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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 12  |  Issue : 3  |  Page : 371-375

Partial lipodystrophy of limbs in type 2 diabetes: A case report


Dia Care – Diabetes and Hormone Clinic, Ahmedabad, Gujarat, India

Date of Submission31-Jan-2021
Date of Decision23-May-2021
Date of Acceptance24-May-2021
Date of Web Publication30-Sep-2021

Correspondence Address:
Dr. Meet Shah
Dia Care – Diabetes and Hormone Clinic 1 & 2 Gandhi Park, Near Nehrunagar Cross Roads, Ambawadi, Ahmedabad 380015, Gujarat.
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/JOD.JOD_15_21

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  Abstract 

Rationale: On account of the diversity and rarity of lipodystrophy, this condition may be frequently unrecognized or misdiagnosed. This tends to be concerning as it is progressive and has potentially life-threatening complications. Patient Concerns: The patient was referred to our clinic with chief complaints of uncontrolled glycemia and an increased frequency of urination since last 3 months. The patient complained of weakness that led to thinning of upper limb muscles, loss of fat from face, neck, and upper thorax with simultaneous increase in abdominal girth, flattening of buttocks, and reduction in girth of lower limb muscles. Diagnosis: The patient was diagnosed with partial lipodystrophy of limbs (PLL) based on unique clinical features, blood investigations, and a DEXA fat scan. Interventions: The patient was treated with basal insulin glargine, metformin 1.5 g, dapagliflozin 10 mg, pioglitazone 7.5 mg, and atorvastatin 40 mg. He was vigorously educated to follow a strict diet and an exercise plan with necessary lifestyle modifications. Outcomes: After 3 months, the patient achieved better glycemic control with a reduction in HbA1c from 9.5% to 7.2%, as well as an improvement in lipid profile. He additionally achieved a weight reduction of 12 kg, with reduction in his abdominal girth from 130 to 118 cm. Lessons: The recognition of PLL in patients with type 2 diabetes can help better clinical management by alerting physicians to the associated comorbidities. Many a times, PLL goes unnoticed on account of the slow progression. Our case highlights the need for greater recognition of PLL as it has specific metabolic features that help in guiding appropriate clinical management.

Keywords: Insulin resistance, lipodystrophy, partial lipodystrophy of limbs


How to cite this article:
Shah M, Saboo B. Partial lipodystrophy of limbs in type 2 diabetes: A case report. J Diabetol 2021;12:371-5

How to cite this URL:
Shah M, Saboo B. Partial lipodystrophy of limbs in type 2 diabetes: A case report. J Diabetol [serial online] 2021 [cited 2021 Nov 30];12:371-5. Available from: https://www.journalofdiabetology.org/text.asp?2021/12/3/371/327301




  Introduction Top


Lipodystrophy syndromes are a diverse group of diseases with loss of functional adipocytes as the primary defect that eventually results in severe dyslipidemia, ectopic steatosis, and insulin resistance.[1] Lipodystrophy, based on the type of inheritance, can be classified as congenital or acquired. It is also classified as generalized or partial depending on the loss of adipose tissue.[2] Between 2012 and 2014, the estimated prevalence of diagnosed lipodystrophy was 3.07 cases/million, with 0.23 cases/million for generalized lipodystrophy and 2.84 cases/million for partial lipodystrophy.[3]

Partial lipodystrophy of limbs (PLL), a relatively common form of lipodystrophy in diabetes, is characterized by insulin-resistant diabetes and symmetrical distal lipoatrophy of the limbs. The common phenotypic presentations include loss of subcutaneous fat in the forearms, thighs, and calves and loss of fat in forearms and calves.[4] However, diagnosis of partial lipodystrophy is frequently delayed, particularly in males and frequently in females.[5]

Here, we present the case of an adult male with PLL and type 2 diabetes. Peculiarities of this case that differentiate it from that of metabolic syndrome include specific clinical features, marked insulin resistance, need for aggressive dyslipidemia management, and periodic screening of associated comorbidities/consequences of lipodystrophy. We believe that PLL in type 2 diabetes requires greater recognition; knowledge of PLL may help in identifying such not-so-uncommon cases.


  Case Report Top


A 48-year-old male, known case of type 2 diabetes, was referred to our clinic with chief complaints of uncontrolled glycemia and increased frequency of urination since last 3 months. The patient was incidentally diagnosed with type 2 diabetes 8 years ago, at which point his weight was 102 kg with central obesity and mild thinning of both upper and lower limb muscles. The thinning was progressive in nature but the patient noticed prominent changes over the last 3 years. It started with weakness leading to thinning of upper limb muscles, loss of fat from face, neck, and upper thorax with simultaneous increase in abdominal girth, and flattening of both buttocks with reduction in girth of lower limb muscles. The patient continued to gain weight since diagnosis. He had no comorbidities and did not require any major hospitalization. Neither of his parents were diabetic and had never observed any such change in physique in other family members; he had a sedentary lifestyle with irregular food habits.

The referring primary care physician was treating the patient with a varied combination of oral antidiabetic agents; when referred, the patient was on metformin 1 g twice daily, glimepiride 1 mg twice daily, and teneligliptin 20 mg once daily for the previous 6 months. As the patient did not achieve good glycemic control despite multiple oral agents, he was referred to us by the primary care physician for glycemia control. Cushing’s syndrome was ruled out on the basis of the normal morning serum cortisol level [Table 1].
Table 1: Laboratory investigations at baseline

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Physical examination revealed a height of 183 cm, weight of 112 kg, abdominal girth of 130 cm, and hip girth of 121 cm; the calculated body mass index was 33.4 kg/m2. His vitals were within normal limits. Presence of acanthosis nigricans was noted over the patient’s neck. [Figure 1] shows features of marked fat loss and thinning of all four limbs, flat buttocks, with significant central obesity. The patient’s laboratory investigations at baseline are elucidated in [Table 1]. His liver and renal function tests were within normal limits and HIV was negative.
Figure 1: Shows classical features of PLL characterized by central obesity, flat buttocks and thinning of both upper limb and lower limb muscles

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An abdominal ultrasound (USG) revealed marked hepatomegaly with changes of fatty liver. All other remaining findings were normal. A Dexa fat scan recorded the total android/gynecoid (A/G) ratio as 1.14, indicative of marked central obesity. The fat (in g) was least in the arms followed by legs, compared with the trunk. The fat mass ratio seemed to clearly correlate with clinical features of thinning of limbs [Figure 2].
Figure 2: DEXA SCAN which shows fat distribution in different body compositions

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  Management Top


Diagnosis of type 2 diabetes with lipodystrophy, contributing to significant insulin resistance, was based on clinical features and laboratory and radiological investigations. For glycemic control, the patient was initiated on basal insulin glargine (16 U at bed time, with titration as per self-monitoring of blood glucose), metformin 1.5 g, dapagliflozin 10 mg, and pioglitazone 7.5 mg in view of marked insulin resistance, and atorvastatin 40 mg was added for management of dyslipidemia.

The patient was vigorously educated to follow a strict diet and exercise plan with necessary lifestyle modifications; a high protein with low fat and low carbohydrate diet was advised. Resistance training exercises were explained in view of muscle strengthening.

The patient was followed up via teleconsultation on a regular basis to optimize the dose of insulin, and dietary recall was reinforced. After 3 months, his glucose level was relatively well controlled with a reduction in HbA1c to 7.2%, along with an improvement in lipid profile. Moreover, he achieved a weight reduction of 12 kg. Though his abdominal girth reduced to 118 cm, there were no changes in his limb girths.


  Discussion Top


PLL in type 2 diabetes represents a phenotype that further epitomizes the heterogeneous nature of type 2 diabetes. This form does not follow the conventional diagnostic structure of either congenital vs. acquired or generalized vs. partial. Considering the absence of family history and onset in adulthood, this form of lipodystrophy is indeed partial and seems to be acquired. These individuals are discerned by the presence of prediabetes or type 2 diabetes, symmetrical lipodystrophy of the forearms, or both forearms and calves, or infrequently, whole limbs, and acanthosis nigricans. Such patients have higher insulin resistance and worse glycemia, suggesting clinical significance of this form of lipodystrophy.

This form of lipodystrophy also has implications in terms of clinical management. Pharmacotherapy in conjunction with dietary intervention plays a critical role in management of diabetes and dyslipidemia.[6] The choice and dose of antidiabetic medications must be based on the degree of insulin resistance and dearth of insulin-mediated glucose uptake; careful evaluation and aggressive therapy should be undertaken for steatohepatitis and marked hypertriglyceridemia.[7]

Insulin resistance should be treated with metformin, pioglitazone, and insulin (wherever required), whereas statins, fenofibrate, and omega-3 fatty acids must be used for dyslipidemia, and bariatric surgery is recommended in selected patients.[1] The recombinant form of leptin, “Metreleptin,” is available and approved by the Food and Drug Administration (FDA) as an adjuvant drug in patients with generalized lipodystrophy.[8] Metreleptin therapy has been found to be effective for metabolic complications in selected patients with partial lipodystrophy.[6] There is a need for the addressal of appropriate screening and treatment of associated comorbidities.


  Conclusion Top


In patients with abnormal deposition of subcutaneous fat, one must include a differential diagnosis of lipodystrophy, confirmation of which could be based on genetic testing. PLL with diabetes is not an uncommon entity and is characterized by marked insulin resistance. Aggressive treatment of diabetes with insulin, metformin, and pioglitazone along with that of dyslipidemia plays a significant role. Presently, lipodystrophy remains unnoticed among primary care physicians, and it is essential to identify this clinical entity. Early detection of lipodystrophy could help prevent the possible comorbidities/complications by reinforcing diet, lifestyle modifications, and apt treatment.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Akinci B, Sahinoz M, Oral E. Lipodystrophy syndromes: Presentation and treatment. [Updated April 24, 2018]. In: Feingold KR, Anawalt B, Boyce A, et al, editors. Endotext [Internet]. South Dartmouth, MA: MDText.com, Inc.; 2000.  Back to cited text no. 1
    
2.
Araújo-Vilar D, Santini F. Diagnosis and treatment of lipodystrophy: A step-by-step approach. J Endocrinol Invest 2019;42:61-73.  Back to cited text no. 2
    
3.
Chiquette E, Oral EA, Garg A, Araújo-Vilar D, Dhankhar P. Estimating the prevalence of generalized and partial lipodystrophy: Findings and challenges. Diabetes Metab Syndr Obes 2017;10:375-83.  Back to cited text no. 3
    
4.
Demir T, Akinci B, Demir L, Altay C, Atik T, Cavdar U, et al. Partial lipodystrophy of the limbs in a diabetes clinic setting. Prim Care Diabetes 2016;10:293-9.  Back to cited text no. 4
    
5.
Stears A, Hames C. Diagnosis and management of lipodystrophy: A practical update. Clin Lipidol 2014;9:235-59.  Back to cited text no. 5
    
6.
Brown RJ, Araujo-Vilar D, Cheung PT, Dunger D, Garg A, Jack M, et al. The diagnosis and management of lipodystrophy syndromes: A multi-society practice guideline. J Clin Endocrinol Metab 2016;101:4500-11.  Back to cited text no. 6
    
7.
Strickland LR, Guo F, Lok K, Garvey WT. Type 2 diabetes with partial lipodystrophy of the limbs: A new lipodystrophy phenotype. Diabetes Care 2013;36:2247-53.  Back to cited text no. 7
    
8.
Handelsman Y, Oral EA, Bloomgarden ZT, Brown RJ, Chan JL, Einhorn D, et al; American Association of Clinical Endocrinologists. The clinical approach to the detection of lipodystrophy—An AACE consensus statement. Endocr Pract 2013;19: 107-16.  Back to cited text no. 8
    


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