Clinical type or subtype No. of reported cases** Age at onset and gender Clinical presentation and distribution Associations Histopathology*** Prognosis
Generalized papular and sclero-dermoid eruption (sclero-myxedema) 117 Middle-aged adults
M=F
· Widespread symmetric eruption of 2-3 mm papules on hands, forearms, face, neck, upper trunk, thighs
· Shiny sclerodermoid perilesional skin
·Deep glabellar furrows
· "Doughnut sign" over PIP joints
· Progresses to more diffuse infiltrative plaques with internal organ involvement
· Paraprotein-emia (usu IgG ? light chains)
· Bone marrow plasmacytosis
· Multiple myeloma (in 10%)
· Lymphoma
· Leukemia
·Waldenstrom's macroglobul-inemia
· Diffuse mucin in upper and mid dermis
· Increased fibroblasts
· Collagen deposition
· May also have:
thinned epidermis
atrophic follicles
superficial perivascular lymphocytes and plasma cells
fragmented and deceased elastic fibers
Poor (can be fatal)
Localized LM (papular mucinosis)
(see below for features of subtypes)
Discrete papular LM 27 Adults
M>F****
· Few to numerous 2-5 mm symmetric papules on proximal limbs and trunk
· Sparing of face
· No induration
· HIV
· Obesity
· Focal or diffuse mucin in upper and mid dermis
· Slight fibrosis
· Variable increase in fibroblasts
Slow local progression but no systemic involvement; rare cases resolve in weeks to months
Acral persistent papular mucinosis 20 Adults
F>M (4.7:1)
· Multiple papules on dorsal hands and extensor wrists · HIV · Focal mucin in upper dermis
· No fibrosis or increase in fibroblasts
· Grenz zone
Slow local progression but no systemic involvement
Papular mucinosis of infancy 4 Infants · Papules on the elbows, upper arms, and trunk · None · Focal mucin in papillary dermis "hugged" by epidermis Does not resolve
Nodular LM 2 Adults · Multiple nodules on the limbs and trunk · None · Mucin in upper dermis Does not resolve
Atypical forms
variable features

*          As described in Rongioletti F. Lichen myxedematosus (papular mucinosis): new concepts and perspectives for an old disease. Semin Cutan Med Surg 2006;25:100-4
**        As reported in Rongioletti F, Rebora A. Updated classification of papular mucinosis, lichen myxedematosus, and scleromyxedema. J Am Acad Dermatol 2001;44:273-81(and including cases reported in that article) and in Rongioletti F. Lichen myxedematosus (papular mucinosis): new concepts and perspectives for an old disease. Semin Cutan Med Surg 2006;25:100-4
***      According to Rongioletti F, Rebora A. Cutaneous mucinoses: microscopic criteria for diagnosis. Am J Dermatopathol 2001;23:257-67
****     According to Sulit DJ, Harford R, et al. Discrete papular form of lichen myxedematosus: a case report and review of the literature. Cutis 2005;75:105-112