Reticular erythematous mucinosis

Reticular erythematous mucinosis
Other names	Midline mucinosis,[1] Plaque-like cutaneous mucinosis,[2] and REM syndrome[2]
Specialty	Dermatology

Reticular erythematous mucinosis (REM) is a skin condition caused by fibroblasts producing abnormally large amounts of mucopolysaccharides. It is a disease that tends to affect women in the third and fourth decades of life.[2]: 187 

Signs and symptoms

Clinically, there are papules and pink to red macules that eventually combine to form annular and reticulated lesions.[3] Although they are usually found on the upper mid-back or mid-chest, these lesions have also been reported on unusual sites like the face, arms, legs, and abdomen.[4] There's a chance that the lesions have telangiectasias and are mildly itchy.[5] Although exposure to the sun has been known to occasionally be beneficial, it usually makes the eruption worse.[3] UVA and/or UVB provocative phototests have the potential to replicate reticular erythematous mucinosis lesions.[6]

Causes

Patients with reticular erythematous mucinosis have also been reported to have other conditions like myxedema, hypothyroidism, Hashimoto's thyroiditis, monoclonal gammopathy, and HIV infection.[7][8] Menstruation, heat, x-ray therapy, oral contraceptives, pregnancy, and perspiration can all induce or worsen reticular erythematous mucinosis.[9] Reticular erythematous mucinosis's specific link to lung cancer is being investigated. Different cytokines, such as transforming growth factor β, interleukins, tumor necrosis factor, and interferon, are known to modulate mucin synthesis.[10]

Diagnosis

Histologically, reticular erythematous mucinosis is linked to a variable deep perivascular extension and a mild superficial and middermal perivascular infiltrate.[11][12] A primarily lymphocytic perifollicular infiltrate may exist,[13] along with a small number of histiocytes, factor XIIIa-positive dendrocytes, and admixed mast cells.[14] In the papillary dermis, there is occasionally focal, mild hemorrhage as well as slight vascular dilatation.[15]

A characteristic of reticular erythematous mucinosis is the separation of dermal collagen bundles, and the upper and mid dermis are the primary areas where variable amounts of basophilic mucin are visible.[3] The areas of the upper dermis, appendages, and the infiltrate are where the mucin is most noticeable.[16] There might be a few stellate cells as well.[3] Although mild spongiosis and focal lichenoid inflammation have been reported, the epidermis is usually normal. Sporadic elastic fiber fragmentation and mild basal layer degeneration are possible in certain situations.[12] The staining reactions of the mucin are variable. Alcian blue has occasionally produced false negative results; however, colloidal iron staining has been shown to be superior.[17][18]

Direct immunofluorescence has demonstrated the accumulation of immunoglobulins, specifically IgM, along the basal layer in multiple instances.[19][20] It may be possible to see focal elastic fiber fragmentation, expanding intercollagenous spaces, and active fibroblasts when the lesions are viewed under an electron microscope.[21] Electron microscopy has also revealed numerous tubular inclusions in keratinocytes, dermal macrophages, pericytes, and endothelial cells.[12][22]

Treatment

Antimalarial medications are the preferred treatment for REM. After beginning treatment, they frequently result in a quick clinical improvement, but recurrence is frequent.[3] Generally speaking, treating the illness with hydroxychloroquine at a dosage of 200–400 mg/d has proven successful.[23][24]

Many treatments have been tried, but with varying degrees of success, including oral antihistamines, topical tacrolimus, systemic and topical corticosteroids, tetracycline, cyclosporine, and UVB radiation.[4][9][25]

See also

• Mucinosis
• List of cutaneous conditions

References

1. Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 978-1-4160-2999-1.
2. James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 978-0-7216-2921-6.
3. Thareja, Sumeet; Paghdal, Kapila; Lien, Mary H.; Fenske, Neil A. (2012). "Reticular erythematous mucinosis – a review". International Journal of Dermatology. 51 (8): 903–909. doi:10.1111/j.1365-4632.2011.05292.x. ISSN 0011-9059. PMID 22788804. S2CID 205398751.
4. Suárez-Amor, O.; Pérez-Bustillo, A.; González-Morán, M.A.; Ramírez-Santos, A.; Rodríguez-Prieto, M.A. (2010). "Reticular Erythematous Mucinosis: Partial Response to Treatment With Topical Tacrolimus". Actas Dermo-Sifiliográficas (English Edition). 101 (1): 105–106. doi:10.1016/S1578-2190(10)70595-3. PMID 20109408.
5. Weindorf, N; Schultz-Ehrenburg, U; Altmeyer, P (September 1988). "Plaque-formed cutaneous mucinosis with telangiectasis". Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete (in German). 39 (9): 589–592. PMID 3182272.
6. Adamski, H.; Le Gall, F.; Chevrant-Breton, J. (September 20, 2004). "Positive photobiological investigation in reticular erythematous mucinosis syndrome". Photodermatology, Photoimmunology & Photomedicine. 20 (5). Wiley: 235–238. doi:10.1111/j.1600-0781.2004.00113.x. ISSN 0905-4383. PMID 15379872. S2CID 14167793.
7. Rongioletti; Ghigliotti; De Marchi; Rebora (1998). "Cutaneous mucinoses and HIV infection". British Journal of Dermatology. 139 (6). Oxford University Press (OUP): 1077–1080. doi:10.1046/j.1365-2133.1998.02570.x. ISSN 0007-0963. PMID 9990377. S2CID 32104032.
8. Velasco, J.A.; Santos, J.C.; Villabona, V.; Santana, J. (1992). "Reticular Erythematous Mucinosis and Acral Papulokeratotic Lesions Associated with Myxoedema due to Hashimoto Thyroiditis". Dermatology. 184 (1). S. Karger AG: 73–77. doi:10.1159/000247505. ISSN 1018-8665. PMID 1559001.
9. Bolognia, JL; Jorizzo, JL; Rapini, RP; Callen, JP; Horn, TD; Mancini, AJ; Salasche, SJ; Schaffer, JV; Schwarz, T; Stingl, G; Stone, MS (2008). Dermatology (2 ed.). London: Mosby Elsevier. pp. 540–555. ISBN 9781416029991.
10. Izumi, T.; Tajima, S.; Harada, R.; Nishikawa, T. (1996). "Reticular Erythematous Mucinosis Syndrome: Glycosaminoglycan Synthesis by Fibroblasts and Abnormal Response to Interleukin-1β". Dermatology. 192 (1). S. Karger AG: 41–45. doi:10.1159/000246312. ISSN 1018-8665. PMID 8832951.
11. Quimby, Steven R.; Perry, Harold O. (1982). "Plaquelike cutaneous mucinosis: Its relationship to reticular erythematous mucinosis". Journal of the American Academy of Dermatology. 6 (5). Elsevier BV: 856–861. doi:10.1016/s0190-9622(82)70075-1. ISSN 0190-9622. PMID 7096649.
12. BLEEHEN, S.S.; SLATER, D.N.; MAHOOD, J.; CHURCH, R.E. (1982). "Reticular erythematous mucinosis: light and electron microscopy, irnmunofluorescence and histochemical findings". British Journal of Dermatology. 106 (1). Oxford University Press (OUP): 9–18. doi:10.1111/j.1365-2133.1982.tb00896.x. ISSN 0007-0963. PMID 7037039. S2CID 30956403.
13. Braddock, Suzanne W.; Kay, H. David; Maennle, Diane; McDonald, Thomas L.; Pirruccello, Samuel J.; Masih, Aneal; Klassen, Lynell W.; Sawka, Alisa R. (1993). "Clinical and immunologic studies in reticular erythematous mucinosis and Jessner's lymphocytic infiltrate of skin". Journal of the American Academy of Dermatology. 28 (5). Elsevier BV: 691–695. doi:10.1016/0190-9622(93)70094-a. ISSN 0190-9622. PMID 8496412.
14. Tominaga, A.; Tajima, S.; Ishibashi, A.; Kimata, K. (2001). "Reticular erythematous mucinosis syndrome with an infiltration of factor XIIIa+ and hyaluronan synthase 2+ dermal dendrocytes". British Journal of Dermatology. 145 (1). Oxford University Press (OUP): 141–145. doi:10.1046/j.1365-2133.2001.04299.x. ISSN 0007-0963. PMID 11453924. S2CID 25332994.
15. Morison, Warwick L. (1979-11-01). "Reticular Erythematous Mucinosis Syndrome: Report of Two Cases". Archives of Dermatology. 115 (11): 1340–1342. doi:10.1001/archderm.1979.04010110046025. ISSN 0003-987X. PMID 507890.
16. Triffet Trevino, M; Ahmed, I (1996). "Plaque-like cutaneous mucinosis (reticular erythematous mucinosis): a clinicopathologic analysis". J Cutan Pathol. 23: 63.
17. SMITH, NEIL P.; SANDERSON, K.V.; CROW, K.D. (1976). "Reticular erythematous mucinosis syndrome*". Clinical and Experimental Dermatology. 1 (1). Oxford University Press (OUP): 99–103. doi:10.1111/j.1365-2230.1976.tb01404.x. ISSN 0307-6938. PMID 1269183. S2CID 34221275.
18. DEL POZO, J.; MARTINEZ, W.; ALMAGRO, M.; YEBRA, M.T.; GARCIA-SILVA, J.; FONSECA, E. (1997). "Reticular erythematous mucinosis syndrome. Report of a case with positive immunofluorescence". Clinical and Experimental Dermatology. 22 (5). Oxford University Press (OUP): 234–236. doi:10.1046/j.1365-2230.1997.2670672.x. ISSN 0307-6938. PMID 9536545.
19. Gasior-Chrzan, B; Husebekk, A (April 15, 2004). "Reticular erythematous mucinosis syndrome: report of a case with positive immunofluorescence". Journal of the European Academy of Dermatology and Venereology. 18 (3). Wiley: 375–378. doi:10.1111/j.1468-3083.2004.00813.x. ISSN 0926-9959. PMID 15096164.
20. DODD, H. J.; SARKANY, I.; SADRUDIN, A. (1987). "Reticular erythematous mucinosis syndrome". Clinical and Experimental Dermatology. 12 (1). Oxford University Press (OUP): 36–39. doi:10.1111/j.1365-2230.1987.tb01853.x. ISSN 0307-6938. PMID 3652502. S2CID 41205459.
21. Vanuytrecht-Henderickx, D.; Dewolf-Peeters, C.; Degreef, H. (1984). "Morphological Study of the Reticular Erythematous Mucinosis Syndrome". Dermatology. 168 (4). S. Karger AG: 163–169. doi:10.1159/000249692. ISSN 1018-8665. PMID 6202567.
22. MIYOSHI, Ken; MIYAJIMA, Osamu; YOKOGAWA, Maki; SANO, Shigetoshi (2010). "Favorable response of reticular erythematous mucinosis to ultraviolet B irradiation using a 308-nm excimer lamp". The Journal of Dermatology. 37 (2). Wiley: 163–166. doi:10.1111/j.1346-8138.2009.00779.x. ISSN 0385-2407. PMID 20175851. S2CID 11858253.
23. Woollons, A.; Darley, C. R. (September 1, 2002). "Erythematous Rash on the Chest". Archives of Dermatology. 138 (9). American Medical Association (AMA): 1245–c–1250. doi:10.1001/archderm.138.9.1245-c. ISSN 0003-987X. PMID 12224994.
24. Sidwell, R.U.; Francis, N.; Bunker, C.B. (2001). "Hormonal influence on reticular erythematous mucinosis". British Journal of Dermatology. 144 (3). Oxford University Press (OUP): 633–634. doi:10.1046/j.1365-2133.2001.04105.x. ISSN 0007-0963. PMID 11260035.
25. Meewes, Christian (June 1, 2004). "Treatment of Reticular Erythematous Mucinosis With UV-A1 Radiation". Archives of Dermatology. 140 (6). American Medical Association (AMA): 660–662. doi:10.1001/archderm.140.6.660. ISSN 0003-987X. PMID 15210454.

Further reading

• STEIGLEDER, GERD KLAUS; GARTMANN, HEINZ; LINKER, UTE (1974). "REM syndrome: reticular erythematous mucinosis (round-cell erythematosis), a new entity?". British Journal of Dermatology. 91 (2). Oxford University Press (OUP): 191–199. doi:10.1111/j.1365-2133.1974.tb15865.x. ISSN 0007-0963. PMID 4472292. S2CID 20659319.
• Izumi, Tatsua; Tajima, Shingo (1995). "Reticular Erythematous Mucinosis Syndrome Associated with Psoriasis-like Eruptions". The Journal of Dermatology. 22 (9). Wiley: 700–703. doi:10.1111/j.1346-8138.1995.tb03903.x. ISSN 0385-2407. PMID 8537561. S2CID 23123120.

External links

• DermNet
• VisualDx