Rem syndrome L98.5

Author: Prof. Dr. med. Peter Altmeyer

Last updated on: 21.09.2023

Synonym(s)

Mucinosis erythematosa reticularis; Mucinosis reticular erythematous; Reticular erythematous mucinosis; Reticular erythomatous mucinosis; Reticulum Erythema Mucinosis; Round-cell erythematosis

Definition
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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.

Etiopathogenesis
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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).

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Dermatology

Papel

Flat, spherical or pointed conical efflorescence protruding above the skin, up to 1.0 cm in diameter (note:...

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

Women are affected twice as often as men.

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

Clinical features
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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.

Histology
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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.

Direct Immunofluorescence
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Mostly negative, rarely IgM, IgA,_{C3 precipitates} are present at the dermatoepidermal junction zone.

Differential diagnosis
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Seborrheic eczema; lupus erythematosus; mucinosis; granuloma anulare; light dermatoses.

External therapy
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Glucocorticoids such as 0.1% mometasone (e.g. Ecural fat cream) or 0.1% triamcinolone cream (e.g. Triamgalen, R259 ) are indicated. Alternatively, inject intralesional glucocorticoids such as triamcinolone crystal suspension (e.g. Volon A diluted 1:3 with LA, e.g. scandicain) every 2 weeks. Recently, successful therapy approaches with a 0.1% tacrolimus ointment have been described (first 2 times/day, later as maintenance dose 1 time / day; off-label use!)

Internal therapy
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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.

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

Literature
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Braddock SW, Davis CS, Davis RB (1988) Reticular erythematous mucinosis and thrombocytopenic purpura. Report of a case and review of the world literature, including plaguelike cutaneous mucinosis. J Am Acad Dermatol 19: 986-988.
Brandl P et al (1991) Reticular erythematous mucinosis (REM). Dt Derm 39: 1438-1441
Cinotti E et al (2014) Reticular erythematous mucinosis: histopathological and immunohistochemical features of 25 patients compared with 25 cases of lupus erythematosus tumidus. J Eur Acad Dermatol Venereol doi: 10.1111/jdv.12654.
Haendchen LC et al (2014) Reticular erythematous mucinosis. Cutis 93:E21-24
Kucukunal A et al (2014) Reticular erythematous mucinosis on the midline of the back. Cutis 93:294-296.
Perry HO et al (1960) Plaque-like form of cutaneous mucinosis. Arch Dermatol 82:980-985.
Quimby SR, Perry HO (1982) Plaque-like cutaneous mucinosis: its relationship to reticular erythematous mucinosis. J Am Acad Dermatol 6: 856-861.
Rongioletti Fet al. (2013) Reticular erythematous mucinosis: a review of patients' characteristics, associated conditions, therapy and outcome in 25 cases. Br J Dermatol 169:1207-1211
Rubegnis R et al (2004) A case of reticular erythematous mucinosis treated with topical tacrolimus. Br J Dermatol 150: 173-174
Simon M et al (1985) Unusually localized REM syndrome. Z Hautkr 60: 1555-1558.
Steigleder GK, Gartmann H, Linker U (1974) REM syndrome: reticular erythematous mucinosis (round-cell erythematosis), a new entity? Br J Dermatol 91: 191-199
Tenea D et al (2023) Reticular erythematous mucinosis in an African woman with HIV infection: case report and literature review. Case Rep Dermatol 15:117-125.
Tominaga A et al (2001) Reticular erythematous mucinosis syndrome with an infiltration of factor XIIIa+ and hyaluronan synthase 2+ dermal dendrocytes. Br J Dermatol 145: 141-145