Association of dermal melanocytosis with lysosomal storage disease: clinical features and hypotheses regarding pathogenesis.

Association of dermal melanocytosis with lysosomal storage disease: clinical features and hypotheses regarding pathogenesis. Research Abstract Details 

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Association of dermal melanocytosis with lysosomal storage disease: clinical features and hypotheses regarding pathogenesis. Abstract Text:

BACKGROUND: The potential association of dermal melanocytosis with lysosomal storage disease in infancy is an uncommonly known and poorly understood entity. OBSERVATIONS: We describe 2 infants with extensive dermal melanocytosis in association with GM1 gangliosidosis type 1 and Hurler syndrome, respectively. A literature analysis revealed 37 additional cases. Clinically, dermal melanocytosis associated with lysosomal storage disease is characterized by extensive, blue cutaneous pigmentation with dorsal and ventral distribution, indistinct borders, and persistent and/or "progressive" behavior. GM1 gangliosidosis type 1 and Hurler syndrome are the most common underlying disorders associated with these cutaneous features. CONCLUSIONS: In the appropriate clinical setting, an unusual presentation of dermal melanocytosis in an infant may be a cutaneous sign of an underlying lysosomal storage disease. The pathogenetic mechanisms behind this association remain to be elucidated.

Association of dermal melanocytosis with lysosomal storage disease: clinical features and hypotheses regarding pathogenesis. Publishing Authors By Initials

For similar skin and connective tissue diseases: skin diseases: pigmentation disorders research abstracts see: skin and connective tissue diseases: skin diseases: pigmentation disorders research

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Association of dermal melanocytosis with lysosomal storage disease: clinical features and hypotheses regarding pathogenesis. Journal Published:

PUBLICATION TYPE: Review

Journal: Archives of dermatology

VOLUME: 139

Page Numbers: 916-20

Journal Abbreviation: Arch Dermatol

ISSN: 0003-987X

DAY: 17

MONTH: Jul

YEAR: 2003

Association of dermal melanocytosis with lysosomal storage disease: clinical features and hypotheses regarding pathogenesis. Information

Number of References: 37

LANGUAGE: eng

NlmUniqueID: 372433

Association of dermal melanocytosis with lysosomal storage disease: clinical features and hypotheses regarding pathogenesis. Keywords Mesh Terms:

KEYWORDS: Pigmentation Disorders

MESH TERMS: pathology

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Grant and Affiliation Information for Association of dermal melanocytosis with lysosomal storage disease: clinical features and hypotheses regarding pathogenesis.

AFFILIATION: Department of Dermatology, Texas Children's Hospital, Baylor College of Medicine, Houston, 77030, USA.

Country: United States

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MEDLINETA: Arch Dermatol

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