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Proteus syndrome

Proteus Syndrome (PS) is a congenital disorder that causes skin overgrowth, atypical bone development, and tumour appearance over half the body. Proteus Syndrome is extremely rare. Since Dr. Michael Cohen identified it in 1979, only a few more than 100 cases have been confirmed worldwide. more...

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There may be many more than this, but those individuals correctly diagnosed usually have the most obvious manifestations of Proteus syndrome, leaving them severely disfigured.

This extremely rare condition would have remained obscure, were it not for the fact that Joseph Merrick - immortalized as the "Elephant Man" for a look imparted by his large facial tumour and the grayish hue of his overgrown skin - was lately diagnosed as having a particularly severe case of Proteus syndrome rather than, or in addition to, the neurofibromatosis (NF) doctors once thought he had.

Proteus syndrome is a progressive condition, wherein children are usually born without any obvious deformities. As they age, tumours as well as skin and bone growths appear. Some affected individuals may suffer from learning disabilities as a result of these growths, and a significantly shortened lifespan. The disorder affects both genders equally, and can be found in all ethnicities.

Like Merrick, however, many people with Proteus syndrome are of normal or above-average intelligence. Their greatest difficulty lies in how society treats them on account of their serious deformities.

Researchers are still trying to determine the cause(s) of Proteus syndrome. While doctors can treat some of the symptoms (by removing tumours, for example), there is no cure.

Many sources classify Proteus syndrome to be a type of epidermal nevus syndrome (see external links).


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Tropical diabetic hand syndrome — Dar es Salaam, Tanzania, 1998-2002 - hand sepsis
From Morbidity and Mortality Weekly Report, 11/1/02

Patients with diabetes mellitus have impaired immunologic responses to combat infections (1). Infection and ulceration of the hand is a major cause of morbidity and mortality in certain populations in Africa (2,3); however, the condition is less well recognized than foot infections and is not classified generally as a specific diabetes complication. Hand ulceration and infection in diabetic patients was first described in the United States in 1977 (4) and in Africa in 1984 (5). Subsequently, the majority of reported cases have been from various parts of the African continent (2,6,7). The term "tropical diabetic hand syndrome" (TDHS) has been used to describe diabetes among patients who have progressive, fulminant hand sepsis (3,8,9). More recently, TDHS has been reported among patients in India (10). These data suggest that TDHS occurs primarily in diabetic patients who live in tropical or coastal areas and might result in loss of hand function, amputation, or death (2). This report describes the characterist ics of 72 patients with TDHS examined at Muhimbili National Hospital (MNH) in Dar es Salaam, Tanzania. Early recognition by patients, prompt medical attention, and improved glycemic control might reduce the incidence of disability or death.

A patient with TDHS was defined as any adult diabetes patient with hand cellulitis, infection, and gangrene who sought medical attention at MNH during February 9, 1998-August 22, 2002. A total of 72 patients had illnesses that met the case definition; 36 (50%) were male, 44 (61%) had type 2 diabetes, and all had first episodes of diabetes. Median age of patients was 52 years (range: 20-89 years), median interval since diagnosis of diabetes was 5 years (range: 2 weeks-19 years), and median body mass index was 23.4 kg/[m.sup.2] (range: 15-39 kg/[m.sup.2]). Patients' median blood glucose level at initial presentation was 280 mg/dL (range: 56-626 mg/dL). Peripheral neuropathy was present in 10 (14%) patients; one patient had evidence of peripheral vascular disease, which was ascertained through Doppler studies. The initial precipitating causes of TDHS varied: hand trauma was reported in 19 (26%); itching, caused possibly by insect bites, occurred in 11 (15%); boils were the precipitating cause in 10 (14%); seemin gly innocuous papules were the cause in nine (13%) patients; and the cause was unknown in 23 (28%) patients. All 72 patients had hand ulcerations; 61(85%) were purulent, 23 (32%) had a deep ulcer which involved the bone, and 17 (24%) had localized or widespread gangrene of the arm. The median time between onset of symptoms and initial clinical evaluation by a physician was 14 days (range: 2-252 days).

Superficial swab cultures of hand lesions were obtained for the majority of patients. These cultures all yielded polymicrobial growth that included Streptococcus spp., Staphylococcus aureus, S. epidermidis, Kiebsiella pneumoniae, Pseudomonas aeruginosa, Escherichia coli, or Proteus mirabilis.

Patients for whom delay in seeking treatment was >14 days (median) from onset of symptoms were significantly more likely to undergo a surgical procedure after hospital admission (relative risk [RR]=1.8; 95% confidence interval [CI]=1.0-3.3; p<0.05) or to have acquired a long-term hand deformity at follow-up (RR=2.0; 95% CI=1.1-3.9; p<0.05). Patients who delayed seeking medical attention were twice as likely as those who did not delay to have gangrene of the hand or arm. Patients with a random blood glucose level of [greater than or equal to]280 mg/dL (median) were significantly more likely than those with a random blood glucose level of <280 mg/dL to undergo surgery (RR=1.7;95% CI=1.02-2.8; p<0.05). Patients with random blood glucose levels above the median were twice as likely as those below the median to have gangrene (11 of 37 versus five of 35).

All 72 patients received antimicrobial therapy after initial clinical evaluation. Overall, 36 (50%) patients underwent surgery; 16 (44%) had gangrene of the hand. Of these 16 patients, seven (44%) required amputation of fingers, hand, or arm because of very rapid progression to gangrene. The remaining 29 patients who had surgery underwent incision and drainage and debridement.

Follow-up was completed for 64 (89%) patients. Of these, 51 (80%) had complete healing of their hand ulcer and resolution of inflammation; eight (13%) had ulcers that did not heal, and five (8%) died. During follow-up, 33 (52%) patients were found to have substantially impaired hand function that adversely affected their daily living activities. Damages included wasting, strictures, deformities, chronic lymphedema, or chronic pain. Of the 51 patients with healed ulcers, 20 (39%) reported chronic, severe neuropathic pain.

Reported by: ZG Abbas, MMed, J Lutale, MMed, Muhimbili Univ College of Health Sciences, Dar es Salaam, Tanzania. LK Archibald, MBBS, WR Jarvis, MD, Div of Healthcare Quality Promotion; G Beckles, MD, Div of Diabetes Translation; K Moore, MD, EIS Officer, CDC.

Editorial Note: TDHS is a complication of diabetes that has been reported in tropical regions of Africa (8,9) and in India (10). It is both poorly understood by patients and clinicians and severe in consequence without prompt and aggressive treatment. Given its innocuous initial stages, patients and clinicians might assume that hand ulceration and infection is analogous to the more familiar and indolent diabetic foot ulcer. Previous small series or case reports indicate the severe consequences of TDHS, including permanent disability and death (2,3,5,6,8).

The findings in this report illustrate important characteristics that distinguish TDHS from diabetic foot ulcer syndrome. Patients with TDHS have poorly controlled blood glucose levels; neither peripheral vascular disease nor peripheral neuropathy appear to play a substantial role in the pathogenesis of TDHS. In contrast, peripheral vascular disease and peripheral neuropathy are well-known risk factors for diabetic foot ulcers and foot infections.

TDHS can develop into a rapidly progressive, synergistic gangrene (Meleney's gangrene) confined to the superficial fascia that can result in death within days of onset of symptoms (2,3). Although the majority of patients survive, permanent disability is likely. The most common cause of polymicrobial synergistic gangrene is a symbiotic relationship of aerobic gram-negative rods in combination with different enteric anaerobes (3). Culture of tissue biopsy specimens yields a single bacterial species in >75% of cases, whereas swab cultures yield polymicrobial flora in the majority of cases, probably because of contamination (3). Therefore, routine swabs of open, infected hands cannot guide optimal antimicrobial therapy and might not be appropriate use of resources in hospitals with limited laboratory facilities.

The likelihood of permanent disability or death might be increased because of delays in medical treatment. Such delays might occur because of limited access to medical care or because the patient is unaware of the risk for life-threatening infection. Many patients reported initial symptoms that seemed insignificant, such as itching or an insect bite. These symptoms did not alarm patients enough to seek medical care until much further into the course of the infection. Educating diabetes patients can minimize delays in seeking prompt medical attention once they recognize the initial signs and symptoms of TDHS.

Appropriate treatment for the majority of patients includes incision and drainage of the wound, debridement, or amputation. Antimicrobial therapy must be broad-spectrum because of the potential for development of polymicrobial gangrene. Physicians treating patients with diabetes should examine patients' hands and educate them about TDHS.

Prevention of permanent disability and death resulting from TDHS will require more study. The effects of demographic, socioeconomic, and behavioral factors on the occurrence of TDHS remain unknown. A case-control study is under way in Tanzania to identify risk factors for development of TDHS among diabetic patients. Poor glucose control is evident in this cohort and might be an important factor contributing to the development of TDHS, highlighting the need for improved management of glycemic levels. Further laboratory studies are needed to characterize the causative organisms, especially in coastal regions of Africa. As diabetes becomes more prevalent worldwide, especially in resource-limited tropical countries, patients and health-care providers in these regions should be educated about TDHS to prevent its life-threatening and crippling complications.


(1.) Robertson HD, Polk HG Jr. The mechanism of infection in patients with diabetes mellitus: a review of leukocyre malfunction. Surgery 1974;75:123-8.

(2.) Archibald LK, Gill GV, Abbas Z. Fatal hand sepsis in Tanzanian diabetic patients. Diabet Med 1997;14:607-10.

(3.) Gill GV, Famuyiwa OO, Rolfe M, Archibald LK. Serious hand sepsis and diabetes mellitus: specific tropical syndrome with western counterparts. Diabet Med 1998;15:858-62.

(4.) Mann RJ, Peacock JM. Hand infections in patients with diabetes mellitus. J Trauma 1977;17:376-80.

(5.) Akintewe TA. The diabetic hand--5 illustrative case reports. Br J Clin Pract 1984;38:368-71.

(6.) Bosseri S, Gill G. Hand and foot sepsis in Libyan diabetic patients. Trop Doct 1997;27:232-3.

(7.) Ezeldeen K. Management of hand infection in Khartoum. East Afr Med J 1992;69:616-8.

(8.) Abbas ZG, Lutale J, Gill GV, Archibald LK. Tropical diabetic hand syndrome: risk factors in an adult diabetes population. Int J Infect Dis 2001;5:19-23.

(9.) Gill GV, Famuyiwa OO, Rolfe M, Archibald LK. Tropical diabetic hand syndrome. Lancer 1998;351:113-4.

(10.) Bajaj S, Bajaj AK. Tropical diabetic hand syndrome-Indian experience. J Assoc Physicians India 1999;47:1118-9.

COPYRIGHT 2002 U.S. Government Printing Office
COPYRIGHT 2004 Gale Group

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