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[Classification and scope of clinical variations of Melkersson-Rosenthal syndrome]
O P Hornstein1, N Stosiek, A Schönberger
1Dermatologische Universitäts-Klinik Erlangen.
Abstract:
Apart from the apparent trias of oro-facial swellings, facial paresis, and lingua plicata (LP), Melkersson-Rosenthal syndrome (MRS) comprises a variety of complex signs and symptoms. During the last 18 years, 73 patients suffering from MRS were admitted to our hospital. Re-examination of 42 out of these patients and evaluation of all data available proved preceding facial paresis(es) in only 34% of all cases and LP of various degrees in 52%. In more than 80%, however, we found vasomotoric, sialo-secretory, or other neurovegetative "minor signs", locally and/or temporally connected with swellings of either skin or mucosa. Since such minor signs are essential for the diagnosis and the understanding of both "complete" and "incomplete" forms of MRS (either associated with or without typical cheilitis granulomatosa), we worked out a classification of MRS considering the dermal, neurological, and neurovegetative affections observed in our cases and according to the relevant literature. A systematic classification like this, which takes into account the diagnostic signification of the findings, allows exact recognition of "incomplete" forms of MRS and represents a conditio sine qua non with regard to family studies and the follow-up of patients concerned.
Insights
Melkersson-Rosenthal syndrome (MRS) often presents with less common neurovegetative "minor signs" alongside facial swelling and lingua plicata. A new classification aids in diagnosing incomplete MRS forms and understanding patient follow-up.
Area of Science:
- Neurology
- Dermatology
- Genetics
Context:
- Melkersson-Rosenthal syndrome (MRS) is characterized by facial swelling, facial paralysis, and plicated tongue.
- Previous studies focused on the classic triad, potentially overlooking other manifestations.
- A significant number of MRS patients exhibit less recognized neurovegetative symptoms.
Purpose:
- To re-evaluate the clinical spectrum of Melkersson-Rosenthal syndrome (MRS).
- To identify the prevalence of classic and subtle symptoms in a cohort of MRS patients.
- To propose a refined classification system for MRS, including incomplete forms.
Summary:
- Facial paresis occurred in 34% and lingua plicata in 52% of 42 re-examined MRS patients.
- Over 80% of patients displayed vasomotoric, sialo-secretory, or other neurovegetative signs.
- A classification integrating dermal, neurological, and neurovegetative findings was developed.
Impact:
- The proposed classification facilitates the accurate diagnosis of incomplete MRS.
- This systematic approach is crucial for familial studies and long-term patient management.
- Understanding the full spectrum of MRS aids in better patient care and research.