Surg Today Jpn J Surg (1994) 24:1097-1100

~ SUaC, ERvTooAv © Springer-Verlag 1994

Clostridial Gas Gangrene Associated with Congenital Generalized Lipodystrophy: Report of a Case

HAJIME NAKAE, SHIGEATSU ENDO, YASUHIKO YAMADA, TETSUYA TAKAKUWA, SHIGERU TANIGUCHI, and MASATOMO KIKUCHI

The Critical Care and Emergency Center, Iwate Medical University, 19-1 Uchimaru, Morioka, 020 Japan

Abstract: Congenital generalized lipodystrophy (CGL) is a rare disease, the main symptoms of which are a reduction of systemic subcutaneous fat, characteristic facial features, hypertrichosis, and insulin-resistant diabetes. We report herein the unusual case of a 25-year-old man with CGL in whom gas gangrene developed, an association which has never before been encountered.

Key Words: clostridial gas gangrene, congenital generalized lipodystrophy, hyperbaric oxygen therapy

Introduct ion

Congenital generalized lipodystrophy (CGL) is a rare disease the main symptoms of which are a reduction of systemic subcutaneous fat, characteristic facial features, hypertrichosis, and insulin-resistant diabeteS. Although patients with CGL have been reported to develop heart failure and renal failure, this appears to be the first case of gas gangrene developing in a patient with CGL.

Case Report

A 25-year-old man who presented at a local hospital with pain and swelling in the area extending from the right chest to the axilla was transferred to our hospital following surgical drainage. He had a history of dia- betes mellitus since 12 years of age and exhibited hollow cheeks, prominent eyebrows, and a bulging upper jaw (Fig. la). His body and extremities were lacking in subcutaneous fat, and showed a tendency

Reprint requests to: H. Nakae (Received for publication on Oct. 18, 1993; accepted on July 1, 1994)

toward excessive hair growth (Fig. lb). Intertriginous chromatosis was also observed. A family history revealed that his male cousin also suffered from gener- alized lipodystrophy. Hematologi'cal findings were normal except for a markedly elevated WBC count of 22,000. His blood glucose level was 175 mg/dl.

The area from the right thoracic region to the brachium was very painful with an odor of putre- faction, and the surrounding tissues were red and inflamed. There was a crackling sensation in that area in response to touch. Roentgenographic findings showed subcutaneous gases in the right brachium, axilla, and lateral thoracic region (Fig. 2). Clostridium hastatitorme was isolated from a swab culture from an infected lesion. Thus, an additional wide skin incision was made in the lateral thoracic region, and hyperbaric oxygen therapy of 3.0 ATA for 90min twice a day, and antibiotic therapy of penicillin G, ten million units twice a day, were initiated. The gases disappeared from the lesions within 2 weeks after admission and a border of necrotic skin became apparent within 3 weeks, after which skin grafting was performed. Con- sidering the underlying CGL, the patient had insulin- resistant diabetes without ketosis, but no hyperlipemia. His basal metabolism and tests of endocrinological function were within the normal range. A 75-g glucose tolerance test gave the following venous plasma glucose levels and serum insulin levels: 134mg/dl and 34 ~tU/ ml at fasting; 165mg/dl and 86~tU/ml at 30rain; and 270 mg/dl and 262 ~tU/ml at 180 rain, respectively.

Abdominal ultrasonographic examination revealed splenomegaly and an enlarged portal vein, while laparoscopic and liver biopsy findings showed liver cirrhosis. Histopathological examination of the skin revealed a virtual absence of adipose tissue in the tunica muscularis (Fig. 3). We had difficulty in con- trolling his blood sugar; however, the serum glucose levels were finally able to be stabilized by diet alone as the gas gangrene was alleviated. The patient was dis-

1098 H. Nakae et al.: Gas Gangrene with Generalized Lipodystrophy

Fig. 1. a Characteristic face of the patient with congenital generalized lipodystrophy. Hollow cheeks, prominent eye-brows, and a bulging upper jaw are typical, b Body and extremities are lacking in subcutaneous fat, and show a tendency toward excessive hair growth and myopachynsis

Fig. 2. Initial radiographs show the presence of subcutaneous gas in the right brachium, axilla, and lateral thoracic region (arrows)

H. Nakae et al. : Gas Gangrene with Generalized Lipodystrophy 1099

Fig. 3. Photomicrograph of the skin biopsy specimen. There is a definite depletion of fat in the subcutaneous tissue with replacement of dense collagenous tissue. (H&E, ×30)

charged without any further complications 2 months after his operation.

Discussion

Generalized lipodystrophy is a rare disease first re- ported by Berardinelli in 1954. ~ Its features include reduced systemic fat tissues, myopachynsis, melan- opathy, acanthosis nigricans, hypertrichosis universalis, characteristic facial features, visceral hypertrophy of the liver, kidney, and/or spleen, and acromegaty-like characteristics due to growth acceleration. The associ- ated metabolic abnormalities include basal hypermet- abolism, hyperlipemia, and reduced glucose tolerance. These patients are believed to develop diabetes mel- litus, which is characterized by insulin resistance and a low potential for ketosis before 10 years of age. 2 The mechanism responsible for these abnormalities is not known, but GL has been classified into congenital and acquired types. The congenital type is an autosomal recessive disorder with an onset at birth, whereas the acquired type is an entirely sporadic disorder, the onset of which occurs either in childhood or young adulthood. A diagnosis of CGL was easily established in our patient because of his characteristic physical appearance, insulin-resistant diabetes, and splen- omegaly. Moreover, because he had a cousin with generalized lipodystrophy, his condition was con- sidered to be of the congenital type. Complications of this disease include fatty liver, liver cirrhosis, hyper- tension, cardiomegaly, and renal dysfunction) '4 We conducted a literature search concerning gas gangrene

as a complication of CGL, and found that our patient was apparently the first case of CGL to be associated with gas gangrene.

Nontraumatic gas gangrene often develops in the extremities, especially the feet; 5 however, our patient had no history of trauma, and his infection developed in the axilla and the thorax, having possibly begun as axillary folliculitis. Because C. hastatitorme was detected, the following four measures were employed:

1. Surgical treatment: the incision wound was enlarged with minimum d6bridement.

2. Massive doses of antibiotics, mainly penicillins, were administered.

3. Hyperbaric oxygen therapy was employed. 6 4. Skin grafting was performed when the necrotic

tissue border had become clear.

Although the prognosis of CGL is still uncertain due to the limited number of reported patients, close observation to detect the occurrence of any possible complications related to diabetes mellitus, particularly insulin-resistant diabetes, as well as cardiac dysfunc- tion due to hyperlipemia, hepatic dysfunction due to liver cirrhosis, and malignant transformation of acanthosis is mandatory. Moreover, since patients with CGL are at risk of developing infections due to their diabetes, it is possible that severe infectious dis- eases may occur, and this must be kept in mind, particularly in adult patients.

1100 H. Nakae et al.: Gas Gangrene with General ized Lipodystrophy

References

1. Berardinelli W (1954) An undiagnosed endocrinometabolic syn- drome: Report of 2 cases. J Clin Endocrinol Metab 14:193-204

2. Oseld S, Beck-Nielsen H, Pederson O, Sovik O (1977) Decreased binding of insulin to its receptor in patients with congenital generalized lipodystrophy. New Engl J Med 296:245-248

3. Ishii J, Abe M, Kubo M, Sumitomo T, Hamada M, Hiwada K, Kokubo T, Osawa G (1989) Total lipodystrophy with heart failure and renal failure: Report of a case. Jpn J Med 28:534-537

4. Rheuban KS, Blizzard RM, Parker MR, Carter T, Wilson T, Gutgesell HP (1986) Hypertrophic cardiomyopathy in total lipodystrophy. J Pediatr 109:301

5. Bessman AN, Wabner W (1975) Nonclostridial gas gangrene: Report of 48 cases and review of the literature. JAMA 233: 958-963

6. Brummelkamp WH, Boerema I, Hoogendyk L (1963) Treatment of clostridial infection with hyperbaric oxygen drenching. A report on 26 cases. Lancet 1:235-238