Reticular Erythematous Mucinosis Syndrome: Review of the World Literature and Report of the Syndrome in a Prepubertal Child

Abstract

An 8-year-old boy with reticular erythematous mucinosis syndrome had erythematous plaques on his chest, face, and arms for three years. Sun exposure resulted in pruritus and increased lesions. Histologic examination revealed a perivascular mononuclear cell infiltrate with hematoxylin and eosin staining, positive staining material between the dermal collagen bundles with alcian blue (pH 2.5) staining, and granular basement membrane deposits of IgM with direct immunofluorescence staining. Results of all lupus erythematosus serologies and porphyrin studies were negative. Minimal erythema dose determinations to ultraviolet A and B were normal, and the lesions could not be induced with high doses of irradiation. Topical sunscreens, corticosteroid cream, and systemic beta-carotene produced no therapeutic benefit.

Reticular erythematous mucinosis syndrome: report of a case with positive immunofluorescence

“…Three previously described cases with IgM deposits in the dermo‐epidermal junction seem to support the theory of REM as being a subset of lupus erythematosus 8–10 …”

“…The REM syndrome has been described worldwide in patients with different ethnic backgrounds. The disease initially appears between the ages of 11 and 40 in 80% of women and 63% of men and rarely arises before age 10 and after age 60 8 . Untreated, the condition usually has a prolonged duration for decades.…”

Lupus Erythematosus Tumidus

“…Histological features of REM show a mild to dense perivascular infiltrate of lymphocytes with abundant mucin deposition in the dermis. 40,41 Furthermore, pseudolymphoma also can simulate LET clinically; however, in most of the cases, histological evaluation shows a top-heavy, usually wedgeshaped infiltrate of small lymphocytes as well as plasma cells and eosinophils. 42,43,64 In summary, LET presents a rare, but distinct, subset of CCLE with characteristic clinical features requiring correlation with histological, photobiological, and laboratory findings, since, taken in isolation, other diagnoses can be indicated.…”

“…10 Reticular erythematous mucinosis is also considered to be a variant of DLE or LET by some authors, especially because REM can be induced by sun exposure and antimalarials have been reported as the most effective therapy. 40,47 In contrast to LET, young to middleaged women are mostly affected, and the skin lesions, ranging from erythematous, indurated papules to reticulated, macular erythema, are mainly localized on the central chest or upper back. Histological features of REM show a mild to dense perivascular infiltrate of lymphocytes with abundant mucin deposition in the dermis.…”

Reticular Erythematous Mucinosis Associated with Human Immunodeficiency Virus Infection

“…Braddock et al [8] consider that REM may be related to alterations in immune function. In fact, different cases of REM associated with thyroid dis ease, arthritis, diabetes mellitus, discoid lupus erythemato sus, idiopathic thrombocytopenic purpura and uveitis, all of which are disorders somehow related to autoimmune dis ease, have been reported, being closely reviewed by Ingber and Sandbank [9] and Cohen et al [10]. Some other cases have been associated with malignancy [5,11 ].…”