Self-healing Juvenile Cutaneous Mucinosis

Nodular Lesions of Self-healing Juvenile Cutaneous Mucinosis

“…14 Three types of classic skin lesions have been described: (1) nontender ivory-white papules on the head, neck, trunk, and periarticular regions, which may be grouped in a linear array on the chest and abdomen; (2) deep nodules on the face and periarticular regions; and (3) hard edema of the periorbital and zygomatic areas. 4 However, in some cases, nodules clearly predominate or they can be only the first clinical presentation, as in our 3 cases. Such rapidly proliferating nodules in young children are usually worrisome for pediatricians, even if the children remain in good heath.…”

Self-healing juvenile cutaneous mucinosis: Cases highlighting subcutaneous/fascial involvement

“…Painful polyarthritis may be present, as well as swelling of the knees, elbows, and hands. 7 In contrast to mucinoses affecting adults, SHJCM has not been associated with systemic disorders such as paraproteinemia, bone marrow plasmacytosis, or thyroid disease. 1,4 One case has been reported coincident with nephroblastoma, whereas another case was associated with bilateral carpal tunnel syndrome.…”

“…7 The majority of reported cases have included descriptions of biopsy specimens taken from papular lesions rather than deep nodules. The histopathologic findings have classically described edema of the papillary and reticular dermis with separation of the collagen bundles, slightly increased number of fibroblasts, a mild perivascular infiltrate, and Alcian blue staining at pH 2.5, but not at pH 0.5.…”

“…The histopathologic findings have classically described edema of the papillary and reticular dermis with separation of the collagen bundles, slightly increased number of fibroblasts, a mild perivascular infiltrate, and Alcian blue staining at pH 2.5, but not at pH 0.5. 1,5,7,9 Deep dermal and subcutaneous features of SHJCM have only been described in two reports. Wadee, Roode, and Schulz 8 included histopathologic data from nodular lesions, which exhibited mucin in the mid and deep reticular dermis, arborizing thinwalled vessels, and prominent plump to spindleshaped fibroblasts.…”

“…Case reports and small series reporting the clinical and histologic features of self-healing juvenile cutaneous mucinosis (SHJCM) have been described since 1973. [1][2][3][4][5][6][7][8][9][10][11] Reports of subcutaneous histologic features were first mentioned by Wadee, Roode, and Schulz 8 in 1994 and then again by Cowen, Scott, and Mercurio 2 in 2004. Three cases of SHJCM are presented to highlight subcutaneous histopathologic features which are a potential cause of diagnostic confusion.…”