| Mouth and genital ulcers with inflamed cartilage syndrome | |
|---|---|
| Other names | MAGIC syndrome |
| Specialty | Dermatology |
Mouth and genital ulcers with inflamed cartilage syndrome or MAGIC syndrome refers to a condition in which an individual exhibits symptoms of both relapsing polychondritis (RP) and Behcet's disease (BD). [1] Inflammatory ulcers in the mouth, genitalia, and skin are the hallmark of Behcet's disease (BD), a multisystem illness that is chronic and relapsing. [2] Autoimmune recurrent chondritis of the larynx, tracheobronchial tree, nose, ears, and mouth is known as relapsing polychondritis (RP). [3]
Signs and symptoms
The median time interval between the onset of symptoms and the diagnosis was 6 years, with a range of 26 days to 14 years. This suggests that the symptoms of MAGIC syndrome may manifest relatively long after the initial onset of symptoms. During the course of MAGIC syndrome, the signs and symptoms of BD may typically occur before those of RP. [4]
Causes
An autoimmune response to Type II collagen, which is found in cartilage and the sclera of the eye, may play a role in the pathophysiology of RP. [5] A genetic connection to HLA-DR4 (DRB1*04 subtype alleles) may also exist. [5] About 30% of cases of RP are linked to additional illnesses, such as autoimmune disorders. [5] Seventy percent of patients with BD have HLA-B51, which is linked to the multisystem disorder that is associated with histopathological leukocytoclastic vasculitis. [5] Only two of the five cases that could be evaluated, though, had HLA-B51 positivity. Two of the four cases that could be evaluated had HLA-DR4 positivity. [4] MAGIC syndrome may not be a distinct medical condition, according to some writers. [5] [6] It could be RP developing as a result of BD, another autoimmune disease association, or RP and vasculitis. [5]
Diagnosis
When the symptoms of BD and RP are combined into a single clinical entity, the diagnosis of MAGIC syndrome is made. [7]
Treatment
The available pharmacological treatments for MAGIC syndrome include biologics ( tocilizumab and infliximab), immunosuppressants ( cyclosporin A, methotrexate, cyclophosphamide, and azathioprine), steroids ( corticosteroids, methylprednisolone, prednisolone, and prednisone), colchicines, dapsone, and nonsteroidal anti-inflammatory drugs. [4]
Epidemiology
In the English-language published work as of 2016, there have been 16 reports of MAGIC syndrome (21 patients). The patients, who were 8 men and 13 women, ranged in age from 10 to 59 years old (mean, 35.8; median, 37.0), with early middle age being the most common age range. [4]
History
Firestein initially identified the syndrome in 1985. It was characterized by recurrent oral and genital ulcers as well as inflammation of the cartilage in the ears, nose, throat, and rib cage. [7]
See also
References
- ^ Pak, Stella; Logemann, Shaina; Dee, Christine; Fershko, Adam (October 4, 2017). "Breaking the Magic: Mouth and Genital Ulcers with Inflamed Cartilage Syndrome". Cureus. 9 (10). Cureus, Inc.: e1743. doi: 10.7759/cureus.1743. ISSN 2168-8184. PMC 5714402. PMID 29218258.
- ^ Alpsoy, Erkan (April 14, 2016). "Behçet's disease: A comprehensive review with a focus on epidemiology, etiology and clinical features, and management of mucocutaneous lesions". The Journal of Dermatology. 43 (6). Wiley: 620–632. doi: 10.1111/1346-8138.13381. ISSN 0385-2407. PMID 27075942.
- ^ Cantarini, Luca; Vitale, Antonio; Brizi, Maria Giuseppina; Caso, Francesco; Frediani, Bruno; Punzi, Leonardo; Galeazzi, Mauro; Rigante, Donato (2014). "Diagnosis and classification of relapsing polychondritis". Journal of Autoimmunity. 48–49. Elsevier BV: 53–59. doi: 10.1016/j.jaut.2014.01.026. ISSN 0896-8411. PMID 24461536.
- ^ a b c d Nakai, Noriaki; Kaneko, Yuka; Kida, Takashi; Kawahito, Yutaka; Katoh, Norito (2016). "Mouth and genital ulcers with inflamed cartilage syndrome: Case report and review of the published work". Indian Journal of Dermatology. 61 (3). Medknow: 347. doi: 10.4103/0019-5154.182463. ISSN 0019-5154. PMC 4885202. PMID 27293269.
- ^ a b c d e f Kötter, I; Deuter, C; Günaydin, I; Zierhut, M (2006). "MAGIC or not MAGIC--does the MAGIC (mouth and genital ulcers with inflamed cartilage) syndrome really exist? A case report and review of the literature". Clinical and Experimental Rheumatology. 24 (5 Suppl 42): S108–S112. PMID 17067439. Retrieved 13 January 2024.
- ^ Nascimento, Ana Claudia Mendes do; Gaspardo, Daniela Barros Cortez; Cortez, Tatiana Mimura; Miot, Helio Amante (2014). "Syndrome In Question". Anais Brasileiros de Dermatologia. 89 (1). FapUNIFESP (SciELO): 177–179. doi: 10.1590/abd1806-4841.20142615. ISSN 0365-0596. PMC 3938379. PMID 24626673.
- ^ a b Firestein, Gary S.; Gruber, Harry E.; Weisman, Michael H.; Zvaifler, Nathan J.; Barber, Joan; O'Duffy, J.Desmond (1985). "Mouth and genital ulcers with inflamed cartilage: MAGIC syndrome. Five patients with features of relapsing polychondritis and behçet's disease". The American Journal of Medicine. 79 (1). Elsevier BV: 65–72. doi: 10.1016/0002-9343(85)90547-9. ISSN 0002-9343. PMID 4014306.
Further reading
- Geissal, Erik D.; Wernick, Richard (2010). "A Case of Severe MAGIC Syndrome Treated Successfully With the Tumor Necrosis Factor-Alpha Inhibitor Infliximab". JCR: Journal of Clinical Rheumatology. 16 (4). Ovid Technologies (Wolters Kluwer Health): 185–187. doi: 10.1097/rhu.0b013e3181dfce16. ISSN 1076-1608. PMID 20414125.
- IMAI, HIROKAZU; MOTEGI, MUTSUHITO; MIZUKI, NOBUHISA; OHTANI, HIROSHI; KOMATSUDA, ATSUSHI; HAMAI, KEIKO; MIURA, AKIRA B. (1997). "Mouth and Genital Ulcers With Inflamed Cartilage (MAGIC Syndrome): A Case Report and Literature Review". The American Journal of the Medical Sciences. 314 (5). Elsevier BV: 330–332. doi: 10.1097/00000441-199711000-00010. ISSN 0002-9629. PMID 9365335.
External links
| Mouth and genital ulcers with inflamed cartilage syndrome | |
|---|---|
| Other names | MAGIC syndrome |
| Specialty | Dermatology |
Mouth and genital ulcers with inflamed cartilage syndrome or MAGIC syndrome refers to a condition in which an individual exhibits symptoms of both relapsing polychondritis (RP) and Behcet's disease (BD). [1] Inflammatory ulcers in the mouth, genitalia, and skin are the hallmark of Behcet's disease (BD), a multisystem illness that is chronic and relapsing. [2] Autoimmune recurrent chondritis of the larynx, tracheobronchial tree, nose, ears, and mouth is known as relapsing polychondritis (RP). [3]
Signs and symptoms
The median time interval between the onset of symptoms and the diagnosis was 6 years, with a range of 26 days to 14 years. This suggests that the symptoms of MAGIC syndrome may manifest relatively long after the initial onset of symptoms. During the course of MAGIC syndrome, the signs and symptoms of BD may typically occur before those of RP. [4]
Causes
An autoimmune response to Type II collagen, which is found in cartilage and the sclera of the eye, may play a role in the pathophysiology of RP. [5] A genetic connection to HLA-DR4 (DRB1*04 subtype alleles) may also exist. [5] About 30% of cases of RP are linked to additional illnesses, such as autoimmune disorders. [5] Seventy percent of patients with BD have HLA-B51, which is linked to the multisystem disorder that is associated with histopathological leukocytoclastic vasculitis. [5] Only two of the five cases that could be evaluated, though, had HLA-B51 positivity. Two of the four cases that could be evaluated had HLA-DR4 positivity. [4] MAGIC syndrome may not be a distinct medical condition, according to some writers. [5] [6] It could be RP developing as a result of BD, another autoimmune disease association, or RP and vasculitis. [5]
Diagnosis
When the symptoms of BD and RP are combined into a single clinical entity, the diagnosis of MAGIC syndrome is made. [7]
Treatment
The available pharmacological treatments for MAGIC syndrome include biologics ( tocilizumab and infliximab), immunosuppressants ( cyclosporin A, methotrexate, cyclophosphamide, and azathioprine), steroids ( corticosteroids, methylprednisolone, prednisolone, and prednisone), colchicines, dapsone, and nonsteroidal anti-inflammatory drugs. [4]
Epidemiology
In the English-language published work as of 2016, there have been 16 reports of MAGIC syndrome (21 patients). The patients, who were 8 men and 13 women, ranged in age from 10 to 59 years old (mean, 35.8; median, 37.0), with early middle age being the most common age range. [4]
History
Firestein initially identified the syndrome in 1985. It was characterized by recurrent oral and genital ulcers as well as inflammation of the cartilage in the ears, nose, throat, and rib cage. [7]
See also
References
- ^ Pak, Stella; Logemann, Shaina; Dee, Christine; Fershko, Adam (October 4, 2017). "Breaking the Magic: Mouth and Genital Ulcers with Inflamed Cartilage Syndrome". Cureus. 9 (10). Cureus, Inc.: e1743. doi: 10.7759/cureus.1743. ISSN 2168-8184. PMC 5714402. PMID 29218258.
- ^ Alpsoy, Erkan (April 14, 2016). "Behçet's disease: A comprehensive review with a focus on epidemiology, etiology and clinical features, and management of mucocutaneous lesions". The Journal of Dermatology. 43 (6). Wiley: 620–632. doi: 10.1111/1346-8138.13381. ISSN 0385-2407. PMID 27075942.
- ^ Cantarini, Luca; Vitale, Antonio; Brizi, Maria Giuseppina; Caso, Francesco; Frediani, Bruno; Punzi, Leonardo; Galeazzi, Mauro; Rigante, Donato (2014). "Diagnosis and classification of relapsing polychondritis". Journal of Autoimmunity. 48–49. Elsevier BV: 53–59. doi: 10.1016/j.jaut.2014.01.026. ISSN 0896-8411. PMID 24461536.
- ^ a b c d Nakai, Noriaki; Kaneko, Yuka; Kida, Takashi; Kawahito, Yutaka; Katoh, Norito (2016). "Mouth and genital ulcers with inflamed cartilage syndrome: Case report and review of the published work". Indian Journal of Dermatology. 61 (3). Medknow: 347. doi: 10.4103/0019-5154.182463. ISSN 0019-5154. PMC 4885202. PMID 27293269.
- ^ a b c d e f Kötter, I; Deuter, C; Günaydin, I; Zierhut, M (2006). "MAGIC or not MAGIC--does the MAGIC (mouth and genital ulcers with inflamed cartilage) syndrome really exist? A case report and review of the literature". Clinical and Experimental Rheumatology. 24 (5 Suppl 42): S108–S112. PMID 17067439. Retrieved 13 January 2024.
- ^ Nascimento, Ana Claudia Mendes do; Gaspardo, Daniela Barros Cortez; Cortez, Tatiana Mimura; Miot, Helio Amante (2014). "Syndrome In Question". Anais Brasileiros de Dermatologia. 89 (1). FapUNIFESP (SciELO): 177–179. doi: 10.1590/abd1806-4841.20142615. ISSN 0365-0596. PMC 3938379. PMID 24626673.
- ^ a b Firestein, Gary S.; Gruber, Harry E.; Weisman, Michael H.; Zvaifler, Nathan J.; Barber, Joan; O'Duffy, J.Desmond (1985). "Mouth and genital ulcers with inflamed cartilage: MAGIC syndrome. Five patients with features of relapsing polychondritis and behçet's disease". The American Journal of Medicine. 79 (1). Elsevier BV: 65–72. doi: 10.1016/0002-9343(85)90547-9. ISSN 0002-9343. PMID 4014306.
Further reading
- Geissal, Erik D.; Wernick, Richard (2010). "A Case of Severe MAGIC Syndrome Treated Successfully With the Tumor Necrosis Factor-Alpha Inhibitor Infliximab". JCR: Journal of Clinical Rheumatology. 16 (4). Ovid Technologies (Wolters Kluwer Health): 185–187. doi: 10.1097/rhu.0b013e3181dfce16. ISSN 1076-1608. PMID 20414125.
- IMAI, HIROKAZU; MOTEGI, MUTSUHITO; MIZUKI, NOBUHISA; OHTANI, HIROSHI; KOMATSUDA, ATSUSHI; HAMAI, KEIKO; MIURA, AKIRA B. (1997). "Mouth and Genital Ulcers With Inflamed Cartilage (MAGIC Syndrome): A Case Report and Literature Review". The American Journal of the Medical Sciences. 314 (5). Elsevier BV: 330–332. doi: 10.1097/00000441-199711000-00010. ISSN 0002-9629. PMID 9365335.