- Email: [email protected]
Childhood acquired lipodystrophy: A retrospective study
REPORTS
Childhood acquired lipodystrophy: A retrospective study Elena Pope, MD, MSc, FRCPC, Allison Janson, BSc, Amina Khambalia, BSc, MSc, and Brian Feldman, MD, MSc Toronto, Ontario, Canada Objective: We sought to describe the clinical characteristics and complications of children with acquired lipodystrophy (LD). Methods: We conducted a retrospective chart review at a tertiary, academic children’s hospital of children clinically given a diagnosis of acquired LD between January 1997 and December 2004. Results: During the study period, 23 patients were identified. Their mean age at diagnosis was 9.74 6 3.98 years. Of patients, 61% were girls. The length of the follow-up was 4.8 6 3.5 years from the time of LD diagnosis. Of patients, 30% had evidence of localized disease (7 of 23), 26% (6 of 23) had localized partial disease, and 44% (10 of 23) had generalized LD. The most common underlying diagnosis was dermatomyositis (78%), alone or in association with other autoimmune diseases (juvenile rheumatoid arthritis 17%). Panniculitis with autoimmunity was noted in 17% of the patients. More than half of the patients had at least one complication attributable to LD such as acanthosis nigricans (22%), hyperpigmentation (22%), hepatomegaly (13%), hypertension (13%), protuberant abdomen (9%), and hyperlipidemia (4%). The only predictor for development of complications was the age of diagnosis of LD, with younger age being associated with increased risk (7 vs 12 years, P = .04). Limitations: Limitations were inherent to the retrospective design. Conclusions: Childhood acquired LD is seen more frequently in the context of autoimmunity. Affected children should be monitored for the development of complications, particularly if given a diagnosis of LD at a younger age. ( J Am Acad Dermatol 2006;55:947-50.)
L
ipodystrophy (LD) is a rare heterogeneous disease characterized by the depletion of subcutaneous adipose tissue. Although uncommon in childhood, acquired LD may have its onset during childhood or adolescence. In children, acquired LD is strongly associated with panniculitis,1 juvenile dermatomyositis (JDM),2 and systemic lupus erythematosus.3 Very localized forms of the disease have been noted after local trauma, particularly subcutaneous injections of various medications.3-5 In addition to cosmetic implications, LD has been associated with metabolic disorders, most notably From the Hospital for Sick Children, University of Toronto. Funding sources: None. Conflicts of interest: None identified. Accepted for publication May 3, 2006. Reprint requests: Elena Pope, MD, MSc, FRCPC, Section of Dermatology, Hospital for Sick Children, 555 University Ave, Toronto, Ontario, Canada M5G 1X8. E-mail: [email protected]. Published online June 13, 2006. 0190-9622/$32.00 ª 2006 by the American Academy of Dermatology, Inc. doi:10.1016/j.jaad.2006.05.005
Abbreviations used: JDM: juvenile dermatomyositis LD: lipodystrophy RA: rheumatoid arthritis
insulin resistance, hepatomegaly, hyperlipidemia, acanthosis nigricans, and diabetes mellitus.6 To date, there is very little information on childhood acquired LD, particularly related to the clinical characteristics and the risk factors predictive of future metabolic complications. The purpose of this study was to review our institution’s experience with childhood onset acquired LD (excluding those secondary to HIV infection and localized scleroderma,) to increase our understanding about patients at risk for the development of LD and its complications.
METHODS The study was approved by our institution’s ethics research board. Our institution is a tertiary/ 947
948 Pope et al
J AM ACAD DERMATOL DECEMBER 2006
Table I. Complications of lipodystrophy
Complications
No. (%)
Ages of diagnosis for the complication, y
Acanthosis nigricans Hyperpigmentation Transaminitis Hepatomegaly Menstrual dysfunction Protuberant abdomen Hyperlipidemia
5 5 5 3 2 2 1
8, 11, 12, 14, 15 NS NS 4, 8, 12 NS NS NS
(22) (22) (22) (13) (9) (9) (4)
NS, Not specified.
quaternary referral center with a provincial catchment area. The inclusion criteria for this study were pediatric patients 0 to 18 years of age with a diagnosis of acquired LD from January 1997 to December 2004. Cases of presumed congenital LD and acquired LD secondary to HIV and localized scleroderma were excluded. A search by diagnosis (LD/lipoatrophy) was performed using dermatology and rheumatology databases. To ensure consistency, we included only patients who had the LD diagnosis mentioned in both the written record and the dictated letter and/or in the written record from at least two subsequent follow-up visits. Because there is no standard diagnostic tool for LD, diagnosis date was recorded as the date of the first written record of ‘‘lipodystrophy’’ or ‘‘lipoatrophy.’’ The follow-up date was recorded as the date of the last patient visit to our institution, regardless of whether LD or lipoatrophy was mentioned. Data collection included patient characteristics, underlying diagnosis, personal medical history, duration of follow-up, laboratory investigations at diagnosis and last follow-up visit, treatment of the underlying disease, and new medical diagnoses during the study period. Patients were categorized as having localized LD if only one body part was affected (ie, one arm), partial LD if face and arms were affected, and generalized LD if more than two body parts were affected, based on the location described in the medical records. Descriptive statistics were performed using mean, median, and proportions. Univariate analysis was used to compare patients with normal versus abnormal investigation findings. Fisher’s exact test was used for categorical variables because of small cell sizes and the Mann-Whitney U test was used for nonnormally distributed continuous data. A P value of .05% was considered statistically significant. Statistical analyses were conducted using software (SPSS for Windows 11.0, SPSS Inc, Chicago, Ill).
RESULTS During the study period, 23 patients met the inclusion criteria. Their mean age at diagnosis was 9.74 6 3.98 years. Girls represented 61% of patients. Of patients, 30% had evidence of localized disease (7 of 23), 26% (6 of 23) had partial disease, and 44% (10 of 23) had generalized LD. The most common affected body area was the upper extremity (78%, 18 of 23), followed by the face (73%, 17 of 23) and the lower extremity (39%, 9 of 23). The average length of follow-up was 4.8 6 3.5 years from the time of the LD diagnosis. The most common underlying diagnosis was JDM (78%), alone (65%) or in association with other autoimmune diseases such as juvenile rheumatoid arthritis (RA) (13%). One patient had juvenile RA without evidence of other autoimmune disease (4%). Panniculitis in the context of other autoimmune disorders (ie, Graves’ disease, JDM, and vasculitis) was noted in 17% of the patients. One patient’s LD was considered to be idiopathic (4%). The diagnosis of LD was made shortly after the initial underlying diagnosis of panniculitis (mean 0.06 6 0.1 SD years). In cases where JDM and juvenile RA were the underlying diagnoses, LD was diagnosed after a mean lag period of 4.71 6 2.76 years and 3.68 6 4.36 years, respectively. The most common complications that were found to be potentially related to the LD diagnosis were acanthosis nigricans (22%), hyperpigmentation (22%), transaminitis (22%), hepatomegaly (13%), and hypertension (13%). Less frequently noted were complications such as protuberant abdomen (9%), menstrual dysfunction (9%), and hyperlipidemia (4%) (Table I). Interestingly, none of the study patients had a diagnosis of diabetes mellitus. Of patients, 52% had at least one complication that was attributable to LD. Possible risk factors such as sex, duration of follow-up, underlying diagnosis, and the location of LD were found to be poor predictors for development of complications (Table II). The only positive predictor factor for complications attributable to LD was younger age at diagnosis (7 vs 12 years, P = .04).
DISCUSSION Little is known about the cause, diagnosis, prognosis, and treatment of LD, because of complex associations with other preceding and subsequent conditions. In comparing our data with adult studies, some notable differences were observed. In our population, the most common underlying diagnosis predisposing to LD was JDM (78%). Two recent studies summarizing the existing literature reported that 15% of patients with partial acquired LD have associated dermatomyositis7 and only 6% of patients with generalized LD have a history of dermatomyositis.8
Pope et al 949
J AM ACAD DERMATOL VOLUME 55, NUMBER 6
Table II. Risk factors for development of lipodystrophy complications Complications of LD Yes N = 12
Potential predictors
Female, n (%) Age of diagnosis of LD (y), median (range) Length of follow-up, median (range) [N] Dermatomyositis, median (range) [N] Panniculitis, median (range) [N] Juvenile rheumatoid arthritis, median (range) [N] Age of dermatomyositis (y), median (range) [N] Age of panniculitis (y), median (range) [N] Age of juvenile rheumatoid arthritis (y), median (range) [N] LD spread Generalized Localized Partial
7 7 5 9 3 1 3.70 8.08 5.87
(58) (4, 14) (0, 11) (75) (25) (8) (1.96, 5.43) (4.68, 13.04) [3] [1]
7 (58) 1 (8) 4 (33)
No N = 11
7 12 3 9 1 2 5.21 11.95 7.78
(64) (6, 17) (0, 11) (82) (9) (18) (1.96, 10.78) [1] (6.56, 9.00) [2]
4 (36) 5 (45) 2 (18)
P value
1.00 .04* .10 1.00 .59 .59 .18 1.00 .60 1.00 .13 1.00
LD, Lipodystrophy. Complications: Yes if had at least one of the following: acanthosis nigricans, hepatomegaly, hyperlipidemia, protuberant abdomen, transaminitis, menstrual dysfunction; No if had none of the above items. *Statistically significant.
This discrepancy may be partially explained as a result of our institution having a specialized JDM clinic. However, the prevalence of LD in JDM in our institution is 13% in comparison with 25% in a reported similar pediatric population with JDM.2 The next most common underlying diagnosis was panniculitis with only one patient being categorized under the idiopathic group. Demographic characteristics, such as age of diagnosis and sex, were not very different from previous studies. The age of diagnosis of LD highlighted some important considerations: patients with panniculitis were given a diagnosis of LD at or shortly after their first visit whereas patients with JDM or RA developed notable LD on average after 3 to 4 years from their initial diagnosis. It is possible that all these patients have some degree of underlying panniculitis that is not clinically detectable and/or follow a more subacute or chronic course. The medical significance of LD is generally related to the longterm complications that develop as a result of it. We were particularly interested in assessing the range, timing, and frequency of commonly reported complications in our population. Although almost half of our patients had at least one documented complication thought to be related to LD, the frequency of complications was less than the reported figures in the other series. This could be explained by the ages of the patients and their length of follow-up. Somewhat contrary to the existing literature, none of our patients had a diagnosis of diabetes, which is the most commonly reported complication.7,8 It is conceivable that our
patients had biological evidence of diabetes without being symptomatic, particularly patients with acanthosis nigricans. Unfortunately, none of our patients had insulin levels or oral glucose tolerance tests performed. In a study looking at the metabolic abnormalities of patients with JDM, Huemer et al2 found that 20% of patients with LD and JDM had diabetes and a further 40% of them had evidence of metabolic disturbances (abnormal glucose or lipids) in the absence of LD. Another plausible explanation is that the length of followup (4.8 6 3.5 years) in this study was not long enough to allow for a more accurate estimation of this occurrence. Aside from asking what the complications of LD are in children, a more clinically relevant question is whether there are clinical or biological risk factors for the development of complications. In our small sample, the only predictor for development of complications was diagnosis at a young age. Other possible contributors such as underlying diagnosis and spread of the LD were not significant in our population; a finding that is likely explained by the small sample size. Unavoidable limitations of a retrospective review are the availability of the medical records and the inability to collect data on important cofounders. In addition, LD was a secondary diagnosis in a significant proportion of our patients and, therefore, clinical data and laboratory investigations were not tailored to this condition. Despite limitations, we believe that this study is representative for childhood LD, as most of the patients with either underlying
950 Pope et al
J AM ACAD DERMATOL DECEMBER 2006
diagnoses leading to LD or a diagnosis of LD will be referred to a tertiary institution. However, further prospective data are required. In children with LD, monitoring for complications should be performed on a routine basis, particularly in younger age groups. Until further research data are available, we recommend yearly investigations consisting of lipid profile, liver function tests, fasting glucose, and glucose tolerance testing for patients with abnormal fasting glucose. Patients who are symptomatic, have acanthosis nigricans, or have hepatomegaly may require closer monitoring for development of complications. REFERENCES 1. Eberhard BA, Ilowite NT. Panniculitis and lipodystrophy. Curr Opin Rheumatol 2002;14:566-70. 2. Huemer C, Kitson H, Malleson PN, Sanderson S, Huemer M, Cabral DA, et al. Lipodystrophy in patients with juvenile
3.
4.
5.
6. 7.
8.
dermatomyositiseevaluation of clinical and metabolic abnormalities. J Rheumatol 2001;28:610-5. Haas N, Henz BM, Bunikowski R, Keitzer R. Semicircular lipoatrophy in a child with systemic lupus erythematosus after subcutaneous injections with methotrexate. Pediatr Dermatol 2002;19:432-5. Dahl PR, Zalla MJ, Winkelmann RK. Localized involutional lipoatrophy: a clinicopathologic study of 16 patients. J Am Acad Dermatol 1996;35:523-8. Griffin ME, Feder A, Tamborlane WV. Lipoatrophy associated with lispro insulin in insulin pump therapy: an old complication, a new cause? Diabetes Care 2001;24:174. Garg A. Acquired and inherited lipodystrophies. N Engl J Med 2004;350:1220-34. Misra A, Peethambaram A, Garg A. Clinical features and metabolic and autoimmune derangements in acquired partial lipodystrophy: report of 35 cases and review of the literature. Medicine (Baltimore) 2004;83:18-34. Misra A, Garg A. Clinical features and metabolic derangements in acquired generalized lipodystrophy: case reports and review of the literature. Medicine (Baltimore) 2003;82: 129-46.
Childhood acquired lipodystrophy: A retrospective study Elena Pope, MD, MSc, FRCPC, Allison Janson, BSc, Amina Khambalia, BSc, MSc, and Brian Feldman, MD, MSc Toronto, Ontario, Canada Objective: We sought to describe the clinical characteristics and complications of children with acquired lipodystrophy (LD). Methods: We conducted a retrospective chart review at a tertiary, academic children’s hospital of children clinically given a diagnosis of acquired LD between January 1997 and December 2004. Results: During the study period, 23 patients were identified. Their mean age at diagnosis was 9.74 6 3.98 years. Of patients, 61% were girls. The length of the follow-up was 4.8 6 3.5 years from the time of LD diagnosis. Of patients, 30% had evidence of localized disease (7 of 23), 26% (6 of 23) had localized partial disease, and 44% (10 of 23) had generalized LD. The most common underlying diagnosis was dermatomyositis (78%), alone or in association with other autoimmune diseases (juvenile rheumatoid arthritis 17%). Panniculitis with autoimmunity was noted in 17% of the patients. More than half of the patients had at least one complication attributable to LD such as acanthosis nigricans (22%), hyperpigmentation (22%), hepatomegaly (13%), hypertension (13%), protuberant abdomen (9%), and hyperlipidemia (4%). The only predictor for development of complications was the age of diagnosis of LD, with younger age being associated with increased risk (7 vs 12 years, P = .04). Limitations: Limitations were inherent to the retrospective design. Conclusions: Childhood acquired LD is seen more frequently in the context of autoimmunity. Affected children should be monitored for the development of complications, particularly if given a diagnosis of LD at a younger age. ( J Am Acad Dermatol 2006;55:947-50.)
L
ipodystrophy (LD) is a rare heterogeneous disease characterized by the depletion of subcutaneous adipose tissue. Although uncommon in childhood, acquired LD may have its onset during childhood or adolescence. In children, acquired LD is strongly associated with panniculitis,1 juvenile dermatomyositis (JDM),2 and systemic lupus erythematosus.3 Very localized forms of the disease have been noted after local trauma, particularly subcutaneous injections of various medications.3-5 In addition to cosmetic implications, LD has been associated with metabolic disorders, most notably From the Hospital for Sick Children, University of Toronto. Funding sources: None. Conflicts of interest: None identified. Accepted for publication May 3, 2006. Reprint requests: Elena Pope, MD, MSc, FRCPC, Section of Dermatology, Hospital for Sick Children, 555 University Ave, Toronto, Ontario, Canada M5G 1X8. E-mail: [email protected]. Published online June 13, 2006. 0190-9622/$32.00 ª 2006 by the American Academy of Dermatology, Inc. doi:10.1016/j.jaad.2006.05.005
Abbreviations used: JDM: juvenile dermatomyositis LD: lipodystrophy RA: rheumatoid arthritis
insulin resistance, hepatomegaly, hyperlipidemia, acanthosis nigricans, and diabetes mellitus.6 To date, there is very little information on childhood acquired LD, particularly related to the clinical characteristics and the risk factors predictive of future metabolic complications. The purpose of this study was to review our institution’s experience with childhood onset acquired LD (excluding those secondary to HIV infection and localized scleroderma,) to increase our understanding about patients at risk for the development of LD and its complications.
METHODS The study was approved by our institution’s ethics research board. Our institution is a tertiary/ 947
948 Pope et al
J AM ACAD DERMATOL DECEMBER 2006
Table I. Complications of lipodystrophy
Complications
No. (%)
Ages of diagnosis for the complication, y
Acanthosis nigricans Hyperpigmentation Transaminitis Hepatomegaly Menstrual dysfunction Protuberant abdomen Hyperlipidemia
5 5 5 3 2 2 1
8, 11, 12, 14, 15 NS NS 4, 8, 12 NS NS NS
(22) (22) (22) (13) (9) (9) (4)
NS, Not specified.
quaternary referral center with a provincial catchment area. The inclusion criteria for this study were pediatric patients 0 to 18 years of age with a diagnosis of acquired LD from January 1997 to December 2004. Cases of presumed congenital LD and acquired LD secondary to HIV and localized scleroderma were excluded. A search by diagnosis (LD/lipoatrophy) was performed using dermatology and rheumatology databases. To ensure consistency, we included only patients who had the LD diagnosis mentioned in both the written record and the dictated letter and/or in the written record from at least two subsequent follow-up visits. Because there is no standard diagnostic tool for LD, diagnosis date was recorded as the date of the first written record of ‘‘lipodystrophy’’ or ‘‘lipoatrophy.’’ The follow-up date was recorded as the date of the last patient visit to our institution, regardless of whether LD or lipoatrophy was mentioned. Data collection included patient characteristics, underlying diagnosis, personal medical history, duration of follow-up, laboratory investigations at diagnosis and last follow-up visit, treatment of the underlying disease, and new medical diagnoses during the study period. Patients were categorized as having localized LD if only one body part was affected (ie, one arm), partial LD if face and arms were affected, and generalized LD if more than two body parts were affected, based on the location described in the medical records. Descriptive statistics were performed using mean, median, and proportions. Univariate analysis was used to compare patients with normal versus abnormal investigation findings. Fisher’s exact test was used for categorical variables because of small cell sizes and the Mann-Whitney U test was used for nonnormally distributed continuous data. A P value of .05% was considered statistically significant. Statistical analyses were conducted using software (SPSS for Windows 11.0, SPSS Inc, Chicago, Ill).
RESULTS During the study period, 23 patients met the inclusion criteria. Their mean age at diagnosis was 9.74 6 3.98 years. Girls represented 61% of patients. Of patients, 30% had evidence of localized disease (7 of 23), 26% (6 of 23) had partial disease, and 44% (10 of 23) had generalized LD. The most common affected body area was the upper extremity (78%, 18 of 23), followed by the face (73%, 17 of 23) and the lower extremity (39%, 9 of 23). The average length of follow-up was 4.8 6 3.5 years from the time of the LD diagnosis. The most common underlying diagnosis was JDM (78%), alone (65%) or in association with other autoimmune diseases such as juvenile rheumatoid arthritis (RA) (13%). One patient had juvenile RA without evidence of other autoimmune disease (4%). Panniculitis in the context of other autoimmune disorders (ie, Graves’ disease, JDM, and vasculitis) was noted in 17% of the patients. One patient’s LD was considered to be idiopathic (4%). The diagnosis of LD was made shortly after the initial underlying diagnosis of panniculitis (mean 0.06 6 0.1 SD years). In cases where JDM and juvenile RA were the underlying diagnoses, LD was diagnosed after a mean lag period of 4.71 6 2.76 years and 3.68 6 4.36 years, respectively. The most common complications that were found to be potentially related to the LD diagnosis were acanthosis nigricans (22%), hyperpigmentation (22%), transaminitis (22%), hepatomegaly (13%), and hypertension (13%). Less frequently noted were complications such as protuberant abdomen (9%), menstrual dysfunction (9%), and hyperlipidemia (4%) (Table I). Interestingly, none of the study patients had a diagnosis of diabetes mellitus. Of patients, 52% had at least one complication that was attributable to LD. Possible risk factors such as sex, duration of follow-up, underlying diagnosis, and the location of LD were found to be poor predictors for development of complications (Table II). The only positive predictor factor for complications attributable to LD was younger age at diagnosis (7 vs 12 years, P = .04).
DISCUSSION Little is known about the cause, diagnosis, prognosis, and treatment of LD, because of complex associations with other preceding and subsequent conditions. In comparing our data with adult studies, some notable differences were observed. In our population, the most common underlying diagnosis predisposing to LD was JDM (78%). Two recent studies summarizing the existing literature reported that 15% of patients with partial acquired LD have associated dermatomyositis7 and only 6% of patients with generalized LD have a history of dermatomyositis.8
Pope et al 949
J AM ACAD DERMATOL VOLUME 55, NUMBER 6
Table II. Risk factors for development of lipodystrophy complications Complications of LD Yes N = 12
Potential predictors
Female, n (%) Age of diagnosis of LD (y), median (range) Length of follow-up, median (range) [N] Dermatomyositis, median (range) [N] Panniculitis, median (range) [N] Juvenile rheumatoid arthritis, median (range) [N] Age of dermatomyositis (y), median (range) [N] Age of panniculitis (y), median (range) [N] Age of juvenile rheumatoid arthritis (y), median (range) [N] LD spread Generalized Localized Partial
7 7 5 9 3 1 3.70 8.08 5.87
(58) (4, 14) (0, 11) (75) (25) (8) (1.96, 5.43) (4.68, 13.04) [3] [1]
7 (58) 1 (8) 4 (33)
No N = 11
7 12 3 9 1 2 5.21 11.95 7.78
(64) (6, 17) (0, 11) (82) (9) (18) (1.96, 10.78) [1] (6.56, 9.00) [2]
4 (36) 5 (45) 2 (18)
P value
1.00 .04* .10 1.00 .59 .59 .18 1.00 .60 1.00 .13 1.00
LD, Lipodystrophy. Complications: Yes if had at least one of the following: acanthosis nigricans, hepatomegaly, hyperlipidemia, protuberant abdomen, transaminitis, menstrual dysfunction; No if had none of the above items. *Statistically significant.
This discrepancy may be partially explained as a result of our institution having a specialized JDM clinic. However, the prevalence of LD in JDM in our institution is 13% in comparison with 25% in a reported similar pediatric population with JDM.2 The next most common underlying diagnosis was panniculitis with only one patient being categorized under the idiopathic group. Demographic characteristics, such as age of diagnosis and sex, were not very different from previous studies. The age of diagnosis of LD highlighted some important considerations: patients with panniculitis were given a diagnosis of LD at or shortly after their first visit whereas patients with JDM or RA developed notable LD on average after 3 to 4 years from their initial diagnosis. It is possible that all these patients have some degree of underlying panniculitis that is not clinically detectable and/or follow a more subacute or chronic course. The medical significance of LD is generally related to the longterm complications that develop as a result of it. We were particularly interested in assessing the range, timing, and frequency of commonly reported complications in our population. Although almost half of our patients had at least one documented complication thought to be related to LD, the frequency of complications was less than the reported figures in the other series. This could be explained by the ages of the patients and their length of follow-up. Somewhat contrary to the existing literature, none of our patients had a diagnosis of diabetes, which is the most commonly reported complication.7,8 It is conceivable that our
patients had biological evidence of diabetes without being symptomatic, particularly patients with acanthosis nigricans. Unfortunately, none of our patients had insulin levels or oral glucose tolerance tests performed. In a study looking at the metabolic abnormalities of patients with JDM, Huemer et al2 found that 20% of patients with LD and JDM had diabetes and a further 40% of them had evidence of metabolic disturbances (abnormal glucose or lipids) in the absence of LD. Another plausible explanation is that the length of followup (4.8 6 3.5 years) in this study was not long enough to allow for a more accurate estimation of this occurrence. Aside from asking what the complications of LD are in children, a more clinically relevant question is whether there are clinical or biological risk factors for the development of complications. In our small sample, the only predictor for development of complications was diagnosis at a young age. Other possible contributors such as underlying diagnosis and spread of the LD were not significant in our population; a finding that is likely explained by the small sample size. Unavoidable limitations of a retrospective review are the availability of the medical records and the inability to collect data on important cofounders. In addition, LD was a secondary diagnosis in a significant proportion of our patients and, therefore, clinical data and laboratory investigations were not tailored to this condition. Despite limitations, we believe that this study is representative for childhood LD, as most of the patients with either underlying
950 Pope et al
J AM ACAD DERMATOL DECEMBER 2006
diagnoses leading to LD or a diagnosis of LD will be referred to a tertiary institution. However, further prospective data are required. In children with LD, monitoring for complications should be performed on a routine basis, particularly in younger age groups. Until further research data are available, we recommend yearly investigations consisting of lipid profile, liver function tests, fasting glucose, and glucose tolerance testing for patients with abnormal fasting glucose. Patients who are symptomatic, have acanthosis nigricans, or have hepatomegaly may require closer monitoring for development of complications. REFERENCES 1. Eberhard BA, Ilowite NT. Panniculitis and lipodystrophy. Curr Opin Rheumatol 2002;14:566-70. 2. Huemer C, Kitson H, Malleson PN, Sanderson S, Huemer M, Cabral DA, et al. Lipodystrophy in patients with juvenile
3.
4.
5.
6. 7.
8.
dermatomyositiseevaluation of clinical and metabolic abnormalities. J Rheumatol 2001;28:610-5. Haas N, Henz BM, Bunikowski R, Keitzer R. Semicircular lipoatrophy in a child with systemic lupus erythematosus after subcutaneous injections with methotrexate. Pediatr Dermatol 2002;19:432-5. Dahl PR, Zalla MJ, Winkelmann RK. Localized involutional lipoatrophy: a clinicopathologic study of 16 patients. J Am Acad Dermatol 1996;35:523-8. Griffin ME, Feder A, Tamborlane WV. Lipoatrophy associated with lispro insulin in insulin pump therapy: an old complication, a new cause? Diabetes Care 2001;24:174. Garg A. Acquired and inherited lipodystrophies. N Engl J Med 2004;350:1220-34. Misra A, Peethambaram A, Garg A. Clinical features and metabolic and autoimmune derangements in acquired partial lipodystrophy: report of 35 cases and review of the literature. Medicine (Baltimore) 2004;83:18-34. Misra A, Garg A. Clinical features and metabolic derangements in acquired generalized lipodystrophy: case reports and review of the literature. Medicine (Baltimore) 2003;82: 129-46.