Review Note

Clinical description and distribution of scleromyxoedema

- Numerous, 2–3 mm, firm, waxy, closely aligned papules develop in a relatively widespread symmetrical pattern.
- Papules often arranged in a strikingly linear array.
- The surrounding skin is shiny and indurated, i.e. sclerodermoid in appearance
- Erythema, edema, and a brownish discoloration may also be seen in involved areas
- Erythematous and infiltrated plaques --> skin stiffening, sclerodactyly, and decreased motility of the mouth and joints.

- Severe involvement of the face can result in a leonine facies
- Deep furrowing can also occur on the trunk and extremities and is referred to as the “Shar-Pei sign”
- Donut sign: Overlying proximal interphalangeal joints - Central depression surrounded by elevated rim due to skin thickening

- Pruritus is not uncommon.
- Mucous membranes and scalp are spared.
- Mat and cuticular telangiectasias and calcinosis, as seen in systemic sclerosis, are absent.

Most common sites: head and neck region - glabella is typically involved with deep longitudinal furrowing, upper trunk, hands, forearms, and thighs