Pluriorificial Ectodermosis L51.-

Last updated on: 19.01.2025

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Definition
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Pluriorificial ectodermosis is a rare mucocutaneous inflammatory syndrome (minus variant of erythema multiforme), which is characterized by a preferential infestation of the mucous membranes rather than the skin. The mucous membranes of the mouth and lips are frequently affected. Initially, blisters appear, which can develop into painful, polycyclic erosions. Eye, genital, anal or nasal infestations can be affected. Skin lesions are usually absent. The general symptoms vary in intensity.

Etiopathogenesis
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In children, pluriorificial ectodermosis is mainly associated with infections caused by Mycoplasma pneumoniae or Herpes simplex. M>w! Recurrences occur but are rather rare. In adults, recurrences may be associated with recurrent herpes simplex.

Case report(s)
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Four male patients with an average age of 15.2 years had hemorrhagic cheilitis, erosive gingivostomatitis and bilateral conjunctivitis. Three of them had erosive infestation of the external genitalia. Two patients had cocardial lesions on the hands.

A post-herpetic cause was present in one case, in which an episode of cutaneous herpes had occurred one week previously. In the other three cases, an infection with Mycoplasma pneumoniae (MP) was suspected due to the presence of a dry cough, asthenia and an interstitial syndrome on chest X-ray. This could only be confirmed in one of the three cases, where the MP serology was IgG- and IgM-positive. All patients were treated with systemic corticosteroid therapy and corticosteroids as mouth rinses, which led to a significant improvement. Two out of four cases also received clarythromicin-based antibiotic therapy (Lofgren D et al. 2021).

Literature
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  1. Lofgren D et al. (2021) Mycoplasma Pneumoniae-Induced Rash and Mucositis: A Systematic Review of the Literature. Spartan Med Res J 30: 6:25284.
  2. Kircher W (1958). On a case of ectodermosis érosive pluriorificielle (Flessinger-Rendu); a further contribution to the question of the connection of this disease with staphylococcal sepsis. Helv Paediatr Acta 13:239-243.
  3. Keizer DP (1951) Ectodermosis erosiva pluriorificialis rendu-Fiessinger-Stevens Johnson. Geneeskd Gids 29:351-356.
  4. Kumer L. (1936) Ectodermosis érosive pluriorificielle and erythema exsudativum multiforme Dermatologische Zeitschrift 72: 62-70.
  5. Martínez-Pérez M et al. (2019) Mycoplasma pneumoniae-Induced Mucocutaneous Rash: A New Syndrome Distinct from Erythema Multiforme? Report of a New Case and Review of the Literature. Actas Dermosifiliogr 107:e47-51.
  6. Gise R et al. (2020) Mycoplasma pneumonia-induced rash and mucositis: a longitudinal perspective and proposed management criteria. Am J Ophthalmol 219: 351-356
  7. Schwarze E (1966) On the ectodermosis pluriorificialis erosiva Fiessinger-Rendu (Syndroma muco-cutaneo-oculare acutum Fuchs). Z Haut Geschlechtskr 21:167-169.

Incoming links (1)

RIME;

Outgoing links (1)

Erythema multiforme;

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

Last updated on: 19.01.2025