Lupus Erythematosus and Cutaneous Mucinosis

Mucin is an acid mucopolysaccharide consisting of hyaluronic acid bound to small amounts of chondroitin sulphate and heparin. In 1991, Rongioletti and Rebora (Ron-gioletti and Rebora 1991) divided cutaneous mucinosis into two groups: the first has distinctive clinical characteristics, and the second appears in various diseases as an additional feature. In patients with LE and other connective tissue diseases, microscopic amounts of mucin in the dermis could be detected, but without clinical evidence (Choi et al. 1992). Gold (Gold 1954), in 1954, first reported an occurrence of mucinosis lesions in two patients with SLE. His patients had papular eruptions on the neck, the upper part of the trunk, and the extremities that preceded the development of SLE lesions. This condition, later named "papular and nodular mucinosis," (PNM) is a well-characterized cutaneous mucinosis predominantly associated with SLE. Rongioletti et al. (Rongioletti et al. 1990b) believed that PNM may herald a severe systemic disease. PNM typically presents as indolent, flesh-colored, centrally depressed papules and nodules on the neck, trunk, and extremities (Kanda et al. 1997, Rongioletti and Rebora 1986). Biopsies did not reveal the typical LE epidermal changes, but the immunofluorescence study detected immunoglobulin deposits and C3 at the der-moepidermal junction.

The temporal relationship with LE is different: in some cases PNM precedes LE and in others it appears with it. In 70% of patients, PNM appears in association with SLE, and in 30% it appears with DLE (Rongioletti et al. 1990b). DLE with dermal mucinosis has been reported by Weigand et al. (Weigand et al. 1981) in 1981 and Lowe et al. in 1992 (Lowe et al. 1992). In DLE, an acute periorbital mucinosis has been reported by Williams and Ramos-Caro (Williams and Ramos-Caro 1999) in 1999. Why cutaneous mucinosis appears in LE is still not known. In some patients, one could find a link between sun exposure and development of mucinosis. The two cases described by Weidner and Djawari (Weidner and Djawari 1982) in 1982 had DLE and dermal mucinosis, and both appeared after sun exposure. The patient described by Nishimoto et al. (Nishimoto et al. 1989) developed SLE and cutaneous mucinosis provoked by exposure to psoralen-UVA. Cutaneous mucinosis has been reported by Kuhn et al. (Kuhn et al. 1995) in association with SCLE. Several years later, Kuhn et al. (Kuhn et al. 2000) found deposits of mucin in patients with LE tumidus (LET). Both subtypes of LE, SCLE and LET, are recognized as being extremely photosensitive. Various clinical pictures of mucinosis in association with LE have been reported: massive cutaneous mucinosis (Maryama et al. 1997), plaque-like (Kobayashi et al. 1993), and atrophie blanche-like lesions (Egawa et al. 1994). In addition, lichen myxede-matosus was described in two patients by Salomon et al. (Salomon et al. 1977) in 1977. The first patient had DLE and the other had SLE. Since in the most cases dermal mucinosis and especially PNM preceded LE, the early recognition of LE is important.

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