PANNICULITIS




PATIENT STORY



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A 26-year-old Caucasian woman presents for evaluation of painful, red nodules on her bilateral shins that started several days ago. The nodules were initially bright red. They are now turning darker and becoming flatter. She had a similar occurrence several months ago where she developed a bruise-like discoloration for several weeks that resolved without scarring. Her review of systems is positive for a low-grade fever, malaise, and some joint pains. Additionally, she admits to intermittent bouts of abdominal pain, as well as a few episodes of bloody diarrhea.



This patient was ultimately diagnosed with erythema nodosum that was found to be associated with inflammatory bowel disease. Figures 59-1 and 59-2 demonstrate characteristic erythema nodosum on the classic locations. She was prescribed rest and nonsteroidal anti-inflammatory drugs, and was referred to a gastroenterologist. After initiating treatment for Crohn disease, the nodules healed without scarring and did not recur.




FIGURE 59-1


Erythema nodosum in a patient with underlying Kikuchi disease. (Photograph courtesy of Matthew Zirwas, MD.)






FIGURE 59-2


Erythema nodosum in a patient with no underlying abnormality. (Photograph courtesy of Matthew Zirwas, MD.)






EPIDEMIOLOGY



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Erythema Nodosum





  • The most common panniculitis that is typically seen in young women between the ages of 20 and 40. Erythema nodosum is seen 3 to 6 times more frequently in women than men. An underlying cause can be found in approximately two-thirds of cases.1



  • In children, it is most commonly associated with streptococcal pharyngitis or perianal infection.



  • In adults, it is most commonly associated with drug ingestion, sarcoidosis, or upper respiratory tract infection. Table 59-1 presents the most common associations seen in teenagers and adults.





TABLE 59-1.Common Associations of Erythema Nodosum and Clinical Pearls



Panniculitis Associated With Systemic Disease





  • Lupus panniculitis (or lupus profundus)—may be seen in up to 2% to 3% of patients with systemic lupus.2 It may also be seen independently of systemic lupus. Similar but less frequent panniculitides may be seen in dermatomyositis and scleroderma.



  • Pancreatic panniculitis—occurs in 2% to 3% of patients with pancreatic disease.3



  • Alpha-1-antitrypsin deficiency—this manifestation is less frequent than the characteristic liver and lung disease.4




Infectious Panniculitis





  • Occurs primarily in patients who are immunocompromised. This panniculitis is usually the result of hematogenous spread with seeding of organisms into the subcutaneous fat.




Traumatic and Factitial Panniculitis





  • Traumatic panniculitis—prevalence is difficult to assess. In adults this is most commonly seen as a tender breast mass that resolves without treatment. Cold panniculitis occurs in cold weather and is associated with low-grade trauma, classically in horseback riders corresponding to the areas of the thighs that rest on the saddle.



  • Factitial panniculitis—occurs after the injection of exogenous substances into the subcutaneous fat.





ETIOLOGY AND PATHOPHYSIOLOGY



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Erythema Nodosum





  • Hypersensitivity reaction to an underlying inflammatory state.




Panniculitis Associated With Systemic Disease





  • Lupus panniculitis—a specific autoimmune inflammatory condition that may be partially related to sensitization from traumatized subcutaneous fat.



  • Pancreatic panniculitis—associated but not fully explained by lipase release into the blood stream from an inflamed pancreas.



  • Alpha-1-antitrypsin deficiency—triggered by unopposed activity of numerous catabolic enzymes.




Infectious Panniculitis





  • Caused by hematogenous spread of infection, resulting in thrombosis of deep subcutaneous vasculature and extension of organisms into the deep subcutaneous fat. In hematogenous cases, the primary entry point for the organism is via the lungs.




Traumatic and Factitial Panniculitis





  • Traumatic panniculitis—initiated by abrupt vascular insufficiency, and results in an inflammatory response to encapsulate necrotic adipocytes.



  • Factitial panniculitis—induced by inflammatory histiocytes attempting to encapsulate foreign material.





DIAGNOSIS



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  • There are no pathognomonic clinical findings; consequently, an accurate diagnosis of all cases of panniculitis requires an early deep incisional or punch biopsy containing copious subcutaneous fat (Figure 59-3).





FIGURE 59-3


Demonstration of fat architecture that underlies the primary classification of panniculitis. Inflammation is classified as predominantly lobular or septal. (Photograph courtesy of Rebecca Ziegler, MD.)

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Jan 13, 2019 | Posted by in CARDIOLOGY | Comments Off on PANNICULITIS

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