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Dry Gangrene of Sudden Onset (possibly purpura fulminans)

Case: Dry gangrene

Reported by Ahmad Bo-Eisa


2-year-old boy, who has a sudden dry massive gangrene of both hands and feet. He was with his family in a desert trip when he was noticed to have dark discoloration in both hands followed by a low grade fever. In 2 days all 4 extremities are gangrenous. He has a dry gangrene from both wrist distally, nothing proximal to this. In the lower limbs, he has a large patch of gangrene on the left thigh anterolaterally. the distal 2/3rd of the leg and distally is dry gangrene. No pulses distal to popliteal are felt or detected. both radial pulses are also not detected.

The patient has no neurological defecits or convulsions. and his temp remains at a low grade to no fever. The gangrene remains the same and demarkation stops at the same levels. Blood cultures and viral cultures did not show any growth. His renal, hepatic and cardiac profiles are normal.

These finding are thought to be due to a memengotoxemia but the distribution and clinical picture is not compatible. A snake or poisonous bite was also in working diagnosis. but we have no experience in a similar picture.

It would be great if someone has any suggestion or have seen a similar case before.

Comments

From: Charles Mehlman
Date: 14 Apr 1998
Sure sounds like PURPURA FULMINANS. Could be due to meningococcal septiciemia or perhaps other agents such as Haemophilus infuenzae or those native to the region. It is felt that an antigen-antibody-mediated
reaction occurs, the severity of which is determined by the amount of endotoxin exposure, leading at times to true disseminated intravascular coagulation (DIC). The mortality rate is disturbingly high.

Although usually present, fever need not always be present- the host response may simply be overwhelmed. I agree that the "picture" may not be classic, but the treatment (unfortunately) remains the same - medical supportive care & wait for tissue demarcation to guide your final amputation levels.

Charles T Mehlman, DO
Division of Pediatric Orthopaedic Surgery
Children's Hospitla Medical Center
Cincinnati, Ohio [USA]

Jacobsen ST, Crawford AH. Amputation Following Meningococcemia: A Sequelae to Purpura Fulminans. Clin Orthop Rel Res 185:214-219, 1984.

Dabney KW, Bowen JR. Complications of Musculoskeletal Infections. Chapter 32 In: CH Epps & JR Bowen [eds] Complications in Pediatric Orthopaecic Surgery. JB Lippincott. Philadelphia. 1995.

Saez-Llorens X, Lagrutta F. The Acute Phase Host Reaction During Bacterial Infection and its Clinical Imapct in Children. Pediatr Infect Dis J 12:83-87, 1993.

Styrt B, Sugarman B. Antipyresis and Fever. Arch Int Med 150:1589-1597, 1990.

From: Richard Strain
Date: 14 Apr 1998
I had one case in a healthy 27 year old female that developed Acute Gangrene of 4 limbs after significant weight loss and she was on a number of "healthfood" supplements we never found the cause even at post mortem.
Rick Strain

From: Iain Thirsk
Date: 14 Apr 1998
We have seen this not infrequently in very young children following severe dehydration caused by diarrhoea and vomiting. I'm not sure what "a desert journey" involves and how well the child was cared for and watched. Our
patients come with all sorts of weird and wonderful histories which do more to cloud issues than help. We do see a lot of venomous bites mainly from snakes (about 250 admissions per year) and they do not present like this, but such things tend to be very regional. The chances are if something venomous did do that to a child you would know about it locally.
I think the advice already given was good - treat supportively and allow the extremities to demarcate before attempting debridement, and then just do the minimum.
Iain Thirsk it@pixie.co.za
Surgeon, Ngwelezana Hospital, Kwa Zulu Natal, South Africa

From: Albert B. Accettola Jr. MD
Date: 15 Apr 1998
Could it be a Protein-C defficiency, with the hypercoagulable state being brought on by relative dehydration?
Albert B. Accettola Jr. MD

From: Wosk
Date: 15 Apr 1998
I know about same features that happen in small children in M. East deserts, but never saw so spreaded multifocal gangrenas. The reason of lesion is cold injury. Usually malnutrition and some degree of dehydration are predisposing factors. Children have been exposured for low temperature in local tribes tents. The problem is in desert the great fluctuation of high midday temperatures that drops sharply to very low night temperatures.
I believe, you just have very good advices from some fellows of the list. I am only worry about thigh injury. This is location where dry gangrena can perform to wet with sepsis etc.I think that antibiotic administration, hyperalimentation and closed supervision are avaiable.

From: Ahmad Bo-eisa (originator)
Date: 15 Apr 1998
Yes the desert at this time of the year is very cold at night. We never see this type of ischemic gangrene even if the child is left uncovered. The patient has somehow a pregressive gangrene during the daytime when the family have noticed that upper limbs started first. His general condition remains stable. His coagulation profile was in a reasonable status with a moderately high platelet's count.

From: Ahmad Bo-eisa (originator_
Date: 15 Apr 1998
Thank you. I started to believe that it is in favor of PURPURA FULMINANS. Now it is 3 weeks since the onset. The patient is otherwise in good stable condition. demarkation is well established now and he is ready for amputations.

From: COBDEN
Date: 16 Apr 1998
Have seen this once before, in a young man with a history of splenectomy and subsequent pneumococcal infection and sepsis.
COBDEN@aol.com

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