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]
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
basophilicmucin 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]
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]
^Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby.
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^
abcJames, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier.
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^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.
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^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.
ISSN0190-9622.
PMID7096649.
^
abcBLEEHEN, 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.
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^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:
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^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.
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^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 (inactive 2024-02-16).
ISSN0307-6938.
PMID9536545.{{
cite journal}}: CS1 maint: DOI inactive as of February 2024 (
link)
^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.
ISSN0926-9959.
PMID15096164.
^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.
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^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.
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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.
ISSN0007-0963.
PMID4472292.
S2CID20659319.