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Papular lesions over face have a wide range of differential diagnoses. The clinical differentiation among these is difficult and often requires clinicopathological correlation. We hereby report the case of a 28 year old female presenting with numerous skin colored papular lesions over face, pinna and upper limbs.

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Background: Hyaline fibromatosis syndrome (HFS) is a recently introduced alternative term for two disorders that were previously known as juvenile hyaline fibromatosis (JHF) and infantile systemic hyalinosis (ISH). These two variants are secondary to mutations in the anthrax toxin receptor 2 gene (ANTXR2) located on chromosome 4q21. The main clinical features of both entities include papular and/or nodular skin lesions, gingival hyperplasia, joint contractures and osteolytic bone lesions that appear in the first few years of life, and the syndrome typically progresses with the appearance of new lesions.

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Self-healing juvenile cutaneous mucinosis.

Arch Dermatol

April 1995

Institute of Dermatologic Sciences, University of Milan, Ospedale Maggiore Institute of Research With Scientific Character, Italy.

Background: Mucinoses represent a puzzling and heterogeneous group of rare diseases, and self-healing juvenile cutaneous mucinosis is an extremely rare disease among them.

Observations: A scleroedematous condition of the face, associated with papular lesions and arthropathies, had occurred in a 5-year-old boy 10 days after onset of fever, arthralgia, muscle tenderness, and weakness.

Results: Histologic examination revealed an edematous dermis, occupied by mucin.

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