Lipoatrophic panniculitis of children
Alerts and Notices
Important News & Links
Synopsis
Lipoatrophic panniculitis, also known as lipophagocytic panniculitis and annular lipoatrophic panniculitis of the ankles, is an extremely rare, predominately lobular panniculitis that most commonly affects the lower extremities of children. It is characterized by recurrent, tender subcutaneous nodules with associated systemic symptoms (eg, fever, malaise, arthralgias). After the acute inflammation subsides, permanent lipoatrophy ensues.
Although this condition can affect adults, it is more common in children, predominantly affecting ages 3-13 years, and girls are affected more frequently than boys. Fewer than 5 of the reported cases to date have also presented with hepatomegaly or splenomegaly.
While the cause of lipoatrophic panniculitis is unknown, it may be associated with other autoimmune or autoinflammatory conditions (eg, insulin-dependent diabetes mellitus, Hashimoto thyroiditis, and juvenile rheumatoid arthritis). Neither physical trauma nor specific viral etiologies have been linked, although prior upper respiratory infections have been reported in some patients prior to onset. A genetic link has been postulated but not confirmed. One report suggests that a mutation in chromosome 10p (where alleles for the interleukin-2 [IL-2] receptor gene reside) is implicated.
Although this condition can affect adults, it is more common in children, predominantly affecting ages 3-13 years, and girls are affected more frequently than boys. Fewer than 5 of the reported cases to date have also presented with hepatomegaly or splenomegaly.
While the cause of lipoatrophic panniculitis is unknown, it may be associated with other autoimmune or autoinflammatory conditions (eg, insulin-dependent diabetes mellitus, Hashimoto thyroiditis, and juvenile rheumatoid arthritis). Neither physical trauma nor specific viral etiologies have been linked, although prior upper respiratory infections have been reported in some patients prior to onset. A genetic link has been postulated but not confirmed. One report suggests that a mutation in chromosome 10p (where alleles for the interleukin-2 [IL-2] receptor gene reside) is implicated.
Codes
ICD10CM:
M79.3 – Panniculitis, unspecified
SNOMEDCT:
238881001 – Lipoatrophic panniculitis
M79.3 – Panniculitis, unspecified
SNOMEDCT:
238881001 – Lipoatrophic panniculitis
Look For
Subscription Required
Diagnostic Pearls
Subscription Required
Differential Diagnosis & Pitfalls
To perform a comparison, select diagnoses from the classic differential
Subscription Required
Best Tests
Subscription Required
Management Pearls
Subscription Required
Therapy
Subscription Required
References
Subscription Required
Last Reviewed:01/31/2022
Last Updated:02/01/2022
Last Updated:02/01/2022