Acral persistent papular mucinosis

Classification according to ICD-10
L98.5	Mucinosis of the skin
ICD-10 online (WHO version 2019)

The acral persistent papular mucinosis is a rare form of localized Lichen myxoedematosus (a skin disease ), usually with symmetrical weave-like papules exclusively at back of the hands and forearms.

The first description comes from 1953 by the US dermatologists Hamilton Montgomery and Laurence J. Underwood.

Spread and cause

The frequency and cause are not known, the disease occurs more frequently around the age of 40, and women are three times more likely to be affected.

Clinical manifestations

Clinical criteria are:

mostly symmetrical, disseminated , pale papules without itching on the distal forearms , the back of the hands, the edges of the hands, the wrists on the extensor side and in the neck

Increased occurrence of malignancies such as uterine cancer , breast cancer or thyroid cancer .

Differential diagnosis

Must be distinguished are granuloma annulare and Akrokeratoelastoidose Costa .

therapy

Treatment is usually not required. Individual papules can be removed using laser ablation .

literature

A. Iglesias-Plaza, G. Melé-Ninot, N. Pérez-Muñoz, M. Salleras-Redonnet: Acral persistent papular mucinosis with pruritic skin lesions. In: Anais brasileiros de dermatologia. Volume 93, number 5, 2018 Sep-Oct, pp. 769-770, doi: 10.1590 / abd1806-4841.20187878 , PMID 30156641 , PMC 6106683 (free full text).
C. Navarrete-Dechent, S. Bajaj, A. Marghoob, S. González, A. Jaque: Acral persistent papular mucinosis (APPM): Dermoscopy of an uncommon disease. In: Journal of the American Academy of Dermatology. Volume 76, number 2S1, February 2017, pp. S10 – S11, doi: 10.1016 / j.jaad.2016.03.046 , PMID 28087015 .

Individual evidence

↑ ^a ^b ^c ^d ^e Encyclopedia Dermatology
↑ Mucinosis, papular, acral-persistent. In: Orphanet (Rare Disease Database).
^ H. Montgomery, LJ Underwood: Lichen myxedematosus; differentiation from cutaneous myxedemas or mucoid states. In: The Journal of investigative dermatology. Volume 20, Number 3, March 1953, pp. 213-236, PMID 13044990 .
↑ MS Graves, AA Lloyd, EV Ross: Treatment of acral persistent papular mucinosis using an Erbium-YAG laser. In: Lasers in Surgery and Medicine . Volume 47, Number 6, August 2015, pp. 467-468, doi: 10.1002 / lsm.22368 , PMID 25952726 .

[Altmeyer-1] Encyclopedia Dermatology

[2] Mucinosis, papular, acral-persistent. In: Orphanet (Rare Disease Database).

[3] H. Montgomery, LJ Underwood: Lichen myxedematosus; differentiation from cutaneous myxedemas or mucoid states. In: The Journal of investigative dermatology. Volume 20, Number 3, March 1953, pp. 213-236, PMID 13044990 .

[4] MS Graves, AA Lloyd, EV Ross: Treatment of acral persistent papular mucinosis using an Erbium-YAG laser. In: Lasers in Surgery and Medicine . Volume 47, Number 6, August 2015, pp. 467-468, doi: 10.1002 / lsm.22368 , PMID 25952726 .