Cytophagic Histiocytic Panniculitis (CHP) was first described in 1980 by Winkelmann as a chronic histiocytic disease of the subcutaneous adipose tissue

Adipose tissue

In histology, adipose tissue or body fat or fat depot or just fat is loose connective tissue composed of adipocytes. It is technically composed of roughly only 80% fat; fat in its solitary state exists in the liver and muscles. Adipose tissue is derived from lipoblasts...

, which is characterized clinically by tender erythematous nodules

Nodule (medicine)

For use of the term nodule in dermatology, see Nodule In medicine, a nodule refers to a relatively hard, roughly spherical abnormal structure....

, recurrent high fever

Fever

Fever is a common medical sign characterized by an elevation of temperature above the normal range of due to an increase in the body temperature regulatory set-point. This increase in set-point triggers increased muscle tone and shivering.As a person's temperature increases, there is, in...

, malaise

Malaise

Malaise is a feeling of general discomfort or uneasiness, of being "out of sorts", often the first indication of an infection or other disease. Malaise is often defined in medicinal research as a "general feeling of being unwell"...

, jaundice

Jaundice

Jaundice is a yellowish pigmentation of the skin, the conjunctival membranes over the sclerae , and other mucous membranes caused by hyperbilirubinemia . This hyperbilirubinemia subsequently causes increased levels of bilirubin in the extracellular fluid...

, organomegaly

Organomegaly

Organomegaly is the abnormal enlargement of organs. For example, clitoromegaly is the enlargement of the clitoris, hepatomegaly is enlargement of the liver, cardiomegaly is enlargement of the heart, and splenomegaly is enlargement of the spleen....

, serosal effusions, pancytopenia

Pancytopenia

Pancytopenia is a medical condition in which there is a reduction in the number of red and white blood cells, as well as platelets.If only two parameters from the full blood count are low, the term bicytopenia can be used...

, hepatic dysfunction and Coagulation

Coagulation

Coagulation is a complex process by which blood forms clots. It is an important part of hemostasis, the cessation of blood loss from a damaged vessel, wherein a damaged blood vessel wall is covered by a platelet and fibrin-containing clot to stop bleeding and begin repair of the damaged vessel...

 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

Panniculitis

Panniculitis is a group of diseases whose hallmark is inflammation of subcutaneous adipose tissue...

with multisystem involvement. But it can also present in a benign form involving only the subcutaneous tissue, thus having a broad clinical spectrum.