Panniculitis is a group of diseases whose hallmark is
inflammation
Inflammation (from la, wikt:en:inflammatio#Latin, inflammatio) is part of the complex biological response of body tissues to harmful stimuli, such as pathogens, damaged cells, or Irritation, irritants, and is a protective response involving im ...
of
subcutaneous adipose tissue (the fatty layer under the skin –
panniculus adiposus
The panniculus adiposus is the fatty layer of the subcutaneous tissues, superficial to a deeper vestigial layer of muscle, the panniculus carnosus.McGrath, J.A.; Eady, R.A.; Pope, F.M. (2004). ''Rook's Textbook of Dermatology'' (Seventh Edition). B ...
). Symptoms include tender skin
nodules
Nodule may refer to:
*Nodule (geology), a small rock or mineral cluster
*Manganese nodule, a metallic concretion found on the seafloor
*Nodule (medicine), a small aggregation of cells
*Root nodule
Root nodules are found on the roots of plants, p ...
, and systemic signs such as
weight loss
Weight loss, in the context of medicine, health, or physical fitness, refers to a reduction of the total body mass, by a mean loss of fluid, body fat (adipose tissue), or lean mass (namely bone mineral deposits, muscle, tendon, and other conn ...
and
fatigue
Fatigue describes a state of tiredness that does not resolve with rest or sleep. In general usage, fatigue is synonymous with extreme tiredness or exhaustion that normally follows prolonged physical or mental activity. When it does not resolve ...
.
Restated, an inflammatory disorder primarily localized in the subcutaneous fat is termed a "panniculitis", a group of disorders that may be challenging both for the clinician and the dermatopathologist.
The general term for inflammation of any adipose tissue is
steatitis.
Signs and symptoms
Panniculitis can also be classified based on the presence or absence of systemic
symptoms
Signs and symptoms are the observed or detectable signs, and experienced symptoms of an disease, illness, injury, or condition. A sign for example may be a higher or lower temperature than normal, raised or lowered blood pressure or an abnormali ...
. Panniculitis without systemic disease can be a result of
trauma
Trauma most often refers to:
*Major trauma, in physical medicine, severe physical injury caused by an external source
*Psychological trauma, a type of damage to the psyche that occurs as a result of a severely distressing event
*Traumatic inju ...
or cold.
Panniculitis with systemic disease can be caused by:
*
connective tissue
Connective tissue is one of the four primary types of animal tissue, along with epithelial tissue, muscle tissue, and nervous tissue. It develops from the mesenchyme derived from the mesoderm the middle embryonic germ layer. Connective tiss ...
disorders such as
lupus erythematosus or
scleroderma;
*
lymphoproliferative
Lymphoproliferative disorders (LPDs) refer to a specific class of diagnoses, comprising a group of several conditions, in which lymphocytes are produced in excessive quantities. These disorders primarily present in patients who have a compromised ...
disease such as
lymphoma
Lymphoma is a group of blood and lymph tumors that develop from lymphocytes (a type of white blood cell). In current usage the name usually refers to just the cancerous versions rather than all such tumours. Signs and symptoms may include enlar ...
or
histiocytosis;
*
pancreatitis
Pancreatitis is a condition characterized by inflammation of the pancreas. The pancreas is a large organ behind the stomach that produces digestive enzymes and a number of hormones. There are two main types: acute pancreatitis, and chronic pancr ...
or
pancreatic cancer
Pancreatic cancer arises when cell (biology), cells in the pancreas, a glandular organ behind the stomach, begin to multiply out of control and form a Neoplasm, mass. These cancerous cells have the malignant, ability to invade other parts of t ...
;
* sarcoidosis with cutaneous involvement (seen in up to 20 percent);
*
Alpha 1-antitrypsin deficiency
Alpha-1 antitrypsin deficiency (A1AD or AATD) is a genetic disorder that may result in lung disease or liver disease. Onset of lung problems is typically between 20 and 50 years of age. This may result in shortness of breath, wheezing, or an inc ...
*
Crohn's disease
Crohn's disease is a type of inflammatory bowel disease (IBD) that may affect any segment of the gastrointestinal tract. Symptoms often include abdominal pain, diarrhea (which may be bloody if inflammation is severe), fever, abdominal distension ...
This is not a complete list of possible causes.
Associated conditions
Lipoatrophy
Lipoatrophy is the term describing the localized loss of fat tissue. This may occur as a result of subcutaneous injections of insulin in the treatment of diabetes, from the use of human growth hormone or from subcutaneous injections of copaxone use ...
or
lipodystrophy (the loss of subcutaneous adipose tissue) can occur in any of these conditions.
Diagnosis
Classification
It can occur in any fatty tissue (
cutaneous or visceral) and is often diagnosed on the basis of a deep skin
biopsy
A biopsy is a medical test commonly performed by a surgeon, interventional radiologist, or an interventional cardiologist. The process involves extraction of sample cells or tissues for examination to determine the presence or extent of a diseas ...
, and can be further classified by
histological characteristics based on the location of the inflammatory cells (within fatty lobules or in the septa which separate them) and on the presence or absence of
vasculitis
Vasculitis is a group of disorders that destroy blood vessels by inflammation. Both arteries and veins are affected. Lymphangitis (inflammation of lymphatic vessels) is sometimes considered a type of vasculitis. Vasculitis is primarily caused b ...
.
There are thus four main histological subtypes:
# lobular panniculitis without vasculitis (acute panniculitis, previously termed
Weber–Christian disease
Weber–Christian disease, is a cutaneous condition characterized by recurrent subcutaneous nodules that heal with depression of the overlying skin.
It is a type of panniculitis.
It is a rare disease seen in females 30–60 years of age. It is a r ...
,
systemic nodular panniculitis)
# lobular panniculitis with vasculitis
# septal panniculitis without vasculitis
# septal panniculitis with vasculitis
Lobular
=With vasculitis
=
Erythema induratum, or "Bazin disease", is a panniculitis on the back of the calves. It was formerly thought to be a reaction to the
tuberculum bacillus. It is now considered a panniculitis that is not associated with a single defined pathogen.
Nodular vasculitis is a skin condition characterized by small, tender, reddened nodules on the legs, mostly on the calves and shins. Microscopically there are epithelioid granulomas and vasculitis in the subcutaneous tissue, making it a form of panniculitis. Most of these cases are now thought to be manifestation of tuberculosis and indeed they respond well to anti-tuberculous treatment.
=Without vasculitis
=
Non-vasculitis forms of panniculitis that may occur include:
* ''Cytophagic histiocytic panniculitis'' was first described in 1980 by Winkelmann as a chronic
histiocytic disease of the
subcutaneous adipose tissue, which is characterized clinically by tender
erythematous
Erythema (from the Greek , meaning red) is redness of the skin or mucous membranes, caused by hyperemia (increased blood flow) in superficial capillaries. It occurs with any skin injury, infection, or inflammation. Examples of erythema not assoc ...
nodules
Nodule may refer to:
*Nodule (geology), a small rock or mineral cluster
*Manganese nodule, a metallic concretion found on the seafloor
*Nodule (medicine), a small aggregation of cells
*Root nodule
Root nodules are found on the roots of plants, p ...
, recurrent high
fever
Fever, also referred to as pyrexia, is defined as having a body temperature, temperature above the human body temperature, normal range due to an increase in the body's temperature Human body temperature#Fever, set point. There is not a single ...
,
malaise
As a medical term, malaise is a feeling of general discomfort, uneasiness or lack of wellbeing and often the first sign of an infection or other disease. The word has existed in French since at least the 12th century.
The term is often used ...
,
jaundice
Jaundice, also known as icterus, is a yellowish or greenish pigmentation of the skin and sclera due to high bilirubin levels. Jaundice in adults is typically a sign indicating the presence of underlying diseases involving abnormal heme meta ...
,
organomegaly Organomegaly is the abnormal enlargement of organs. For example, cardiomegaly is enlargement of the heart. ''Visceromegaly'' is the enlargement of abdominal organs. Examples of visceromegaly are enlarged liver (hepatomegaly), spleen (splenomegaly
...
,
serosal
The serous membrane (or serosa) is a smooth membrane, tissue membrane of mesothelium lining the contents and inner walls of body cavity, body cavities, which secrete serous fluid to allow lubricated sliding (motion), sliding movements between op ...
effusions,
pancytopenia
Pancytopenia is a medical condition in which there is significant reduction in the number of almost all blood cells (red blood cells, white blood cells, platelets, monocytes, lymphocytes, etc.).
If only two parameters from the complete blood coun ...
,
hepatic
The liver is a major organ only found in vertebrates which performs many essential biological functions such as detoxification of the organism, and the synthesis of proteins and biochemicals necessary for digestion and growth. In humans, it i ...
dysfunction and
coagulation
Coagulation, also known as clotting, is the process by which blood changes from a liquid to a gel, forming a blood clot. It potentially results in hemostasis, the cessation of blood loss from a damaged vessel, followed by repair. The mechanism o ...
abnormalities.
CHP may occur either isolated or as part of cutaneous manifestations of
hemophagocytic syndrome
Hemophagocytic lymphohistiocytosis (HLH), also known as haemophagocytic lymphohistiocytosis (British spelling), and hemophagocytic or haemophagocytic syndrome, is an uncommon hematologic disorder seen more often in children than in adults. It is a ...
(HPS). CHP is a rare and often fatal form of panniculitis with multisystem involvement. But it can also present in a benign form involving only the subcutaneous tissue, thus having a broad clinical spectrum.
* ''Traumatic panniculitis'' is a panniculitis that occurs following trauma to the skin.
* ''Cold panniculitis'' is a panniculitis occurring after exposure to cold, most often seen in infants and young children.
This condition has been described in children who suck ice or
popsicles, and therefore is sometimes referred to as "popsicle panniculitis."
The term was coined when a patient with a rash of unknown origin on her cheek was taken to a
dermatologist.
* ''Gouty panniculitis'' is a panniculitis caused by deposition of
uric acid crystals in
gout
Gout ( ) is a form of inflammatory arthritis characterized by recurrent attacks of a red, tender, hot and swollen joint, caused by deposition of monosodium urate monohydrate crystals. Pain typically comes on rapidly, reaching maximal intensit ...
.
* ''Pancreatic panniculitis'' (also known as enzymatic panniculitis, Pancreatic fat necrosis,
and subcutaneous fat necrosis) is a panniculitis most commonly associated with
pancreatic carcinoma
Pancreatic cancer arises when cells in the pancreas, a glandular organ behind the stomach, begin to multiply out of control and form a mass. These cancerous cells have the ability to invade other parts of the body. A number of types of pancre ...
, and more rarely with anatomic pancreatic abnormalities,
pseudocysts, or drug-induced pancreatitis.
* ''Factitial panniculitis'' is a panniculitis that may be induced by the injection of organic materials,
povidone,
feces
Feces ( or faeces), known colloquially and in slang as poo and poop, are the solid or semi-solid remains of food that was not digested in the small intestine, and has been broken down by bacteria in the large intestine. Feces contain a relati ...
,
saliva
Saliva (commonly referred to as spit) is an extracellular fluid produced and secreted by salivary glands in the mouth. In humans, saliva is around 99% water, plus electrolytes, mucus, white blood cells, epithelial cells (from which DNA can be ...
,
vaginal fluid
Vaginal discharge is a mixture of liquid, cells, and bacteria that lubricate and protect the vagina. This mixture is constantly produced by the cells of the vagina and cervix, and it exits the body through the vaginal opening. The composition, amou ...
, and oils.
With needle-shaped clefts
Lipodermatosclerosis
Lipodermatosclerosis is a skin and connective tissue disease. It is a form of lower extremity panniculitis,Bruce AJ. et al., Lipodermatosclerosis: Review of cases evaluated at Mayo Clinic. J Am Acad Dermatol. 2002. an inflammation of the layer of f ...
is a form of panniculitis associated with
chronic venous insufficiency
Chronic venous insufficiency (CVI) is a medical condition in which blood pools in the veins, straining the walls of the vein. The most common cause of CVI is superficial venous reflux which is a treatable condition. As functional venous valves are ...
that presents with brown indurations on the front of the shins. It may be associated with pain and other signs of
chronic venous insufficiency
Chronic venous insufficiency (CVI) is a medical condition in which blood pools in the veins, straining the walls of the vein. The most common cause of CVI is superficial venous reflux which is a treatable condition. As functional venous valves are ...
. The exact cause is unknown.
[Bruce AJ. et al., Lipodermatosclerosis: Review of cases evaluated at Mayo Clinic. J Am Acad Dermatol. 2002.]
Other forms include:
*
Subcutaneous fat necrosis of the newborn, a form of panniculitis occurring in
newborns that is usually self-resolving, that may be a result of
hypoxic injury to relatively high levels of
brown fat.
*
Sclerema neonatorum
Sclerema neonatorum is a rare and severe skin condition that is characterized by diffuse hardening of the subcutaneous tissue with minimal inflammation. It usually affects premature, ill newborns. Prognosis is poor.
Minimal inflammation helps di ...
, affecting
premature birth
Preterm birth, also known as premature birth, is the birth of a baby at fewer than 37 weeks gestational age, as opposed to full-term delivery at approximately 40 weeks. Extreme preterm is less than 28 weeks, very early preterm birth is between 2 ...
s.
*
Weber–Christian disease
Weber–Christian disease, is a cutaneous condition characterized by recurrent subcutaneous nodules that heal with depression of the overlying skin.
It is a type of panniculitis.
It is a rare disease seen in females 30–60 years of age. It is a r ...
, a symmetrical form of the disease of unknown origin occurring in middle-aged women.
*
Lupus erythematosus panniculitis
Lupus erythematosus panniculitis presents with subcutaneous nodules that are commonly firm, sharply defined and nontender.
See also
* Lupus erythematosus
* List of cutaneous conditions
Many skin conditions affect the human integumentar ...
, panniculitis associated with
lupus erythematosus.
* Forms associated with use of high doses of systemic corticosteroids during rapid corticosteroid withdrawal, and from the injection of
silicone
A silicone or polysiloxane is a polymer made up of siloxane (−R2Si−O−SiR2−, where R = organic group). They are typically colorless oils or rubber-like substances. Silicones are used in sealants, adhesives, lubricants, medicine, cooking ...
or
mineral oil
Mineral oil is any of various colorless, odorless, light mixtures of higher alkanes from a mineral source, particularly a distillate of petroleum, as distinct from usually edible vegetable oils.
The name 'mineral oil' by itself is imprecise, ...
s.
Septal
=Erythema nodosum
=
Erythema nodosum is a form of panniculitis characterised by tender red nodules, 1–10 cm, associated with systemic symptoms including fever, malaise, and joint pain. Nodules may become bluish-purple, yellowing, and green, and subside over a period of 2–6 weeks without ulcerating or scarring. Erythema nodosum is associated with
infection
An infection is the invasion of tissues by pathogens, their multiplication, and the reaction of host tissues to the infectious agent and the toxins they produce. An infectious disease, also known as a transmissible disease or communicable dise ...
s, including
Hepatitis C
Hepatitis C is an infectious disease caused by the hepatitis C virus (HCV) that primarily affects the liver; it is a type of viral hepatitis. During the initial infection people often have mild or no symptoms. Occasionally a fever, dark urine, a ...
,
EBV and
tuberculosis
Tuberculosis (TB) is an infectious disease usually caused by '' Mycobacterium tuberculosis'' (MTB) bacteria. Tuberculosis generally affects the lungs, but it can also affect other parts of the body. Most infections show no symptoms, in ...
,
Crohn's disease
Crohn's disease is a type of inflammatory bowel disease (IBD) that may affect any segment of the gastrointestinal tract. Symptoms often include abdominal pain, diarrhea (which may be bloody if inflammation is severe), fever, abdominal distension ...
and
sarcoidosis
Sarcoidosis (also known as ''Besnier-Boeck-Schaumann disease'') is a disease involving abnormal collections of inflammatory cells that form lumps known as granulomata. The disease usually begins in the lungs, skin, or lymph nodes. Less commonly af ...
,
pregnancy
Pregnancy is the time during which one or more offspring develops ( gestates) inside a woman's uterus (womb). A multiple pregnancy involves more than one offspring, such as with twins.
Pregnancy usually occurs by sexual intercourse, but ca ...
, medications including
sulfonamides
In organic chemistry, the sulfonamide functional group (also spelled sulphonamide) is an organosulfur group with the structure . It consists of a sulfonyl group () connected to an amine group (). Relatively speaking this group is unreactive. ...
, and some
cancer
Cancer is a group of diseases involving abnormal cell growth with the potential to invade or spread to other parts of the body. These contrast with benign tumors, which do not spread. Possible signs and symptoms include a lump, abnormal b ...
s, including
Non-Hodgkin lymphoma
Non-Hodgkin lymphoma (NHL), also known as non-Hodgkin's lymphoma, is a group of blood cancers that includes all types of lymphomas except Hodgkin lymphomas. Symptoms include enlarged lymph nodes, fever, night sweats, weight loss, and tiredness. ...
and
pancreatic cancer
Pancreatic cancer arises when cell (biology), cells in the pancreas, a glandular organ behind the stomach, begin to multiply out of control and form a Neoplasm, mass. These cancerous cells have the malignant, ability to invade other parts of t ...
.
=A1AT-deficiency-associated
=
Alpha-1 antitrypsin deficiency
Alpha-1 antitrypsin deficiency (A1AD or AATD) is a genetic disorder that may result in lung disease or liver disease. Onset of lung problems is typically between 20 and 50 years of age. This may result in shortness of breath, wheezing, or an inc ...
panniculitis
is a panniculitis associated with a deficiency of the α
1-antitrypsin enzyme.
Treatment
See also
*
Panniculus carnosus
The panniculus carnosus is a part of the subcutaneous tissues in vertebrates. It is a layer of striated muscle deep to the panniculus adiposus.McGrath, J.A.; Eady, R.A.; Pope, F.M. (2004). ''Rook's Textbook of Dermatology'' (Seventh Edition). Black ...
*
List of cutaneous conditions
Many skin conditions affect the human integumentary system—the organ system covering the entire surface of the body and composed of skin, hair, nails, and related muscle and glands. The major function of this system is as a barrier against t ...
References
External links
*
*
{{Disorders of subcutaneous fat
Conditions of the subcutaneous fat
Soft tissue disorders