A Practical Approach to Panniculitis

Transcription

A Practical Approach to Panniculitis
2 0 0 7
www.dermatologyrounds.ca
DERMATOLOGY
AS
Rounds
A Practical Approach to Panniculitis
By SUSAN M POELMAN, MD, DENIS SASSE VILLE, MD
Vo l u m e 6 ,
TM
I s s u e
5
PRESENTED IN THE ROUNDS OF
THE
D IVISION
OF
D ERMATOLOGY,
®
M C G ILL U NIVERSITY H EALTH C ENTRE
Members of the
Division of Dermatology
Denis Sasseville, MD, Director
Editor, Dermatology Rounds
Panniculitis has always been a difficult topic in dermatology, mainly due to the diverse
spectrum of disorders that may present as inflammation of subcutaneous fat. Traditionally,
most textbooks classify the panniculitides based on histologic features (Figure 1) because
they are difficult to differentiate clinically (most present with deep-seated tender nodules with
surrounding erythema and edema); however, the histologic approach is not helpful in the clinic. This issue of Dermatology Rounds presents a practical approach to panniculitis that will
allow clinicians to develop a differential diagnosis in the office and direct the investigation
and management of patients with panniculitis. A brief commentary on diagnostic histopathological features and treatment of each entity is also given. For a comprehensive review of
the histopathologic features of panniculitis, the reader is referred to an excellent article by
Requena et al.1, 2
Alfred Balbul, MD
The clinical approach to panniculitis
David Gratton, MD
The first thing to consider when panniculitis is suspected is whether it is caused by an
exogenous or endogenous source (Table 1). After exogenous causes have been ruled-out,
endogenous causes may be expanded by carefully eliciting the medical history and reviewing
body systems (Table 2). On physical examination, the appearance and location of the nodules
may narrow the differential diagnosis as indicated in Table 2. The skin biopsy should be a deep
excisional or incisional biopsy to the level of subcutaneous fat. A portion of the biopsy should
be sent to microbiology for special stains and culture. The features listed in Table 3 should be
included in the pathology report. Once a clinical entity is suspected and the biopsy performed,
laboratory or radiologic investigations may be indicated to confirm the diagnosis. These
include, but are not limited to, the tests outlined in Table 3. Basic supportive care for patients
with panniculitis includes rest, leg elevation, elastic compression stockings (Comprilan® or
ACE® bandages), and salicylates or nonsteroidal anti-inflammatory drugs (NSAIDs) for pain.
Manish Khanna, MD
Alain Brassard, MD
Judith Cameron, MD
Wayne D. Carey, MD
Ari Demirjian, MD
Anna Doellinger, MD
Odette Fournier-Blake, MD
Roy R. Forsey, MD
William Gerstein, MD
Raynald Molinari, MD
Linda Moreau, MD
Brenda Moroz, MD
Khue Huu Nguyen, MD
Elizabeth A. O’Brien, MD
Wendy R. Sissons, MD
Beatrice Wang, MD
Ralph D. Wilkinson, MD
Noninfectious granulomatous panniculitis
Erythema nodosum (EN): EN, the most common type of panniculitis, is recognized as a reactive disorder to a variety of stimuli. Streptococcal infection is the most common precipitant of
EN in children while, in adults, sarcoidosis, drugs, and inflammatory bowel disease are the most
common causes. EN typically presents in young women with tender warm nodules and plaques
on the shins that change to a colour that is similar to deep bruises and resolve without ulceration, atrophy, or scarring. Commonly associated symptoms include fever, fatigue, malaise,
arthralgias, headache, cough, abdominal pain, vomiting, and diarrhea. A characteristic change
on histology are Miescher’s radial granulomas, small collections of macrophages surrounding a
stellate-shaped cleft. The clinical course of EN lasts 3 to 6 weeks, but lesions may persist and
frequently recur. Treatment involves supportive care3-5 and potassium iodide 400 to 900 mg
daily or 2 to 10 drops in water or orange juice TID may be used adjunctively.6, 7
Panniculitis involving vessels
Elastin stains are useful in cases of panniculitis with vasculitis because arteries have elastic
lamina and will stain positively, whereas veins do not.
Nodular vasculitis: Nodular vasculitis typically occurs in obese, middle-aged women with
venous insufficiency who present with ulcerating nodules on the posterior legs that are aggravated by cold weather. When tuberculosis (TB), the most common cause of nodular vasculitis,
is present, the nodules are renamed erythema induratum of Bazin. Nodular vasculitis has also
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Figure 1: Histologic classification of panniculitis1,2
Septal
With vasculitis
• Leukocytoclastic
vasculitis (small vessels)
• Cutaneous PAN
(medium-sized arteries)
• Superficial migratory
thrombophlebitis
(large veins)
Lobular
With vasculitis
Without vasculitis
• Erythema nodosum
• Scleroderma/deep
morphea/eosinophilic
fasciitis
• Erythema induratum
• Erythema nodosum
leprosum
• Lucio’s phenomenon
Without vasculitis
• Trauma (cold, blunt
trauma, injection)
• Infection
• Pancreatic
• Childhood/neonatal
• Cytophagic histiocytic
• CTD (DM, SLE)
• Lipodermatosclerosis
• Calciphylaxis,
• α1-antitrypsin deficiency
PAN = polyarteritis nodosa; CTD = connective tissue disease; DM = dermatomyositis; SLE = systemic lupus erythematosus.
been associated with hepatitis C8 and treatment with
propylthiouracil.9 Lesions commonly turn bluish-red,
break down into ulcers with violaceous borders and,
after many years, eventually heal with atrophic scars.
They frequently recur. In cases associated with TB,
caseating necrosis and multinucleated giant cells may be
seen on histology. Chest x-ray and a Mantoux test are
recommended to rule-out TB, followed by triple therapy
if indicated. Oral corticosteroids, tetracycline, and potassium iodide are occasionally indicated as adjuncts to
supportive care.10
Superficial migratory thrombophlebitis (SMT): Patients
with SMT often have a history of venous insufficiency
and present with linear, tender, cord-like, erythematous
nodules along an involved vein. SMT has been associated
with malignancy11-14 and/or a hypercoagulable state;15, 16
therefore, a full coagulation and malignancy work-up is
indicated. Treatment is supportive.
Cutaneous polyarteritis nodosa (PAN): What separates
cutaneous PAN from other panniculitides is the livedo
reticularis and ulceration that accompanies the bilateral
tender red nodules on the lower legs. The medium-sized
arteries of the septa are involved and the process is more
inflammatory than thrombotic, as in SMT. Patients with
cutaneous PAN frequently have low-grade fever, arthralgias, malaise, myalgias, and fatigue.17 If there is no systemic
vasculitis, the prognosis is good and patients respond well
to NSAIDs, and low-dose prednisone (20 mg daily).
Erythema nodosum leprosum and Lucio’s phenomenon: Patients with lepromatous leprosy may present
with painful erythematous to violaceous nodules on the
extremities that are associated with severe systemic
symptoms. Erythema nodosum leprosum is an immune
complex-mediated vasculitis that involves the dermis
and occasionally extends into the subcutaneous fat.
Treatment with thalidomide 400 mg nightly or clofazimine 300 mg daily and prednisone 30 mg daily is
recommended.1 Lucio’s phenomenon is an uncommon
diffuse form of lepromatous leprosy characterized by
painful hemorrhagic ulcers and severe systemic symptoms; it occasionally leads to death. The treatment of
choice for this necrotizing vasculitis is thalidomide.18
Table 1: Exogenous vs. endogenous causes of panniculitis
Exogenous
Endogenous
• Infection: erythema nodosum,
erythema induratum of Bazin,
viral/fungal/bacterial
• Medications: penicillin, sulfonamides,
bromides/iodides, oral contraceptive
pills, post-steroid panniculitis
• Trauma: blunt trauma, cold trauma,
injection granuloma, paraffin
• Self antigens/autoantibodies: lupus panniculitis, dermatomyositis,
scleroderma/morphea
• Immune complex: superficial migratory thrombophlebitis, polyarteritis
nodosa
• Venous insufficiency: lipodermatosclerosis, nodular vasculitis
• Malignancies: Pancreatic carcinoma
• Abnormal histiocytes: cytophagic
• Humoral factors: α1-antitrypsin deficiency, calciphylaxis
• Hormonal factors: pregnancy (erythema nodosum)
• Neonatal abnormal fatty acid ratio with increased propensity to
crystallize with exposure to cold temperatures: sclerema neonatorum,
subcutaneous fat necrosis of newborn
• Associated with other diseases: sarcoidosis, inflammatory bowel disease,
Behcet's disease (erythema nodosum)
Table 2: Clues to the etiology of panniculitis on
history and physical examination
History
• No systemic symptoms (trauma-induced: chemical,
thermal, physical)
• Fever, arthritis, abdominal pain, or history of
pancreatic disease (pancreatic panniculitis)
• Immunosuppressed (infectious etiology)
• Pancreatitis, glomerulonephritis, emphysema,
cirrhosis, cutaneous vasculitis, rheumatoid arthritis
(α1-antitrypsin deficiency)
• Arthritis, photosensitivity, Raynaud’s, dysphagia,
and oral ulcers (connective tissue disease)
• Fever, sore throat, arthralgias, malaise, bowel
symptoms, history of oral contraceptives,
sulfonamides, bromides/iodides (erythema
nodosum)
• Fever, weight loss (CHP)
Physical Examination
Nodules:
• Fluctuant, ulcerating, draining
– Pancreatic
– Traumatic
– α1-antitrypsin deficiency
– Infection
• Bilateral, tender on posterior legs of middle-aged
obese female with venous insufficiency
– E. induratum/nodular vasculitis,
Lipodermatosclerosis (acute form)
• Hemorrhagic/purpuric
– α1-antitrypsin deficiency, CHP
• Linear configuration (with history of varicose veins
and/or hypercoagulable state)
– Superficial migratory thrombophlebitis
Other observations:
• Venous stasis
– Lipodermatosclerosis, nodular vasculitis
• Livedo reticularis, small nodules in distribution of
superficial arteries
– Polyarteritis nodosa
• Hepatosplenomegaly
– CHP
E. induratum = erythema induratum of Bazin,
CHP = cytophagic histiocytic panniculitis
Vascular disorders
Sclerosing panniculitis (lipodermatosclerosis): Similar
to nodular vasculitis, lipodermatosclerosis is common in
middle-aged obese women with venous insufficiency.
Patients with lipodermatosclerosis present with woody
indurated plaques on “inverted champagne bottle”shaped lower legs. Treatment is supportive. Stanozolol
2 to 5 mg twice daily19 or pentoxifylline20 have been
effective in pain control.
Calciphylaxis: Approximately 4% of all patients on
hemodialysis present with calciphylaxis due to calcification of vessel walls and occlusion of small arterioles.
Although the most common cause of calciphylaxis is
end-stage renal failure, it is also associated with secondary hyperparathyroidism and, rarely, malignancy21-23
and end-stage liver cirrhosis.24 Clinically, patients present
with symmetrical violaceous to black livedo reticularis-
Table 3: Investigations for panniculitis
Skin biopsy: what to look for on the pathology report
• Pattern: lobular, septal, or mixed
• Vasculitis or no vasculitis
• Characteristic histologic findings
(ie, needle-shaped clefts, ghost cells, etc.)
Laboratory tests
• Pancreatic enzymes (amylase, lipase)
• SPEP, CBC, LDH, peripheral blood smear
• Calcium, phosphate, creatinine, PTH
• ANA, ENA, ANCA’s, dsDNA, rheumatoid factor
• α1-antitrypsin levels
• Fasting glucose, HbA1c
Radiologic tests
• Chest x-ray
• CT chest, abdomen, and pelvis
• Lower leg venous Doppler studies
like patches and plaques, most often on the legs, but also
on the upper extremities, trunk, and penis. With time,
lesions enlarge into ulcers and become painful and
necrotic with black eschars. On histology, fat necrosis
with calcification of vessel walls is characteristic. Mortality rates of 60% to 70% have been reported, mainly due
to sepsis from secondarily-infected ulcers. Treatment
options are limited, but include parathyroidectomy and
hyperbaric oxygen with subcutaneous low-molecular
weight heparin.25-27
Connective tissue disorders
Lupus panniculitis: Approximately 1% to 3% of
patients with lupus erythematosus (LE) will develop
lupus panniculitis. It is more common in patients with
discoid LE than systemic LE (SLE), and usually precedes
(but may occur synchronously or after) the onset of LE.
Patients with SLE who present with lupus panniculitis
usually have a milder disease course. Clinically, multiple
symmetric painful nodules or plaques are localized to
the proximal extremities, face, and trunk. When lesions
regress, they result in lipoatrophy (commonly seen on
the shoulders and upper arms).
On histology, epidermal changes of discoid LE such
as follicular plugging, atrophy, dyspigmentation, telangiectasias, and ulceration are characteristic.28, 29 The clinical course is chronic and recurrent. Avoidance of trauma
and sun protection is important. Potent topical or
intralesional corticosteroids and antimalarials have been
successfully used.30-32 Alternatively, dapsone, cyclophosphamide, and thalidomide are reported to be successful,
with a second antimalarial added if there is no response
with a single agent.
Deep morphea “scleroderma panniculitis”: Patients with
deep morphea present with bound down plaques or
nodules that heal with atrophy and hyperpigmentation
and respond poorly to treatment. Intralesional and oral
corticosteroids, antimalarials, penicillamine, and methotrexate33 have been used unsuccessfully.
Other: In case reports, dermatomyositis, Sjögren’s
disease, and mixed connective tissue disease have been
described as being associated with panniculitis.34-36
Panniculitis associated with
other systemic disorders
Pancreatic panniculitis: Panniculitis in patients with
pancreatic disorders is rare (~2%)1 and may precede
the onset of pancreatic disease.37 Pancreatic panniculitis may be associated with acute or chronic pancreatitis, pancreatic carcinomas, or rarely anatomic
ductal anomalies,38 pseudocysts,39 and vasculopancreatic fistulas.40 Clinically, painful erythematous
nodules that ulcerate and discharge a brownish oily
exudate (if fat necrosis is severe) are typically seen
on the lower legs. Patients may also present with
abdominal pain or acute arthritis if peritoneal or
periarticular fat is involved, respectively. Histologically, fat necrosis with saponification and “ghost
cells” (adipocytes with absent nuclei) is pathognomonic.41 The pathogenesis of pancreatic panniculitis
is thought to be related to a combination of fat
necrosis by pancreatic enzymes and immunologic
factors.42 In pancreatic cancer patients, panniculitis
may herald the development of metastatic disease
and predict a poor clinical outcome.37 Treatment of
patients with pancreatic panniculitis is based on surgical repair of the underlying pancreatic abnormality
and supportive care (which is often minimally
effective). For patients with pancreatic tumours,
octreotide, a synthetic somatostatin analog that
inhibits secretion of pancreatic enzymes, has been
reported to be of some benefit in a few cases.43
α1-antitrypsin deficiency-associated panniculitis:
α1-antitrypsin is an important enzyme produced by
the liver that prevents the autodigestion of tissues in
the body by proteases. A deficiency in this enzyme
may result in fat necrosis and panniculitis, cirrhosis,
emphysema, pancreatitis, glomerulonephritis, rheumatoid arthritis, cutaneous vasculitis, or angioedema.
Clinically, patients present with erythematous to
purpuric painful plaques and nodules that may
ulcerate and drain a brownish oily fluid. After a
prolonged course, plaques heal with atrophy and
scarring. Histologically, fat necrosis with splaying of
neutrophils between collagen bundles in the deep
dermis is a characteristic finding. Lesions are often
resistant to treatment; however, oral corticosteroids,
antimalarials, doxycycline, dapsone, colchicine,
intravenous infusions of exogenous α1-antitrypsin
inhibitor, and plasma exchange have been found to
be effective.44-46
Cytophagic histiocytic panniculitis and subcutaneous panniculitis-like T-cell lymphoma: Cytophagic histiocytic panniculitis (CHP) is so-named
because of histiocyte phagocytosis of various cells
and debris, resulting in “bean bag cells” histologically. It is thought to represent the early stage of a lymphoproliferative disorder because, after a prolonged
course, patients may develop T-cell, B-cell, and NK
cell lymphomas, grouped together as subcutaneous
panniculitis-like T-cell lymphoma (SPTL). Clinically, deep-seated erythematous nodules with overly-
ing ecchymoses, symptoms of fever and weight loss,
and findings of lymphadenopathy and hepatosplenomegaly are found.
Patients with the benign variant of CHP , which
does not transform into lymphoma, tend to respond
well to prednisone or cyclosporine.1 Unlike SPTL,
this nonfatal form is not associated with EpsteinBarr virus (EBV) and is most commonly seen in
patients without systemic symptoms. Patients with
CHP that transforms to SPTL have a poor prognosis
and, although therapies such as prednisone, cyclosporine, dapsone, and high-dose chemotherapy
alone or in combination with peripheral stem cell
rescue have been reportedly effective, prolonged
remissions are uncommon.
Infectious panniculitides
Infectious panniculitis is commonly found in,
but is not exclusive to, immunosuppressed patients.
It is most often due to the hematogenous spread of
bacteria, mycobacteria, or fungi, but may also result
from direct inoculation of these infectious agents.
Patients with diabetes mellitus, malignancy, connective tissue disease, acquired immune deficiency syndrome (AIDS), or a history of organ transplant have
been reported with infectious panniculitis1 and
typically present with ulcerating fluctuant nodules
similar to those of pancreatic or α1-antitrypsin deficiency panniculitis. The histology is nondescript.
Diagnosis is made on special stains for organisms
and culture, and patients usually respond to antibiotics or surgery in selected cases.
Traumatic panniculitis
Traumatic panniculitis results from accidental,
intentional, or iatrogenic injury. Typical scenarios
include children who suck on popsicles, ice cubes,
or ice packs and develop firm nodules on the cheeks
and chin, women who wear tight pants and go
horseback riding who develop erythematous to violaceous plaques on their inner thighs, and women
with large breasts who develop indurated nodules
that mimic inflammatory breast cancer. Traumatic
panniculitis from injections most commonly occurs
with substances such as mineral oil, silicones,
camphor, cottonseed, and sesame oil. On histology,
fat necrosis and a characteristic “swiss cheese”
appearance is seen. Treatment requires removal of
the inciting agent and oral or intralesional corticosteroids to control the inflammation.
Childhood panniculitis
Children are uniquely susceptible to panniculitis due to the increased ratio of saturated to unsaturated fatty acids, resulting in a higher melting point
and increased propensity towards crystallization
upon exposure to cold.
Sclerema neonatorum: The typical patient with
sclerema neonatorum is a premature baby with congestive heart failure (CHF) who presents in the first
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week of life with a generalized distribution of cold,
rigid, wooden board-like skin. This child may have
underlying hypothermia, lung abnormalities, CHF,
diarrhea, or intestinal blockage, and commonly dies
of septicemia. There is sparing of the palms, soles,
and genitalia and common precipitants include
exposure to cold, defective complement, and
dehydration. Needle-shaped clefts in lipocytes are
characteristic on histology. There is no effective
treatment and supportive measures to control sepsis
are indicated.
Subcutaneous fat necrosis of the newborn (SFN):
In contrast to sclerema neonatorum, SFN is a localized self-limited disorder that is much less severe.
Newborns between 2 to 3 weeks old present with
red to violaceous plaques or nodules on the cheeks,
shoulders, trunk, buttocks, and thighs that often
resolve within a few days. Predisposing factors
include hypothermia, gestational diabetes, hypoglycemia, meconium aspiration, placenta previa,
seizures, and preeclampsia.47,48 Common complications of SFN are hypercalcemia and thrombocytopenia; therefore, monitoring of serum calcium is
recommended and dietary restriction of calcium
and vitamin D, hydration, and furosemide may be
indicated to control calcium levels. On histology,
needle-shaped clefts in both adipocytes and giant
cells are characteristic. Treatment involves supportive care. Patients recover quickly, but corticosteroids
may occasionally be indicated.
Post-steroid panniculitis: Post-steroid panniculitis
presents in children aged 2 to 14 years who have
recently (within the last 1 to 40 days) undergone
rapid withdrawal of corticosteroids. Firm red plaques
on the cheeks, arms, and trunk are typically seen.
The histologic appearance is identical to subcutaneous fat necrosis of the newborn. There is no treatment as lesions spontaneously resolve after months
to one year or after corticosteroids are restarted.
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Abstract of Interest
Normal subcutaneous fat, necrosis of adipocytes
and classification of the panniculitides
more vasculitis than panniculitis and include superficial thrombophlebitis and cutaneous polyarteritis nodosa. Mostly septal
panniculitides with no vasculitis include erythema nodosum,
necrobiosis lipoidica, deep morphea, subcutaneous granuloma
annulare, rheumatoid nodule, and necrobiotic xanthogranuloma. Mostly lobular panniculitis with vasculitis is only represented by erythema induratum of Bazin. In contrast, mostly lobular
panniculitides without vasculitis comprise a large series of disparate disorders, including sclerosing panniculitis, calciphylaxis,
sclerema neonatorum, subcutaneous fat necrosis of the newborn, poststeroid panniculitis, lupus erythematosus profundus,
pancreatic panniculitis, alpha(1)-antitrypsin deficiency panniculitis, subcutaneous Sweet syndrome, infective panniculitis,
factitial panniculitis, lipodystrophy, traumatic panniculitis,
subcutaneous sarcoidosis, and sclerosing postirradiation panniculitis. Finally, some cutaneous lymphomas may simulate
panniculitis, both from clinical and histopathologic points of
view and, for that reason, they will be included in this review,
although they are not inflammatory processes, but authentic
lymphocytic neoplasms involving subcutaneous tissue.
Semin Cutan Med Surg 2007;26(2):66-70.
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R E Q U E N A L, M A D R I D , S PA I N .
The panniculitides represent a group of heterogeneous inflammatory diseases that involve the subcutaneous fat. The specific
diagnosis of these diseases requires histopathologic study
because different panniculitides usually show the same clinical
appearance, which consists of erythematous nodules on the
lower extremities. However, the histopathologic study of panniculitis is difficult because of an inadequate clinicopathologic
correlation and the changing evolutive nature of the lesions. In
addition, large scalpel incisional biopsies are required. From
histopathologic point of view, all panniculitides are somewhat
mixed because the inflammatory infiltrate involves both the
septa and lobules. However, nearly always the differential diagnosis between a mostly septal and a mostly lobular panniculitis
is straightforward at scanning magnification on the basis of the
structures more intensely involved by the inflammatory infiltrate. Mostly septal panniculitides with vasculitis are actually
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