Two Cases of Hemihyperplasia–Multiple Lipomatosis Syndrome and Review of Asymmetric Hemihyperplasia Syndromes

Craiglow, Brittany G.; Ko, Christine J.; Antaya, Richard J.

doi:10.1111/pde.12071

Cited by 6 publications

(4 citation statements)

References 10 publications

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“…Asymmetrically distributed growing regions of lipomatous tissue are seen in Proteus syndrome and hemihyperplasia-multiple lipomatosis syndrome ( 6 ). However, these conditions are often congenital and are associated with vascular malformations ( 7 ). In our patient, asymmetrically distributed non-tender lipomatosis developed around the age of 65 years; therefore, our case does not fit into either of these entities.…”

Section: Discussionmentioning

confidence: 99%

Adult-onset Asymmetrical Lipomatosis

Aoki

Miyagawa²,

Ogawa³

et al. 2020

Acta Derm Venereol

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Section: Discussionmentioning

confidence: 99%

Adult-onset Asymmetrical Lipomatosis

Aoki

Miyagawa²,

Ogawa³

et al. 2020

Acta Derm Venereol

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“…HHML syndrome (hemihyperplasia-multiple lipomatosis syndrome) is a rare, genetic overgrowth syndrome (progressive, asymmetrical, moderate hemihyperplasia) associated with multiple, slow-growing, painless, subcutaneous lipomas distributed throughout entire body [54].…”

Section: Diagnosismentioning

confidence: 99%

Rare Forms of Lipomatosis: Dercum’s Disease and Roch-Leri Mesosomatous Lipomatosis

Lemaître

Aubert

Chevalier³

et al. 2021

JCM

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In contrast to obesity, which is very frequent, lipomatosis and lipodystrophy syndromes are rare diseases of adipose tissue. Lipodystrophy syndromes are characterized by metabolic abnormalities associated with partial or generalized lipoatrophy. Lipomatosis is defined by the presence of several body lipomas without lipoatrophy. Dercum’s disease (DD) and Roch-Leri mesosomatous lipomatosis (RLML) are rare and poorly characterized forms of lipomatosis. They have raised little clinical interest despite the non-negligible consequences of DD on quality of life. The main clinical presentation of these diseases includes multiple lipomas, which are painful in DD (in contrast to RLML). The two diseases are frequently associated with obesity and metabolic syndrome, with hypertension, diabetes, or dyslipidemia. The long-term course of the diseases remains poorly described. DD affects mainly women, whereas RLML mostly affects men. In both diseases lipomas are found on the back and thighs, as well as on the abdomen in DD and the forearms in RLML. The painful lipomas tend to recur after surgery in DD (in contrast to RLML). Most cases are sporadic. No specific treatment has been identified, as the pathophysiology remains unknown. Nevertheless, low-grade fat inflammation and specific abnormalities such as hyperbasophilia deserve further investigation. The aim of this review is to analyze the available literature on the topic.

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“…In addition to the lipomas, vascular anomalies and connective tissue nevi are present in the various phenotype subtypes including the CLOVES and Proteus syndromes. [326][327][328][329] Facial infiltrating lipomatosis is another phenotypic expression of the PIK3CA spectrum. [330][331][332] Encephalocraniocutaneous lipomatosis not only has spinal and intracranial lipomas but aplasia cutis congenita with an associated localized lipoma.…”

Section: Adipocytic-lipomatous Tumorsmentioning

confidence: 99%

Nonepithelial Tumors and Tumor-like Lesions of the Skin and Subcutis in Children

Dehner

Gru

2018

Pediatr Dev Pathol

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This overview of mesenchymal tumors presenting in the skin and/or subcutis in children brings together the range of neoplasms and hamartomas which are seen in this age-group. It is not surprising from the perspective of the pediatric or general surgical pathologist that vascular anomalies, including true neoplasms and vascular malformations, are the common phenotypic category. Since there is considerable morphologic overlap among these lesions, clinicopathologic correlation may be more important than for many of the other mesenchymal tumors. The skin and subcutis are the most common sites of clinical presentation for the infantile myofibroma which is the most common of fibrous mesenchymal tumors in children. Several of the other mesenchymal tumors are more common adults-like dermatofibrosarcoma protuberans, but nonetheless have an important presence in children, even as a congenital neoplasm. A lipomatous tumor in a young child should be considered as a possible manifestation of an overgrowth syndrome.

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Two Cases of Hemihyperplasia–Multiple Lipomatosis Syndrome and Review of Asymmetric Hemihyperplasia Syndromes

Cited by 6 publications

References 10 publications

Adult-onset Asymmetrical Lipomatosis

Adult-onset Asymmetrical Lipomatosis

Rare Forms of Lipomatosis: Dercum’s Disease and Roch-Leri Mesosomatous Lipomatosis

Nonepithelial Tumors and Tumor-like Lesions of the Skin and Subcutis in Children

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