| 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
|