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

Abstract

SUMMARY 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.

Reticular erythematous mucinosis: a review of patients' characteristics, associated conditions, therapy and outcome in 25 cases

“…Reticular erythematous mucinosis (REM) is a rare condition first described in 1974 by Steigleder et al 1 and classified into the group of primary cutaneous mucinoses. However, its nosology, and in particular its relationship with cutaneous lupus erythematosus (CLE), has been the subject of controversies.…”

“…1,[5][6][7] This controversy is not easy to resolve, as there is also some debate as to whether LET itself is a separate subtype of chronic CLE, or a nonspecific manifestation within the lupus erythematosus classification system. 8,9 Our present study includes the largest series of cases of REM ever reported in the literature.…”

Treatment of Reticular Erythematous Mucinosis With UV-A1 Radiation

“…[1][2][3][4][5][6][7][8][9][10][11][12] Other treatment alternatives (UV-B, laser, glucocorticoids, cyclosporine) have had variable efficacy.…”

“…[1][2][3][4][5][6][7][8][9][10][11][12] Other treatment alternatives (UV-B, laser, glucocorticoids, cyclosporine) have had variable efficacy. [1][2][3][4][5][6][7][8][9][10][11][12] Since treatment with chloroquine was refused by the patient, another therapeutic option was needed. Therefore, UV-A1 (340-400 nm) therapy was considered.…”

“…Several factors have been associated with the induction of the syndrome, including viral processes, immunological disturbances, and solar irradiation. [1][2][3][4][5][6][7][8] …”