Rothmann Makai Syndrome

At a glance

A very rare variant of lobular panniculitis, presenting with numerous large subcutaneous lesions without visceral involvement.

Synonym

Lipogranulomatosis Subcutanea.

History

First described by M. Rothman, a German pathologist, and E. Makai, a Hungarian surgeon, in 1900.

Incidence

Very rare. Occurs most frequently in children and middle-aged females.

Genetic inheritance

Belongs to the group of panniculitis. The differential diagnosis between Weber-Christian disease, Rothmann-Makai, and lipophagic panniculitis is made histologically.

Clinical aspects

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 raised. Oral corticosteroids and tetracycline have been reported to be effective treatment.

Anesthetic considerations

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.

Other condition to be considered

• ☞Weber-Christian Disease: Rare skin disorders characterized by single or multiple, tender or painful subcutaneous nodules (1-2 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.

References