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Comèl-Netherton-Syndrom mit bakterieller Superinfektion

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Zusammenfassung

Wir berichten über einen 10-jährigen, bosnischen Jungen von nicht konsanguinen Eltern, der sich mit einer ichthyotischen Erythrodermie unklarer Genese und zusätzlich impetigenisierten Herden vorstellte. Das klinische Bild, die feingewebliche Untersuchung sowie die Haarschaftanalysen ergaben die Diagnose eines autosomal-rezessiv vererbten Comèl-Netherton-Syndroms mit bakterieller Superinfektion. Unter einer behutsamen Therapie mit dünn aufgetragenem topischem Tacrolimus (Protopic 0,1%®) kam es zu einer deutlichen Besserung und zu einer Verlängerung der beschwerdefreien Intervalle. Tacrolimus wurde intermittierend und nicht während der akuten Schübe verwandt, sodass auch nach längerer Anwendung eine systemische Resorption von FK506 trotz der gestörten epidermalen Barriere nicht stattfand. Während der zuweilen mit rezidivierenden Pyodermien einhergehenden Schübe wurde Enterotoxin C bildender Staphylococcus aureus isoliert. Es ist möglich, dass dieses Superantigen an der Aggravation beteiligt ist. Die topische Therapie mit Tacrolimus bietet eine einfach durchführbare, bedarfsadaptierte therapeutische Option für diese seltene Genodermatose.

Abstract

A 10-year old boy, the child of unrelated Bosnian parents presented with a superinfected ichthyotic erythroderma. The clinical features, histological findings and hair analysis led to the diagnosis of the autosomal-recessive inherited Comel-Netherton syndrome witch bacterial superinfection. Under careful therapy with small amounts of topical tacrolimus (Protopic 0.1% ®), he improved and had longer disease-free intervals. Tacrolimus was administered intermittently and not during acute flares, thus avoiding systemic resorption even after long-time treatment despite the disturbed epidermal barrier in Comel-Netherton syndrome. Staphylococcus aureus producing enterotoxin C was isolated during flares which were sometimes accompanied by marked bacterial superinfection. It is possible, that this superantigen is involved in the observed aggravation of disease. The topical therapy with tacrolimus is an easy, flexible therapeutic option for this rare genodermatosis.

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Danksagung

Wir danken Dr. H. Ständer für die mikroskopische Haaranalyse.

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Authors and Affiliations

  1. Hautklinik der Westfälischen-Wilhelms-Universität Münster, Münster

    G. Beljan, H. Traupe & D. Metze

  2. Klinik und Poliklinik für Dermatologie und Allergologie, Ulm

    C. Sunderkötter

  3. Hautklinik und Poliklinik für Dermatologie und Venerologie, Westfälische-Wilhelms-Universität Münster, Von-Esmarch-Straße 58, 48149 , Münster

    G. Beljan

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  1. G. Beljan
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  2. H. Traupe
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  3. D. Metze
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  4. C. Sunderkötter
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Correspondence to G. Beljan.

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Beljan, G., Traupe, H., Metze, D. et al. Comèl-Netherton-Syndrom mit bakterieller Superinfektion. Hautarzt 54, 1198–1202 (2003). https://doi.org/10.1007/s00105-003-0572-8

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  • DOI: https://doi.org/10.1007/s00105-003-0572-8

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