Jpn. J. Clin. Immunol.

, 34 (1) 4952 (2011) 2011 The Japan Society for Clinical Immunology 49

Case report

A case of WeberChristian disease with later development of rheumatoid arthritis

Yuki NANKE1, Naoko ISHIGURO2, Toru YAGO1, Tsuyoshi KOBASHIGAWA1,

Michiko KAWAKAMI, Makoto KAWASHIMA and Shigeru KOTAKE

2 2 1

1Institute of Rheumatology, Tokyo Women's Medical University

2Department of Dermatology Tokyo Women's Medical University

(Received October 12, 2010)

summary

WeberChristian disease (WCD) is a syndrome characterized by recurrent subcutaneous nodules, fever, occasional
lipoatrophy, fatigue, arthralgia, and myalgia. We report a case of WCD associated with rheumatoid arthritis. A 65
yearold woman consulted our outpatient clinic because of bilateral hand swelling. The patient had presented with fever
and subcutaneous nodules in her trunk and upper and lower extremities in 1983. At that time, the dermatology depart-
ment diagnosed this patient as having WCD after biopsy of the nodules demonstrated lobular panniculitis. She has been
treated with corticosteroid (515 mg/day) since then. The patient continued to have recurrent episodes of transient in-
ammatory arthritis in the small joints of the ngers and fever, and was initially assessed at our institution in October
2007. Finally, in November 2007, she was diagnosed as having both WCD and rheumatoid arthritis (RA) and treated
with corticosteroid (5 mg/day) and methotrexate (MTX) (7.5 mg/week). Thereafter, her clinical symptoms gradually
improved. This is the second case of WCD showing the subsequent development of RA, successfully treated with MTX,
in the English literature. This case may provide clinical insight into WCD and RA.

Key wordsWeberChristian disease (WCD); panniculitis; rheumatoid arthritis; collagen disease

Introduction Case

Weber Christian disease (WCD) was rst
described in German by Pfeifer1) in 1892. Weber2)
reported a case of relapsing nonsuppurative pannicu-
litis showing phagocytosis of the subcutaneous fat
cells by macrophages in 1925, while Christian3)
reported relapsing febrile nodular nonsuppurative
panniculitis in 1928. Thereafter, Baily4) reported
relapsing febrile nodular nonsuppurative panniculitis
as WCD in JAMA in 1937. WCD is a syndrome
characterized by recurrent subcutaneous nodules,
fever, occasional lipoatrophy, fatigue, arthralgia, and
myalgia5). Biopsy of the nodules demonstrates lobu-
lar panniculitis. The prognosis is variable6), but that
of lobular panniculitis associated with prominent
visceral involvement may eventually be poor.
Rheumatoid arthritis (RA) is a chronic inamma-
tory disease and involves the joint. It can cause syno-
vitis and progressive joint destruction. However, the
association of WCD showing subsequent develop-
ment of RA has not previously been described in En-
glish reports. Here, we describe the rst case, which
may provides clinical insight into WCD and RA.
1Institute of Rheumatology, Tokyo Women's Medical
University.
In December 2007, a 65 yearold woman consulted
our outpatient clinic because of bilateral hand swell-
ing. The patient had demonstrated fever and sub-
cutaneous nodules in her trunk and upper and lower
extremities in 1983. Physical examination at the
Department of Dermatology demonstrated multiple,
tender, nger tipsized nodules on both lower ex-
tremities, both forearms, the abdomen and buttocks,
and the formation of skin depressions on the both
upper arms. She was diagnosed as having WCD by
biopsy of the nodules, which showed degeneration of
fat cells, and predominantly lymphocytic inltrations
with histiocytes and plasma cells in the fat lobules
(Figure 1). She has been treated with corticosteroid
(515 mg/day) since 1983 and remained free of sym-
ptoms over the next few years. In 2007, the patient de-
veloped recurrent episodes of transient inammatory
arthritis involving the PIP and MTP joints of the
ngers accompanied by fever. Despite treatment with
corticosteroid (10 mg/day) by the dermatology
department, serum C reactive protein (CRP) grad-
ually became elevated to 2 mg/dl and ANA increased
a serum dilution of 1 : 160. She was initially assessed
at our institution in October 2007. On admission, her
temperature was 36 C. There were no subcutaneous
erythematous tender nodules. She complained of
 50 (Vol. 34 No. 1)

Figure 1. Histopathological staining of nodules (hematoxy-
lineosin, original magnication 40, 100).
Degeneration of fat cells, and predominantly lymphocytic in-
ltration with histiocytes and plasma cells in the fat lobules.
Figure 2. The photo of upper extremities (taken in February
2010)
Lipoatrophy of the forearms and swelling of the wrists and
ngers were seen in the bilateral extremities.
Figure 3. XP of the hands showed erosive changes and nar-
rowing of the joint spaces in February 2010.
morning stiness that lasted for half a day. In the
past, she had not shown Raynaud's phenomenon.
There was no palpable lymphoadenopathy or
hepatosplenomegaly. In both lower lung elds, ne
crackle was audible. Laboratory data were as fol-
lows : WBC 8700/mL (neutrophils 78.4, lympho-
cytes 16.2, monocytes 4.7), Hb 12.2 g/dl, PLT
30.9104/mL, and CRP 2.26 mg/dl. Urinalysis did
not demonstrate any protein on dipstick. KL 6 was
326 U/ml. Renal and liver functions were normal.
Immunological tests demonstrated immunoglobulin
(Ig) G, IgA and IgM were 1602, 141 and 175 mg/dl,
respectively. Rheumatoid factor was 68 IU/ml.
Anticyclic citrullinated peptide antibody was 100
U/ml. Matrix metalloproteinase (MMP) 3 was 122.6
ng/ml. Antinuclear antibody was 1 : 320 (speckled,
nucleolar). Both anti SS A antibody and anti SS B
antibody were negative. Anti RNP antibody was 1
index. Anti Topo1 antibody was negative. CH 50,
41.4 U/ml ; C3, 120 mg/d and C4, 33 mg/dl. Chest
 NANKEWeberChristian and rheumatoid arthritis
CT demonstrated honeycombing change in the
bilateral lower lung elds and SPO2 was 96. ECG
was within normal limits. X P of the hands and feet
showed periarticular osteoporosis, but there were no
erosive changes. Although anti RNP antibody was
weakly positive, she did not satisfy the criteria for
other collagen diseases such as mixed connective
tissue disease.
In November 2007, nally, she was diagnosed as
having WCD, interstitial pneumonia and RA and
treated with corticosteroid (5 mg/day) and MTX
(7.5 mg/week) at our outpatient clinic. Thereafter,
CRP and hand swelling gradually decreased. Arthritis
was seen in both wrists and lipoatrophy was seen in
both upper extremities (Figure 2). X P of the hands
showed erosive changes and narrowing of the joint
spaces in February 2010 (Figure 3).
Discussion
Here, we present a case of WCD associated with
RA. Biopsy of the nodules demonstrates lobular pan-
niculitis. The present case satised WCD's criteria.
Rehman et al7) reported a patient with WCD who
demonstrated polyarthritis. The patient showed
recurrent episodes of transient inammatory arthritis
involving the ankles, knees, shoulders, elbows and
small joints of the hands, resulting in signicant joint
damage. Radiographs of the knees demonstrated evi-
dence of signicant periosteitis. Magnetic resonance
image scans of both knees showed bilateral femoral
lesions characteristic of multifocal bone infarcts and
extensive inammatory change within the subcutane-
ous tissue of both the thighs and knees. Since fat
necrosis is an important component of WCD, Reh-
man et al. considered that polyarthritis in that case
was secondary to periarticular and intraarticular fat
necrosis. The osteolytic bone lesions seen in WCD are
also thought to be secondary to medullary fat necro-
sis. The patient in their report nally became wheel-
chair bound.
Yamamoto et al8). also reported a case showing
osteoarthropathy associated with WCD. The patient
developed pain in the left knee and right ankle 13
months after onset of WCD. The left distal femur and
the right distal tibia showed cortical hyperostosis with
periosteal reaction in radiographs. T1 weighted
magnetic resonance (MRI) images demonstrated an
irregularly thickened cortex with hypointensity, while
T2 weighted MR images showed inhomogeneous
hyperintensity in the bone medulla. The patient un-
derwent open biopsy and specimens from the left
51
femoral bone marrow showed an admixture of fat
necrosis and scattered chronic inammatory cells.
In WCD, acute and chronic arthritis and periarthri-
tis predominantly aect the ankles and knees. Radio-
graphs of the aected bones may demonstrate diuse
osteolysis, endosteal scalloping, and mild cortical
hyperostosis911). In this case, radiological ndings in
the hands demonstrated periarticular osteoporosis,
erosions and clinical synovitis. She complained of
morning stiness that persisted for half a day. Anticy-
clic citrullinated peptide antibody was 100 U/ml
and MMP 3 was 122.6 ng/ml. Based on these data,
arthropathy in the current case was associated with
RA. Taken together, our patient was diagnosed as
having WCD and RA.
While we were preparing the current manuscript,
Pongratz et al12) reported a patient with RA who was
diagnosed as having WCD during immunosuppres-
sive therapy. A biopsy from a painful subcutaneous
nodule demonstrated lobular panniculitis compatible
with WCD. Thus, contrary to the course shown in our
case, WCD developed subsequent to RA in their
patient.
Iwasaki et al13) reported a patient with WCD show-
ing increased serum levels of soluble IL 2 receptor,
IFN g, IL 6, IL 4 and IL 10, who was successfully
treated with cyclosporine A. Hojo et al14) also report-
ed a patient with WCD associated with myelodysplas-
tic syndrome, in whom the levels of soluble IL 2
receptor, IFN g, IL 1b, IL 6 and tumor necrosis
factor a were elevated during the active state and
then returned to normal after prednisolone therapy.
Pongratz et al12) reported a patient with WCD suc-
cessfully treated with cyclosporine A. T cell immune
response may be involved in the pathogenesis of
WCD.
Conclusion
We described herein a case of WCD associated with
RA. This is the second reported case of WCD show-
ing subsequent development of RA, successfully
treated with MTX, providing clinical insight into
WCD and RA.
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pannicultis showing phagocytosis of subcutane-
ous fat cells by macrophages. Br J Dermatol
 52 (Vol. 34 No. 1)

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