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Lupus erythematosus tumidus: Benign disease in children?

Lupus erythematosus tumidus (LET) is a rare subtype of cutaneous lupus erythematosus. It usually occurs on sun-exposed areas of skin and is characterized by erythematous, urticariallike, non-scarring plaque and papules with a tendency to produce annular formations. Most LET patients are negative for antinuclear antibodies (ANA), and the association with systemic lupus erythematosus (SLE) is extremely rare. Although predominant in adults, some pediatric cases have been reported. A 12-year-old girl presented with a 5 day history of symptoms, consisting of non-pruritic skin lesions on the face and upper chest accompanied by daily and high-grade fever. She had no relevant medical or family history. Physical examination indicated a well-appearing girl, with a remarkable rash on the face and upper chest and back characterized by erythematous, edematous and confluent papules and plaques that disappeared with pressure (Fig. 1a,b). Laboratory analysis indicated leukopenia (1,920/lL), and neutropenia (600/lL) confirmed on peripheral blood smear; normocytic anemia (11 g/dL), and normal platelet count (165 000/lL). Coagulation and all routine serum chemistries were normal, except for erythrocyte sedimentation rate (ESR; 50 mm/h). Wide infection screening was negative except for Salmonella typhi Ag O serology with a low titer. The patient received ceftriaxone with no response. At this point, on suspicion of a primary diagnosis of rheumatic disease such as SLE, laboratory studies were extended, and were negative for all antibodies except for immunoglobulin M anti-b2-glycoprotein-I antibodies (32 EU/mL). Finally, punch biopsy of the skin lesions indicated the typical histology of LET (Fig. 1c). Analysis of systemic manifestations to rule out SLE was normal. Given the systemic disease and the possibility of incomplete SLE, oral corticosteroids (1 mg/kg/day) and antimalarials (5 mg/kg/day) were started. The course was initially favorable, with remission of fever with steroids, although it increased again when the dose was reduced. Skin lesions disappeared without scarring, and the neutropenia and leukopenia were partly resolved (3,640/1,620/ lL); ESR control decreased to 24 mm/h. On control laboratory analysis 6 months after diagnosis, significant leukocytosis (31 080/lL) with mild anemia and thrombocytopenia were identified. The blood smear showed blastic cells and markers compatible with acute lymphoblastic leukemia (ALL-B CD10+), which were confirmed on aspirated bone marrow biopsy. The patient received chemotherapy and bone marrow transplantation and did not develop new skin lesions. The patient died 4 years later due to graft-versus-host disease. This clinical report is useful to highline some questions. First, the controversy regarding the diagnostic criteria of LET. Although the specificity of the histological features of LET remains a topic of debate, Kuhn et al. published an extensive review of this subject on the centenary of LET (2009), in which it was concluded that LET should be valued as a specific entity in the classification of cutaneous lesions of lupus erythematosus. In the present case the lesions had the typical characteristics of lupus, both clinically and histologically (perivascular lymphocytic infiltrate and interstitial mucin deposition, as well as minimal vacuolar degeneration of basement membrane). Regarding the systemic manifestations of LET, they are usually absent, although some cases have been reported, including an association with SLE. In most cases ANA is negative, and blood disorders are also infrequent. The current patient presented with fever, leukopenia with neutropenia and anemia, which were attributed to a possible lupus-like syndrome. The present patient was finally diagnosed with ALL. Subacute cutaneous lupus and other connective tissue diseases have been reported as rare paraneoplastic syndromes in connection with lung, breast, gastric, hepatocellular tumors, non-Hodgkin lymphoma, laryngeal, uterine, esophageal adenocarcinoma and cholangiocarcinoma. This is the first case, however, of LET associated with ALL. It is not known whether the hematological disease was present at the beginning of the course, or whether its diagnosis could have been delayed because of the corticosteroid treatment. Hence this case emphasizes that when patients present with LET and systemic manifestations, other Correspondence: Pilar S anchez Y a~nez, MD, Pediatric Intensive Care Unit, Children’s Hospital, M alaga Regional University Hospital, Avenue Arroyo de Los Angeles, M alaga 29011, Spain. Email: pili_sy@hotmail.com Received 22 March 2017; revised 19 September 2017; accepted 27 October 2017. doi: 10.1111/ped.13452 196 P S anchez Y a~nez et al.