Chronic autoimmune urticaria as the first manifestation of juvenile systemic lupus erythematosus

Abstract

Chronic urticaria (daily or almost daily symptoms lasting for more than six weeks) is characterized by wheals and erythema, with or without itching. A few case reports have shown chronic autoimmune urticaria at the beginning of systemic lupus erythematosus (SLE), particularly in adults. However, the prevalence of this manifestation in a lupus paediatric population was not studied. During 27 consecutive years, 5419 patients were followed up at our University Hospital and 271 (5%) had juvenile SLE (American College of Rheumatology criteria). Two of them (0.7%) had chronic and painless autoimmune urticaria as the first manifestation of juvenile SLE, and were reported herein. One case was a five-year old female with continuous widespread urticaria (duration 120 days), antinuclear antibodies (ANA) 1:640 (dense fine speckled pattern) and elevated complement levels. The juvenile SLE diagnosis was established after one year. The other case was a 13-year old female who had chronic widespread urticaria (lasting 45 days), ANA 1:160 (fine speckled pattern) and normal complement levels. The juvenile SLE diagnosis was established after three years. In conclusion, chronic autoimmune urticaria is very rare and may be the first lupus manifestation, particularly associated with the presence of autoantibodies. This study reinforces the importance of a rigorous follow-up in children and adolescents suffering from autoimmune urticaria due to the possibility of connective tissue disorders, such as paediatric lupus.

Chronic Spontaneous Urticaria: A Survey of 852 Cases of Childhood-Onset Systemic Lupus Erythematosus

“…We describe a low frequency of CSU in a large population of cSLE patients. Of note, the majority of cSLE patients presented this skin manifestation before or at disease onset, as was also observed previously in case reports of adult and cSLE patients [6,8] .…”

“…Few case reports have evaluated urticaria either in adult cSLE patients [6][7][8] . However, the prevalence of this manifestation in the pediatric lupus population and the possible associated factors have not been studied.…”

“…Indeed, a variety of cutaneous manifestations can be observed in lupus patients [1,3,4] , including urticaria [5][6][7][8] . This skin involvement is defined by the sudden onset of wheals, angioedema or both.…”

Impact of chronic urticaria on systemic lupus erythematosus: A nationwide population‐based study in Taiwan

“…Early diagnosis and appropriate treatment involving clinicians across many different specialties are crucial for preventing further manifestations and achieving beneficial long‐term outcomes for patients . Urticaria may present as the initial symptom in these patients before the more classical form of SLE is confirmed . CU‐like lesions may occur in lupus tumidus, urticarial plaque of LE, which is an uncommon LE‐specific but distinct subtype of chronic cutaneous LE…”

Autoimmune urticaria

“…They often suffer additionally from other autoimmune diseases (vitiligo, insulin-dependent diabetes, rheumatoid arthritis, autoimmune hepatitis or autoimmune Hashimoto thyroiditis) [30]. Spadoni et al have reported on cases of urticaria, as the first manifestation of juvenile systemic lupus erythematosus [31]. In terms of treatment, it seems that patients diagnosed with AU need higher doses of antihistamines and/or general modification of therapy, including alternative therapeutic modalities [32,33].…”