptoms less frequent, but progno-

Ho et al. 1998, Ward and Polisson 1989)

sis better than in early-onset LE

NLE. The skin lesions of NLE typically appear during the first few weeks of life, although in a few cases they have been reported to be present at birth. The skin lesions are similar to those of SCLE in adults (Lee and Weston 1997).

The expected outcome of cutaneous NLE is spontaneous resolution by a few months of life. Most babies have resolution of active skin lesions by age 6 months (Lee and Weston 1997). In 57 infants with NLE, mean age at diagnosis of the cutaneous manifestations was 6 weeks, and mean duration was 17 weeks. In 37 infants, the rash resolved without sequelae; 43% of which were untreated. A quarter of the patients had residual sequelae with telangiectasia and dyspigmentation (Neiman et al. 2000). Long-term rheumatologic outcome of children is good in general. However, rare cases of NLE are described with the development of autoimmune disorder in adulthood (Brucato et al. 1997).

CLE of childhood is identical morphologically and histologically to CLE in adults. CLE may occur in association with SLE or in children who have no evidence of internal involvement. In children who have SLE, malar rash of acute CLE is present in 30%-80% (Font et al. 1998, Lehmann 1995, Schaller 1982). Cutaneous manifestations are a prominent finding in children with SLE. In a group of 57 children followed for 6 years, 77% had cutaneous findings, followed by renal involvement (74%). The skin changes noted were malar rash (74%), oral ulcers (46%), vasculitis (42%), photosensitivity (40%), alopecia (32%), and discoid LE (19%). The ANA reaction was positive in 93% of patients, and anti-ds-DNA was positive in 46%. Eight patients died, six of severe infection and two of renal failure (Wanakul et al. 1998). Comparison of the disease course in childhood-onset SLE and adulthood-onset SLE reveals that malar rash, anemia, leukopenia, and anti-ds-DNA antibodies were significantly higher in childhood-onset SLE (Rood et al. 1999). The presence of arthritis, anemia, and seizures at onset of the disease resulted in a 2.6-3.9 times higher chance of a severe disease course (Rood et al. 1999). In a large study of 135 pediatric SLE cases, cumulative 5- and 10-year survival was 80.2% and 65%, respectively, without a sex difference (Lo et al. 1999).

DLE may occur in children who have SLE or as an isolated finding. In a study of 16 children with discoid lesions, it was noted that DLE is similar to its adult counterpart concerning its presentation and chronic course. Important differences are a lack of female predominance, a low incidence of photosensitivity, and a high rate of progression to systemic disease. Five of the 10 children followed into adulthood developed SLE (George and Tunnessen 1993). SCLE has rarely been noted in children (Wanakul et al. 1998).

Lupus profundus/panniculitis is also described in pediatric cases. Its main characteristics are a female predominance and the location of the skin lesions, electively on the face and the lateral aspect of the shoulders. Usually the lesions regress, leaving a characteristic atrophic scar (Bachmeyer et al. 1992).

Onset of SLE in later life, usually defined as after age 50 years, constitutes 6%-18% of the lupus population. Characteristic findings are a reduction of the female predominance from 9:1 in early-onset disease to 6-7:1 in late-onset SLE (Kammer and Mishra 2000, Pu et al. 2000). The interval from onset to diagnosis is usually longer in older patients with LE than in the younger ones. The mean interval between onset of symptoms and signs for diagnosis is approximately 32.5 months (Font et al. 1991, Kammer and Mishra 2000, Maddisin 1987). Racial differences are reported, with a higher proportion of blacks having early-onset LE than late onset (Domenech et al. 1992). Although rash and arthritis/arthralgia remain the most frequent signs in both early- and late-onset SLE, there is an increase in the frequency of interstitial pneu-monitis, serositis, and hematocytopenias in late-onset LE (Kammer and Mishra 2000). In contrast, malar rash, livedo reticularis, alopecia, photosensitivity, oral and nasal ulcers, glomerulonephritis, and lymphadenopathy have a lower prevalence in late-onset compared with early-onset SLE (Domenech et al. 1992, Kammer and Mishra 2000, Ward and Polisson 1989). Late-onset LE has been associated with a milder clinical course and a better prognosis compared with earlier onset (Formiga et al. 1999, Ho et al. 1998, Koh and Boey 1994, Shaikh and Wang 1995). However, comparative retrospective and prospective studies have provided conflicting data on the pattern of presentation and the relationship of serologic abnormalities and disease expression (Baker et al. 1979, Kammer and Mishra 2000, Mak et al. 1998).

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