Laboratory Data

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Ecthyma gangrenosum

What are your opinions about the diagnosis and treatment?

Luemsamran P, Pornpanich K, Vangveeravong S, Mekanandha P. Orbital cellulitis and endophthalmitis in pseudomonas septicemia. Orbit. 2008;27(6):455-7

Department of Ophthalmology, Mahidol University, Siriraj Hospital, Bangkok, Thailand.

We report a case of concurrent orbital cellulitis and endophthalmitis that resulted from endogenous complications of community-acquired Pseudomonas aeruginosa bacteremia in an apparently healthy individual. Pseudomonas pneumonia and extensive focal skin lesions of ecthyma gangrenosum also complicated the condition. The presence of drug-induced neutropenia was a risk factor in this patient. Simultaneous orbital cellulitis and endophthalmitis developed and rapidly progressed. Intravenous, intravitreal, and topical antibiotics were administered along with frequent eye wash with normal saline to dilute copious purulent discharge from a deep subcutaneous abscess of lower eyelid. Because of the exocellular products of Pseudomonas aeruginosa, the sclera and corneal stroma were degraded, resulting in nearly perforated cornea. Tarsoconjunctival flap from the upper eyelid was performed to reconstruct the thinning areas. After the infection was controlled, the patient's ultimate visual acuity was light perception.

Ecthyma gangrenosum: an important feature of pseudomonal sepsis in a previously well child.Goolamali SI, Fogo A, Killian L, Shaikh H, Brathwaite N, Ford-Adams M, Macfarlane S. Clin Exp Dermatol. 2009 Jul;34(5):e180-2.

Department of Dermatology, King's College Hospital, Denmark Hill, London, UK.

Ecthyma gangrenosum is a rare, distinctive skin disorder associated with potentially fatal underlying pseudomonal sepsis. Although typically occurring in neutropenic or immunocompromised patients, it can occasionally affect healthy children. The appearances are characteristic with small indurated vesicular papules progressing rapidly to infarcted necrotic areas with surrounding erythema and a typical black eschar. In young children, these are often accompanied by fever and diarrhoea. The absence of suppuration and slough distinguishes it from the more recognized pyoderma gangrenosum. Lesions can occur at any site although are most commonly found over the buttocks, limbs, axillae and perineum. We describe the case of a 28-month-old, previously well child who presented with typical features of ecthyma gangrenosum secondary to Pseudomonas infection who responded to appropriate antibiotic treatment. Despite a thorough search, no underlying cause was found. Early recognition and prompt treatment with antipseudomonal antibiotics is vital to reduce morbidity and potential mortality.

ecthyma, pseudomonas

Henry Foong FRCP, Consultant Dermatologist, Foong Skin Specialist Clinic, Ipoh, Malaysia on September 9, 2009

I have just reviewed the patient’s photos.  Based on the clinical findings, I think this patient has pseudomonas septicemia.  He needs urgent IV fluids, IV antibiotics and toilet debridement of the lesions on the body (with dil hydrogen peroxide/saline).  Agree that the clinical lesions are those of ecthyma gangrenosum. The characteristic lesions of ecthyma gangrenosum are hemorrhagic pustules or infarcted-appearing areas with surrounding erythema that evolve into necrotic ulcers surrounded by erythema. As with the choice of antibiotics, I would use a combination but depends on local culture and sensitivity results.  The combination of IV piperacillin and genta/amikacin is OK. Try to figure out why he has septicemia.  Is he immunocompromised?

Eye consult is crucial and urgent but best left to the ophthalmologist. Early recognition and prompt treatment with antipseudomonal antibiotics is important to reduce morbidity and mortality. Good luck.

1. Goolamali SI, Fogo A, Killian L, Shaikh H, Brathwaite N, Ford-Adams M, Macfarlane S. Ecthyma gangrenosum: an important feature of pseudomonal sepsis in a previously well child. Clin Exp Dermatol. 2009 Jul;34(5):e180-2.
2. Pavithran K. Ecthyma gangrenosum with pseudomonas septicemia. Indian J Dermatol Venereol Leprol 1991;57:192-3

David Elpern MD, Consultant Dermatologist, The Skin Clinic, Williamstown, USA on September 9, 2009

Dear Dr. Jabir, Thank you for presenting this instructive case. Please keep us posted on how this child does. There is a good review of E.G. on emedicine.com. http://emedicine.medscape.com/article/1053997-overview In the U.S. such a patient would probably be managed by an infectious disease specialist. I suspect you could do as well with a pediatrician as a colleague. Ophthalmic involvement has been reported (see PubMed). You and your colleagues will be looking for immunodeficiency -- even where it was not there initially, it may follow an episode. I think you have done all the right things to date. Here's a nice review I found:

Zomorrodi A, Wald ER. Ecthyma gangrenosum: considerations in a previously healthy child. Pediatr Infect Dis J. 2002 Dec;21(12):1161-4.
Department of Pediatrics, University of Pittsburgh School of Medicine, PA, USA.

Ecthyma gangrenosum is a skin lesion that is most commonly caused by. Although ecthyma gangrenosum usually develops in patients with underlying immunodeficiencies or chronic diseases, there have been reports of its appearance in previously healthy children. A review of such patients in the English literature showed that most of them had either previously undetected immunodeficiencies or transient risk factors that predisposed them to the development of ecthyma gangrenosum. We report a patient without apparent antecedent predisposing risk factors for ecthyma gangrenosum who developed chronic neutropenia 1 week after presentation. It is important for the primary care provider to recognize ecthyma gangrenosum, treat it with appropriate antimicrobial agents and investigate the patient for occult immunodeficiencies.

Sunil Dogra MD, Assistant Professor, Dermatology, PGIMER, Chandigarh, India on September 9, 2009

I agree with the comments given by David and Henry.

Apart from that, based on morphology of skin lesions in the index case, one may consider following differential diagnoses - Bullous/ necrotizing Fixed drug eruption (mucosal involvement and preceeding drug history), Vasculitis (in a suggestive clinical setting), Purpura fulminance (lesions are irregular, more dusky, hemorrhagic).

Thanks for this case illustration.

Julian Manzur Katrib M.D., Prof. Faculty "Enrique Cabrera", Havana, Cuba, on September 11, 2009

I agree this baby has a diagnosis of pseudomonas septicemia. Frequently it is an important event in a complex illness with bad prognosis.

Good luck and great wishes that the therapy will be effective and this 8 month child has a complete recovery. Please would like to know follow up of this boy.

Ken Fowler MD, Consultant Pediatrician, South Shore, Massachusetts, USA on September 13, 2009

Regarding the immediate concerns, I agree with piperacillin (we use pip + tazo in the USA) along with amikacin for empiric therapy until the culture results are available. I agree that in the U.S. a pedi ID consultant would be involved, but in this case the sensitivity results are really what you need. There is a controversy about "double coverage" of pseudomonas - the need to use two different antibiotics to treat this rather difficult to treat organism. In this case, I would use two different agents (as you have done) and adjust based on culture results.

In the USA, we see pseudomonas sepsis most commonly in the setting of chemotherapy-induced neutropenia. However, transient and intermittent neutropenias do happen in otherwise healthy-appearing children and perhaps more often than we realize. If this unfortunate little boy survives the sepsis, I would consider followup with checking occasional CBCs. In the U.S. people often associate pseudomonas with cystic fibrosis but that does not seem to fit in this case based on the info so far.

Wound care and the ophthamologist's care will be critically important. Please let us know how this child does; if he is doing poorly, please post on this forum and I can try to find a Boston specialist for a long-distant consult.

Mardziah Alias M.D., Consultant Pediatric Dermatologist, Damansara Specialist Hospital, Kuala Lumpur, Malaysia on September 13, 2009

I would agree with the diagnosis of Pseudomonas septicaemia with ecthyma gangrenosum and orbital cellulitis. The clinical presentation and the skin sign is typical.The antiboitics used is suitable; combination of aminoglycoside and piperacillin. Get the culture & sensitivity right. The response varies. Make sure that the duration of treatment is adequate. Good luck and keep us updated.

Munqithe M Jabir MD, FICMS, Consultant Dermatologist, Baghdad, Iraq on September 13, 2009

The ophthalmologist (this is the third ophthalmic consultation) wrote today after examining the baby's eye:

This is a case of endophthalmitis (endogenous) which is very rare. The eye is lost and some of the intraocular structures, mainly the ciliary body and choroid are visible. Imminent rupture is expected. The safest way is to do evisceration of the eye. His father refused that and asked for medical treatment. I told him about the risk of meningitis, encephalitis or intracranial abscess. Suggest continuation of systemic antipseudomonal treatment, Oflox (Ofloxacin 0.3%) ophthalmic solution every hour and fucithalmic (Fucidic acid) viscous eye drops 6 hourly)

During the last few days the eye was treated by washing, atropin drops, TobraDex drops, Chloramphenicol eye ointment and drops, and Dexamethazone drops. And we unfortunately failed to save the eye. The baby, otherwise, is much better regarding the skin lesions (that are getting dry and smaller) and general health.

Bushan Kumar MD, Former Head of Dermatology, Postgraduate Institute of Medical Education & Research, Chandigarh, India on September 13, 2009

Erthema around the eschar is very bright and prominent and the lesions look like a severe form of erythema multiforme with central necrosis. In addition to the appropriate antibiotics acyclovir to cover herpes simplex infection might have been an useful addition.