Modern Plastic Surgery, 2012, 2, 99-102 Published Online October 2012 ( 99
Primary Cutaneous Diffuse Large B-Cell Lymphoma
(Leg Type) Presenting as Necrotising Fasciitis
Niall M. McInerney*, Kieran T. Power, Alan J. Hussey
Department of Plastic and Reconstructive Surgery, Galway University Hopsital, Galway, Ireland.
Email: *
Received April 27th, 2012; revised May 25th, 2012; accepted June 21st, 2012
Necrotising fasciitis is a rare rapidly progressive, life threatening, soft tissue infection which spreads along fascial
planes. We present a patient who was diagnosed with a primary cutaneous Diffuse Large B-Cell Lymphoma (leg type)
following initial presentation as probable necrotising fasciitis. Presentation was of a painful swollen leg, septic shock
and MRI findings consistent with the clinical sig ns. Diagnosis of ne crotisin g fasciitis remains ch allen ging and it is o ften
missed in the early stages. Early fascial biopsy and histopathological analysis is useful in cases where the diagnosis is
unclear. We feel that this case highlights these important issues and will benefit others in their management of similar
cases in the future.
Keywords: Cutaneous; Lymphoma; Necrotising; Fasciitis
1. Introduction
Necrotising fasciitis is a rare rapidly progressive, life
threatening, soft tissue infection which spreads along
fascial planes [1]. If left untreated it is invariably fatal. A
high index of clinical suspicion is therefore warranted.
There has been a 5-fold increase in incidence over the
past decade, with increasing age being an important risk
factor [2]. It can be caused by group A Streptococcal
infection often incombinatio n with Staphlococcus Aureu s.
It may also be polymicrobial with several aerobic and
anaerobic organisms implicated, including Bacteroides,
Clostridium, Pep tostrep tococcus , Enterobacteriaceae, co-
liforms, Proteus, Pseudomonas, and Klebsiella. Presen-
tation can occur anywhere in the body but the lower limb
is most commonly affected, varying from 32% - 58%
[3,4]. Diagnosis remains challenging and it is often
missed in the early stages. Early fascial biopsy and his-
topathological analysis is useful in cases where the diag-
nosis is unclear. Magnetic Resonanace Imaging (MRI)
may also be useful as it can differentiate between necro-
tising fasciitis and cellulites [5 ].
2. Case Report
A 82-year-old male who was referred a local district gen-
eral hospital with a two week history of pain, redness,
swelling and bruising of his left medial thigh; (Figure 1).
He had failed to respond to standard intravenous antibi-
otic therapy for a presumed diagnosis of cellulitis. Upon
presentation the thigh was very swollen and tense. There
was no ulceration or other skin lesion noted.
Clinically he was in septic shock. His inflammatory
markers were noted to be elevated (WCC 13.3, CRP 202),
with a deteriorating renal function and markedly elevated
lactate levels. Blood gas analysis showed a metaboloic
acidosis. The patient was transferred to the intensive care
unit (ICU) and commenced on intravenous Clindamycin,
Ciprofloxacin and Vancomycin. Tissue for culture failed
to isolate any organism.
MRI scan on the day of admission demonstrated skin
and subcutaneous thickening with oedema of the poste-
rior compartment crossing the fascial planes to the ante-
rior compartment (Figure 1). The muscles of the medial
compartment of the upper leg were preserved but grossly
oedematous (Figure 2). These findings in conjunction
with the history were highly suggestive of a severe in-
flammatory process.
After resuscitation the patient underwent urgent surgi-
cal debridement under general anaesthesia. The intraop-
erative findings revealed overlying necrotic skin and fas-
cia in the medial compartment of the thigh. There was
thrombosis of cutaneous vessels. The underlying muscles
were necrotic and there was extensive, purulent appear-
ing exudate. Samples were sent for histology and micro-
biology. A presumptive diagnosis of necrotising fasciitis
was made.
The patient was returned to ICU where he continued to
*Corresponding a uthor.
Copyright © 2012 SciRes. MPS
Primary Cutaneous Diffuse Large B-Cell Lymphoma (Leg Type) Presenting as Necrotising Fasciitis
Figure 1. Axial MRI image of left thigh showing gross oe-
dema of the posterior compartment crosssing the fascial
planes into the anterior compartment.
Figure 2. Saggital MRI image of the left thigh showing skin
thickening of the left medial thigh with adductor oede ma.
be administered with intravenous antibiotics. He also
required haemodialysis and inotropic support. Further
debridements were undertaken on the third and fifth day.
A negative pressure dressing was applied. Although there
was no clinical evidence of infection, haematological
indices including CRP (325), WBC (21.4) and lactate
dehydrogenase (22 (normal 0.05 - 2.20)) continued to
deteriorate. A Computed Tomography (CT) scan of tho-
rax, abdomen and pelvis showed no other occult sepsis.
On day 15 the wounds were covered with split thickness
skin graft (Figure 3).
A diagnosis of primary cutaneous large B-cell lym-
(a) (b)
Figure 3. (a) Day 2 wound debridement: wound internally
packed with bethadine soaked gauze. (b), (c) Day 14 wound
debridement and split thickness skin grafting. Note a clean
wound with healthy skin edges and significant amount of
muscle resected. Note the extension of the lymphoma over
the patella.
phoma of the leg (PCLBCL-leg) was made after histo-
logical analysis of the fascia and attached adipose tissue
showed diffuse infiltration by atypical lymphoid cells
(Figure 4). The proliferation of lymphoid cells shows
so-called starry-sky appearance with the pres ence of large
histiocytes phagocyting nuclear debris. Mitotic figures
were also present. The atypical lymphoid cells showed
CD20 and BCL6 positivity.
He was referred to the haematology service, but he
continued to deteriorate in ITU and died before chemo-
therapy was commenced.
3. Discussion
Necrotising fasciitis typically presents with early signs of
hot overlying skin, intense erythema, small serous bullae,
and firm induration signifying underlying fascial in-
volvement but may appear as mild cellulites [6]. Connec-
tive tissue and fat is broken down by hyaluronidase and
lipases released by bacteria which allows rapid spread of
infection along fascial planes. Aggressive debridement is
the fundamental principle in the treatment of necrotising
Copyright © 2012 SciRes. MPS
Primary Cutaneous Diffuse Large B-Cell Lymphoma (Leg Type) Presenting as Necrotising Fasciitis 101
(a) (b)
Figure 4. (a) Adipose tissue shows diffuse infiltraion by
lymphoid cells (×10); (b) Atypical lymphoblasts and im-
munoblasts predominate the picture with several mitotic
figures and apoptotic bodies (×40); (c) CD20 immunohisto-
chemistry shows strong positivity confirming B-cell origin
of the lesion.
fasciitis [7]. The va lue of obtaining a fresh frozen b iopsy
is evident not only in terms of improved diagnosis but
also survival [8,9].
Various B-cell lymphomas can present primarily in the
skin without evidence of extracutaneous disease [10].
Primary cutaneous large B-cell lymphomas [PCLBCLs] are
responsible for the majority of these [11]. These lypm-
homas are divided into three main subtypes; primary cu-
taneous marginal zone B-cell lymphoma (PCMZL), pri-
mary cutaneous follicle center-cell lymphomas (PCFCCLs)
and primary cutaneous large B-cell lymphoma of the leg
(PCLBCL-leg). PCLBCL-leg differs by being rapidly
progressive, having a poorer prognosis, higher age of
onset, and expressing bcl-2 protein in the vast majority of
cases [10,12]. The neoplastic B cells also express the
B-cell-associated antigen CD20. PCLBCL-leg develop
extracutaneous disease more frequently and patients have
a 5-year survival of 52% compared with 94% with
PCFCCL [13].
PCLPBL commonly presents with one or both legs
exhibiting a rapidly growing red or bluish tumour (Is it
cutaneous or subcutaneous?). An elevated serum lactate
deydrogenase has been identified in 11.7% of cases [14].
Approximately 25% of patients will develop extracuta-
neous disease at a mean time of 22 months while rarely
lesions may present in a primary location other than the
leg and carry a better prognosis [13,15].
Radiotherapy, chemotherapy and immunotherapy are
the mainstays of treatment [17]. Regimens typically in-
clude an anti-CD20 antibody (rituximab) in comination
with the antracycline based chemotherapy regimen
CHOP (r-CHOP) [15,17,18]. Radiotherapy may also be
considered particularly in presentations of a solitary
small skin tumour. Relapse rates following radiation
have been reported as 58% with extracutaneous progres-
sion rates of 30% [17]. Surgical excision is highly effec-
tive in PCMZL and PCFCCL with a similar efficacy to
radiotherapy, however its efficacy is not well described
in PCLPBL [19].
This case highlights the need for urgent tissue diagno-
sis even when you think the diag nosis is obvio us. Had an
earlier tissue diagnosis been made Initial treatment of
this patient with radiation and/or r-CHOP may have im-
proved his prognosis. The presence of a raised lactate
should also lead one to suspect cutaneous lymphoma. We
therefore recommend that urgent tissue biopsy be per-
formed in all cases of presumed necrotising fasciitis.
[1] E. J. Whallett, J. H. Stevenson and A. D. Wilmshurst,
“Necrotising Fasciitis of the Extremity,” Journal of Plas-
tic, Reconstructive & Aesthetic Surgery, Vol. 63, No. 5,
2010, pp. e469-e473. doi:10.1016/j.bjps.2009.09.011
[2] R. Kaul, A. McGeer, D. E. Low, et al., “Population-
Based Surveillance for Group a Streptococcal Necrotizing
Fasciitis: Clinical Features, Prognostic Indicators, and
Microbiologic Analysis of Seventy-Seven Cases. Ontario
Group A Streptococcal Study,” American Journal of Me-
dicine, Vol. 103, No. 1, 1997, pp. 18-24.
[3] D. A. Anaya and E. P. Dellinger, “Necrotizing Soft-Tis-
sue Infection: Diagnosis and Management,” Clinical In-
fectious Diseases, Vol. 44, No. 5, 2007, pp. 705-710.
[4] B. J. Childers, L. D. Potyondy, R. Nachreiner, et al., “Ne-
crotizing Fasciitis: A Fourteen-Year Retrospective Study
of 163 Consecutive Patients,” The American Journal of
Surgery, Vol. 68, No. 2, 2002, pp. 109-116.
[5] A. Rahmouni, O. Chosidow, D. Mathieu, et al., “MR
Imaging in Acute Infectious Cellulites,” Radiology, Vol.
192, No. 2, 1994, pp. 493-496.
[6] J. D. Urschel, “Necrotiz ing Soft Tissue Infections,” Post-
graduate Medical Journal, Vol. 75, No. 889, 1999, pp.
645-649. doi:10.1136/pgmj.75.889.645
[7] C. H. Wong, A. K. Yam, A. B. Tan, et al., “Approach to
Debridement in Necrotizing Fasciitis,” The American
Journal of Surgery, Vol. 196, No. 3, 2008, pp. e19-e24.
Copyright © 2012 SciRes. MPS
Primary Cutaneous Diffuse Large B-Cell Lymphoma (Leg Type) Presenting as Necrotising Fasciitis
Copyright © 2012 SciRes. MPS
[8] I. Stamenkovic and P. D. Lew, “Early Recognition of
Potentially Fatal Necrotizing Fasciitis. The Use of Fro-
zen-Section Biopsy,” The New England Journal of Medi-
cine, Vol. 310, No. 26, 1984, pp. 1689-1693.
[9] J. Majeski and E. Majeski, “Necrotizing Fasciitis: Im-
proved Survival with Early Recognition by Tissue Biopsy
and Aggressive Surgical Treatment,” Southern Medical
Journal, Vol. 90, No. 11, 1997, pp. 1065-1068.
[10] R. Willemze, H. Kerl, W. Sterry, et al., “EORTC Classi-
fication for Primary Cutaneous Lymphomas: A Proposal
from the Cutaneous Lymphoma Study Group of the
European Organization for Research and Treatment of
Cancer,” Blood, Vol. 90, No. 1, 1997, pp. 354-371.
[11] P. Isaacson and A. Norton, “Cutaneous Lymphomas,” In:
P. G. Isaacson and N. A. Wright, Eds., Extranodal Lym-
phomas, Churchill Livingstone, London, 1994, p. 172.
[12] F. A. Geelen, M. H. Vermeer, C. J. Meijer, et al., “Bcl-2
Protein Expression in Primary Cutaneous Large B-Cell
Lymphoma is Site-Related,” Journal of Clinical Oncol-
ogy, Vol. 16, No. 6, 1998, pp. 2080-2085.
[13] F. Grange, M. W. Bekkenk, J. Wechsler, et al., “Prognos-
tic Factors in Primary Cutaneous Large B-Cell Lympho-
mas: A European Multicenter Study,” Journal of Clinical
Oncology, Vol. 19, No. 16, 2001, pp. 3602-3610.
[14] F. Grange, M. Beylot-Barry, P. Courville, et al., “Primary
Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type:
Clinicopathologic Features and Prognostic Analysis in 60
Cases,” Archives of Dermatology, Vol. 143, No. 9, 2007,
pp. 1144-1150. doi:10.1001/archderm.143.9.1144
[15] R. Willemze, E. S. Jaffe, G. Burg, et al., “WHO-EORTC
Classification for Cutaneous Lymphomas,” Blood, Vol.
105, No. 10, 2005, pp. 3768-3785.
[16] P. L. Zinzani, P. Quaglino, N. Pimpinelli, et al., “Prog-
nostic Factors in Primary Cutaneous B-Cell Lymphoma:
the Italian Study Group for Cutaneous Lymphomas,”
Journal of Clinical Oncology, Vol. 24, No. 9, 2006, pp.
1376-1382. doi:10.1200/JCO.2005.03.6285
[17] N. J. Senff, E. M. Noordijk, Y. H. Kim, et al., “European
Organization for Research and Treatment of Cancer and
International Society for Cutaneous Lymphoma Consen-
sus Recommendations for the Management of Cutaneous
B-Cell Lymphomas,” Blood, Vol. 112, No. 5, 2008, pp.
1600-1609. doi:10.1182/blood-2008-04-152850
[18] B. Dreno, “Standard and New Treatments in Cutaneous
B-Cell Lymphomas,” Journal of Cutaneous Pathology,
Vol. 33, No. S1, 2006, pp. 47-51.
[19] P. Golling, A. Cozzio, R. Dummer, et al., “Primary Cu-
taneous B-Cell Lymphomas—Clinicopathological, Prog-
nostic and Therapeutic Characterisation of 54 Cases ac-
cording to the WHO-EORTC Classification and the
ISCL/EORTC TNM Classification System for Primary
Cutaneous Lymphomas Other than Mycosis Fungoides
and Sezary Syndrome,” Leuk Lymphoma, Vol. 49, No. 6,
2008, pp. 1094-1103. doi:10.1080/10428190802064925