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[The REM-syndrome (author's transl)].

J Herzberg

    Zeitschrift Fur Hautkrankheiten
    |October 15, 1981
    PubMed
    Summary

    Five new cases of REM-Syndromes were identified, with four aligning to the original description. These patients exhibit unique clinical and histochemical signs, responding only to antimalarial treatments.

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    Area of Science:

    • Dermatology
    • Histopathology
    • Rare Diseases

    Context:

    • REM-Syndromes represent a rare group of mucinoses.
    • Previous descriptions by Steigleder, Gartmann, and Linker established key characteristics.
    • Distinguishing REM-Syndromes from other cutaneous mucinoses, like Perry et al.'s plaque-like form, is crucial for accurate diagnosis and management.

    Purpose:

    • To report and characterize five new patients diagnosed with REM-Syndromes.
    • To compare these new cases with the established clinical and histochemical features of REM-Syndromes.
    • To highlight the diagnostic challenges and therapeutic responses observed in these patients.

    Summary:

    • Five new patients with REM-Syndromes are presented, with four conforming to the initial Steigleder, Gartmann, and Linker description.
    • The fifth patient presented with distinct clinical and histochemical findings but was still classified within the REM-syndrome spectrum.
    • All reported cases differed from the plaque-like cutaneous mucinosis described by Perry et al.
    • Notable features include asymptomatic presentation, resistance to most therapies except synthetic antimalarials, characteristic localization, and stereotypical clinical signs.
    • Histochemical findings were characteristic, and even standard HE staining suggested the diagnosis.

    Impact:

    • This study expands the understanding of REM-Syndromes by presenting new cases with varied presentations.
    • It reinforces the diagnostic criteria and emphasizes the unique therapeutic response to synthetic antimalarials.
    • The findings aid in differentiating REM-Syndromes from other mucinosis conditions, improving diagnostic accuracy for rare dermatological disorders.

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