Rem syndrome L98.5

A mucinous disease with erythema, papules and plaques on the chest (décolleté) and upper back.

It is debated whether REM syndrome is closely related or identical to the plaque-like form of cutaneous mucinosis described by HO Perry as early as 1960.

Unexplained. Increased photosensitivity (the opposite has also been reported), viral triggering or other immunological triggers are discussed. Connections with tumor diseases (in larger collectives increased rectal and breast carcinomas were detectable), hyperthyroidism, hepatitis C, ulcerative colitis and Borrelia infections have to be examined. Furthermore, relations to lupus erythematosus have to be clarified.

Of note is the coincidence of REM syndrome with HIV infection in a young African woman (Tenea D et al. 2023).

Mostly occurring in adulthood (30-50 years, average 45 years).

Women are affected twice as often as men.

The centre of the chest and/or back is affected, more rarely the upper abdomen, elbows and face.

Reticular to areal, irregularly configured, bright red, rarely brownish, possibly slightly urticarial, mostly asymptomatic (in 10% of patients itching and/or burning), surface-smooth, disseminated erythema, papules and plaques. Reticular patterns are often recognizable.

Approximately 30% of patients exacerbate after sun exposure.

However, experimental UV provocations are usually not triggerable.

Other patients report improvement after sunlight exposure.

The epidermis is inconspicuous except for minor spongiosis. The basement membrane zone is inconspicuous. Mucin deposits are present in the dermis. Perivascular, partly perifollicular round cell infiltrate, predominantly of lymphocytes, is localized in the upper stratum reticulare. Furthermore, Alzian blue or Hale reactive precipitates are detectable.

Good successes have been described with the administration of chloroquine (e.g. Resochin: 3-4 mg/kgkgKG p.o.) and hydroxychloroquine (e.g. Quensyl) as mono- or combination therapy at an average dosage of approx. 250 mg/day. Reduction of the dose to 125 mg/day after 2-3 weeks. Average duration of therapy until the skin changes heal 2-3 months. Maximum cumulative total dose of 100 g! Cave! Initial quarterly ophthalmological check-ups.

Chronic course. Spontaneous remission possible at any time. The average duration of illness was 14.7 months (1-20 months).

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