Lymphedema and Panniculitis

[patoco]

Lymphedema and Panniculitis

Our Home Page: Lymphedema People

http://www.lymphedemapeople.com

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Lymphedema and Panniculitis

What is panniculitis and why would I have a section on it in a lymphedema website. Very simply, in the skin, most of the lymphatic system is in the subcytaneous tissue and most of the institial fluids that collect as a result of lymphedema are therefire in the subcutaneous tissues.

The subcutaneous tissue is the third of the three layers of skin. The subcutaneous layer contains fat and connective tissue that houses larger blood vessels and nerves. This layer is important is the regulation of temperature of the skin itself and the body. The size of this layer varies throughout the body and from person to person.

Panniculitis is an inflammation of the subcutaneous tissue and can be caused by a number of factors, including lymphedema.

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Panniculitis

Definition of Panniculitis

Panniculitis is an inflammation of the layer of subcutaneous fat underlying the epidermis of the skin. Acute panniculitis is also called nodular fat necrosis.

Causes and Risk Factors of Panniculitis

Panniculitis may result from a variety of conditions. While it is not possible to develop a firm classification of acute panniculitis, one may distinguish between panniculitis without systemic (throughout the body) disease and panniculitis with systemic disease.

Panniculitis without systemic disease is usually due to trauma or a cold. One variant, subcutaneous fat necrosis of the newborn, may be due to a combination of obstetric trauma and hypothermia (low body temperature).

Panniculitis with systemic disease includes collagen vascular disease (lupus and scleroderma), lymphoma, pancreatic cancer and pancreatitis.

Symptoms of Panniculitis

The appearance of single or multiple crops of nodules in subcutaneous fat is the hallmark of acute panniculitis. The nodules are usually, but not always, tender. On occasion, they drain an oily solution and suppuration (pus) may occur. Individual lesions last from one to eight weeks before disappearing, and a pigmented depressed area may be left at the involved site.

While some patients have only nodular panniculitis, which may or may not be relapsing, others may develop fever, abnormal liver function, involvement of the bone marrow, bleeding tendencies, nodular pulmonary lesions and evidence of pancreatic disease. This constellation of findings has been called Weber-Christian disease.

Diagnosis of Panniculitis

Acute panniculitis can be diagnosed only histologically, usually by biopsy of tissue. Once the lesion is identified, a search for the cause must be made. If systemic symptoms are present, the primary differential diagnosis is between collagen vascular disease, lympho-proliferative disorder and pancreatitis or pancreatic cancer.

Treatment of Panniculitis

Treatment is often unsatisfactory. Some physicians may use anti-inflammatory medications. Some patients with certain types of panniculitis may respond to combined chemotherapy with cyclophosphamide, bleomycin and prednisone.

Questions To Ask Your Doctor About Panniculitis

Do any more tests need to be done to diagnose the disease or the cause of the disease?

What is the cause of panniculitis?

What type of treatment will you be recommending?

How effective is this treatment?

Will you be prescribing any medications?

What are the side effects?

http://www.healthscout.com/ency/416/175/main.html

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PANNICULITIS AND DISORDERS OF THE SUBCUTANEOUS FAT

Gerald S. Lazarus

INTRODUCTION. The subcutaneous tissue is a fibrofatty layer spread between skin and muscles. It functions not only as a thermal and mechanical insulator but also as an active metabolic organ. The characteristic signet ring lipocytes are organized into lobules by fibrous septa, which are continuous with the dermis and contain the blood and lymph vessels and reticuloendothelial cells.

The diagnosis of panniculitis frequently requires deep skin biopsy. The most important histologic characteristic is the location of the inflammatory process. Inflammation primarily in the septa is designated septal panniculitis, whereas inflammatory cells primarily in the fat lobules designate lobular panniculitis. The presence or absence of vasculitis further differentiates panniculitis into the four major groups.

LOBULAR PANNICULITIS WITHOUT VASCULITIS

Nodular Panniculitis-Weber-Christian Disease. Nodular panniculitis describes a group of syndromes or diseases characterized by subcutaneous nodules and inflammatory cells in the fat lobules. The term Weber-Christian disease is applied when cutaneous lesions are associated with systemic complaints.

The etiology of this group of diseases is unknown. In the early stages the fat lobules are infiltrated with polymorphonuclear leukocytes. Later, macrophages appear and ingest fat, producing the characteristic lipophagic granuloma. The lesions heal with lobular fibrosis. Modest septal vasculitis may be observed. Lobular panniculitis most commonly presents in females between the ages of 30 and 60, although cases have been reported in all age groups. The lesions begin as red, slightly tender nodules deep in the skin. They appear more or less in symmetric crops on thighs and lower legs, but lesions may also occur on arms, trunk, and face. The number of lesions may vary enormously. The lesions become firmer, less red, and less tender over a period of weeks. They heal, leaving a depressed hyperpigmented scar. Liquefying panniculitis is a variant in which the lesions become necrotic and drain an oily, yellow-brown fluid. Rothmann-Makai syndrome is a very rare variant of lobular parmiculitis, affecting children with numerous large lesions; the lesions do not liquefy, and healing usually occurs within 12 months.

Systemic nodular parmiculitis or Weber-Christian disease is a widespread proces~ affecting cutaneous and visceral fat. Patients usually present with unequivocal cutaneous nodules and arthralgias, malaise, fatigue, weight loss, and abdominal pain. Involvement of the bone marrow may produce anemia, leukocytosis or leukopenia, and bone pain. Hepatomegaly, steatorrhea, and intestinal perforation have also been reported. Inflammation may occur in other internal organs such as lungs, pleura, pericardium, spleen, kidney, and adrenal glands. Visceral involvement may be confined to the retroperitoneal space, producing abdominal pain, nausea, and vomiting. Alpha,-antitrypsin deficiency is associated with this syndrome not infrequently. Malignant histiocytosis (cytophagic histiocytic panniculitis) may present as Weber-Christian disease. The prognosis of nodular panniculitis is good in patients with only cutaneous involvement. There are frequent remissions and exacerbations of the lesions. Some patients recover after a few months, and permanent remission is usual after several years. On rare occasions visceral involvement may be fatal.

There is no specific therapy for this disease. Saturated potassium iodide, increasing from 5 drops three times daily by 1 drop per day to 30 drops three times daily, has been suggested. Hydroxychloroquine,* 200 mg twice per day, has also been advocated as treatment. High dose prednisone, 40 to 60 mg for one to two weeks with gradual tapering over six to eight weeks, has also been reported to be of value in patients with severe disease; steroids should be used only for acute attacks and for limited periods of time.

Lobular Panniculitis Associated with Pancreatic Disease. The diagnosis is made by skin biopsy, which discloses acute fat necrosis with characteristic ghost cells. These patients often have associated arthritis, ascites, and eosinophilia. Acute pancreatitis, trauma to the pancreas, chronic pancreatitis, pancreatic cysts, and pancreatic carcinoma have been reported to be associated with this syndrome. Diagnosis depends upon the histologic findings at skin biopsy and documentation of a specific pancreatic abnormality. Therapy is directed at the underlying pancreatic disease.

Poststeroid Lobular Panniculitis. Children who receive large doses of steroid for a short period of time, followed by abrupt discontinuance, may develop lobular parmiculitis. Lesions may occur in the viscera, and a fatal case has been reported. Physical Lobular Panniculitis. Physical trauma of any kind and cold injury, especially in children, can produce lobular panniculitis. A unique traumatic panniculitis occurs in the obese breasts of women in their 50's. Injection of silicones or other foreign materials into female breasts or buttocks and into the male genitalia may induce a granulomatous foreign body nodular panniculitis. Similar inflammatory lesions may be seen following injections of Talwin.

Lobular Panniculitis Associated with Systemic Disease. Lupus erythematosus, sarcoidosis, granuloma annulare, Sweet's disease, acute sudden weight loss from gastrointestinal surgery, and infections including deep fungi and pyogens may present as lobular parmiculitis. Lymphoma or leukemia may also present as panniculitis; histologically, these lesions demonstrate malignant cells in the fat lobules. Lupus erythematosus confined primarily to the fat is known as lupus profundus. The skin may be exclusively involved, or the panniculitis may be associated with systemic disease.

LOBULAR PANNICULITIS WITH VASCULITIS.

This category of disease includes nodular vasculitis and erythema induratum. The eruption consists of recurring, tender, painful nodules on the calves, which often ulcerate and heal with scarring. It is much more common in females than in males. Increased erythrocyte sedimentation rate and hypertension have been associated with this syndrome. Bazin gave the name erythema induraturn to this disease when histologic examination revealed caseation necrosis and the lesions were associated with tuberculosis. There is no specific therapy. Most patients have remission of lesions with bed rest. Severe cases have been successfully treated with nonsteroidal anti-inflammatory drugs, dapsone, and prednisone. In the very rare case of nodular vasculitis associated with tuberculosis, appropriate antituberculous therapy is indicated.

SEPTAL PANNICULITIS WITHOUT VASCULITI

This histology in a patient with nodular, painful, tender lesions, especially on the anterior leg, is diagnostic of erythema nodosum, which is discussed in Ch. 534. A chronic disease similar to erythema nodosum clinically and histologically except that the lesions spread peripherally over months, forming rings, is called subacute migratory panniculitis. This disease responds to therapy with increasing doses of saturated *This use is not listed in the manufacturer's directive.

potassium iodide as described for nodular panniculitis. Septal parmiculitis without vasculitis can also be seen in scleroderma, eosinophilic fasciitis, dermatomyositis, and necrobiosis lipoidica diabeticorum.

SEPTAL PANNICULITIS WITH VASCULITIS

Thrombophlebitis may present with subcutaneous nodules. Histology reveals inflammation of veins with adjacent parmiculitis (see Ch. 57). Cutaneous polyarteritis is a chronic, recurring, painful nodular eruption, primarily of the legs. There is often an associated mottled livedo vascular pattern. Cutaneous polyarteritis is associated with myalgias, arthralgias, and increased erythrocyte sedimentation rate. Histologic examination demonstrates leukocytoclastic vasculitis of medium-sized arterioles. This disease is usually not associated with systemic involvement. It has a benign course, but lesions may recur for years. Therapy includes nonsteroidal anti-inflammatory agents and short courses of corticosteroids. Cutaneous polyarteritis associated with granulornatous bowel disease has responded to short courses of Cytoxan.

LIPOATROPHY

Loss of subcutaneous tissue can occur as a consequence of healing in almost any of the parmiculitides described previously. The most common diagnosable cause of lipoatrophy is recurrent insulin injection. Insulin lipoatrophy is usually associated with repetitive injections of high doses of insulin in exactly the same location in females. Talwin injections may also produce panniculitis and severe lipoatrophy.

Total lipoatrophy associated with diabetes may occur in children and adults. The clinical picture is dramatic, and there is almost complete loss of subcutaneous fat. Partial lipoatrophy usually begins in children or young adults. It is five times more common in females than in males. Patients often lose the fat in the face and the upper half of the body. In some cases, there is hypertrophy of the fat on the lower half of the body. Patients with partial lipodystrophy often develop progressive mesangiocapillary glomerulonephritis and hypocomplementernia. Diabetes develops in one third of these patients. Retinitis pigmentosum has also been reported with this disease. The prognosis depends upon the severity of the renal disease

http://www.geocities.com/SoHo/Gallery/6 ... ulitis.htm

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Erythema Nodosum Support Group

http://health.groups.yahoo.com/group/er ... sum_Group/

The website for the group Is

Erythema Nodosum

http://www.geocities.com/erythema_nodos ... _Page.html

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Panniculitis at Yahoo Groups

http://health.groups.yahoo.com/group/panniculitis/

Subscribe: panniculitis-subscribe@yahoogroups.com

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For Further Information

Subacute Nodular Migratory Panniculitis (Vilanova Disease)

eMedicine

Last Updated: July 28, 2003

Synonyms and related keywords: chronic erythema nodosum, erythema nodosum migrans

Author: Sarah M Boyce, MD, Staff Physician, Department of Dermatology, University of Alabama School of Medicine

Coauthor(s): Daniel Davis, MD, Program Director, Assistant Professor, Departments of Dermatology and Pathology, University of Alabama at Birmingham School of Medicine

http://www.emedicine.com/derm/topic408.htm

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Cold Panniculitis

eMedicine

Last Updated: November 29, 2001

Author: Geromanta Baleviciene, MD, Head, Professor, Department of Dermatology, Vilnius University, Medical Faculty, Lithuania

Coauthor(s): Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School; Richard J Cervin, MD, Consulting Staff, Department of Dermatology, Baltic-American Medical and Surgical Clinic, Lithuania

http://www.emedicine.com/derm/topic781.htm

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Panniculitis, Idiopathic Nodular

http://my.webmd.com/hw/health_guide_atoz/nord297.asp

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Related Links Page for Pub Med / National Library of Medicine

http://www.ncbi.nlm.nih.gov/entrez/quer ... id=9674398

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PANNICULITIS
HELLENIC ASSOCIATION DERMATOLOGY - VENEREOLOGY

Links to Articles

http://www.edae.gr/panniculitis.html

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ERYTHEMA NODOSUM PANNICULITIS

eMedicine

Last Updated: April 12, 2002

Synonyms and related keywords: EN, delayed hypersensitivity reaction

Author: Jeanette Hebel Matthews, MD, Department of Dermatology, The Skin Surgery Center

Coauthor(s): Thomas Habif, MD, Adjunct Professor, Department of Internal Medicine, Section of Dermatology, Dartmouth Medical School

http://www.emedicine.com/derm/topic138.htm

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ERYTHEMA NODOSUM PANNICULITIS

Medline Plus

http://www.nlm.nih.gov/medlineplus/ency ... 000881.htm

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Panniculitis - Diagnostic Images

Atlas of Dermatology

http://atlases.muni.cz/atl_en/main nenadory pann.html

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Panniculitis - Diagnostic Images

Dermatlas

http://dermatlas.med.jhmi.edu/derm/resu ... -639418194

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Panniculitis - Diagnostic Images

Google

http://images.google.com/images?q=panni ... rt=20&sa=N

Yahoo

http://images.search.yahoo.com/search/i ... 1&ei=UTF-8

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Panniculitis

Panniculitis refers to a group of conditions that involve inflammation of the fat under the skin. Despite having very diverse causes, most forms of panniculitis have the same clinical appearance. The diagnosis is established by a skin biopsy as there are characteristic microscopic features depending on the cause.

The classification of panniculitis is complicated but, in general, different types can be divided into mostly septal or mostly lobular panniculitis depending on where the microscopic inflammation is most concentrated. In reality, most types of panniculitis have both lobular and septal inflammation. Further classification is based on whether or not there is inflammation involving the blood vessels of the fat, i.e. vasculitis.

Classification of panniculitis

Mostly septal panniculitis with vasculitis

Leukocytoclastic vasculitis
Superficial thrombophlebitis
Cutaneous polyarteritis nodosa
Mostly septal panniculitis without vasculitis

Necrobiosis lipoidica
Scleroderma, which may be localised (morphoea)
Subcutaneous granuloma annulare
Rheumatoid nodule
Necrobiotic xanthogranuloma
Erythema nodosum
Mostly lobular panniculitis with vasculitis

Erythema nodosum leprosum (leprosy)
Lucio's phenomenon
Neutrophilic lobular panniculitis associated with rheumatoid arthritis
Erythema induratum of Bazin
Crohn's disease
Mostly lobular panniculitis without vasculitis

Sclerosing panniculitis (lipodermatosclerosis)
Calciphylaxis
Oxalosis
Sclerema neonatorum
Cold panniculitis
Lupus panniculitis
Panniculitis in dermatomyositis
Pancreatic panniculitis
Alpha-1 antitrypsin deficiency
Infective panniculitis
Factitial panniculitis
Subcutaneous sarcoidosis
Traumatic panniculitis
Lipoatrophy
Subcutaneous fat necrosis of the newborn
Post-steroid panniculitis
Gout panniculitis
Crystal-storing panniculitis
Cytophagic histiocytic panniculitis
Post-irradiation pseudosclerodermatous panniculitis

Clinical Features

An area of skin involved with panniculitis feels thickened and woody to touch. It may or may not demonstrate discolouration of the overlying skin, either reddening or darker, brownish pigmentation. The area is often tender. Most often, the affected areas appear as raised nodules or lumps under the skin, but may be a plaque or large flat area of thickened skin.

Treatment

Treatment includes:

Treat the underlying cause, if known (e.g. stop a medication, treat an infection)

Rest and elevate the affected area
Compression hosiery (18-25mm Hg pressure) if these can be tolerated
Pain relief using anti-inflammatory medications such as aspirin, ibuprofen or diclofenac
Systemic steroids (oral or injected) to settle the inflammation
Anti-inflammatory antibiotics including tetracycline or hydroxychloroquine
Potassium iodide
Surgical removal of persistent or ulcerated lesions.

http://dermnetnz.org/dermal-infiltrativ ... litis.html

Related topics

On DermNet:
Erythema nodosum
Necrobiosis lipoidica
Morphoea (localised scleroderma)

www.dermnetnz.org/

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Weber-Christian panniculitis: a review of 30 cases with this diagnosis.

White JW Jr, Winkelmann RK.

Department of Dermatology, Mayo Clinic Jacksonville, Florida 32224, USA.

BACKGROUND:

More than 60 years ago, Christian described a panniculitis that was later termed Weber-Christian disease. OBJECTIVE: The purpose of this study was to investigate whether this is a specific disease or a nonspecific disease that embraces several specific conditions.

METHODS:

We studied 30 cases diagnosed as Weber-Christian panniculitis and found it possible to make a more specific diagnosis. RESULTS: In 12 patients, findings were compatible with erythema nodosum. Six patients had phlebitis or postphlebitic syndrome. Factitial panniculitis was diagnosed in five patients, and trauma had a role in the conditions of another three patients. Cytophagic panniculitis, lymphoma, and leukemia were recognized in one patient each. The lesion was lobular in almost all cases, and the presence of lipophagia was noted in 19 biopsy specimens. Granulomatous, neutrophilic, and lymphocytic pathologic changes were present in nine, eight, and eight tissue specimens, respectively.

CONCLUSION:

The recognition of distinct disease patterns of fat lesions as fat necrosis with pancreatic disease, alpha1-antitrypsin panniculitis, lupus and connective tissue disease panniculitis, involution lipoatrophy, lipomembranous panniculitis, factitial panniculitis syndromes, calcification panniculitis, lipophagic lipoatrophy, and cytophagic panniculitis has lessened the need for a less specific panniculitis category. All these diseases have been reported in the literature as "Weber-Christian disease." Because separate and distinct forms of fat lesions have been described, we believe that the eponym should be abandoned and that more specific diagnoses should be made on the basis of pathogenesis or cause.

MeSH Terms:
Adult
Aged
Diagnosis, Differential
Erythema Nodosum/diagnosis
Factitious Disorders/diagnosis
Fat Necrosis/diagnosis
Fat Necrosis/pathology
Female
Granuloma/pathology
Humans
Leukemia/diagnosis
Lymphocytes/pathology
Lymphoma/diagnosis
Male
Middle Aged
Neutrophils/pathology
Panniculitis/classification
Panniculitis/diagnosis
Panniculitis/pathology
Panniculitis, Nodular Nonsuppurative/diagnosis*
Panniculitis, Nodular Nonsuppurative/pathology
Panniculitis, Nodular Nonsuppurative/psychology
Phlebitis/diagnosis
Postphlebitic Syndrome/diagnosis
Skin/injuries
Skin Neoplasms/diagnosis

PMID: 9674398 [PubMed - indexed for MEDLINE]

http://www.ncbi.nlm.nih.gov/entrez/quer ... t=Citation

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Mesenteric Panniculitis

http://brighamrad.harvard.edu/Cases/bwh ... /full.html

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Sclerosing panniculitis

Lipomembranous (Membranocystic) Fat Necrosis: Clinicopathologic Correlation of 38 Cases.

http://www.amjdermatopathology.com/pt/r ... -00007.htm jsessionid=CVqYN16lrvE2iQJVU3MYRAZdBpA2rpStujIiO7OZ9rGbNVSfF2Iv!884695698!-949856031!9001!-1

I wanted to share about two great resources I just learned about for Panniculitis.

This website and Yahoo support group focuses on Erythema Nodosum, which is a form of panniculitis.

The online support group is:

Erythema Nodosum Support Group

http://health.groups.yahoo.com/group/er ... sum_Group/

The website for the group Is

Erythema Nodosum

http://www.geocities.com/erythema_nodos ... _Page.html

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Erythema nodosum

Semin Cutan Med Surg. 2007 Jun

Requena L, Sánchez Yus E.
Department of Dermatology, Fundación Jiménez Díaz, Universidad Autónoma, Madrid, Spain. lrequena@fjd.es

Erythema nodosum is the most frequent clinicopathologic variant of panniculitis. The process is a cutaneous reaction that may be associated with a wide variety of disorders, including infections, sarcoidosis, rheumatologic diseases, inflammatory bowel diseases, medications, autoimmune disorders, pregnancy, and malignancies. Erythema nodosum typically manifest by the sudden onset of symmetrical, tender, erythematous, warm nodules and raised plaques usually located on the lower limbs.

Often the lesions are bilaterally distributed. At first, the nodules show a bright red color, but within a few days they become livid red or purplish and, finally, they exhibit a yellow or greenish appearance, taking on the look of a deep bruise. Ulceration is never seen, and the nodules heal without atrophy or scarring. Histopathologically, erythema nodosum is the stereotypical example of a mostly septal panniculitis with no vasculitis. The septa of subcutaneous fat are always thickened and variously infiltrated by inflammatory cells that extend to the periseptal areas of the fat lobules. The composition of the inflammatory infiltrate in the septa varies with age of the lesion.

In early lesions edema, hemorrhage, and neutrophils are responsible for the septal thickening, whereas fibrosis, periseptal granulation tissue, lymphocytes, and multinucleated giant cells are the main findings in late stage lesions of erythema nodosum. A histopathologic hallmark of erythema nodosum is the presence of the so-called Miescher's radial granulomas, which consist of small, well-defined nodular aggregations of small histiocytes arranged radially around a central cleft of variable shape.

Treatment of erythema nodosum should be directed to the underlying associated condition, if identified. Usually, nodules of erythema nodosum regress spontaneously within a few weeks, and bed rest is often sufficient treatment. Aspirin, nonsteroidal antiinflammatory drugs, such as oxyphenbutazone, indomethacin or naproxen, and potassium iodide may be helpful drugs to enhance analgesia and resolution. Systemic corticosteroids are rarely indicated in erythema nodosum and before these drugs are administered an underlying infection should be ruled out.

Keywords: septal panniculitis; erythema nodosum; Miescher radial granuloma

http://www.sciencedirect.com/science?_o ... b656a644ea

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Erythema nodosum: a sign of systemic disease

Am Fam Physician. 2007 Mar

Schwartz RA, Nervi SJ.
University of Medicine and Dentistry of New Jersey-New Jersey Medical School, Newark, New Jersey, USA. roschwar@cal.berkeley.edu

Erythema nodosum, a painful disorder of the subcutaneous fat, is the most common type of panniculitis. Generally, it is idiopathic, although the most common identifiable cause is streptococcal pharyngitis. Erythema nodosum may be the first sign of a systemic disease such as tuberculosis, bacterial or deep fungal infection, sarcoidosis, inflammatory bowel disease, or cancer. Certain drugs, including oral contraceptives and some antibiotics, also may be etiologic.

The hallmark of erythema nodosum is tender, erythematous, subcutaneous nodules that typically are located symmetrically on the anterior surface of the lower extremities. Erythema nodosum does not ulcerate and usually resolves without atrophy or scarring. Most direct and indirect evidence supports the involvement of a type IV delayed hypersensitivity response to numerous antigens. A deep incisional or excisional biopsy specimen should be obtained for adequate visualization. Erythema nodosum represents an inflammatory process involving the septa between subcutaneous fat lobules, with an absence of vasculitis and the presence of radial granulomas. Diagnostic evaluation after comprehensive history and physical examination includes complete blood count with differential; erythrocyte sedimentation rate, C-reactive protein level, or both; testing for streptococcal infection (i.e., throat culture, rapid antigen test, antistreptoly-sin-O titer, and polymerase chain reaction assay); and biopsy.

Patients should be stratified by risk for tuberculosis. Further evaluation (e.g., purified protein derivative test, chest radiography, stool cultures) varies based on the individual. Erythema nodosum tends to be self-limited. Any underlying disorders should be treated and supportive care provided. Pain can be managed with nonsteroidal anti-inflammatory drugs.

http://www.aafp.org/afp/20070301/695.html

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Pat O'Connor
Lymphedema People
http://www.lymphedemapeople.ocm

[patoco]

Normal subcutaneous fat, necrosis of adipocytes and classification of the panniculitides.

Semin Cutan Med Surg. 2007 Jun

Requena L.
Department of Dermatology, Fundación Jiménez Díaz, Universidad Autónoma, Madrid, Spain. lrequena@fjd.es

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 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.

Keywords: panniculitis; subcutaneous fat; adipocytes; necrosis of the adipocytes

http://www.sciencedirect.com/science?_o ... f05d8d1b21

[patoco]

Neutrophilic panniculitis secondary to bacterial cystitis

Author(s) : Margarete Niebuhr, Bernward Völker, Alexander Kapp, Bettina Wedi

Summary : Neutrophilic panniculitis (NP) is a widely unknown inflammatory disorder of the subcutaneous fat. The diagnosis and proper classification of panniculitis relies on histologic features, mainly the location of the subcutaneous inflammation (septal, lobular, mixed or vascular). In contrast to erythema nodosum, where the inflammation predominates in the fat septa, NP is localized in the fat lobules. However, little is known about this rare condition. We report a 54-year-old woman with a history of several episodes with subcutaneous nodules occurring on the lower legs secondary to bacterial cystitis (successfully treated with antibiotics prior to admission to our Department), that responded immediately to treatment with oral corticosteroids. An infective aetiology for NP has not been described so far, although this can be found in other neutrophilic dermatoses. The originality of our case is the recurrence of the disease on several occasions in association with bacterial cystitis. This case confirms that infections can be a cause of NP.

Keywords : neutrophilic panniculitis, bacterial cystitis

http://www.john-libbey-eurotext.fr/en/r ... /resume.md

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Etiologic spectrum of mesenteric panniculitis: report of 7 cases

Béchade D, Durand X, Desramé J, Rambelo A, Corberand D, Baranger B, Farge D, Algayres JP.
Service de clinique médicale, hôpital du Val-de-Grâce, 74, boulevard de Port-Royal, 75230 Paris cedex 05, France. bechade.dominique@wanadoo.fr

OBJECTIVE: Mesenteric panniculitis is a rare disorder characterized by nonspecific inflammation and/or necrosis and/or fibrosis in the adipose tissue of the bowel mesentery. Its signification, primary or associated with other diseases, is a subject of controversy.

METHODS: A descriptive and retrospective study of patients with an abdominal CT examination showing features of mesenteric panniculitis and for whom biopsy with immunohistochemical examination was obtained in all cases.

RESULTS: Seven patients were enrolled (4 men and 3 women) with a median age of 62,1 years. None of the patients without an identified etiology had a history of abdominal surgery. An associated disease was identified in 4 cases: breast cancer (1), non-Hodgkin's lymphoma based on peripheric lymph nodes biopsy (2) and cryoglobulinemic vascularitis based on renal biopsy (1). In the 3 remaining cases, isolated mesenteric panniculitis was the only abnormality despite thorough imaging and pathologic investigations.

CONCLUSION: Except an obvious malignancy context or a history of abdominal surgery, a pathologic examination of the mesenteric panniculitis lesions is necessary, especially to eliminate another mesenteric disorder. Mesenteric panniculitis is often associated with lymphoma.

http://www.sciencedirect.com/science?_o ... c984db1c53

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Staphylococcus aureus Panniculitis Complicating Juvenile Dermatomyositis

Published online January 29, 2007
PEDIATRICS Vol. 119 No. 2 February 2007, pp. e528-e530 (doi:10.1542/peds.2006-1518

Steven J. Spalding, MD, Manuel P. Meza, MD, Sarangarajan Ranganathan, MD and Raphael Hirsch, MD

Division of Rheumatology, Department of Pediatrics, Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania

ABSTRACT

Panniculitis is a rarely reported manifestation of juvenile dermatomyositis. The 3 previously reported cases of juvenile dermatomyositis and panniculitis were attributed to flare of underlying disease, rather than infection, and were treated with increased immunosuppression. Here we describe a patient with juvenile dermatomyositis who developed panniculitis secondary to Staphylococcus aureus. Patients with juvenile dermatomyositis and panniculitis should have extensive testing for infectious etiologies before increasing their immunosuppressive regimens.

Key Words: dermatomyositis • panniculitis • Staphylococcus

Abbreviations: DM, dermatomyositis • JDM, juvenile dermatomyositis • MSSA, methicillin-sensitive Staphylococcus aureus

Juvenile dermatomyositis (JDM) is a nonsuppurative, inflammatory condition that affects the skin and muscle. Panniculitis is an inflammatory condition of the subcutaneous tissues that typically occurs in association with systemic illnesses such as infection, malignancy, systemic lupus erythematosus,1 juvenile rheumatoid arthritis,2 and DM. Thirteen cases of DM-associated panniculitis have been described in the literature since 1924, and only 3 cases have pertained to children.3–5 None of these children were found to have an infectious source of their panniculitis. Here we present a 15-year-old boy with JDM who was diagnosed with panniculitis caused by methicillin-sensitive Staphylococcus aureus (MSSA).

CASE REPORTS

A 15-year-old white boy with a 2-year history of JDM was referred to our institution by his primary rheumatologist after 7 days of left upper-arm pain, redness, swelling, and low-grade fever. His immunosuppressive regimen consisted of subcutaneous methotrexate (19.4 mg/m2 per week) and intravenous immunoglobulin (2 g/kg per month). His initial presentation of JDM was primarily cutaneous, with mild muscle enzyme elevation and no evidence of weakness. No myositis-specific or -associated antibody testing was performed. Corticosteroids, hydroxychloroquine, and cyclophosphamide had previously failed to control the cutaneous manifestations of his JDM. Eighteen months before this presentation, the patient developed persistent soft tissue abscesses of the upper back caused by MSSA, which required surgical intervention. This infection had been attributed to his immunosuppressive medications.

Physical examination revealed an uncomfortable 15-year-old boy. His BMI was 22.1 kg/m2. Inspection of the skin showed diffuse facial erythema with scaling; heliotrope rash; facial telangiectasias; poikilodermatous macules over the chest, shoulders, and upper back; Gottron's papules over the fingers, elbows, knees, and ankles; and dilated, tortuous nail-fold capillaries. A 12 x 6-cm, intensely tender, firm, warm, erythematous, nonfluctuant mass was palpable within the subcutaneous tissue along the medial aspect of his left arm. There were no other areas of muscle tenderness, and his muscle strength was normal. No lipodystrophy, acanthosis nigricans, or calcinosis was noted.

Initial studies revealed a total white blood cell count of 9.0 x 109/L (reference: 4.5–13.5 x 109/L) with normal differential counts, hemoglobin level of 12.1 g/dL (reference: 13.5–17.5 g/dL), platelet count of 435 x 109/L (reference: 150–350 x 109/L), erythrocyte sedimentation rate of 51 mm/hour (reference: 0–20 mm/hour), C-reactive protein level of 17.7 mg/dL (reference: 0.3–0.7 mg/dL), neopterin level of 48.2 nmol/L (reference: 2–10 nmol/L), and von Willebrand's antigen level of 2.21 U/mL (reference: 0.5–1.60 U/mL). His serum aldolase, creatinine kinase, lactate dehydrogenase, and aspartate aminotransferase levels were all within reference ranges. Plain radiographs of the left upper arm revealed diffuse soft tissue swelling without evidence of bony involvement or calcinosis.

The patient was diagnosed with panniculitis secondary to JDM. An MRI of the left upper limb on the second hospital day was interpreted by a pediatric radiologist to be consistent with this diagnosis (Fig 1). Clinical suspicion for infection was low, but the patient was placed on clindamycin at admission. A wedge biopsy of the lesion was performed on the fourth hospital day as a last confirmatory investigation before beginning pulse methylprednisolone. At biopsy, the muscle and connective tissues were "woody" and indurated. An abscess pocket (not observed on MRI) was found, and fluid was obtained for culture. A Gram-stain of the tissues and fluid showed few white blood cells but no organisms. Surprisingly, cultures of the abscess fluid, muscle, and subcutaneous tissue grew MSSA. Histopathological examination of the biopsy by a pediatric pathologist revealed extensive areas of necrosis with abundant neutrophilic infiltrate involving the fascia, fat, and muscle, with necrotic vessels within the exudate (Fig 2). Lymphoplasmacytic infiltrate was noted in the superficial portions of the panniculus and in a perivascular location within the dermis. Antibiotics were continued and immunosuppressants were held. A neutrophil oxidative burst assay was normal. The patient improved and was discharged to complete a 21-day course of oral clindamycin.

DISCUSSION

Abnormalities of the subcutaneous tissue, including panniculitis and lipodystrophy, have been rarely reported in JDM.3–6 Panniculitis in patients with DM is classically described as painful, erythematous, indurated plaques and nodules that are located primarily on the arms, thighs, and buttocks.3,4 Panniculitis may also be subclinical and only evidenced on biopsy.7 The typical histopathologic appearance includes lobular panniculitis with lymphocytes and plasma cells, perivascular lymphocytic infiltrate, and vacuolar interface changes.3,4,7 These lesions usually respond to corticosteroids but may require other immunomodulating agents including cyclosporine A or methotrexate. This patient's physical examination findings and radiographic studies supported the diagnosis of panniculitis secondary to his underlying disease. However, the neutrophil-predominated histology and positive bacterial culture were evidence of active infection. On biopsy, there was extensive muscle necrosis associated with a suppurative and organizing exudate. The limited lymphoplasmacytic infiltrate was septal and adjacent to areas of suppuration, raising the question as to whether it represented inflammatory changes caused by underlying DM or evidence of chronic infection. The necrotic vessels were also related to the infectious process because they showed coagulative necrosis rather than the typical fibrinoid and neutrophilic vasculitis encountered in DM. Our patient also had histopathologic and radiographic evidence of fasciitis. Fasciitis has not been described in children with JDM and, when described in adults, has been mainly related to infections such as tuberculosis or histoplasmosis.8,9 A Gomori stain and fungal culture of all tissues were negative.

The pathogenesis of the patient's infection is unclear. The severe inflammatory nature of his skin disease, coupled with skin atrophy from long-term steroid use, may have resulted in epidermal microtrauma, which allowed bacteria to gain access to the subcutaneous tissue with compromised perfusion. An alternative mechanism could have been a transient bacteremia that seeded an area of poor vascular flow as a result of preexisting vasculopathy. Finally, the patient's immunosuppressive medications may have increased his susceptibility to bacterial pathogens. This patient was on relatively high doses of methotrexate in conjunction with intravenous immunoglobulin at the time he developed these complications. Methotrexate has been shown to inhibit neutrophil chemotaxis, decrease circulating interleukin 6 levels, and decrease the effects of interleukin 1 on inflammatory cells.10 The resultant antiinflammatory effect may also suppress the immune system's response to bacterial pathogens. Several of the children previously reported were on immunosuppressive therapies at the time they developed panniculitis.4,5 However, none of these children were reported to have had an infectious cause of their panniculitis.

CONCLUSIONS

We have reported the first case of infectious panniculitis and fasciitis in a child with JDM. We present this case as evidence that the possibility of infection must be ruled out via biopsy and tissue culture before increasing the immunosuppressive regimen in children with presumed autoimmune panniculitis.

FOOTNOTES

Accepted Aug 24, 2006.

Address correspondence to Steven J. Spalding, MD, Division of Rheumatology, Children's Hospital of Pittsburgh, 3705 Fifth Ave, Pittsburgh, PA 15213. E-mail: steven.spalding@chp.edu

The authors have indicated they have no financial relationships relevant to this article to disclose.

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