Dercum's disease (adiposis dolorosa): A review of clinical presentation and management

Abstract

Dercum's disease (adiposis dolorosa) is a rare disease of unknown etiology characterized by painful subcutaneous adipose tissue deposits with various localization over the body. The deposits occur histologically as lipomas and are associated with overweight or obesity and a variety of psychiatric disturbances (anxiety, depression, sleep disturbances). Classification of Dercum's disease is related to size and location of adipose nodules (generalized diffuse, generalized nodular, localized nodular and juxta-articular forms). Diagnosis in based on clinical presentation and exclusion of a number of other disorders associated with lipomas. There is no generally accepted management of the patients. Liposuction or lidocaine application has been reported successful in some cases. Other therapeutic methods have been reported but their effectiveness is based on anecdotal descriptions only, and were not confirmed in clinical trials.

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Review paper Reumatologia 2019; 57, 5: 281–287
DOI: https://doi.org/10.5114/reum.2019.89521
Dercum’s disease (adiposis dolorosa): areview of clinical
presentation and management
Eugeniusz J. Kucharz, Magdalena Kopeć-Mędrek, Justyna Kramza, Monika Chrzanowska,
Przemysław Kotyla
Department of Internal Medicine, Rheumatology and Clinical Immunology, Medical University of Silesia, Katowice, Poland
Abstract
Dercum’s disease (adiposis dolorosa) is a rare disease of unknown etiology characterized by painful
subcutaneous adipose tissue deposits with various localization over the body. The deposits occur
histologically as lipomas and are associated with overweight or obesity and a variety of psychiatric
disturbances (anxiety, depression, sleep disturbances). Classification of Dercum’s disease is related
to size and location of adipose nodules (generalized diffuse, generalized nodular, localized nodular
and juxta-articular forms). Diagnosis in based on clinical presentation and exclusion of a number
of other disorders associated with lipomas. There is no generally accepted management of the pa-
tients. Liposuction or lidocaine application has been reported successful in some cases. Other thera-
peutic methods have been reported but their effectiveness is based on anecdotal descriptions only,
and were not confirmed in clinical trials.
Key words: Dercum’s disease, adiposis dolorosa, subcutaneous fat masses.
Introduction
Dercum’s disease, also known as adiposis dolorosa,
is a rare disease typically characterized by development
of painful subcutaneous adipose tissue deposits of dif-
ferent size, multiplicity and localization.
The associated symptoms include overweight or
obesity, fatigue or weakness and a number of psychi-
atric manifestations (e.g. sleep disturbances, emotional
instability, depression, and anxiety) but the associated
symptoms are not a constant component of clinical pre-
sentation of the disease.
The etiology of Dercum’s disease remains unknown,
and disease classification, diagnostic criteria and thera-
peutic strategy are subject to controversy.
The disease was described for the first time by an
American neurologist working in Philadelphia, Francis
Xavier Dercum (1856–1931) [1]. He published two papers
on the disease in 1888 [2] and 1892 [3], and he used the
term “adiposis dolorosa”. Further, the disease was re-
ported also in Philadelphia by the American physician
James Meschter Anders (1854–1936) [4] and the British
physician and medical biographer at Guy’s Hospital in
London, Sir William Hale White (1857–1949) [5].
Several synonyms of Dercum’s disease beside adi-
posis dolorosa have appeared in medical literature. They
include: lipomatosis dolorosa, adiposalgia, adipose tis-
sue rheumatism, fatty tissue rheumatism, lipalgia, neu-
rolipomatosis, and Ander syndrome. Due to unclear eti-
ology and the lack of clear definition, Dercum’s disease
is also known as Dercum’s syndrome.
Dercum’s disease has been recognized by the World
Health Organization in ICD-10 and is classified as lipo-
matosis not elsewhere classified (IV – E88.2). Orphanet
and the National Organization of Rare Disorders also
listed the disease.
The total number of publications on Dercum’s dis-
ease is estimated at 140–160 references. A significant
majority of them are case reports and only a few papers
are analyses of patient cohorts. An excellent review was
published in 2012 by Hansson et al. [6], and contained
a proposal of classification criteria. The present review
is intended to summarize the recent data on the disease
Address for correspondence:
Eugeniusz J. Kucharz, Department of Internal Medicine, Rheumatology and Clinical Immunology, Medical University of Silesia,
45/47 Ziołowa St., 40-635 Katowice, Poland, e-mail: ejkucharz@poczta.onet.pl
Submitted: 23.07.2019; Accepted: 17.09.2019

282 Eugeniusz J. Kucharz, Magdalena Kopeć-Mędrek, Justyna Kramza, et al.
Reumatologia 2019; 57/5
with emphasis of clinical presentation, differential diag-
nosis and management.
Epidemiology
Dercum’s disease is a rare disorder appearing almost
exclusively in adults, and is predominant in individuals
aged 35–50 years [7, 8]. A very few cases of affected chil-
dren have been published only [9]. It is predominantly
a disease affecting women, and the ratio of women to
men is estimated at 5–30 : 1 [4]. The majority of the re-
ported cases concerned Caucasian patients.
Some papers suggested that Dercum’s disease oc-
curs predominantly in postmenopausal women. In con-
trast, studies of Herbst and Asare-Bediako [7] revealed
that four-fifths of the female patients developed the dis-
ease before menopause. There have been no studies on
prevalence or incidence of the disease.
Almost all case descriptions of Dercum’s disease are
sporadic. A very few reports described familial occur-
rence of Dercum’s disease. The main problem of these
case reports is differential diagnosis. Familial tendency
was shown in some forms of benign symmetric lipoma-
tosis (Madelung’s syndrome), and as implied by name in
patients with familial multiple lipomatosis.
Familial clustering of Dercum’s disease was report-
ed by Campen et al. [10]. They described two affected
siblings whose family included at least 5 affected first-
degree relatives in two generations. Some of the lipo-
mas were painless. The genetic evaluation excluded the
8344A to G mitochondrial mutation detectable in pa-
tients with multiple lipomatoses other than Dercum’s
disease. They suggested autosomal dominant form of
inheritance of the trait. They also suggested that pheno-
type expression of the disease may vary and Dercum’s
disease may be one of clinical forms of lipomatosis con-
sidered in a broad sense.
Autosomal dominant inheritance of the disease was
also suggested by Cantu et al. [11], and its familial oc-
currence was reported by Lynch and Harlan [12]. Despite
the above-mentioned reports, all available literature in-
dicates sporadic occurrence of Dercum’s disease. It is
possible that the disease is heterogeneous and inher-
ited cases are a small subgroup of Dercum’s syndrome.
Clinical presentation
Dercum’s disease is characterized by pronounced
pain in the adipose tissue. The pain is burning and sear-
ing. The pain can be severe, is chronic (> 3 months) and
is disabling. It is believed that pain is always associated
with smaller or bigger subcutaneous adipose lumps.
In patients with so-called generalized diffuse form
of Dercum’s disease, the painful deposits can be small
and difficult to palpate as opposed to those occurring
in nodule form when the lipomas can achieve a large
size and mass. The skin over the affected area remains
unchanged and lumps are soft, usually well demarcat-
ed. There are no signs of altered skin pigmentation or
cutaneous breakage. The typical changes of Dercum’s
disease are presented in Figure 1.
Subcutaneous lipomas may appear in all possible
locations. Hansson et al. [6] summarized several de-
scriptions and showed that legs, arms and trunk (ante-
rior or posterior) are the most common body parts with
lipomas. The face is affected in about one-fifth of the
patients while the scalp and neck are locations of the
lipomas in about one-third of individuals suffering from
Dercum’s disease. Common places of lipomas’ develop-
ment are the buttocks (about 70%) and thighs. Painful
adipose tissue is also common in the subcostal area, the
medial aspect of the upper extremity and the lateral and
medial parts of the thighs.
The onset of pain is rather slow, but it is also possible
that the pain is recognized by the patients with some
delay due to its other possible explanations (small inju-
ries, etc.).
Overweight or obesity is considered as the second
cardinal symptom after multiple painful adipose mass-
es. Most of the descriptions are concomitant with oc-
currence of this symptom. Historically, Roux and Vitaut
[13] described in 1901 two other cardinal symptoms, i.e.
fatigue or weakness and a variety of psychiatric mani-
festations.
Other investigators suggested that weakness and
psychiatric manifestations can be secondary to obesity
and chronic pain syndrome. These symptoms are com-
mon in patients with Dercum’s disease and should be
Fig. 1. Changes in the subcutaneous tissue on the
patient’s shoulder, typical for Dercum’s disease.

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Reumatologia 2019; 57/5
taken into account in the clinical diagnosis. They are
proposed to be used as diagnostic criteria but there are
controversies over which symptoms are cardinal and
which are minor.
Psychiatric manifestations associated with Dercum’s
disease are not found in all the patients. They include
depression and emotional instability, sleep disturbances,
and less frequently cognitive impairment, dementia and
epilepsy. The nature of these symptoms and their rela-
tionship with the disease remain unclear.
Depression may be related to chronic pain and oc-
curs in patients with other disorders associated with
a long-lasting pain. The same applies to sleep distur-
bances. Cognitive impairment and even dementia can
be associated with metabolic disturbances found in
obese individuals. Obesity may also be related to weak-
ness and fatigue. Prominent neurological involvement
associated with depression and cognitive impairment
was reported in a patient with Dercum’s disease [14].
A case control study on depression in Dercum’s
disease was published by Hansson et al. [15]. They in-
vestigated 111 women with the disease and applied the
Montgomery Åsberg Depression Rating Scale. The study
revealed that 55% of the patients had light or moder-
ate depression, 44% were classified as having “no de-
pression”, and one patient had severe depression. The
conclusion indicated for more common depression is
patients with Dercum’s disease.
Case descriptions of patients with Dercum’s dis-
ease indicated a number of other symptoms or signs
associated with the disease. It is difficult to distinguish
those which are a part of clinical symptomatology of the
disease from those that are randomly associated only.
Commonly reported symptoms or accompanying disor-
ders include joint and/or muscular pain, constipation,
tachycardia, shortness of breath and diabetes. Many of
them can also be associated with obesity and all are not
specific for Dercum’s disease.
Medical literature contains a significant number of
atypical presentations of Dercum’s disease. Some of
them reported different locations of fatty deposits. It
should be mentioned that painful lipomas can cause
pain of the breast [16]. Despite it being very uncommon,
it should be taken into consideration in diagnosis of
mastalgia as well as the occurrence of breast lumps. Ab-
dominal pain as a main symptom of the disease [17] and
arthralgia as a presenting symptom of Dercum’s disease
[18] were reported in rare cases. A patient with Dercum’s
disease complicated by steato-cutaneous necrosis and
septic shock was reported by Haddad et al. [19]. Kawale
et al. [20] described a patient with fatty lumps of the
scalp with severe headache.
Metabolic alterations related to or associated with
Dercum’s disease are a subject of interesting hypothe-
ses. We reported a case of Dercum’s disease in a patient
suffering from severe hypercholesterolemia [21]. It is
possible that this is unrelated coexistence of two sepa-
rate disorders, but on the other hand obese patients are
prone to various metabolic alterations.
Izar et al. [9] reported an eight-year-old girl suffering
from Dercum’s disease and hyperbetalipoproteinemia
as well as hyperinsulinemia.
Hansson et al. [6] described excess of glucocorti-
coids, due to medication with hormones as a hypo-
thetical cause of Dercum’s disease. Glucocorticoids are
known to induce dyslipidemia and it is possible that
Dercum’s disease has some relationship to altered lipid
metabolism.
Łabuzek et al. [22] reported a case of Dercum’s dis-
ease treated with metformin. The drug is used primarily
in obese patients with diabetes mellitus and has a vari-
ety of metabolic actions. They found favorable effects of
medication on adipokines, β-endorphin and pro-inflam-
matory cytokines associated with a decrease in pain.
Both metabolic and other mechanisms of action of met-
formin (e.g. influence on synaptic plasticity, activation of
microglia) were hypothetically considered as a mecha-
nism of drug-induced pain reduction in the patient.
Hansson et al. [23] measured different neuropep-
tides in cerebrospinal fluid and plasma of 53 patients
with Dercum’s disease. They found a lower level of sub-
stance P in cerebrospinal fluid of the patients. Neuro-
peptide Y level was also borderline lower while some el-
evation in β-endorphin level was found in cerebrospinal
fluid of the patients. The etiological role of these alter-
ations remains unknown.
Campen et al. [10] proposed an inflammatory patho-
mechanism, possibly leading to the release of pain-
related neuropeptides. The role of trauma has been
suggested in some patients [24]. The role of lymphatic
vessel dysfunction has also been suggested. According
to Rasmussen et al. [25], lymphovascular impairment
leads to lymphatic transport deficiency and lipomas
appeared to have resulted from defective drainage by
lymphatic vessels.
Classification
A few authors have proposed a classification of sub-
forms of clinical presentation of Dercum’s disease. Most
of the classifications distinguish three forms of the dis-
ease. Recently, Hansson et al. [6] proposed four types of
the disease as summarized in Table I.
The classification is based on the following criteria:
size of lumps (small in generalized diffuse form, bigger

284 Eugeniusz J. Kucharz, Magdalena Kopeć-Mędrek, Justyna Kramza, et al.
Reumatologia 2019; 57/5
in patients with Dercum’s disease is indistinguishable
from lipomas [29].
Imaging appearance
Ultrasound and magnetic resonance imaging re-
vealed that the lesions were located in the superficial
subcutaneous fat. There were no changes in the deep
fat. In most of the cases, imaging techniques detected
more lesions than physical evaluation of the patients. It
indicates that most of the lesions are clinically asymp-
tomatic.
In study of Tins et al. [30] most lesions were < 2 cm
in diameter and were oblong with the long axis parallel
to the skin. All lesions were hyperechoic on ultrasound
and showed no demonstrable flow on Doppler imaging.
There was no edema in or around the lesions. Magnetic
resonance imaging revealed a blush-like appearance
with a decreased T1-weighted signal and an increased
signal in the water-sensitive sequence (STIR). Larger le-
sions were more inhomogeneous in appearance. There
was no difference between symptomatic and asymp-
tomatic lesions [30].
Diagnosis and differentiation
Physical examination and medical history revealing
painful lipomas consisting of normal adipose tissue and
pain localized in the adipose tissue in a person with at
least overweight should be followed by careful exclusion
of other painful conditions. The accompanying symp-
toms and signs should be taken into consideration.
A number of disorders can be akin to Dercum’s dis-
ease and should be excluded. Differential diagnoses are
listed in Table II.
The most common and sometimes difficult to differ-
entiate with the generalized form of Dercum’s disease
are fibromyalgia, lipoedema, panniculitis, lymphedema,
and others.
Fibromyalgia is in some cases difficult to distinguish
from Dercum’s disease. It is a widespread muscular pain
in all other forms of the disease) and distribution of fat-
ty deposits (widespread versus localized, localized near
or within the joints). Thus, the classification is based on
descriptive findings and mixed and difficult to categorize
forms of Dercum’s disease were also reported [26, 27].
Lack of understanding of the etiology and natural histo-
ry of the disease results in a lack of possibility to create
a pathophysiological classification.
Laboratory and pathological findings
There are no specific laboratory tests facilitating
diagnosis of Dercum’s disease. Histopathological eval-
uation of fat mass reveals lipomas without signs of in-
flammation. Herbst at al. [28] described a higher level
of connective tissue in fat biopsy samples in patients
with Dercum’s disease. In general, most of the reports
indicated that the pathological picture of the fat masses
Table I. Classification of types of Dercum’s disease (based on Hansson et al. [6] with descriptions partially based
on Kosseifi et al. [26])
Type Description
Type I
Generalized diffuse form
Widespread pain from fatty tissue, very small adipose deposits can be palpated in various
parts of the body but pain can occur in areas without detectable lumps
Type II
Generalized nodular form
Pain around and in lipomas localized in multiple parts of the body
Type III
Localized nodular form
Painful lipomas in limited locations
Type IV
Juxta-articular form
Painful folds of fat located inside or very near big joints (the knee, hip or elbow)
Table II. Differential diagnosis of Dercum’s disease
Generalized diffuse and generalized nodular forms
Fibromyalgia
Lipoedema
Lymphedema
Panniculitis
Proteus syndrome
Weber-Christian disease
Fröhlich’s syndrome
Progressive lipodystrophy
Cushing’s syndrome
Hypothyroidism
Nodular forms
Benign symmetric lipomatosis (Madelung’s syndrome,
Launois-Bensaude syndrome)
Familial multiple lipomatosis
Neurofibromatosis type 1
Adenolipomatosis
Multiple endocrine neoplasia I (MEN I)

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with several psychiatric manifestations. Detection of
painful lipomas may be helpful in the distinction of these
two disorders. Lipoedema is usually limited to lower ex-
tremities, and is characterized by bilateral symmetric oc-
currence and homogeneous deposition of fat [31].
Panniculitis is associated with inflammation and in
some cases with vasculitis. The nodules are erythem-
atous and some of them heal and others develop. The
lower extremities are common locations of panniculitis.
Lymphedema results from accumulation of protein-rich
interstitial fluid within the skin and responds to com-
pression or lymphatic massage [32]. Differential diagno-
sis of Dercum’s disease massive localized lymphedema
may be difficult [33].
Treatment
The treatment strategy in patients with Dercum’s
disease is based on the individual decision of the phy-
sician, because only a few studies have been published
and most of the reports described a single case.
Liposuction is a method of management of Dercum’s
disease that is relatively commonly reported in the lit-
erature. Most of the described case were operated on
with “dry” technique and only a few with tumescence
technique [6, 34].
Hansson et al. [35] reported a decrease in pain in 53
patients operated on with liposuction. Diminished pain
was revealed with both subjective and objective mea-
surements. In a long-term observation, it was found that
the pain relief diminished over time, but after 5 years
still pain indices were lower than in inoperable subjects
with Dercum’s disease and were lower than baseline
values before liposuction. The same group of research-
ers also found a slight improvement in quality of life af-
ter liposuction [36].
The mechanism of pain reduction after liposuction
remains unclear. Destruction of nerve plexuses within
the adipose tissue was suggested. Reduction of altered
fat masses seems to be the main cause of pain reduc-
tion because pain diminution lasted longer than sensi-
tivity loss after liposuction [36]. There was no significant
difference in thermal and vibratory thresholds 3 and
12 months after liposuction in patients with Dercum’s
disease [37]. Liposuction was also applied in cases of
juxta-articular form of the disease [38].
Surgical treatment was administered in a few report-
ed cases. Lipectomy or dermolipectomy was described
as a method of amelioration of the disease, also in jux-
ta-articular form [39, 40].
Subcutaneous adipose tissue therapy based on deep
fat tissue, fascia and muscle massage has been recently
suggested as a method facilitating reduction of lipomas
in Dercum’s disease [41].
Pharmacological management mostly focuses on
analgesics. Patients with Dercum’s disease are consid-
ered as refractory to non-steroidal anti-inflammatory
drugs [6], but some reports indicated an opportunity to
obtain pain reduction with non-steroidal anti-inflamma-
tory drugs [7]. Lidocaine is used commonly either in the
form of intralesional injections, transdermal application
or intravenous infusions [26, 42, 43]. The mechanism
of its action is not fully understood but blocking of im-
pulses conduction in peripheral nerves and the effect of
sympathetic activity are suggested mechanisms of ther-
apeutic action of lignocaine.
Transcutaneous frequency rhythmic electrical stimu-
lation was reported as an effective and safe method of
treatment [44].
Single reports on beneficial effects of pregabalin,
interferon α-2b, corticosteroids, metformin and calci-
um-channel modular as well as infliximab with metho-
trexate were published [6, 22, 45–47]. Herbs and Rutledge
[48] performed a study on 10 patients with Dercum’s dis-
ease and revealed that dynamic whole body cyclic pneu-
matic compression resulted in pain relief.
Recently, deoxycholic acid was used for management
of Dercum’s disease. Deoxycholic acid was approved for
the reduction of submental fat. Recently, Wipf et al. [49]
reported novel, highly effective use of deoxycholic acid in
the form of injections to manage lipomas in a patients
with Dercum’s disease. This method of management of
the patients needs further evaluation.
Summing up: There is no recommended strategy
of management of patients with Dercum’s disease. In
general, it is a chronic condition with a strong tendency
to recurrence. The decision on the therapeutic strategy
should be based on an analysis of type of the disease
and severity of symptoms. In more localized forms re-
duction of fat masses resulted in relief and flares were
usually observed after a few years. In generalized forms,
the physician is advised to consider pharmacological
methods, but there is no evidence of their efficacy. Addi-
tional management, including antidepressants, psycho-
therapy and patient education, is suggested.
Conclusions
Dercum’s disease is still a very enigmatic clinical
item, and various aspects of its etiopathogenesis, clini-
cal presentation and management are unresolved.
The disease is diagnosed on the basis of clinical
findings and the question of its heterogeneity should
be addressed. It remains unclear whether Dercum’s dis-
ease is a syndrome or homogeneous nosological unit. It

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Reumatologia 2019; 57/5
can be hypothesized that a few unrelated factors may
result in pain development within accumulations of
fatty tissue. In such cases, Dercum’s disease is a syn-
drome secondary to a group of etiological factors, and
only cases with an evidenced genetic background will
be defined as a nosological unit. Despite this question,
currently diagnosis can be made only on the basis of
pain related to deposits of the adipose tissue.
Management is empirical, focused on pain and in
a limited situation on elimination (with surgical proce-
dures or liposuction) of an excess of fatty tissue. The
mechanism of pain development within the adipose
tissue is unknown; thus various therapeutic methods
have been reported to be effective. Most of the reports
consisted of anecdotic descriptions and there is a lack of
prospective studies.
Despite the lack of understanding of the disease, it
should be better recognized by rheumatologists, general
practitioners and dermatologists, and more investigations
including multicenter studies should be carried out.
The authors declare no conflict of interest.
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