1357
DIGITAL SCLEROSIS IN CHILDREN WITH
INSULIN-DEPENDENT DIABETES MELLITUS
JAMES R. SEIBOLD
Palpable thickening and induration of the skin of
the fingers were found in 47 (34%) of 137 children with
insulin-dependent diabetes mellitus and in none of 52
normal children. Mild flexion contractures of the inter-
phalangeal joints were seen in 26 (19%),mainly in those
children with more extensive and severe skin changes.
Such contractures have been linked to microvascular
complications of diabetes. The clinical and pathogenic
similarities to progressive systemic sclerosis make the
digital sclerosis of childhood diabetes a promising model
for further rheumatologic study.
In 1976 Grgic et a1 reported that 65 (29%) of 229
children with insulin-dependent diabetes mellitus
(IDDM) had flexion contractures of the fingers (1).
Those children with the more severe joint limitation
were also noted to have thickened, adherent skin over
the dorsa of the hands. In 1981 Rosenbloom et a1
reported similar findings in 92 (30%) of 309 childhood
diabetics (2). In those individuals with contractures,
there was an increased risk of microvascular complica-
tions of diabetes, including retinopathy and nephrop-
athy (2).
In neither study, however, was the relation of
From the Division of Rheumatology, Department of Medi-
cine, University of Medicine and Dentistry of New Jersey-Rutgers
Medical School, Piscataway, NJ.
Supported by grants from the Kroc Foundation, Santa
Ynez, CA, and the Cape Atlantic Cumberland Lupus Foundation,
Cape May, NJ.
James R. Seibold, MD: Assistant Professor of Medicine,
Rutgers Medical School.
Address reprints to James R. Seibold, MD, Department of
Medicine, UMDNJ-Rutgers Medical School, P.O. Box 101, Pis-
cataway, NJ 08854.
Submitted for publicaton February 9 , 1982; accepted in
revised form April 5 , 1982.
Arthritis and Rheumatism, Vol. 25, No. 11 (November 1982)
the skin changes to the joint findings made clear. One
group mistakenly observed that the diabetic changes
were clearly distinct from those of scleroderma (pro-
gressive systemic sclerosis), the latter ‘‘. . . character-
ized by thinning of the skin and (without) attendant
joint manifestations” (1). Forty-seven (72%) of the 65
children with contracture in 1 report had no change
other than fifth-digit camptodactyly (1); 33 (36%) in the
second study were said to have mild limitation, de-
fined as involvement of 1 or 2 interphalangeal joints, 1
large joint, or only the metacarpophalangeal joints
bilaterally (2).
The present study was undertaken to confirm
the presence of skin thickening and joint contractures
in childhood diabetes and to define more precisely the
clinical features of this syndrome.
PATIENTS AND METHODS
Rheumatologic histories were taken and physical
examinations performed on 189 children, including 137 with
insulin-dependent diabetes mellitus and 52 age- and sex-
matched normal controls. The diabetic patients ranged in age
from 5 to 24 years (mean 11.7 & 3.2 years) and included 73
girls and 64 boys.
Most of this study was undertaken at a summer camp
for diabetic children. While there were occasional nondia-
betic campers accompanying a diabetic friend, the examiner
was usually aware which children were diabetic and which
were not. The examiner was, however, blinded to the other
information concerning the underlying IDDM, including
duration of illness, insulin dosage, quality of diabetic con-
trol, and presence of complications of diabetes. All patients
were examined by the author, and, in many instances, the
findings were confirmed by camp physicians (pediatricians
or endocrinologists).
Skin and subcutaneous tissue thickening was evalu-
ated by palpation and scored 0 (normal), 1+ (slight but
1358
definite thickening; inability to tent the skin between the
examiner’s thumbs), 2+ (mild to moderate changes), 3 +
(severe thickening), or 4+ (extreme skin thickening). This
scoring was applied to 26 body areas (including fingers,
hands, forearms, arms, shoulders, face, neck, anterior chest,
breasts, abdomen, upper and lower back, thighs, legs, feet,
and toes).
This method of clinical assessment of the extent and
degree of skin involvement has been shown to correlate well
with skin core biopsy weight and total dermal collagen
content in studies of progressive systemic sclerosis (3,4).
This simple clinical technique is easily learned and interob-
server agreement is high. In studies of progressive systemic
sclerosis, clinical palpation has been shown more reliable
than roentgenographic measurements of skin thickening (3).
In our experience, measurement of skin changes by microm-
eter caliper is tedious and subject to great variation, depend-
ing on finger size, patient age, skin area examined, and the
degree of subcutaneous tissue adherence to overlying skin.
In the present study skin findings of uncertain degree were
downgraded to the lower score.
The skin was inspected for pigmentary change and
for telangiectasias. The palms were palpated for thickening
of the palmar flexor tendons and inspected for puckering on
passive hyperextension of the metacarpophalangeal joints.
Tendon friction rubs were sought by palpation of the palms,
forearms, elbows, knees, and lower legs, on active motion of
these areas. Active and passive ranges of motion of the
fingers, wrists, elbows, shoulders, cervical spine, hips,
PIP & Distally
# children 27 (20%)
Figure 1. Frequency and distribution of palpably thickened and adherent skin in 47 (34%) of 137 children with insulin-dependent
diabetes mellitus. PIP = proximal interphalangeal joints; MCP = metacarpophalangeal joints.
SEIBOLD
knees, ankles, subtalar joints, and metatarsophalangeal
joints were recorded. Carpal tunnel syndrome was deter-
mined by questioning and by performance of Tinel’s and
Phalen’s maneuvers.
When the examinations were completed, the chil-
dren’s camp medical records were reviewed for information
on the duration of diabetes; the dosage of insulin; the quality
of control of hyperglycemia by history, as recorded by each
child’s primary physician and by staff while at camp; the
frequency of ketoacidosis; and the history of retinal, renal,
and other complications of IDDM.
RESULTS
We found palpable thickening and induration of
the skin in 47 (34%) of the diabetic children and in
none of the controls. It became readily apparent why
these changes are poorly described in the medical
literature: while the more severely afflicted children
had loss of the transverse digital skin ridges on the
dorsum of the fingers, in most cases the findings were
restricted to abnormalities by palpation. In no instance
was there evidence of dermal atrophy, pigmentary
change, edema, or telangiectasia. In 27 cases, digital
sclerosis was restricted to the proximal interphalan-
geal joints and distally (Figure 1). In an additional 14,
MCP & Distally Proximal to MCP
14 (10%) 6 (4%)
DIGITAL SCLEROSIS IN CHILDHOOD DIABETES 1359

Table 1. Relationship of digital skin thickening to finger-joint diabetes (r = 0.63, P < 0.01). Children with 9- and 10-
contractures in 137 children with insulin-dependent diabetes
year histories of diabetes had 83% and 75% incidence
mellitus
rates, respectively, of digital sclerosis. We were not
Extent of skin No. of No. of patients able to relate the presence of digital sclerosis to the age
involvement* patients with contracture (%)
~ ~~
of the patient or to sex, race, the quality of control of
None 90 0 hyperglycemia, insulin dosage, or measures of the
PIP and distally 27 11 (41%)
MCP and distally 14 10 (71%) severity of the diabetes, including the frequency of
Proximal to MCP 6 5 (83%) ketoacidosis or evidence of renal complications. We
* PIP = proximal interphalangeal joints; MCP = metacarpophalan- did not perform funduscopic examinations, nor did we
geal joints. examine for nailfold capillary abnormalities.

DISCUSSION
skin changes extended from the metacarpophalangeal
Joint contractures have been reported to com-
joints distally. Six children had palpably indurated and
plicate IDDM frequently. Grgic et a1 found finger
adherent skin proximal to the metacarpophalangeal
contractures in 65 (28%) of 229 children with IDDM,
joints, thereby fulfilling preliminary criteria of the
but 47 (72%) of the afflicted children had findings
American Rheumatism Association for the classifica-
restricted to the proximal interphalangeal joint of the
tion of systemic sclerosis (5). Of the 47 children with
fifth digit (1). Traisman et a1 found finger contractures
digital sclerosis, 38 (81%) were considered to have in only 26 (8.4%) of 310 childhood diabetics, but also in
skin thickening on the toes and forefoot; however,
9.4% of 106 nondiabetic siblings and 4.5% of 199
these areas ordinarily have increased skin tautness and
nondiabetic nonsibling controls (6). Again, the major-
are difficult to assess clinically with confidence. In no
ity of afflicted children (38 [95%] of 40) had contrac-
instance was skin change noted on the face, neck, or
ture of only I joint, usually the proximal interphalan-
trunk, or proximal to the wrists and ankles. The gealjoint of the fifth digit. Most recently, Rosenbloom
severity of skin thickening was scored 1+ in 25
et a1 found more generally limited mobility of small
children, 2+ in 19, and 3+ in only 3. and large joints in 92 (30%) of 309 patients with IDDM
We found flexion contractures of the distal and
(2).
proximal interphalangeal joints in 26 of these children. These findings have been variously associated
These contractures were mild, on the order of 5-10", with the duration of diabetes (1,2), the age of the
and in all but 1 case, asymptomatic. We found no
patient (6), short stature (1,2), and thickened, adherent
instance of digital flexion contractures in children with skin on the fingers and dorsa of the hands (1,2). One
apparently normal skin. Furthermore, flexion contrac-
study found an increased risk of microvascular compli-
tures were found mainly in those children with the cations of IDDM in those children with limited joint
more extensive and severe skin changes (Table 1). All mobility (2). No study has linked these changes to sex,
other joints were found to have full ranges of motion.
race, insulin dosage, or level of diabetic control.
No children had palpable thickening, rubs, or nodular-
We found palpably thickened and adherent skin
ity of the palmar flexor tendons, and in no instance did
physical findings suggest that a true arthropathy was
present. In 2 cases, positive Tinel's and Phalen's signs
Table 2. Pattern of joint involvement in 26 children with finger
were elicited: once in a diabetic child with skin contracture complicating digital sclerosis in insulin-dependent
changes and once in a diabetic child without such diabetes rnellitus
changes. In 23 children, the joint contractures in-
Pattern of involvement No. of patients (%)
volved 2 or more digits and were symmetric in distri-
bution (Table 2). The most commonly involved single Symmetric 23 (88%)
Digits 2, 3, 4, 5 6
digits were the middle (24 [92%]) and ring fingers Digits 2, 3, 4 5
(20[77%]) of 26 patients; the index (12 [46%]) and fifth Digits 3, 4 5
digit (8 [31%]) were involved less frequently. The Digits 2, 3 4
Digit 2 or 3 alone 3
pattern of joints of predilection paralleled the relative Asymmetric 3 (12%)
lengths of the affected digits. Digits 314 1
In our study group, the frequency of skin thick- Digits 231345 I
Digits 23134 1
ening was strongly correlated with the duration of
1360
on the digits and occasionally the dorsum of the hands
in 47 (34%) of 137 children with IDDM and in none of
52 controls, Those children with the more severe and
extensive skin changes were found to have mild flex-
ion contractures of the distal and proximal interpha-
langeal joints. For the most part, these contractures
were symmetric and asymptomatic and could not be
attributed to palmar flexor tendon disease or to an
underlying arthropathy. In the present study, the
frequency of digital sclerosis was strongly associated
with the duration of diabetes but not with age, age of
onset of IDDM, sex, race, insulin dosage, or historic
measures of the quality of diabetic control. We consid-
er our clinical findings to support the premise that
digital flexion contractures in IDDM are secondary to
periarticular skin and subcutaneous tissue thickening.
Very little is known about the histopathology of
these skin changes. Limited biopsy studies have re-
vealed thickening of the dermis and an accumulation
of connective tissue in the lower dermis (2,7,8). Buck-
ingham et a1 found decreased extractability of collagen
and increased hexose bound to skin protein in keta-
mine linkage in biopsies of 3 children with IDDM and
skin thickening (8). Increased cross-link formation and
accumulation of collagen of increased resistance to
collagenase are found in both IDDM (9) and experi-
mental diabetes (10).
Lieberman et a1 found lessening of skin thick-
ness in parallel with decreases in glycosylated hemo-
globin in 3 of 4 patients treated with continuous
portable pump infusion of insulin (1 1 ) . However, Ro-
senbloom et a1 found no correlation of levels of
glycosylated hemoglobin and limited joint mobility in
their series of 309 patients (2). While we did not
measure glycosylated hemoglobin levels, we did note
an association of skin thickening with duration of
IDDM. However, we encountered an occasional ex-
ceptional child in whom the clinical findings would be
difficult to explain as a sequela of prolonged hypergly-
cemia and tissue glucosylation. For example, we ex-
amined a 6-year-old boy with IDDM that had been
diagnosed only 6 months earlier; the boy’s diabetes
was well controlled with only 2 units of insulin daily,
but he had 2 + clinical skin thickening extending to the
metacarpophalangeal joints.
Although the digital sclerosis of IDDM has not
been specifically studied, a variety of data serve to link
these changes to alterations at the level of the fibro-
blast. Whereas fibroblasts from subjects with IDDM
have been found to have a reduced doubling rate in
culture (12,131, collagen and total protein synthesis is
SEIBOLD
increased (12,14). A recent study found increased
fibronectin production as well, but noted a decrease in
the fibronectin-to-collagen ratio in diabetic fibroblasts
versus normal (15).
The parallels between the digital sclerosis of
childhood diabetes and progressive systemic sclerosis
are inescapable (16). The clinical features and histopa-
thology are both indicative of dermal accumulation of
connective tissue. Both diseases manifest increased
production of collagen by dermal fibroblasts in cul-
ture. If the findings of Rosenbloom et a1 (2) are
confirmed, both diseases can be strongly linked to
abnormalities of the microvascular circulation (17-19).
The digital sclerosis of IDDM is a promising model for
further rheumatologic investigation.
ACKNOWLEDGMENTS
The author wishes to thank the New Jersey Diabetes
Association, Judy Wentink, RN, and the staff of Camp
Nejeda for their help with this study, and Ms Teri Soriano
for her secretarial skills.
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