A case of Melkersson‐Rosenthal syndrome with endocrine disorders: Extraordinary efficiency of hydroxychloroquine and mechanism hypothesis

Abstract

Background and purpose Melkersson‐Rosenthal syndrome (MRS) is a rare neuro‐mucocutaneous disease. In addition to the traditional clinical triad, there is also a diversity of clinical signs, and it may be related to other systemic diseases. Methods In the present study, we report a case of MRS with endocrine disorders that exhibits extraordinary therapeutic efficiency by using hydroxychloroquine (HCQ), explore whether there is an internal connection between MRS and endocrine disorders, and discuss the mechanism of the therapeutic efficiency of using HCQ. The hypothesis proposed for the first time is that MRS may essentially be a systemic granulomatous disease. Results The physical examination revealed orofacial swelling and fissured tongue. The histopathologic examination showed epithelioid granulomas. Combined with the other examination, this case was diagnosed as incomplete MRS. HCQ and local drugs were introduced. The patient achieved clinical recovery and psychological cure by the 18‐week follow‐up, and the 1‐year follow‐up found no reactivation of MRS. Moreover, the levels of cortisol and adrenocorticotropic were within normal ranges. Conclusions After the drug therapy was targeted at granuloma, not only did all of the symptoms related to MRS disappear, but the endocrine system also returned to normal. It is speculated that the endocrine disorder in this patient may be related to MRS. We further propose the first‐time hypothesis that MRS may essentially be a systemic granulomatous disease. It provides a new medication method with high‐level efficiency.

Queilitis granulomatosa de Miescher tratada con hidroxicloroquina

“…Otros tratamientos incluyen prednisona, hidroxicloroquina, sulfasalazina, antihistamínicos, tetraciclinas, metronidazol, macrólidos, talidomida e infliximab, además de la queiloplastia 7,8 . Dado que la paciente había presentado recidiva de su enfermedad con la administración de corticosteroides tópicos y prefería no recibir inmunosupresores ni terapias invasivas como la corticoterapia intralesional o la cirugía, se optó por un tratamiento de segunda línea como la hidroxicloroquina, con la que se obtuvo una excelente respuesta a los 10 meses 9 .…”