Introduction Demographics Causes Clinical features Complications Diagnosis Differential diagnoses Treatment Outcome
Acral persistent papular mucinosis is a distinct subtype of localised lichen myxoedematosus. It is characterised by discrete papules on the back of the hands, wrists, and forearms. A skin biopsy shows mucin deposition within the dermis.
Acral persistent papular mucinosis is very rare, with about 30 cases reported in the literature.
It occurs most frequently in women, with a ratio of 3:1. The average age of onset is 43 years. The majority of cases have been reported in people of European heritage, but this is thought to be because of increased awareness of acral persistent papular mucinosis in Western countries.
Most cases do not appear to have a familial or genetic link, but there is one report of two sisters both being diagnosed with acral persistent papular mucinosis.
Four cases have reported a history of malignant tumours; it is unclear whether this association is relevant.
The cause of acral persistent papular mucinosis is unknown.
Acral persistent papular mucinosis is characterised by:
Acral persistent papular mucinosis has no association with gammopathy, thyroid disorders, or other systemic abnormalities.
Unlike other forms of lichen myxoedematosus, acral persistent papular mucinosis is a benign disorder and does not affect other organs.
Acral persistent papular mucinosis is diagnosed by recognising its clinical and histological features.
The histological features of a skin biopsy of acral persistent papular mucinosis are:
Mucin deposition can be highlighted using Alcian blue or colloidal iron stains.
Acral persistent papular mucinosis should be distinguished from other forms of mucinosis, including the other types of localised lichen myxoedematosus. These include:
Acral persistent papular mucinosis should also be distinguished from widespread mucinoses such as scleromyxoedema, in which there are widespread papules 2–3 mm in size and arranged linearly. These papules often appear on the head, neck, trunk, hands, forearms, and thighs. Scleromyxoedema is almost always associated with gammopathy.
Papules on the hands and forearms can be due to many disorders, such as:
Several treatments for acral persistent papular mucinosis have been reported, but these have generally been unsuccessful. These include:
As the name suggests, acral persistent papular mucinosis does not resolve spontaneously.
