REM syndrome: reticular erythematous mucinosis (round-cell erythematosis), a new entity?

Four patients, three females and one male, were observed in Cologne with sheet- or net-like erythema of the upper chest and of the back, with a tendency to spread to the abdomen. The patients had no symptoms with the exception of itching in one case and itching after sunburn in another. Histo-logically the epidermis was normal, apart from slight hydropic degeneration and some minor degree of spongiosis. The main features were: dilated blood vessels in the dermis, with a more or less pronounced perivascular infiltrate of round cells, and, in addition, deposits of a substance which stained with alcian blue. Oral treatment with Rhetis was of some benefit, otherwise therapy, including corticosteroids, was not successful. Other antimalarial drugs were not used. There was some relation to sun exposure, but the full spectrum of xenon-light did not cause any abnormal reactions. Since it was not possible to relate these findings to any known syndrome, a combination of the main signs, reticular erythema and mucinosis, was used to name this entity REM syndrome.