A very rare variant of lobular panniculitis, presenting with
numerous large subcutaneous lesions, affecting children and occurring within the
first 12 months of age.
First described by M. Rothman, a German pathologist, and
E. Makai, a Hungarian surgeon, in 1900.
Very rare. Belongs to the group
of panniculitis. The differential diagnosis between Weber-Christian disease,
Rothmann-Makai, and lipophagic panniculitis is only made histologically.
In the Rothmann-Makai type, the nodules are small
and multiple and seem to be self-limited with no severe systemic
involvement. Histologically, the lesions are most marked in the fat. There
are dermal changes with foci of small round cells and macrophages around
blood vessels and appendages. Presence of granulomata composed of
macrophages, fibroblasts, multinucleate cells, and polymorphs. There is absence of
caseation. The panniculitis is an inflammation of subcutaneous fat responsible for
fairly uniform clinical features including nodules and plaques, or swelling
located in the subcutaneous tissue, occasionally progressing to atrophy of
subcutaneous fat. The hypothesis of an abnormal fragility of adipose tissue
or of a failure in the mechanisms of protection against lipophagia has been
The differential diagnosis with the
Weber-Christian Syndrome should be evaluated. In case of doubt, the
patient should be treated as having Weber-Christian Disease. Intraoperative proper
pressure point padding and positioning of the patient should be meticulous
because interruption of vascular and oxygen supply seems to be a trigger.
Vascular access placement and fixation can be very difficult because of skin lesions.
Weber-Christian Disease: Rare skin
disorders characterized by single or multiple, tender or painful
subcutaneous nodules (1 to 2 cm) leading to the development of panniculitis.
The legs and feet are most often affected; however, it can also erupt under
the arms, abdomen, and face. It is associated with fever, generalized
malaise, myalgia, and abdominal pain. The clinical presentation is variable
and may subside after a short period of a few days or weeks and recur months
or years later to become chronic.
Burford JC, Clarke DM: Lipogranulomatosis subcutanea of Rothman-Maki.
Aust J Dermatol 13(3):117, 1972.
Winkelmann RK, Mc Evoy MT, Peters MS: Lipophagic panniculitis of childhood
[review]. J Am Acad Dermatol