Melkersson–Rosenthal syndrome

Medical condition

Melkersson–Rosenthal syndrome
Other names	MRO, Miescher–Melkersson–Rosenthal syndrome

This condition is inherited in an autosomal dominant manner.
Specialty	Neurology

Melkersson–Rosenthal syndrome is a rare neurological disorder characterized by recurring facial paralysis, swelling of the face and lips (usually the upper lip: cheilitis granulomatosis) and the development of folds and furrows in the tongue (fissured tongue). Onset is in childhood or early adolescence. After recurrent attacks (ranging from days to years in between), swelling may persist and increase, eventually becoming permanent. The lip may become hard, cracked, and fissured with a reddish-brown discoloration. The cause of Melkersson–Rosenthal syndrome is unknown, but there may be a genetic predisposition. It has been noted to be especially prevalent among certain ethnic groups in Bolivia. It can be associated with Crohn's disease or sarcoidosis. Approximately 400 cases have been reported worldwide.

Cause

Not to be confused with Rosenthal syndrome a.k.a. hemophilia C which is caused by clotting factor XI deficiency. Only genetic causation is established as it is associated with twins and family members.

Diagnosis

Diagnosis is mainly based on clinical features. However, biopsy has been useful in diagnosis as well as in differentiating between the different types of the disease.

Treatment

Treatment is symptomatic and may include nonsteroidal anti-inflammatory drugs (NSAIDs) and corticosteroids to reduce swelling, antibiotics and immunosuppressants. Surgery may be indicated to relieve pressure on the facial nerves and reduce swelling, but its efficacy is uncertain. Massage and electrical stimulation may also be prescribed.

Prognosis

Melkersson–Rosenthal syndrome may recur intermittently after its first appearance. It can become a chronic disorder. Follow-up care should exclude the development of Crohn's disease or sarcoidosis.

Eponym

The condition is named after Ernst Melkersson and Curt Rosenthal.

Research

The NINDS supports research on neurological disorders such as Melkersson–Rosenthal syndrome. Much of this research is aimed at increasing knowledge of these disorders and finding ways to treat, prevent, and ultimately cure them.

References

Ngan V. "Granulomatous cheilitis". DermNet New Zealand Trust.
James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: Clinical Dermatology. Saunders Elsevier. ISBN 978-0-7216-2921-6.
Bakshi SS. Melkersson–Rosenthal Syndrome. J Allergy Clin Immunol Pract. 2017 Mar - Apr;5(2):471-472. doi: 10.1016/j.jaip.2016.09.034
synd/9 at Who Named It?
Melkersson, E. (1928). "Ett fall av recidiverande facialispares i samband med ett angioneurotiskt ödem". Hygiea. 90. Stockholm: 737–741.
Rosenthal, C. (1931). "Klinisch-erbbiologischer Beitrag zur Konstitutionspathologie. Gemeinsames Auftreten von Facialislähmung, angioneurotischem Gesichtsödem und Lingua plicata in Arthritismus-Familien". Zeitschrift für die Gesamte Neurologie und Psychiatrie. 131: 475–501. doi:10.1007/bf02865984. S2CID 70544781.

The original version of this article was taken from the public domain source at Melkersson–Rosenthal syndrome at nih.gov

External links

Classification	D ICD-10: G51.2 ICD-9-CM: 351.8 OMIM: 155900 MeSH: D008556 DiseasesDB: 31963
External resources	eMedicine: derm/72

Diseases relating to the peripheral nervous system

Mononeuropathy

Arm

median nerve	Carpal tunnel syndrome Ape hand deformity
ulnar nerve	Ulnar nerve entrapment Froment's sign Ulnar tunnel syndrome Ulnar claw
radial nerve	Radial neuropathy Wrist drop Cheiralgia paresthetica
long thoracic nerve	Winged scapula Backpack palsy

Leg

lateral cutaneous nerve of thigh	Meralgia paraesthetica
tibial nerve	Tarsal tunnel syndrome
plantar nerve	Morton's neuroma
superior gluteal nerve	Trendelenburg's sign
sciatic nerve	Piriformis syndrome

Cranial nerves

See Template:Cranial nerve disease

Polyneuropathy and Polyradiculoneuropathy

HMSN	Charcot–Marie–Tooth disease Dejerine–Sottas disease Refsum's disease Hereditary spastic paraplegia Hereditary neuropathy with liability to pressure palsy Familial amyloid neuropathy
Autoimmune and demyelinating disease	Guillain–Barré syndrome Chronic inflammatory demyelinating polyneuropathy
Radiculopathy and plexopathy	Brachial plexus injury Pectoralis minor syndrome Thoracic outlet syndrome Phantom limb
Other	Alcoholic polyneuropathy

Other

General	Complex regional pain syndrome Mononeuritis multiplex Nerve compression syndrome Neuralgia Peripheral neuropathy

Oral and maxillofacial pathology

Lips
Cheilitis Actinic Angular Plasma cell Cleft lip Congenital lip pit Eclabium Herpes labialis Macrocheilia Microcheilia Nasolabial cyst Sun poisoning Trumpeter's wart

Tongue
Ankyloglossia Black hairy tongue Caviar tongue Crenated tongue Cunnilingus tongue Fissured tongue Foliate papillitis Glossitis Geographic tongue Median rhomboid glossitis Transient lingual papillitis Glossoptosis Hypoglossia Lingual thyroid Macroglossia Microglossia Rhabdomyoma

Palate
Bednar's aphthae Cleft palate High-arched palate Palatal cysts of the newborn Inflammatory papillary hyperplasia Stomatitis nicotina Torus palatinus

Oral mucosa – Lining of mouth

Teeth (pulp, dentin, enamel)

Periodontium (gingiva, periodontal ligament, cementum, alveolus) – Gums and tooth-supporting structures

Periapical, mandibular and maxillary hard tissues – Bones of jaws

Temporomandibular joints, muscles of mastication and malocclusions – Jaw joints, chewing muscles and bite abnormalities
Bruxism Condylar resorption Mandibular dislocation Malocclusion Crossbite Open bite Overbite Overeruption Overjet Prognathia Retrognathia Scissor bite Maxillary hypoplasia Temporomandibular joint dysfunction Condylar hypoplasia Condylar hyperplasia

Salivary glands

Stomatognathic system – Teeth, jaws, tongue and associated soft tissues
Bruxism Mouth breathing Sleep apnea Catathrenia Central hypoventilation syndrome Obesity hypoventilation syndrome Obstructive sleep apnea Periodic breathing Snoring

Orofacial soft tissues – Soft tissues around the mouth
Actinomycosis Angioedema Basal cell carcinoma Cutaneous sinus of dental origin Cystic hygroma Gnathophyma Ludwig's angina Macrostomia Melkersson–Rosenthal syndrome Microstomia Noma Oral Crohn's disease Orofacial granulomatosis Perioral dermatitis Pyostomatitis vegetans

Other
Eagle syndrome Hemifacial hypertrophy Facial hemiatrophy Oral manifestations of systemic disease

Categories:

Misplaced Pages