Ecthyma gangrenosum caused by Citrobacter freundii.

A 55-year-old man undergoing chemotherapy for recurrent multiple myeloma presented with a 2-day history of bilateral lower leg rash with pain and oedema. On examination, there were numerous non-palpable retiform pruritic patches over both lower legs. Skin pnch biopsy demonstrated a diffuse interstitial neutrophilic infiltrate with necrosis. Peripheral blood and skin tissue cultures both isolated Citrobacterfreundii, consistent with a rare form of ecthyma gangrenosum. The patient responded with appropriate antibiotic therapy and removal of medical port. He made a full recovery from this infectious complication of his underlying immunosuppression.

Colostomy for Perianal Sepsis With Ecthyma Gangrenosum in Immunocompromised Children.

BACKGROUND: Perianal sepsis with ecthyma gangrenosum is a severe and potentially mutilating complication in immunocompromised children. Therapies include antimicrobial treatment, incision and drainage, generous tissue debridement, and skin transplantation. PROCEDURE: We describe 3 children with acute lymphoblastic leukemia having sepsis with Pseudomonas aeruginosa in febrile neutropenia and severe perianal infections treated relatively early with a protective colostomy. Indications for colostomy were nonhealing wounds, and ceaseless pain. RESULTS: All patients showed a rapid reduction of pain. Complete wound healing was seen in 2 patients, and considerable pain reduction and increased quality of life were seen in a third patient during palliative care. CONCLUSIONS: These results suggest that a protective colostomy should be considered early in the management of immunocompromised children with ecthyma gangrenosum.

Ecthyma gangrenosum skin lesions in previously healthy children.

UNLABELLED: Ecthyma gangrenosum (EG) is an uncommon skin lesion that usually develops in patients with known immune deficiency or who are on immunosuppressive treatment. We report on five previously healthy children presenting with EG. Three of them developed severe neutropaenia. Immunologic work-up revealed chronic neutropaenia in two. CONCLUSION: Recognition of EG is essential for providing early appropriate empiric antibiotic treatment. An immunologic work-up should be done, although a concomitant viral infection can be the predisposing factor.

Autosomal recessive agammaglobulinemia: the third case of Igß deficiency due to a novel non-sense mutation.

This study describes the third case worldwide of autosomal recessive agammaglobulinemia due to a novel non-sense mutation in Igß presenting with neutropenia, ecthyma and mild respiratory infections.

Ecthyma gangrenosum and neutropenia in a previously healthy child.

Ecthyma gangrenosum is the cutaneous manifestation of pseudomonas infection in patients with sepsis. A previously healthy 7-month-old girl who developed ecthyma gangrenosum without apparent inciting factors became neutropenic secondary to autoimmune neutropenia 2 months after initial presentation. She was treated with appropriate surgical and medical intervention and was discharged in stable condition only to die suddenly 2 days after discharge.

Labial ecthyma gangrenosum in an immunocompromised infant with leukemia: heightening awareness for the urologist.

Ecthyma gangrenosum (EG) is a cutaneous infection most commonly associated with Pseudomonas aeruginosa sepsis. EG generally occurs in immunocompromised hosts, such as patients with severe neutropenia. EG presents as erythematous, hemorrhagic, or necrotic macules or plaques, most commonly in the perineal or gluteal areas, but can occur elsewhere. EG is a dermatologic emergency in immunocompromised patients and should be included in the differential diagnosis when urologists are asked to evaluate perineal lesions. We describe the case of a highly immunocompromised infant with labial EG to highlight the importance of prompt clinical diagnosis and of multidisciplinary medical and surgical management.

Successful treatment of Fusarium solani ecthyma gangrenosum in a patient affected by leukocyte adhesion deficiency type 1 with granulocytes transfusions.

BACKGROUND: Ecthyma gangrenosum (EG) manifests as a skin lesion affecting patients suffering extreme neutropenia and is commonly associated with Pseudomonas aeruginosa in immunocompromised patients. Leukocyte adhesion deficiency I (LAD I) which count among primary immunodeficiency syndromes of the innate immunity, is an autosomal recessive disorder characterized in its severe phenotype by a complete defect in CD18 expression on neutrophils, delayed cord separation, chronic skin ulcers mainly due to recurrent bacterial and fungal infections, leucocytosis with high numbers of circulating neutrophils and an accumulation of abnormally low number of neutrophils at sites of infection. CASE PRESENTATION: We report at our knowledge the first case of a child affected by LAD-1, who experienced during her disease course a multi-bacterial and fungal EG lesion caused by fusarium solani. Despite targeted antibiotics and anti-fungi therapy, the lesion extended for as long as 18 months and only massive granulocytes pockets transfusions in association with G-CSF had the capacity to cure this lesion. CONCLUSION: We propose that granulocytes pockets transfusions will be beneficial to heal EG especially in severely immunocompromised patients.

Ecthyma-gangrenosum-like bullous pemphigoid.

Bullous pemphigoid (BP) is a chronic, autoimmune, subepidermal blistering skin disease with varied clinical presentations. Diagnosis is based on the clinical picture, histopathological findings, and direct and indirect immunofluorescence studies. In unclear cases, ELISA or Western blot analysis helps to establish a definite diagnosis by the detection of immunoglobulin G autoantibodies specific for the hemidesmosomal BP antigens BP230 and BP180. We report 3 cases of BP with an as yet not characterized, distinctive ecthyma-gangrenosum-like presentation. Patients were female, above 80 years of age, physically immobile, and skin lesions showed truncal localization and bacterial colonization. Factors contributing to physical immobility were a high body mass index, psychiatric disease, sedative medication and rheumatic disease. The clinical picture resembled ecthyma gangrenosum but lacked systemic infection with Pseudomonas aeruginosa. Lesional bacteriological studies revealed Staphylococcusaureus and/or P. aeruginosa. Diagnosis proved challenging in all cases. Suspicion has to be high, and repeated diagnostic procedures and additional laboratory studies may be necessary to establish a definitive diagnosis of BP. In summary, we propose this combination of truncal ecthyma-gangrenosum-like lesions with bacterial colonization in the context of older age and immobility as a clinically distinct presentation or variant of BP.

Nonpseudomonal ecthyma gangrenosum.

Ecthyma gangrenosum is a cutaneous infection associated most commonly with pseudomonal sepsis in the patient who is immunocompromised. We describe an 8-month-old girl with acute myelocytic leukemia who developed perineal ecthyma gangrenosum caused by Citrobacter freundii, a gram-negative pathogen that has been rarely associated with cutaneous disease. We also review the literature to categorize the range of pseudomonal and nonpseudomonal pathogens associated with ecthyma gangrenosum.

Severe persistent orf in young goats.

Orf (contagious ecthyma) is a viral disease of small and wild ruminants, humans, and less frequently other species. In sheep and goats, the disease is characterized by the formation of vesiculo-proliferative lesions in the skin of lips and nostril. Here, a form of generalized orf in 16 goat kids from 2 different locations in west Texas is described. The disease was characterized by multifocal, severe, proliferative dermatitis that persisted from about 2 months of age until the goat kids were euthanized 3 months later. All affected goats were Boer or Boer crosses under 1 year of age. The mean immunoglobulin concentration in sera of affected goats was elevated compared with healthy control goats. Severe to moderate lymphadenomegaly of the nodes draining the areas of the skin affected with orf lesions was present in all 16 goat kids. Suppurative arthritis, chronic fibrinous pneumonia, and premature thymic involution were found in 3, 5, and 7 of the goat kids, respectively. The skin lesions of 3 goat kids were infested with larvae of the opportunistic black garbage fly (Ophira sp.). The orf virus was identified in skin lesions by isolation in Marbin-Darby ovine kidney cells, electron microscopy, and amplification of viral DNA by polymerase chain reaction. The orf virus was not detected in peripheral blood or lymph node mononuclear cells of any of the goats. Cross-neutralization experiments showed that an ovine orf virus antiserum raised in sheep was more effective in neutralizing a sheep orf virus isolate than a caprine orf virus isolate. The clinical and epidemiological characteristics of these orf cases may be the result of susceptibility factors within some individuals of the Boer breed of goats.

Ecthyma gangrenosum: considerations in a previously healthy child.

Ecthymagangrenosum 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.

A study of nineteen immunocompromised patients with extensive skin lesions caused by Pseudomonas aeruginosa with and without bacteremia.

Nineteen immunocompromised patients with extensive skin lesions caused by Pseudomonas aeruginosa with or without P. aeruginosa bacteremia were analysed. Patients whose lesions originated in the skin were in the majority (14 patients). Skin lesions were located at the site of entry of bacteria (apocrine areas in 12 patients). Cutaneous lesions were pleomorphic but the typical picture of ecthyma gangrenosum was common in this group of patients. Only 2 of them developed P. aeruginosa septicemia and the prognosis was relatively good (7.5% mortality rate). These observations confirm that ecthyma gangrenosum may be a primary cutaneous disorder not systematically associated with bacteremia.

Bacteremia and ecthyma caused by Streptococcus pyogenes in a patient with acquired immunodeficiency syndrome.

Ecthyma is an ulcerated form of impetigo due to Streptococcus pyogenes, seen primarily in children with poor hygiene. The authors report a homosexual man with acquired immunodeficiency syndrome (AIDS) who developed severe ecthyma and bacteremia caused by S. pyogenes. Opsonizing antibody to the M protein of S. pyogenes is important in immunity to this organism. Patients with AIDS may have defective humoral immunity as well as defective cellular immunity, and such a defect may have rendered this patient abnormally susceptible to severe infection with S. pyogenes.