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Ugeskrift For Laeger Nov 2022
Topics: Humans; Adiposis Dolorosa
PubMed: 36345901
DOI: No ID Found -
Journal of the American Academy of... Mar 2023
Topics: Humans; Adiposis Dolorosa; Retrospective Studies; Obesity; Pain
PubMed: 36228943
DOI: 10.1016/j.jaad.2022.09.050 -
Cureus Aug 2022Here, we report a case of a 70-year-old female who presented with a slowly enlarging tender nodule on the right forearm for several months. Physical examination showed a...
Here, we report a case of a 70-year-old female who presented with a slowly enlarging tender nodule on the right forearm for several months. Physical examination showed a faintly blue-tinged freely mobile subcutaneous nodule. Excision was complicated by greater than expected bleeding and revealed an unexpected intravenous mass. Histopathology demonstrated capillary lobules separated by fibrous septae within a vein, consistent with intravenous lobular capillary hemangioma (IVLCH). IVLCH is a rare benign capillary proliferation of unclear etiology. Excision is typically curative and relieves any pain and discomfort the patient might be experiencing. With the addition of IVLCH, we respectfully propose a new acronym for the differential diagnosis of cutaneous tender tumors: intravenous lobular capillary hemangioma, foreign body (reaction), hidradenoma, osteoma cutis, glomus tumor, scar, fibromyxoma, leiomyosarcoma, eccrine angiomatous hamartoma, Dercum's disease (adiposis dolorosa), piezogenic pedal papule, eccrine spiradenoma, neurilemmoma (schwannoma), calcinosis cutis, angioendotheliomatosis, leiomyoma, metastases, angiolipoma, neuroma, dermatofibroma, granular cell tumor, endometriosis, thrombus, blue rubber bleb nevus, angioma, chondrodermatitis nodularis helicis, and keloid ("IF HOGS FLED PEN, CALM AND GET BACK"). Future additions to the cutaneous tender tumor differential diagnosis may require creative additions and rearrangements to this acronym. However, continual updates will allow it to serve both clinicians and pathologists alike as a comprehensive representation of etiologies to consider for cutaneous tender tumors.
PubMed: 36120217
DOI: 10.7759/cureus.28030 -
The Yale Journal of Biology and Medicine Dec 2021Dercum's disease (DD), also described as adiposis dolorosa, is a poorly understood and rare adipose tissue disorder involving obesity and painful adipose tissue masses.... (Review)
Review
Dercum's disease (DD), also described as adiposis dolorosa, is a poorly understood and rare adipose tissue disorder involving obesity and painful adipose tissue masses. Patients may have associated bruising and constitutional symptoms such as fatigue, difficulty concentrating, and sleep disturbance. DD was initially described in 1888 by Francis Xavier Dercum, and was classified into four subtypes, including generalized diffuse, generalized nodular, localized nodular, and juxta-articular subtypes. While this disease has been described for more than 130 years, its etiology and treatment remain elusive. We describe a case of a patient with DD who presented to Ochsner Medical Center, New Orleans, LA, for evaluation of treatment options. We review current knowledge on this rare disease and data on modern treatment methods.
Topics: Adipose Tissue; Adiposis Dolorosa; Diagnosis, Differential; Humans; Obesity; Pain
PubMed: 34970098
DOI: No ID Found -
Medicine Dec 2021Dercum's disease (DD), or adiposis dolorosa, is a rare condition of unknown etiology characterized by growth of painful subcutaneous adipose tissue. No specific...
Dercum's disease (DD), or adiposis dolorosa, is a rare condition of unknown etiology characterized by growth of painful subcutaneous adipose tissue. No specific treatment exists. Pain is often invalidating and resistant to analgesic drugs. We tested the efficacy of Frequency Rhythmic Electrical Modulation System (FREMS) therapy on pain relief. Subcutaneous biopsies were performed for genetic analysis.Nine DD patients were enrolled. Five cycles of FREMS at 3-month intervals during 1 year were administered. Visual analogue scale (VAS), Bartel Index Questionnaire and Short Form 36 questionnaire were used to measure pain and general health status at baseline, 6 and 12 months. Dual-energy X-ray absorptiometry (DEXA) quantified fat mass. Next-Generation Sequencing (NGS) was performed on adipose tissue biopsies and peripheral blood sample to search for somatic variants and specific protein pathway mutation.Seven patients were included in the final analysis. FREMS induced a reduction in VAS score (from 92 to 52.5, P = .0597) and a significant improvement in SF-36 domains (Physical functioning, Role limitation due to physical health, Body pain, Vitality, Social functioning, P < .05). No modification in anthropometrics and DEXA values was observed. The analysis of the mitochondrial Displacement loop (D-loop) region confirmed the clonality of all lipomatous lesions. The presence of the mitochondrially encoded tRNA-Lysine (MT-TK) m.8344A>G variant, occasionally identified in patients with multiple symmetric lipomatosis, was excluded in all subjects. On the other hand, we observed variants in genes belonging to signaling pathways involved in cell cycle and proliferation (Phosphoinositide 3-kinase/AKT/mTOR, MAPK/ERK, and Hippo).FREMS can be a useful tool to alleviate pain and improve overall quality of life in patients with DD. Genetic analysis highlighted the molecular heterogeneity of lipomas.
Topics: Adiposis Dolorosa; Adult; Female; High-Throughput Nucleotide Sequencing; Humans; Lipoma; Male; Middle Aged; Pain; Pain Management; Pilot Projects; Quality of Life; Transcutaneous Electric Nerve Stimulation
PubMed: 34941153
DOI: 10.1097/MD.0000000000028360 -
Cureus Sep 2021Adiposis dolorosa, also known as Dercum's disease, is a rare disorder characterized by debilitating painful lipomas throughout the body. The prevalence and etiology...
Adiposis dolorosa, also known as Dercum's disease, is a rare disorder characterized by debilitating painful lipomas throughout the body. The prevalence and etiology of Dercum's disease are unknown as mentioned in the National Organization of Rare Disorders. We present an interesting case of Dercum's disease in a 53-year-old female who initially presented with a six-week history of painful subcutaneous masses. Ultrasound findings were suggestive of lipomas, however, her symptoms were debilitating beyond that of benign lipomas. She then represented with a rapidly increasing number of soft tissue masses manifesting throughout her body, as well as significant diffuse pain concentrating around these lesions within a short period of time following her initial presentation. The patient underwent surgical excision of a select number of these masses, with histopathology consistent with lipomas. Most cases of Dercum's disease are sporadic, and no guidelines exist regarding the treatment of the disease. Due to the rarity of this condition, in conjunction with simple lipomas typically presenting as painless masses, many patients may be misdiagnosed and neglected due to being falsely labeled as pain seeking or having their symptoms attributed to psychological disorders. Management, therefore, is complex and currently consists of a multidisciplinary approach employing multimodal treatments, including pain control, surgical excision, and psychotherapy. Although this condition has been described in the literature for over 100 years, there have been minimal advancements towards alleviating the suffering of these patients. We aimed to unearth and bring to light the reality and the suffering experienced by patients with Decrum's disease.
PubMed: 34646696
DOI: 10.7759/cureus.17649 -
Georgian Medical News 2021Both lipedema and juxta-articular adiposis dolorsa are painful disorders of subcutaneous adipose tissue. We investigated 297 female patients with lipedema treated at our...
Both lipedema and juxta-articular adiposis dolorsa are painful disorders of subcutaneous adipose tissue. We investigated 297 female patients with lipedema treated at our department for the presence of juxta-articular adiposis dolorsa. Occurrence of both disorders was identified in 4.4% of lipedema patients. The common presence of both disorders was observed only in more advanced lipedema (grade II and III). Juxta-articular adiposis dolorosa of the knees is seen exclusively on the inner knees, and it presents neither bruising nor creases or hypothermia. Choices of surgical treatment are either microcannula liposuction or dermolipectomy. Recurrences have not been observed.
Topics: Adiposis Dolorosa; Female; Humans; Lipedema
PubMed: 34511435
DOI: No ID Found -
JAAD Case Reports Sep 2021
PubMed: 34409145
DOI: 10.1016/j.jdcr.2021.07.009 -
Clinics in Dermatology 2021Francis Xavier Dercum (1856-1931) was a renowned clinician, teacher, and scientist. Although he is widely known as a prominent neurologist and remembered for his...
Francis Xavier Dercum (1856-1931) was a renowned clinician, teacher, and scientist. Although he is widely known as a prominent neurologist and remembered for his treatment of then-President Woodrow Wilson, he also had many contributions to the field of medicine. Specific to dermatology, he provided the first observations and descriptions of adiposis dolorosa, which laid the groundwork for further examination and research. He dedicated his life as a scholar to teaching others and furthering the field of medicine.
Topics: Adiposis Dolorosa; Humans; Male; Medicine; Physicians
PubMed: 34272035
DOI: 10.1016/j.clindermatol.2020.11.008