Melkersson-Rosenthal syndrome with Hashimoto thyroiditis in a 9-year-old girl: an autoimmune disorder.

BACKGROUND: Melkersson-Rosenthal syndrome (MRS) is a rare disorder of unknown cause. The classical triad of MRS is orofacial edema, recurrent facial paralysis, and a fissured tongue. PATIENT: We present a 9-year-old girl with a recurrent peripheral facial paralysis. She experienced the first episode of a peripheral facial paralysis on the same side without orofacial swelling and lingua plicata 1 year ago. She was diagnosed with Hashimoto thyroiditis 9 months earlier, as confirmed by an endocrinologic investigation. RESULTS: While the patient was hospitalized with recurrent facial paralysis, we found that serum levels of free thyroxine (1.3 ng/dL) and thyrotropin (0.4 uIU/mL) were within normal range, but the level of antithyroperoxidase antibodies (772.0 IU/mL) was very increased. She had been taking an oral prednisolone orally for 2 weeks. At the 1-month follow-up, the patient's symptoms had completely disappeared. DISCUSSION: The possible correlation between MRS and autoimmune disorders has been documented in only one report, which described an adult with autoimmune thyroiditis (Hashimoto thyroiditis) and MRS. We suggest that the co-occurrence of MRS and Hashimoto thyroiditis is not coincidental but linked to autoimmunity.

Hashimoto's thyroiditis in Melkersson-Rosenthal syndrome patient: casual association or related diseases?

Melkersson-Rosenthal syndrome (MRS) is a rare disorder of unknown etiology. MRS is classically defined as a triad of recurrent orofacial edema, relapsing paralysis of the facial nerve, and fissured tongue. The authors present the case of a 52-year-old woman with orofacial swelling and facial pain attacks. The patient reported to suffer of hypothyroidism and laboratory findings disclosed free triiodothyronine (FT(3)), free thyroxine (FT(4)), and thyrotropin (TSH) altered. Endocrinological consult led to the diagnosis of Hashimoto's thyroiditis. Antithyroper-oxidase antibodies (anti-TPO) were highly elevated and thyroid function tests had evidenced a clinically significant hypothyroidism. A link between MRS and immunological disorders such as sarcoidosis, Crohn's disease, unilateral anterior uveitis and multiple sclerosis was documented. The literature did not report any association between Hashimoto's thyroiditis and Melkersson-Rosenthal syndrome. The presence of the anti-TPO antibodies in the case reported here could suggest a possible correlation between immunological alteration characteristic of autoimmune thyroiditis and MRS.

Antibodies to mycobacterial stress protein in patients with orofacial granulomatosis.

Immunoglobulin G antibody titers to the mycobacterial stress protein with molecular weight of 65 kDa (mSP65) were determined by ELISA in sera from 10 patients with orofacial granulomatosis (OFG). Four patients with confirmed Crohn's disease had serum antibodies to mSP65 with titers ranging from 400-950. Of six remaining patients, three patients had serum antibodies to mSP65, with titers ranging from 180-850, whilst no serum antibody to this antigen could be detected in 3 patients. However, other laboratory investigations failed to show any consistent pattern of disturbance in the 10 tested. Thus, the presence of serum antibody to mycobacterial 65 kDa stress protein might prove to be of a diagnostic value for Crohn's disease.

Kveim test reactivity in Melkersson-Rosenthal syndrome (cheilitis granulomatosa).

The relation between the Melkersson-Rosenthal syndrome (MRS) and sarcoidosis is unclear. The Kveim test, a test for sarcoidosis, was performed in seven patients, two with complete and five with abortive forms of Melkersson-Rosenthal syndrome. All were found to be negative. Serum levels of angiotensin converting enzyme and calcium were normal. These facts make it unlikely that MRS is a type of sarcoidosis.