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Background

This is a rare cutaneous mucinosis characterized by multiple small papules on the upper extremities and trunk occurring in infants. Under the microscope, there is mucin in the papillary dermis with no fibroblastic proliferation and scattered chronic inflammatory cells. Some investigators speculate that this a connective tissue nevus similar to nevus mucinosis. However, there is growing consenus that this disease is a variant of lichen myxedematosus (localized scleromyxedema) and has been renamed papular mucinosis of infancy.

OUTLINE

Epidemiology  
Disease Associations  
Pathogenesis  
Laboratory/Radiologic/
Other Diagnostic Testing
 
Gross Appearance and Clinical Variants  
Histopathological Features and Variants  
Special Stains/
Immunohistochemistry/
Electron Microscopy
 
Differential Diagnosis  
Prognosis  
Treatment  
Commonly Used Terms  
Internet Links  

EPIDEMIOLOGY CHARACTERIZATION
SYNONYMS  
INCIDENCE/
PREVALENCE
 
AGE  
SEX  
GEOGRAPHY  
EPIDEMIOLOGIC ASSOCIATIONS  

 

DISEASE ASSOCIATIONS CHARACTERIZATION

 

PATHOGENESIS CHARACTERIZATION

 

LABORATORY/
RADIOLOGIC/
OTHER TESTS

CHARACTERIZATION
RADIOLOGIC  
LABORATORY MARKERS  

 

GROSS APPEARANCE/
CLINICAL VARIANTS
CHARACTERIZATION
GENERAL  
VARIANTS  

 

HISTOLOGICAL TYPES CHARACTERIZATION
GENERAL  

 

SPECIAL STAINS/
IMMUNOPEROXIDASE/
OTHER
CHARACTERIZATION
SPECIAL STAINS  
IMMUNOPEROXIDASE  
ELECTRON MICROSCOPY  

 

DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES
DERMAL MUCINOSIS  
SELF-HEALING JUVENILE CUTANEOUS MUCINOSIS  
Self-healing juvenile cutaneous mucinosis: cases highlighting subcutaneous/fascial involvement.

Department of Dermatology, University of North Carolina Hospitals, Chapel Hill, NC, USA.

 

J Am Acad Dermatol. 2006 Dec;55(6):1036-43. Abstract quote

BACKGROUND: Self-healing juvenile cutaneous mucinosis is a rare disease affecting young people characterized by transient cutaneous lesions and sometimes mild inflammatory symptoms. The deep dermal and subcutaneous features of this disorder have not yet been well described.

OBJECTIVE: The purpose of our study was to present 3 cases of self-healing juvenile cutaneous mucinosis in which the histopathologic features caused diagnostic confusion between this disorder and proliferative fasciitis.

METHODS: The study includes clinical and histologic findings of 3 patients, complemented by a literature review. RESULTS: The histologic descriptions of nodular lesions in self-healing juvenile cutaneous mucinosis reveal features of proliferative fasciitis, including a myxoid stroma and gangliocyte-like giant cells.

LIMITATIONS: Self-healing juvenile cutaneous mucinosis is a rare condition and has not been frequently reported in medical literature. Our findings are based on the pathologic features of 3 patients.

CONCLUSIONS: Our findings further elucidate the histologic features of self-healing juvenile cutaneous mucinosis and expand the differential diagnosis for entities in which gangliocyte-like giant cells are noted.

 

PROGNOSIS CHARACTERIZATION

 

TREATMENT CHARACTERIZATION
GENERAL  
   

Macpherson and Pincus. Clinical Diagnosis and Management by Laboratory Methods. Twentyfirst Edition. WB Saunders. 2006.
Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 6th Edition. McGraw-Hill. 2003.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.


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Last Updated December 7, 2006

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