Panniculitis is a group of diseases whose hallmark is
inflammation of
subcutaneous Subcutaneous may refer to:
* Subcutaneous injection
* Subcutaneous tissue
The subcutaneous tissue (), also called the hypodermis, hypoderm (), subcutis, superficial fascia, is the lowermost layer of the integumentary system in vertebrates. The ...
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 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 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 or
histiocytosis;
*
pancreatitis 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
*
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 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, 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.
There are thus four main histological subtypes:
# lobular panniculitis without vasculitis (acute panniculitis, previously termed
Weber–Christian disease,
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 Subcutaneous may refer to:
* Subcutaneous injection
* Subcutaneous tissue
The subcutaneous tissue (), also called the hypodermis, hypoderm (), subcutis, superficial fascia, is the lowermost layer of the integumentary system in vertebrates. The ...
adipose tissue, which is characterized clinically by tender
erythematous 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,
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,
serosal 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 cou ...
,
hepatic dysfunction and
coagulation abnormalities.
CHP may occur either isolated or as part of cutaneous manifestations of
hemophagocytic syndrome (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.
* ''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, and oils.
With needle-shaped clefts
Lipodermatosclerosis is a form of panniculitis associated with
chronic venous insufficiency that presents with brown indurations on the front of the shins. It may be associated with pain and other signs of
chronic venous insufficiency. 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 births.
*
Weber–Christian disease, 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 oils.
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
infections, 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,
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,
pregnancy, 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
cancers, including
Non-Hodgkin lymphoma 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 panniculitis
is a panniculitis associated with a deficiency of the α
1-antitrypsin enzyme.
Treatment
See also
*
Panniculus carnosus
*
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