Author: Dr Emily Shao, Medical Officer, Department of Dermatology, Mater Hospital, Brisbane, Queensland, Australia. DermNet NZ Editor in Chief: Adjunct A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. Copy edited by Gus Mitchell. May 2019.
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. Biopsy shows mucin deposition within the dermis .
Acral persistent papular mucinosis is very rare, with about 30 cases reported in the literature .
There is a female predominance with a ratio of 3:1. The average age of onset is 43 years old. 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 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.
What are the complications of acral persistent papular mucinosis?
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 by using Alcian blue or colloidal iron stains .
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. They often involve 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, including:
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 .
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