The n e w e ng l a n d j o u r na l of
Case Records of the Massachusetts General Hospital
From the Departments of Dermatology
(M.R.Y.), Radiology (L.A.R.), Surgery
(P.J.F.), and Pathology (M.P.H.), Massa‑
chusetts General Hospital, and the De‑
partments of Dermatology (M.R.Y.), Ra‑
diology (L.A.R.), Surgery (P.J.F.), and
Pathology (M.P.H.), Harvard Medical
School — both in Boston.
N Engl J Med 2020;383:1664-71.
DOI: 10.1056/NEJMcpc1916257
Copyright © 2020 Massachusetts Medical Society.
1664
Downloaded from nejm.org at UNIVERSITY LIBRARY UTRECHT on October 21, 2020. For personal use only. No other uses without permission.
Copyright © 2020 Massachusetts Medical Society. All rights reserved.
m e dic i n e
Founded by Richard C. Cabot
Eric S. Rosenberg, M.D., Editor
Virginia M. Pierce, M.D., David M. Dudzinski, M.D., Meridale V. Baggett, M.D.,
Dennis C. Sgroi, M.D., Jo‑Anne O. Shepard, M.D., Associate Editors
Kathy M. Tran, M.D., Assistant Editor
Matthew B. Roberts, M.B., B.S., Case Records Editorial Fellow
Emily K. McDonald, Tara Corpuz, Production Editors
Case 33-2020: A 55-Year-Old Man
with Abdominal Pain, Joint Swelling,
and Skin Lesions
Mariko R. Yasuda, M.D., Lauren A. Roller, M.D., Peter J. Fagenholz, M.D.,
and Mai P. Hoang, M.D.​​
Pr e sen tat ion of C a se
Dr. Kristin M. D’Silva (Medicine): A 55-year-old man was transferred to this hospital
from a rehabilitation facility because of abdominal pain and painful skin lesions.
One year before the current presentation, the patient received a diagnosis of
pancreatitis at another hospital after magnetic resonance cholangiopancreatogra-
phy (MRCP) performed for the evaluation of chronic abdominal pain revealed
pancreatic necrosis and peripancreatic fat stranding involving the pancreatic head
and uncinate process. Blood levels of triglycerides and IgG subclasses were report-
edly normal, and the patient had not been drinking alcohol; the pancreatitis was
attributed to bevacizumab, which had been administered for the treatment of
neurofibromatosis. Bevacizumab therapy was stopped for 2 months. After bevaciz­
umab therapy was restarted, elevated blood lipase levels recurred without any ab-
dominal symptoms, and bevacizumab therapy was discontinued.
Seven months before the current presentation, another MRCP showed pancre-
atic ductal dilatation and a pancreatic pseudocyst with internal calcification. Endo-
scopic ultrasonography revealed changes consistent with chronic pancreatitis and
a pancreatic pseudocyst. The patient was advised to continue to avoid alcohol and
fatty foods. Blood lipase levels returned to normal.
Three and half months before the current presentation, pain in the ankles de-
veloped. The patient reported that the pain was worse after prolonged rest and that
his ankles felt stiff for 1 hour after he awoke in the morning. Two weeks later,
pain, redness, and swelling in the left lower leg developed, with a central fluctuant
nodule on an erythematous base (Fig. 1A). Over the next 2 weeks, swelling and
redness of the left fifth toe and the right first metacarpophalangeal joint devel-
oped (Fig. 1B and 1C). The patient was evaluated by his primary care physician.
Range of motion was limited in the ankles, which had overlying soft-tissue swell-
n engl j med 383;17 nejm.org October 22, 2020
The New England Journal of Medicine
 Case Records of the Massachuset ts Gener al Hospital

A B

C D E

Figure 1. Clinical Photographs.

Three months before the current presentation, the patient presented with erythema and swelling in the left lower
leg, with a central fluctuant nodule on an erythematous base (Panel A). Ten weeks before the current presentation,
erythema and swelling of the left fifth toe (Panel B) and right first metacarpophalangeal joint (Panel C) developed.
During the current evaluation, blanching, erythematous, indurated plaques on the medial aspect of the right knee
(Panel D) and the lateral left calf (Panel E) were seen.

ing and tenderness on palpation. There was also
erythema, tenderness, and soft-tissue swelling
of the right fourth metacarpophalangeal joint,
the left second and third metacarpophalangeal
joints, and the right fourth proximal interpha-
langeal joint of the hand. The erythrocyte sedi-
mentation rate was 76 mm per hour (reference
range, 0 to 15), and the C-reactive protein level
was 102 mg per liter (reference range, 0 to 8).
Blood levels of calcium, uric acid, angiotensin-
converting enzyme, rheumatoid factor, cyclic
citrullinated peptide, streptolysin, and DNase
were normal; tests for antinuclear antibodies
and antineutrophil cytoplasmic antibodies were
negative. A chest radiograph was normal. Empiri-
cal treatment with cephalexin was administered.
After several days, the patient’s condition had
not improved, and he was evaluated by a rheu-
matologist. He received a diagnosis of possible
Löfgren’s syndrome, and prednisone was pre-
scribed. Over the next 2 weeks, there was a de-
crease in the swelling and pain in the left fifth

n engl j med 383;17 nejm.org October 22, 2020 1665

The New England Journal of Medicine
Downloaded from nejm.org at UNIVERSITY LIBRARY UTRECHT on October 21, 2020. For personal use only. No other uses without permission.
Copyright © 2020 Massachusetts Medical Society. All rights reserved.
 The n e w e ng l a n d j o u r na l of m e dic i n e

toe and right first metacarpophalangeal joint

A
but not in the ankle pain or the skin lesion in
the left leg. Pain in the ankles worsened, was
minimally alleviated with the administration of
oxycodone, and limited the patient’s ability to
walk. The dose of prednisone was increased, and
colchicine was administered, with a decrease in
the ankle pain and resolution of the skin lesion
in the left leg.
Five weeks before the current presentation,
the patient was admitted to a second hospital
with septic and hemorrhagic shock in the con-
text of infected pancreatic necrosis and bleeding
from a gastroduodenal artery pseudoaneurysm. B
Dr. Lauren A. Roller: Computed tomographic (CT)
angiography of the abdomen and pelvis revealed
multiple peripancreatic fluid collections and ir-
regularity of the branch vessels of the celiac and
superior mesenteric arteries, including the gas-
troduodenal artery, adjacent to areas of pancre-
atic necrosis and inflammation (Fig. 2A and 2B).
Dr. D’Silva: Percutaneous drainage of the pan-
creatic necrosis and angioembolization of the
gastroduodenal artery pseudoaneurysm were
performed. On the fourth hospital day, the pa-
tient was transferred to this hospital for further
treatment. C
At this hospital, five intraabdominal drainage
catheters were placed, with a decrease in the size
of the multifocal fluid collections in the abdo-
men. Cultures of intraabdominal fluid grew
Pantoea agglomerans (formerly Enterobacter agglom-
erans), abiotrophia species (formerly nutritionally
variant streptococci), and lactobacillus species.
The patient completed a course of piperacillin in
combination with tazobactam and was dis-
charged from this hospital to a rehabilitation Figure 2. CT Scans of the Abdomen.
facility on the 24th hospital day. Axial images obtained 5 weeks before the current pre‑
Seven days after discharge, periumbilical and sentation (Panels A and B) show extensive pancreatic
right lower abdominal pain developed, and the necrosis and multiple large peripancreatic fluid collec‑
patient returned to the emergency department tions (Panel A, white arrows), with a percutaneous drain‑
of this hospital for evaluation. There was no fe- age catheter placed in the dominant collection (Panel A,
dashed arrow). Vessel irregularity and probable pseudo‑
ver, nausea, vomiting, or joint pain. He reported aneurysm involving the gastroduodenal artery (Panel B,
5 days of painful skin lesions and swelling of the arrowhead) are adjacent to areas of pancreatic necrosis.
right leg. An axial image obtained on the current presentation
Other medical history included hypertension, (Panel C) shows a slight decrease in the size of necrotic
neurofibromatosis type 2, gastroesophageal re- peripancreatic fluid collections (white arrows), with a
percutaneous drainage catheter in place (dashed arrow).
flux disease, sensorineural hearing loss, benign
prostatic hypertrophy, and anxiety. There was no
history of sinusitis, inflammatory eye disease, ependymoma, meningiomas, and vestibular
neuropathy, or testicular disease. Neurofibroma- schwannomas and had been treated with bevaciz-
tosis had been complicated by cervical spine umab for 8 months before the diagnosis of pan-

1666 n engl j med 383;17 nejm.org October 22, 2020

The New England Journal of Medicine

Downloaded from nejm.org at UNIVERSITY LIBRARY UTRECHT on October 21, 2020. For personal use only. No other uses without permission.
Copyright © 2020 Massachusetts Medical Society. All rights reserved.
 Case Records of the Massachuset ts Gener al Hospital

creatitis. Medications included aspirin, gabapentin, Table 1. Laboratory Data.

hydrochlorothiazide, lorazepam, losartan, meto-
prolol, omeprazole, oxycodone, pancrelipase, Reference Range,
Variable Adults* On Admission
prednisone, tamsulosin, and tolterodine. There
was a history of heavy alcohol use, but the patient Hematocrit (%) 41–53 25.3
had stopped drinking alcohol 3 years before this Hemoglobin (g/dl) 13.5–17.5 7.6
presentation. There was a remote history of to- White-cell count (per μl) 4500–11,000 12,450
bacco use and no history of illicit drug use. He Differential count (per μl)
lived with his mother in a suburb of Boston.
Neutrophils 1800–7700 11,329.5
The temperature was 37.3°C, the blood pres-
Lymphocytes 1000–4800 435.75
sure 107/68 mm Hg, the pulse 118 beats per
minute, the respiratory rate 20 breaths per min- Monocytes 200–1200 435.75
ute, and the oxygen saturation 98% while the Eosinophils 0–900 0
patient was breathing ambient air. The weight Basophils 0–300 0
was 73.6 kg, and the body-mass index (the Platelet count (per μl) 150,000–400,000 395,000
weight in kilograms divided by the square of the Red-cell count (per μl) 4,500,000– 2,680,000
height in meters) 22. The abdomen was soft and 5,900,000
nontender, with five percutaneous drainage Mean corpuscular volume (fl) 80–100 94.4
catheters in place. There was pitting edema in
Mean corpuscular hemoglobin (pg) 26–34 28.4
the right foot. A blanching, erythematous, indu-
Mean corpuscular hemoglobin con‑ 31–37 30
rated plaque (5 cm in diameter) with superficial centration (g/dl)
scale was present on the medial aspect of the
Lipase (U/liter) 13–60 5338
right knee (Fig. 1D); a similar lesion (1 cm in
diameter) was present on the superior aspect of Total protein (g/dl) 6.0–8.3 5.3
the right knee. There were two blanching, ery- Albumin (g/dl) 3.3–5.0 2.3
thematous, thin plaques (1 cm in diameter) on
* Reference values are affected by many variables, including the patient popu­
the left lateral calf (Fig. 1E). There were several lation and the laboratory methods used. The ranges used at Massachusetts
nontender, hyperpigmented patches on the low- General Hospital are for adults who are not pregnant and do not have medi‑
er legs that were consistent with postinflamma- cal conditions that could affect the results. They may therefore not be appro‑
priate for all patients.
tory hyperpigmentation. There was pain on pal-
pation and diffuse erythema and warmth of the
right lower calf. The remainder of the physical Differ en t i a l Di agnosis
examination was normal.
Blood levels of electrolytes and glucose were Dr. Mariko R. Yasuda: This 55-year-old man with
normal, as were results of kidney- and liver- chronic pancreatitis complicated by infected pan-
function tests. Other laboratory test results are creatic necrosis presented with skin lesions at
shown in Table 1. two discrete time points. The first skin lesion,
Dr. Roller: CT of the abdomen and pelvis, per- which occurred 3 months before the current
formed after the administration of intravenous admission, was a fluctuant nodule on an ery-
contrast material, revealed a slight decrease in thematous base on the distal leg that developed
the size of multiple necrotic peripancreatic fluid in the context of ankle and metacarpal pain. The
collections, with drainage catheters in place lesions that were present on the current admis-
(Fig. 2C). There was evidence of previous embo- sion were different, consisting of blanching, ery-
lization of the gastroduodenal artery without thematous, indurated plaques with superficial
evidence of residual or new pseudoaneurysm. scale on the knee, with blanching, erythema-
Duplex ultrasonography of the right leg showed tous, thin plaques on the calf and several hyper-
nonocclusive thrombosis in the distal right femo- pigmented patches on the lower legs.
ral vein. Although these lesions have different mor-
Dr. D’Silva: A specimen of fluid from the larg- phologic features, the fact that the second le-
est abdominal collection and a specimen of blood sions occurred within 3 months after the first
were obtained for culture; ceftriaxone was ad- lesion suggests that they are part of the same
ministered. A diagnostic test was performed. cutaneous process but are in different stages of

n engl j med 383;17 nejm.org October 22, 2020 1667

The New England Journal of Medicine
Downloaded from nejm.org at UNIVERSITY LIBRARY UTRECHT on October 21, 2020. For personal use only. No other uses without permission.
Copyright © 2020 Massachusetts Medical Society. All rights reserved.
 The n e w e ng l a n d j o u r na l of m e dic i n e

evolution — the fluctuant nodule on a red base
represents the acute phase and the plaques with
fine scale and hyperpigmented patches represent
a chronic or resolving process. I will therefore
focus my differential diagnosis on causes of red
nodules on the legs, including vasculitis, cancer,
infection, and panniculitis.
Vasculitis
Could this patient have cutaneous vasculitis?
Vasculitis involving small or medium-sized ves-
sels can have dermatologic manifestations.1 Typ-
ically, the size of the skin lesion correlates with
the size of the involved vessel.2 Small-vessel
vasculitis, such as Henoch–Schönlein purpura,
typically manifests as crops of small, palpable
purpura.1 The size of this patient’s lesions is
more consistent with medium-vessel vasculitis,
such as polyarteritis nodosa. However, if this
patient were to have medium-vessel vasculitis, I
would expect to see punched-out ulcers or le-
sions that are consistent with livedo reticularis.1
In addition, this patient’s normal renal function,
normal chest radiograph, and negative test for
antineutrophil cytoplasmic antibodies make vas-
culitis unlikely.
Cancer
Cancer can mimic inflammatory skin disorders.
Subcutaneous panniculitis-like T-cell lymphoma
is a rare primary cutaneous lymphoma of mature
cytotoxic T cells, and the disease typically follows
an indolent course. Lesions are usually single or
multiple nodules and plaques on the legs. How-
ever, the disease most commonly occurs in middle-
aged women with coexisting conditions, such as
systemic lupus erythematosus, multiple sclero-
sis, or hypothyroidism.3 Furthermore, the nod-
ules are usually painless. Thus, the diagnosis is
unlikely in this case.
Primary cutaneous gamma/delta T-cell lym-
phoma often causes necrotizing plaques and
tumors or red, scaly plaques that mimic mycosis
fungoides. However, patients with primary cuta-
neous gamma/delta T-cell lymphoma typically
have a more aggressive course than that seen in
this patient, and I would not expect the skin le- Panniculitis
sions to resolve spontaneously.4-6
Infection
Could this patient have cellulitis? Patients with Löfgren’s syndrome is characterized by a classic
cellulitis typically have acute, unilateral, diffuse, triad of erythema nodosum, hilar lymphadenopa-
poorly demarcated, tender erythema.7 This pa-
tient had bilateral, tender nodules that persisted
even after he received a course of cephalexin
early in the disease process, as well as ceftriax-
one later during his illness; thus, uncomplicated
cellulitis is an unlikely diagnosis in this case.
Fungal infections are an important consider-
ation in this patient, who was immunocompro-
mised owing to chronic poor health and predni-
sone use. Invasive candidiasis is the leading
cause of mycosis-associated death in the United
States.8 This patient’s risk factors for invasive
candidiasis include intraperitoneal drainage
catheters, gastrointestinal infection, and recent
bevacizumab use. However, typical dermatologic
manifestations of invasive candidiasis are ec-
thyma gangrenosum–like lesions, oral thrush,
and a folliculitis-like eruption. In addition, cul-
tures of intraabdominal fluid obtained from this
patient did not grow any candida species. Over-
all, systemic candidiasis is unlikely.
The risk of primary cutaneous aspergillosis
increases with prednisone use and can occur at
venipuncture sites,8 but this patient did not have
a history of venipuncture at the lesion sites or of
local trauma. Cutaneous manifestations of sys-
temic aspergillosis vary and can include subcu-
taneous nodules, necrotic papulonodules, pur-
puric nodules, and eschars.8 Mucormycosis has
a predilection for the arms and legs and for sites
of local trauma. Cutaneous manifestations of
systemic mucormycosis also vary and can in-
clude papules, pustules, vesicles, and hemor-
rhagic bullae.8 If this patient’s skin lesions were
due to aspergillosis or mucormycosis, I would
expect the number of lesions to increase quickly
and the disease to spread rapidly in the context
of ongoing prednisone use. Because of the vari-
able cutaneous manifestations and high mortal-
ity associated with angioinvasive fungi, I would
maintain a high degree of suspicion until a biopsy
of the skin or other affected tissue was per-
formed and histopathological and microbiologic
evaluation could reliably rule out aspergillosis
and mucormycosis.
When this patient initially presented with cuta-
neous lesions and joint pain, prednisone therapy
was started for possible Löfgren’s syndrome.

1668 n engl j med 383;17 nejm.org October 22, 2020

The New England Journal of Medicine

Downloaded from nejm.org at UNIVERSITY LIBRARY UTRECHT on October 21, 2020. For personal use only. No other uses without permission.
Copyright © 2020 Massachusetts Medical Society. All rights reserved.
 Case Records of the Massachuset ts Gener al Hospital

thy, and acute polyarthritis, although diagnosis

A
of this condition does not require all three clini-
cal findings.9 This patient had a normal chest
radiograph, without evidence of hilar lymphade-
nopathy. Moreover, his initial lesion, a fluctuant
nodule on a red base, was not consistent with
classic erythema nodosum, a panniculitis that
typically manifests as multiple symmetric, tender
subcutaneous nodules without bullae or ulcer-
ation.9
Could this patient, with elevated levels of in-
flammatory markers and joint pain, have lupus
panniculitis? Lupus panniculitis often precedes
chronic cutaneous (discoid) lupus or systemic B
lupus.10 However, lupus panniculitis is most
common in women and is characterized by ten-
der subcutaneous nodules and plaques on the
face, proximal arms, and trunk but not on the
legs.10 This patient’s skin lesions were confined
to the legs, which makes lupus panniculitis un-
likely.
Another cause of panniculitis to consider in
this patient is pancreatic panniculitis. Patients
with pancreatic panniculitis have tender, some-
times fluctuant subcutaneous nodules that com-
monly involve the legs. The nodules can ulcerate,
Figure 3. Skin-Biopsy Specimen.
releasing oily, thick, yellowish-brown material
Hematoxylin and eosin staining shows lobular pannicu‑
indicative of necrotic adipose tissue.11 Other fea-
litis affecting the subcutaneous tissue (Panel A), with
tures that can be observed in patients with pan- fat necrosis and saponification, which are characterized
creatic panniculitis include pancreatic pseudo- by anucleate and basophilic adipocytes (Panel B, arrows).
cysts and polyarthritis, both of which are
features of this patient’s presentation.10 Exami-
nation of a skin-biopsy specimen would reveal and polyarthritis syndrome, I would perform a
fat saponification by calcium salts, with homo- skin biopsy.
geneous basophilic material or “ghost cells” —
a histologic feature that distinguishes pancreatic Dr . M a r iko R . Y a suda’s Di agnosis
panniculitis from other panniculitides.10 Treat-
ment of the underlying pancreatic disease can Pancreatitis, panniculitis, and polyarthritis syn-
result in resolution of cutaneous lesions. drome.
I think the most likely diagnosis in this pa-
tient is pancreatitis, panniculitis, and polyarthri- Pathol o gic a l Discussion
tis syndrome. This rare syndrome, which is de-
scribed mainly in case reports, is thought to be Dr. Mai P. Hoang: A punch biopsy of the skin on the
due to elevated pancreatic enzymes in the blood medial aspect of the right knee was performed.
that lead to fat necrosis particularly affecting the Histopathological examination of the specimen
subcutaneous adipose tissue, joints, and bones.10,12 revealed lobular panniculitis with marked fat
It is most common in middle-aged men with necrosis and saponification (Fig. 3A). Anucleate
alcohol use disorder.12 Patients with this syn- and necrotic adipocytes with basophilic cyto-
drome have the cutaneous clinical and histo- plasm, which are features of coagulative fat ne-
pathological features of pancreatic panniculitis. crosis, were seen (Fig. 3B). These findings are
To establish the diagnosis of pancreatic pannicu- most consistent with pancreatic panniculitis.
litis, and specifically of pancreatitis, panniculitis, In patients with pancreatic panniculitis, the

n engl j med 383;17 nejm.org October 22, 2020 1669

The New England Journal of Medicine
Downloaded from nejm.org at UNIVERSITY LIBRARY UTRECHT on October 21, 2020. For personal use only. No other uses without permission.
Copyright © 2020 Massachusetts Medical Society. All rights reserved.
 The n e w e ng l a n d j o u r na l
necrotizing inflammation in the subcutaneous
fat is probably a result of adipocyte necrosis due
to systemic release of pancreatic enzymes (lipase,
amylase, phosphorylase, and trypsin) and subse-
quent neutrophilic infiltration. Dystrophic calci-
fication may be present. Chronic changes can
include granulomatous inflammation and hemo-
siderin deposition. Fibrosis and lipoatrophy can
occur late in the disease process.11
Discussion of M a nagemen t
Dr. Peter J. Fagenholz: When this patient presented
to the other hospital just before the current
evaluation, his pancreatic necrosis had probably
been present for months. He was symptomatic,
and a CT scan showed that the pancreatic necro-
sis was walled off and contained gas; these fea-
tures are diagnostic of infection. Therefore,
mechanical intervention was indicated.
When mechanical intervention is undertaken
for infected walled-off necrosis, a step-up ap-
proach is generally used, in which a minimally
invasive percutaneous or endoscopic drainage
procedure is performed first, and then necrosec-
tomy is performed if the patient’s condition does
not improve after initial drainage.13,14 The choice
of initial drainage procedure is primarily based
on the site of the necrosis. Areas of walled-off
necrosis that abut the stomach are usually treated
with endoscopic drainage into the stomach, al-
though a surgical transgastric approach can have
benefits in selected patients.15 The primary ad-
vantage of a transgastric approach, as compared
with techniques involving external drainage, is
the low rate of pancreatic fistula formation.15,16
In this patient, only one of three discrete ar-
eas of walled-off necrosis would have been ac-
cessible with a transgastric approach. Although
the decision to perform percutaneous drainage
was made at another hospital, I agreed with this
initial management strategy, given the necrosis
site. Hemorrhage that developed from branches
of the gastroduodenal artery was managed with
angioembolization. In patients with necrotizing
pancreatitis, visceral arterial hemorrhage results
from blood-vessel degradation due to prolonged
exposure to pancreatic enzymes and infection,
and it is best managed with angioembolization,
since direct surgical control is difficult and asso-
ciated with a high risk of complications.
1670 n engl j med 383;17
The New England Journal of Medicine
Downloaded from nejm.org at UNIVERSITY LIBRARY UTRECHT on October 21, 2020. For personal use only. No other uses without permission.
Copyright © 2020 Massachusetts Medical Society. All rights reserved.
of m e dic i n e
Of patients who are initially treated with per-
cutaneous drainage, 60 to 70% will require “step-
up” to some form of necrosectomy to eradicate
necrosis and facilitate recovery.14 In this patient,
initial drainage alleviated his symptoms and
resolved his septic shock, and he had radiologi-
cally significant improvement in the size of the
necrotic fluid collections. Given these improve-
ments and his state of severe malnutrition and
decondition, he was treated with drainage, anti-
biotics, and enteral nutritional support and was
discharged to a rehabilitation facility.
When the patient was readmitted to this
hospital with abdominal pain and fever due to
persistent necrosis, his nutritional and func-
tional status was markedly improved, and we
thought he would benefit from necrosectomy.
There are no established criteria regarding when
to perform necrosectomy; the decision is indi-
vidualized, depending on clinical and radio-
logic progress. Because this patient had recur-
rent fever and abdominal pain after weeks of
steady progress and had no further diminution
in the volume of walled-off necrosis after ini-
tial drainage, the decision to perform necro-
sectomy was made.
Once the decision is made, the next step is to
determine which technique to use. In this pa-
tient, the technique was largely determined by
the initial approach to drainage, which was in
turn defined by the necrosis site. Percutaneous
drainage had been performed through a combi-
nation of transperitoneal and retroperitoneal
routes into three discrete areas of walled-off
necrosis. One area had resolved completely after
initial drainage. The remaining fluid collections
had been accessed through relatively narrow in-
tercostal and transperitoneal windows. We per-
formed necrosectomy by means of sinus-tract
endoscopy, in which the drainage tract was di-
lated in the operating room under fluoroscopic
guidance to allow for introduction of an operat-
ing scope into the necrotic cavities and removal
of the necrosis through the tract. Dilation tech-
niques are ideal for the treatment of multifocal
walled-off necrosis that can be accessed through
small windows and transperitoneal windows,
because the risk of damage to surrounding struc-
tures and incisional complications is minimized.17,18
In this patient, as in most patients with multifo-
cal walled-off necrosis, we performed débride-
nejm.org October 22, 2020
 Case Records of the Massachuset ts Gener al Hospital

ment of the fluid collections in separate proce-
dures, so if complications did occur, we would
be able to localize them easily.
On postoperative day 15, the patient was dis-
charged to a rehabilitation facility, and on post-
operative day 53, he returned home. The last
drainage catheter was removed on postoperative
day 86. At his most recent follow-up visit, his
weight had returned to his baseline level, and
the albumin level was normal.
Dr. D’Silva: After the necrosectomy procedures,
the patient was treated with a 2-month tapering
course of prednisone, with a starting dose of 20
mg daily. The skin lesions and joint pain re-
solved completely. Two months later, recurrent
pain and swelling in the left knee developed.
Arthrocentesis yielded 10 ml of viscous, brown
fluid. Owing to the viscosity, we were unable to
obtain a cell count; microbiologic cultures were
negative. The patient received an intraarticular
injection of glucocorticoids, which resulted in
alleviation of his symptoms. He has had no fur-
ther skin lesions or joint pain.
Fina l Di agnosis
Pancreatitis, panniculitis, and polyarthritis syn-
drome.
This case was presented at the Medicine Case Conference.
No potential conflict of interest relevant to this article was
reported.
Disclosure forms provided by the authors are available at
NEJM.org.
We thank Dr. Daniela Kroshinsky and Dr. Eli Miloslavsky for
their assistance with the case history.

References
1. Fiorentino DF. Cutaneous vasculitis.
J Am Acad Dermatol 2003;​48:​311-40.
2. Carlson JA. The histological assess-
ment of cutaneous vasculitis. Histopa-
thology 2010;​56:​3-23.
3. López-Lerma I, Peñate Y, Gallardo F,
et al. Subcutaneous panniculitis-like T-cell
lymphoma: clinical features, therapeutic
approach, and outcome in a case series of
16 patients. J Am Acad Dermatol 2018;​79:​
892-8.
4. Merrill ED, Agbay R, Miranda RN,
et al. Primary cutaneous T-cell lympho-
mas showing gamma-delta (γδ) pheno-
type and predominantly epidermotropic
pattern are clinicopathologically distinct
from classic primary cutaneous γδ T-cell
lymphomas. Am J Surg Pathol 2017;​41:​
204-15.
5. Rubio-Gonzalez B, Zain J, Garcia L,
Rosen ST, Querfeld C. Cutaneous gamma-
delta T-cell lymphoma successfully treat-
ed with brentuximab vedotin. JAMA Der-
matol 2016;​152:​1388-90.
6. Guitart J, Weisenburger DD, Subtil A,
et al. Cutaneous γδ T-cell lymphomas:
a spectrum of presentations with overlap
with other cytotoxic lymphomas. Am J
Surg Pathol 2012;​36:​1656-65.
7. Raff AB, Kroshinsky D. Cellulitis:
a review. JAMA 2016;​316:​325-37.
8. Shields BE, Rosenbach M, Brown-Joel
Z, Berger AP, Ford BA, Wanat KA. Angio-
invasive fungal infections impacting the
skin: background, epidemiology, and clini-
cal presentation. J Am Acad Dermatol
2019;​80(4):​869.e5-880.e5.
9. Haimovic A, Sanchez M, Judson MA,
Prystowsky S. Sarcoidosis: a comprehen-
sive review and update for the dermatolo-
gist. I. Cutaneous disease. J Am Acad Der-
matol 2012;​66(5):​699.e1-699.e18, 717-8.
10. Patterson JW. Panniculitis. In:​Bolog-
nia J, ed. Dermatology. 2nd ed. Maryland
Heights, MO:​Mosby, 2008:​1515-37.
11. Dahl PR, Su WP, Cullimore KC, Dicken
CH. Pancreatic panniculitis. J Am Acad
Dermatol 1995;​33:​413-7.
12. Dieker W, Derer J, Henzler T, et al.
Pancreatitis, panniculitis and polyar-
thritis (PPP-) syndrome caused by post-
pancreatitis pseudocyst with mesenteric
fistula: diagnosis and successful surgi-
cal treatment: case report and review of
literature. Int J Surg Case Rep 2017;​31:​
170-5.
13. Working Group IAP/APA Acute Pan-
creatitis Guidelines. IAP/APA evidence-
based guidelines for the management of
acute pancreatitis. Pancreatology 2013;​
13(4):​Suppl 2:​e1-e15.
14. van Santvoort HC, Besselink MG,
Bakker OJ, et al. A step-up approach or
open necrosectomy for necrotizing pan-
creatitis. N Engl J Med 2010;​362:​1491-502.
15. van Brunschot S, van Grinsven J, van
Santvoort HC, et al. Endoscopic or surgi-
cal step-up approach for infected necro-
tising pancreatitis: a multicentre random­
ised trial. Lancet 2018;​391:​51-8.
16. Driedger M, Zyromski NJ, Visser BC,
et al. Surgical transgastric necrosectomy
for necrotizing pancreatitis: a single-stage
procedure for walled-off pancreatic necro-
sis. Ann Surg 2020;​271:​163-8.
17. Fong ZV, Fagenholz PJ. Minimally in-
vasive debridement for infected pancreat-
ic necrosis. J Gastrointest Surg 2019;​23:​
185-91.
18. Luckhurst CM, El Hechi M, Elsharkawy
AE, et al. Improved mortality in necrotiz-
ing pancreatitis with a multidisciplinary
minimally invasive step-up approach: com-
parison with a modern open necrosecto-
my cohort. J Am Coll Surg 2020;​230:​873-
83.
Copyright © 2020 Massachusetts Medical Society.

n engl j med 383;17 nejm.org October 22, 2020 1671

The New England Journal of Medicine
Downloaded from nejm.org at UNIVERSITY LIBRARY UTRECHT on October 21, 2020. For personal use only. No other uses without permission.
Copyright © 2020 Massachusetts Medical Society. All rights reserved.