Lupus erythematosus tumidus: Benign disease in children?
Angel Vera Casa
Antonio Urda Cardona
Pediatric Intensive Care Unit,
Pediatric Rheumatology Unit,
Pediatric Dermatology Unit and
Pediatric UGC. Children’s
Hospital. University Regional Hospital of M
Key words child, cutaneous, leukemia, lupus erythematosus, lupus tumidus.
Lupus erythematosus tumidus (LET) is a rare subtype of cuta-
neous lupus erythematosus. It usually occurs on sun-exposed
areas of skin and is characterized by erythematous, urticarial-
like, non-scarring plaque and papules with a tendency to pro-
duce annular formations. Most LET patients are negative for
antinuclear antibodies (ANA), and the association with sys-
temic lupus erythematosus (SLE) is extremely rare. Although
predominant in adults, some pediatric cases have been
A 12-year-old girl presented with a 5 day history of symp-
toms, 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 examina-
tion indicated a well-appearing girl, with a remarkable rash on
the face and upper chest and back characterized by erythema-
tous, edematous and conﬂuent papules and plaques that disap-
peared 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 sus-
picion 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-gly-
coprotein-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 incom-
plete 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 labora-
tory 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 speci-
ﬁc entity in the classiﬁcation of cutaneous lesions of lupus
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
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
The present patient was ﬁnally diagnosed with ALL. Suba-
cute 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
This is the ﬁrst case, however, of LET
associated with ALL. It is not known whether the hematologi-
cal 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
nez, MD, Pediatric Intensive
Care Unit, Children’s Hospital, M
alaga Regional University
Hospital, Avenue Arroyo de Los
alaga 29011, Spain.
Received 22 March 2017; revised 19 September 2017; accepted
27 October 2017.
196 P S
nez et al.
© 2018 Japan Pediatric Society