Dimitrina Guleva MD, Lyubomir Dourmishev MD, PhD, Ivan Botev MD, Lyubka Miteva MD, PhD Department of Dermatology and Venereology, Medical University of Sofia, Sofia, Bulgaria

Keywords: Lupus erythematosus tumidus; Childhood; Beta thalassemia minor; Antimalarials

Dear Editor,

A 9-year-old boy was consulted regarding an erythematous rash on the face that appeared 6 months ago. The patient, his mother and sister had concomitant β-thalassemia minor with a laboratory established anemia. Clinical examination revealed oval infiltrated erythematous plaques located on the central part of his face (Figure 1) and ears. Laboratory tests were within normal ranges, except for low hemoglobin Genetic diagnosis levels. Antinuclear, anti-dsDNA, anti-Sm, anti-SSA and anti-SSB antibodies were negative. Several conducted phototests were also negative. Histopathology showed discreet vacuolar degeneration of the epidermal basal layer, perivascular and periadnexal lymphoid infiltrate and mucin in dermis (Figure 2). Based on the available clinical and laboratory findings, the diagnosis lupus erythrematosus tumidus (LET) was confirmed.A treatment with systemic corticosteroids was initiated, but shortly discontinued because of the side effects. Regarding the low grade of anemia, we took the risk and initiated treatment with 3mg/kg hydroxychloroquine which resulted in a complete clinical remission. Regular ophthalmological examinations and blood tests were performed every 4 months. No side effects were observed. The patient discontinued his therapy for a year causing a relapse (Figure 3). The antimalarials were reinitiated again resulting in a rapid improvement. Lupus erythematosus tumidus is a variant of lupus erythematosus described by Hoffmann in 1909.1 LET is reported MK-8617 molecular weight to have an increased photosensitivity, which was not established in our case.2 LET affects mostly adults and it is rare in children.

In 2003 Sonntag et al. reported 3 pediatric cases and Aguirre et al. added 2 cases of children with LET all treated with antimalarials.3,4 Lesions of LET may co-exist with other variants of lupus erythematosus and clinicians should be always aware of scomorbidities in pediatric patients.Beta-thalassemia anemia has to be distinguished from hematological manifestations of systemic lupus erythematosus. Limited data about the correlation between lupus erythematosus and β-thalassemia are available in literature.5 Beta-thalassemia is a genetic hemoglobinopathy which causes hemolytic anemia when exposed to oxidative stress.Treatment options in LET are similar Protein Conjugation and Labeling in children and adults. Photoprotection and family education are crucial.6 Topical steroids and calcineurin inhibitors are poorly tolerated. The therapy with antimalarials, such as hydroxychloroquine, can induce hemolytic crises in patients with hemoglobinopathies. 7,8 Few data about the lupus therapy with antimalarial drugs in children are available but there is not enough information about its prescription in patients with comorbidities, especially haemopaths.3,4,9 Our experience showed that hydroxychloroquine has excellent clinical response and a good tolerance from pediatric patients, although β-thalassemia is a relative contraindication for it.