Pediatric Dermatology Vol. 18 No.

4 313±315, 2001

Urticaria Pigmentosa Associated

with Wilms Tumor
Elif GuÈler, M.D., Suna Emir, M.D., Tezer Kutluk, M.D., Ph.D., Ali Varan, M.D.,
and MuÈnevver BuÈyuÈkpamukcËu, M.D.

Department of Pediatric Oncology, Hacettepe University Institute of Oncology, Ankara, Turkey

Abstract: Urticaria pigmentosa is the most common manifestation of

mastocytosis, with the majority of cases undergoing spontaneous reso-
lution, especially in children. Several reports have documented hemato-
logic malignancies developing in patients with urticaria pigmentosa. We
present a 4.5-year-old boy with urticaria pigmentosa who developed
Wilms tumor. To our knowledge, coexisting urticaria pigmentosa and
Wilms tumor have not previously been described.

Urticaria pigmentosa is the most common form of
mastocytosis. It occurs in 90% of patients with indolent
mastocytosis, but its frequency is less than 50% of
patients with systemic mastocytosis. Urticaria pigmen-
tosa is characterized by yellowish tan to red-brown ma-
culopapular or nodular lesions mainly distributed on the
trunk and limbs (1). There have been several reports of
malignancies in patients with urticaria pigmentosa (2,3);
however, this association is rare in childhood. We des-
cribe a patient in whom urticaria pigmentosa coexisted
with Wilms tumor.
CASE REPORT
A 4.5-year-old boy was admitted to our department
with the complaints of an abdominal mass and
maculopapular rash. By history, he had the maculo-
papular rash since infancy. On physical examination,
his vital ®ndings were normal and there was an
abdominal mass 7 cm ´ 7 cm in diameter, located in
the left upper quadrant. A widespread, yellow-brown
maculopapular rash was distributed over his trunk and
extremities (Fig. 1). Darier sign was positive. There
were no symptoms or signs of histamine release such as
¯ushing, abdominal pain, diarrhea, hepatomegaly,
splenomegaly, vasomotor instability, or itching. His
laboratory values were hemoglobin 12 g/dl, WBC
10,000/mm3, and di€erential counts normal. Abdom-
inal ultrasonography and computed tomography (CT)
showed a solid tumoral mass, 62 mm ´ 68 mm in
diameter, arising from the medial part of the left
kidney. Chest radiograph was normal. The left kidney
and mass were removed surgically and were diagnosed
as Wilms tumor. Histopathologic examination con-
®rmed the diagnosis. A skin biopsy specimen was taken
and the pathologic diagnosis was urticaria pigmentosa.
A chemotherapy regimen consisting of actinomycin D
and vincristine was started for the treatment of the
Wilms tumor. Ketotifen was given for the urticaria
pigmentosa. His malignant disease is still in remission,
although there was no change in his urticaria pigmen-
tosa during a 6-month follow-up period.
DISCUSSION
Mast cell disease has a wide clinical spectrum, ranging
from isolated cutaneous involvement to systemic disease.
Systemic mastocytosis, de®ned as mast cell in®ltration of

Address correspondence to Tezer Kutluk, M.D., Department of

Pediatric Oncology, Hacettepe University Institute of Oncology,
06100 Ankara, Turkey, or e-mail: tk06-k@tr-net.

313
314 Pediatric Dermatology Vol. 18 No. 4 July/August 2001
extracutaneous tissue with or without skin involvement,
is rare in children. The proliferating mast cells in the
tissues produce systemic symptoms of histamine liber-
ation such as diarrhea, ¯ushing, vomiting, and vascular
instability. The most common form of mastocytosis in
children is urticaria pigmentosa. The lesions are yellow-
ish tan to red-brown maculopapules or nodules and the
size may vary from a few millimeters to several centim-
eters. They may be present at birth, but frequently erupt
during the ®rst 2 years of life, although they may appear
at any time (1).
The association of hematologic malignancies with
systemic mastocytosis has been well documented (4±6).
These disorders most often involve dysplasia or neo-
plasm of the myeloid cells. Travis et al. (6) described
hematologic malignancies in 13 of 60 adult patients with
systemic mastocytosis. Eight of these patients had acute
nonlymphoblastic leukemia, three patients had lym-
phoma, one had dysmyelopoietic syndrome, and one had
Figure 1. (A,B) Widespread yellow-
brown maculopapular rash of urti-
caria pigmentosa distributed over
the trunk and extremities.
chronic myelomonocytic leukemia. Horny et al. (7)
found hematologic malignancies including myeloprolif-
erative disorder, myelodysplasia, and mast cell leukemia
in 25 of 61 adult patients with systemic mastocytosis.
There are only two reported cases of urticaria pig-
mentosa and hematologic malignancy in children, an
8-year-old child who developed leukemia 3 months
after diagnosis of systemic mastocytosis, and an
11-year-old girl with systemic mastocytosis who died of
acute lymphoblastic lymphoma. Both also had systemic
mastocytosis (8,9).
The association of malignancies with isolated urti-
caria pigmentosa is more rare than that with systemic
mastocytosis (2,3). Cooper et al. (3) documented six
patients with urticaria pigmentosa who developed a
hematologic malignancy. None of them had systemic
mastocytosis. However, there were no reports of coex-
isting isolated urticaria pigmentosa with lymphoprolif-
erative malignancy in children.
 GuÈler et al: Urticaria Pigmentosa Associated with Wilms Tumor
The association of systemic mastocytosis with solid
tumors is more rare than that with hematologic malig-
nancies. The types of solid tumors in adults associated
with systemic mastocytosis include breast carcinoma,
gastric carcinoma, bladder carcinoma, pancreatic ade-
nocarcinoma, pleural mesothelioma, small cell carci-
noma of the lung, and brain tumor (6,10). On the other
hand, there are no reports of the association of systemic
mastocytosis or urticaria pigmentosa with solid tumors
in children. This is the ®rst case of urticaria pigmentosa
associated with a malignant disease in our hospital
records of 8000 patients with lymphoma and solid
tumors for the last 30 years. The absence of systemic
symptoms and signs in our patient rules out the diag-
nosis of the systemic form of the disease. There is
no evidence whether or not this association is coinci-
dental. It is suggested that mastocytosis is a hemato-
poietic stem cell disorder and the factors secreted by
mast cells may have a role on the development of
malignant disease by inducing proliferation and angio-
genesis (11,12). Although there does not seem to be an
increased risk of developing malignancy in patients with
systemic mastocytosis, both solid and hematologic
malignancies may adversely a€ect the prognosis of these
patients (6).
Disodium cromoglycate, ketotifen, and hydroxyzine
have been used for treatment of mastocytosis (13,14) and
topical steroids are advised for the treatment of urticaria
pigmentosa, but the lesions recur after discontinuation of
therapy (15). We have not observed any change in the
urticaria pigmentosa lesions in our patient during his
chemotherapy. It has been reported that cytoreductive
therapy produces no signi®cant modi®cation in the
number and appearance of mast cell disease lesions (3).
For children with urticaria pigmentosa, the prognosis is
good and spontaneous resolution occurs in about 50% of
patients by puberty; another 25% have partial resolution
by adulthood (1).
In conclusion, patients who have isolated urticaria
pigmentosa or systemic mastocytosis should be evalu-
ated and followed for the possibility of solid and hema-
tologic malignancies since the real risk is not known. We
believe that anticancer treatment should not be modi®ed
in patients with isolated urticaria pigmentosa since we
315
have not observed any serious complications of urticaria
pigmentosa produced by anticancer therapy. Pediatri-
cians and dermatologists should collaborate on the
management of this rare association.
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