Scleredema Diabeticorum in a Patient with the Normal Range of the Hemoglobin A1c Level and Impaired Glucose Tolerance

doi:10.4236/jcdsa.2012.23028

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Journal of Cosmetics, Dermatological Sciences and Applications, 2012, 2, 141-143

http://dx.doi.org/10.4236/jcdsa.2012.23028 Published Online September 2012 (http://www.SciRP.org/journal/jcdsa)

141

Scleredema Diabeticorum in a Patient with the Normal

Range of the Hemoglobin A1c Level and Impaired Glucose

Tolerance

Hiromasa Matsuda1, Naoki Oiso1*, Yukie Fujita2, Hiroshi Ikegami2, Akira Kawada1

1Departments of Dermatology, Kinki University Faculty of Medicine, Osaka, Japan; 2Departments of Endocrinology, Metabolism

and Diabetes, Kinki University Faculty of Medicine, Osaka, Japan.

Email: *[email protected]

Received May 7th, 2012; revised June 13th, 2012; accepted June 28th, 2012

ABSTRACT

Scleredema diabeticorum is an uncommon skin disorder showing a diffuse, symmetrical, and non-pitting persistent in-

duration developing initially on the nape. It usually occurs in patients with poorly controlled or insulin-resistant diabetes

mellitus. We present a 57-year-old woman with scleredema diabeticorum with hemoglobin A1c 6.1% (normal, 4.7% -

6.2%) and impaired glucose tolerance. Alcian blue staining at pH 7.0 illustrated deposits of acid mucopolysaccharides

between the collagen bundles in the reticular dermis, but the staining at pH 1.5 did not show the deposits. This case in-

dicates that scleredema diabeticorum may be an early indicator of undiagnosed diabetes mellitus in some cases, and that

impaired glucose tolerance test should be performed for individuals with scleredema even though the hemoglobin A1c

level is within the normal range.

Keywords: Scleredema Diabeticorum; Alcian Blue Staining; Diabetes Mellitus; Hemoglobin A1c; Impaired Glucose

Tolerance; An Early Indicator

1. Introduction

Scleredema diabeticorum, a rare disorder of unknown etio-

logy, is characterized by a diffuse, symmetrical, and non-

pitting persistent induration of the skin [1]. It first ap-

pears on the nape of the neck and then on the shoulders

and back. It is commonly preceded by long-existing, poor-

ly controlled diabetes mellitus [1]. Here, we present a 57-

year-old woman with scleredema diabeticorum associ-

ated with impaired glucose tolerance.

2. Case Report

A 57-year-old woman visited us with a slowly develop-

ing asymptomatic eruption on the nape. She had first no-

ticed it three months earlier. A physical examination re-

vealed the presence of an ill-defined, non-pitting, indura-

tion on the nape (Figure 1). She had not been diagnosed as

having diabetes. She had no recent episode of infectious

disorders. Laboratory blood test results were hemoglobin

A1c (HbA1c): 6.1% (normal, 4.7% - 6.2%); total choles-

terol: 228 mg/dl; triglyceride: 122 mg/dl; highden-

sity-lipoprotein cholesterol: 51 mg/dl; anti-hepatitis B sur-

face antibody: negative; anti-hepatitis C virus antibody:

negative; anti-human immunodeficiency virus type I

(HIV-I) antibody: negative; and anti-HIV-II antibody:

negative. We did not examine the laboratory blood test

for paraproteinemia, hyperparathyroidism and connective

tissue diseases. A biopsy specimen revealed a thickened

dermis with mucinous change (Figures 2(a) and (b)).

Alcian blue staining at pH 7.0 showed deposits of acid

mucopolysaccharides between the collagen bundles in

Figure 1. Clinical appearance of the nape of the neck at the

first visit.

*Corresponding author.

Scleredema Diabeticorum in a Patient with the Normal Range of the Hemoglobin A1c Level

and Impaired Glucose Tolerance

142

Figure 2. (a) and (b) A biopsy specimen from the affected nape

(hematoxylin and eosin stain, original magnification, (a) ×40;

(b) ×100); (c) A specimen stained with alcian blue at pH 7.0

(original magnification, ×100); (d) A specimen stained with

alcian blue at pH 1.5 (original magnification, ×100).

the reticular dermis (Figure 2(c)). Alcian blue staining at

pH 1.5 did not show deposits (Figure 2(d)). A 75 g oral

glucose tolerance test (OGTT) was performed to exam-

ine the serum glucose, serum immunoreactive insulin

(IRI), and serum C-peptide immunoreactivity (CPR)

(Table 1).

We recommended exercising, such as walking, for an

hour per day and eating a nutritionally balanced diet.

Three months later, the induration regressed and flat-

tened (Figure 3). The patient did not wish to undergo an

OGTT after regression.

3. Discussion

Scleredema is caused by deposits of only nonsulfated

acid mucopolysaccharides, which alcian blue stains at pH

7.0 but not at pH 1.5. Scleredema has two main types:

Scleredema adultorum of Buschke and scleredema dia-

beticorum [2]. Buschke-type scleredema is commonly re-

cognized as a post-inflammatory, sudden-onset subtype

that generally regresses within a few weeks to months.

The lack of a recent infectious episode, noted at a first

medical examination, suggests that the eruption was a non-

Buschke-type. Even though the HbA1c level was within

the normal range, we performed an OGTT and identified

impaired glucose tolerance. The eruption was diagnosed

with scleredema diabeticorum associated with impaired

glucose tolerance.

Patients with diabetes mellitus may develop a skin

disorder that is an early indicator of undiagnosed or a

complication of known diabetes mellitus [3]. Cutaneous

signs include scleredema diabeticorum, lichen planus,

acanthosis nigricans, disseminated granuloma annulare,

necrobiosis lipoideca, eruptive xanthomas and others.

Nigam et al. reported that 30.3% patients with lichen

Table 1. Results of 75 g oral glucose tolerance test.

Time (min) 0 30 60 90 120

Serum glucose (mg/dl) 101 147 168 179150

IRI (μU/ ml) 8.1 57.0 44.1 61.857.4

CPR (ng/ ml) 1.37 6.22 6.33 8.219.59

Figure 3. Clinical appearance of the nape of the neck three

months later from the first visit.

planus showed abnormal glucose tolerance [4]. Sclere-

dema diabeticorum is a relatively underestimated com-

plication of diabetes [5,6]. It had been debated whether

scleredema adultorum of Buschke and scleredema dia-

beticorum are identical disease [7], because the condition

of scleredema diabeticorum is believed to be essentially

permanent. Meguerditchian et al. suggested that sclere-

dema of Buschke or scleredema diabetorum is a skin

complication of diabetes and that type 1 or type 2 diabe-

tes may be associated with the condition in more than

50% of cases [6]. Our case would be an instance of scle-

redema diabetorum as a reversible early indicator of dia-

betes.

As shown here, scleredema diabeticorum may be one

of an early indicator of undiagnosed diabetes mellitus.

We hypothesize that regression of scleredema diabeti-

corum in this case may be associated with disappearance

of impaired glucose tolerance as a result of increased

physical activity and a nutritionally balanced diet. Pa-

tients having scleredema on the nape is recommended to

undergo an OGTT even if the HbA1c level is within the

normal range.

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Scleredema Diabeticorum in a Patient with the Normal Range of the Hemoglobin A1c Level

and Impaired Glucose Tolerance

143

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