scleredema Diabeticorum: A Common andDistinct Cutaneous Manifestation of

Diabetes MellitusGARY W. COLE, JOHN HEADLEY, AND RONALD SKOWSKY

Scleredema diabeticorum is characterized by a dramatic increase in the thickness of the skin of theposterior neck and upper back. Of the 17 scleredema patients diagnosed by us in the last 15 yr, 16have had type II diabetes mellitus. In a prospective study of 484 diabetic outpatients we found theprevalence of scleredema to be 2.5%. Angina pectoris was the only complication that occurred sig-nificantly more frequently in scleredematous diabetic patients than in a control group of diabetic patientswithout scleredema. Scleredema diabeticorum is a distinct cutaneous condition peculiar to diabeticindividuals and ought not to be confused with scleredema of Buschke or scleroderma. DIABETES CARE 6:189-192, MARCH-APRIL 1983.

There are a large number of cutaneous abnormalitiesthat have been frequently noted in patients withdiabetes mellitus. The skin lesions most specifi-cally associated with diabetes are necrobiosis li-

poidica and diabetic dermopathy.'A less well-known but rather dramatic cutaneous marker

of diabetes mellitus is scleredema. The scleredema of diabetesis characterized by a remarkable thickening of the dermis ofthe neck and upper back which, although essentially per-manent, causes little morbidity. ' In this study we report theresults of our investigation of the prevalence of scleredemain diabetic patients as well as its clinical implications in theprognosis of diabetes mellitus.

MATERIALS AND METHODS

Initially, a retrospective review of all scleredema patientsseen at the University of California, Irvine, Affiliated Der-matology Clinics within the last 15 yr was conducted. Thevast majority of these patients have been seen and diagnosedby the authors within the last 4 yr at the Veterans Admin-istration Medical Center, Long Beach, California. Skin biop-sies were performed on most patients and processed using thestandard hematoxylin and eosin stain. Occasionally specialstains for mucopolysaccharides were done.

In a prospective study all patients reporting to DiabetesClinic at the Veterans Administration Medical Center, LongBeach, over a 6-mo period were examined for the presenceof scleredema. The records of 12 scleredema patients culledfrom this clinic were compared with the records of 100 ran-

domly selected diabetic patients from the same clinic pop-ulation with reference to important prognostic factors in di-abetes mellitus. These factors included retinopathy (definedas the presence of retinal hemorrhages, exudates, or vascularproliferation); peripheral vascular disease (defined as a historyof ischemic ulceration, gangrene, peripheral arterial bypassgrafts, or a significant decrease in pulse pressure); nephro-pathy (defined by the presence of proteinuria greater than145/95); and neuropathy (defined as a deficit in autonomic,sensory, or motor function referable to diabetes). Statisticalanalysis of the prevalence of diabetic complications was per-formed according to the method of Swinscow using an adap-tion of the Student t test.4

RESULTS

Since 1968 we have documented scleredema in 17 patients.Fifteen new cases were discovered within the last 4 yr at theVeterans Hospital, Long Beach. Ninety-four percent of thesepatients had type II diabetes and most required medicationfor control of their blood sugar.

Individuals with scleredema were virtually asymptomaticand few of them, therefore, were able to accurately date theonset of their disease. In those patients who could date theonset, the duration was prolonged (3-30 yr). In the 4 yr thatmany of these patients have been followed, the sclerede-matous involvement has remained unchanged.

In all our patients the signs of scleredema were similar.The skin of the neck and upper back was always involved

DIABETES CARE, VOL. 6 NO. 2, MARCH-APRIL 1983 189

SCLEREDEMA DIABETICORUM/G. W. COLE, J. HEADLEY, AND R. SKOWSKY

FIG. I Patient with scleredema and diabetesmeUitus. The red area in the center of the backwas indurated and leathery.

with occasional extension to the deltoid and lumbar regions(Figure 1). The scleredematous involvement in these areaswas characterized by an impressive thickening of the dermis,which produced a peau d'orange appearance. This thickening"pitted" under constant forceful thumb pressure for 30 s. Mostpatients appeared to have decreased cutaneous sensation topain and light touch in affected areas. Occasional patientshad a decreased range of motion of the upper extremity ifthe deltoid skin was involved. Affected areas had a modesterythematous tinge.

Pathologic examination obtained from scleredema patientswas carried out on tissues by punch biopsy. The interpretationof such material is difficult because the major criteria for thediagnosis of this condition is an increase in dermal thickness.This thickening is due to an increase in collagen or mucincontent (Figure 2), but neither of these findings is specificfor scleredema. Specimens were therefore interpreted as"consistent with scleredema" by our pathology service.

We prospectively studied the prevalence of scleredema inan unselected group of outpatients reporting to the diabeticclinic at the Veterans Administration Medical Center, Long

Beach. Of 484 patients examined, 12 were found to havescleredema resulting in a prevalence rate of 2.5%.

We selected a number of clinical factors likely to be im-portant in the morbidity and mortality of diabetic patients.We determined the prevalences of these complications in 12scleredema patients discovered in diabetes clinic and com-pared these with prevalences found in a group of 100 age-and sex-matched diabetic controls (Table 1). The averageage of our scleredematous diabetic group was 56.4 yr com-pared with 54.6 yr for our control diabetic group. The averageduration of diabetes in our scleredema group was 14.2 yrcompared with 12 yr for controls. Seventy-five percent ofscleredematous diabetic subjects required insulin comparedwith 70% of control diabetic subjects. Scleredematous dia-betic patients were, on the average, 71.5 pounds overweightcompared with 31.2 pounds for our unaffected diabetic group(Metropolitan Life Insurance Company, New York). Anginawas the only statistically significant factor in which the groupsdiffered. Our diabetic scleredema patients had more thantwice the prevalence of angina found in nonscleredematousdiabetic patients. Although scleredema patients had less ar-

190 DIABETES CARE, VOL. 6 NO. 2, MARCH-APRIL 1983

SCLEREDEMA D1ABETIC0RUM/G. W. COLE, J. HEADLEY, AND R. SKOWSKY

FIG. 2 The histologic appearance of scleredemais characterized by a relative increase in fibroustissue in the dermis. A colloidal iron stain waspositive for mucopolysaccharides (hematoxylin andeosin, X 40).

teriosclerotic vascular disease than did the diabetic controls,this difference was not statistically significant.

DISCUSSION

Some 30% of diabetic patients have some sort of cutaneousinvolvement during their disease.5 Of the skin changes seenin diabetic individuals, most are either nonspecific or notedas part of the systemic response to diabetes.2 Only two der-matologic lesions, necrobiosis lipoidica diabeticorum (NLD)and diabetic dermopathy (DD), are thought to be highlyspecific for diabetes. NLD has the clinical appearance oforange-red telangiectatic plaques on the anterior lower leg,

TABLE 1Frequency of complications in diabetic patients

Complication

RetinopathyCataractsPeripheral vascular diseaseCerebrovascular accidentsAngina pectorisMyocardial infarctionNephropathyNeuropathyHypertension

(+) Scleredema(N = 12)

(%)

251788

67170

4267

(—) Scleredema(N = 100)

(%)

3520211028*21123849

*P<0.05.

often accompanied by ulceration. The prevalence of NLDhas been found to be 0.1-0.3% among diabetic individuals(types I and II), and 35% of patients with NLD do not havecarbohydrate intolerance.6 DD was noted in as many as 60%of diabetic patients but was also noted in 20% of patientswith other endocrine conditions and in 1.5% of normal med-ical students.7 This condition also occurs on the anteriorlower legs and is characterized by multiple hyperpigmenteddepressed scars.

We found the prevalence of scleredema diabeticorum tobe 2.5% in diabetic patients (type II). Ninety-four percentof our patients with scleredema had diabetes mellitus. Scler-edema diabeticorum is, therefore, more prevalent in type IIdiabetic patients than NLD, and is associated with diabetesmore frequently than is DD. Because most of our patientswere seen in a Veterans Medical Center, the majority of ourpatients were male and middle-aged. It is possible that scler-edema diabeticorum could occur more frequently in this groupthan in a group of diabetic individuals more representativeof the whole population.

Part of the difficulty of recognizing the specific relationshipbetween scleredema and diabetes has been the confusionbetween the scleredema seen in diabetic individuals and thescleredema of Buschke.8 Buschke's scleredema is a rare dis-order in which areas of dermal thickening occur, frequentlyafter an upper respiratory infection, and spontaneously clearin months or years.9 The face is frequently involved as wellas arms and hands. Females are more frequently affected thanmales and 29% of the cases occur in childhood. Scleredemadiabeticorum occurs almost exclusively in patients with long-

DIABETES CARE, VOL. 6 NO. 2, MARCH-APRIL 1983 191

SCLEREDEMA DIABETICORUM/G. W. COLE, J. HEADLEY, AND R. SKOWSKY

standing diabetes mellitus, is essentially permanent, has norelationship to infection, always occurs on the posterior neckand upper back, and has not been reported in children.Scleredema diabeticorum causes little morbidity in affectedpatients and there is no effective treatment.

The diagnosis of diabetic scleredema is easily made. Thedramatic increase in dermal thickness is obvious by simplypalpating the involved skin. Ultrasound imaging may be usedto quantify this increase in thickness.10 It is difficult to makean unequivocal diagnosis of scleredema from the histologicexamination of a skin biopsy specimen alone, since dermalthickness is difficult to evaluate unless an attempt is madeto secure a full thickness excisional biopsy. Early lesions ofscleredema are thought to have an excess of mucopolysac-charide, but older lesions apparently lack this change.11 Al-though scleroderma occasionally may be confused with scler-edema, the lack of systemic involvement in scleredema isusually sufficient to distinguish it.12

Rosenbloom et al.13 recently reported a group of childrenwith type I diabetes mellitus, decreased joint mobility, andincreased microvascular complications. These patients had"waxy skin" confined to the dorsal surface of their handsapparently related to an increase in dermal thickness. Al-though our scleredema patients had no special risk of micro-vascular complications, an increase in dermal thickness oc-curred in both pathologic states. One could speculate thatdefective collagen biosynthesis or metabolism may affect thecollagenous component of the basement membrane in dia-betic individuals.

There seems little doubt that scleredema of the type ourpatients exhibited is a specific manifestation of diabetes mel-litus in the skin. If one can accept the latinized adjective"diabeticorum" to describe necrobiosis lipoidica, we can seelittle objection to the use of the term, scleredema diabeti-corum, to label the skin disease herein described.

From the Dermatology Service and Endocrinology Section, Vet-erans Administration Medical Center, Long Beach, California, and

the Department of Dermatology, University of California, Irvine,California.

Address reprint requests to Gary W. Cole, M.D., 5901 E. 7thStreet, Long Beach, California 90822.

REFERENCES1 Hanson, T.: Diabetic dermopathy. In Clinical Dermatology.

Demis, D. J., Dobson, R. L., and McGuire, J., Eds. Hagerstown,Harper and Row, 1979:4-10.

2 Fleischmajer, R., Faludi, G., and Krol, S.: Scleredema anddiabetes mellitus. Arch. Dermatol. 1970; 101:21-35.

3 Cohn, B. A., Wheeler, C. E., and Briggaman, R. A.: Scler-edema adultorum of Buschke and diabetes mellitus. Arch. Der-matol. 1970; 101:27-35.

4 Swinscow, T. D. V.: Statistics at Square One. London, BritishMedical Association, 1980:28-29.

5 Allen, G. E.: Diabetes mellitus and the skin. Practitioner 1969;203:189-93.

6 Mueller, S. A., and Winkelmann, R. K.: Necrobiosis lipoidicadiabeticorum. A clinical and pathologic study of 171 cases. Arch.Dermatol. 1966; 93:272-81.

7 Danowski, T. S., Sabeh, G., Sarver, M. E., Shelkrot, J., andFisher, E. R.: Shin spots and diabetes mellitus. Am. J. Med. Sci.1966; 251:570-75.

8 Curtis, A. C , andShulak, B. M.: Scleredema adultorum. Arch.Dermatol. 1965; 92:526-42.

9 Rook, A., Wilkinson, D. S., and Ebling, F. S. G.: Textbookof Dermatology, 3rd edit. Oxford, Blackwell, 1979.

10 Cole, G. W , Handler, S. J., and Burnett, K.: The ultrasonicevaluation of skin thickness in scleredema. J. Clin. Ultrasound.1981; 9:501-503.

11 Lever, W. F., and Schaumburg-Lever, G.: Histopathology ofthe Skin, 5th edit. Philadelphia, Lippincott, 1975.

12 Joblonska, S.: Scleroderma and Pseudoscleroderma, 2nd edit.Warsaw, Polish Medical Publ., 1975.

13 Rosenbloom, A. L , Silverstein, J. H., Lezotte, D. C , Rich-ardson, K., and McCullum, M.: Limited joint mobility in childhooddiabetes mellitus indicates increased risk for microvascular disease.N. Engl. J. Med. 1981; 305:191-94.

192 DIABETES CARE, VOL. 6 NO. 2, MARCH-APRIL 1983