Clinical Features of Neutrophilic Dermatosis Variants Resembling Necrotizing Fasciitis

Isabelle M. Sanchez, Stefan Lowenstein, Kelly A. Johnson, Jennifer Babik, Carter Haag, Jesse J. Keller, Alex Ortega Loayza, Jarish Cohen, Timothy H. McCalmont, Addison M. Demer, Matthew D. Mansh, Sara A. Hylwa, Jing Liu, Kanade Shinkai

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

Importance: Pyoderma gangrenosum and necrotizing Sweet syndrome are diagnostically challenging variants of neutrophilic dermatosis that can clinically mimic the cutaneous and systemic features of necrotizing fasciitis. Improved characterization of these rare variants is needed, as improper diagnosis may lead to inappropriate or delayed treatment and the potential for morbidity. Objective: To determine the characteristics of necrotizing neutrophilic dermatosis to improve diagnostic accuracy and distinguish from infection. Design, Setting, and Participants: A case series of patients with necrotizing neutrophilic dermatosis treated at 3 academic hospitals (University of California San Francisco, Oregon Health and Science University, and University of Minnesota) from January 1, 2015, to December 31, 2017, was performed along with a literature review of related articles published between January 1, 1980, and December 31, 2017. Data were obtained from medical records as well as Medline and Embase databases. All patients had signs resembling necrotizing infection and had a final diagnosis of pyoderma gangrenosum with systemic features or necrotizing Sweet syndrome. Patients were excluded if a diagnosis other than neutrophilic dermatosis was made, if key clinical information was missing, and if reported in a non-English language. Main Outcomes and Measures: Description of key characteristics of necrotizing neutrophilic dermatosis. Results: Overall, 54 patients with necrotizing neutrophilic dermatosis were included, of which 40 had pyoderma gangrenosum with systemic features and 14 had necrotizing Sweet syndrome. Of the 54 patients, 29 (54%) were male and 25 (46%) were female, with a mean (SD) age of 51 (19) years. Skin lesions commonly occurred on the lower (19 [35%]) and upper (13 [24%]) extremities and developed after a surgical procedure (22 [41%]) or skin trauma (10 [19%]). Shock was reported in 14 patients (26%), and leukemoid reaction was seen in 15 patients (28%). Of the patients with necrotizing neutrophilic dermatosis, 51 (94%) were initially misdiagnosed as necrotizing fasciitis and subsequently received inappropriate treatment. Debridement was performed in 42 patients (78%), with a mean (SD) of 2 (2 [range, 1-12]) debridements per patient. Four amputations (7%) were performed. Forty-nine patients (91%) received antibiotics when necrotizing neutrophilic dermatosis was misdiagnosed as an infection, and 50 patients (93%) received systemic corticosteroids; all patients responded to immunosuppressants. Conclusions and Relevance: A complex spectrum of clinical findings of pyoderma gangrenosum and Sweet syndrome with prominent systemic inflammation exists that defines a new subset of neutrophilic dermatoses, termed necrotizing neutrophilic dermatoses; recognizing the difference between this variant and severe infection may prevent unnecessary surgical procedures and prolonged disease morbidity associated with a misdiagnosis and may expedite appropriate medical management.

Original languageEnglish (US)
Pages (from-to)79-84
Number of pages6
JournalJAMA Dermatology
Volume155
Issue number1
DOIs
StatePublished - Jan 1 2019

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Necrotizing Fasciitis
Skin Diseases
Sweet Syndrome
Pyoderma Gangrenosum
Diagnostic Errors
Debridement
Infection
Skin
Leukemoid Reaction
Morbidity
Unnecessary Procedures
San Francisco
Immunosuppressive Agents
Amputation
Medical Records

ASJC Scopus subject areas

  • Dermatology

Cite this

Sanchez, I. M., Lowenstein, S., Johnson, K. A., Babik, J., Haag, C., Keller, J. J., ... Shinkai, K. (2019). Clinical Features of Neutrophilic Dermatosis Variants Resembling Necrotizing Fasciitis. JAMA Dermatology, 155(1), 79-84. https://doi.org/10.1001/jamadermatol.2018.3890

Clinical Features of Neutrophilic Dermatosis Variants Resembling Necrotizing Fasciitis. / Sanchez, Isabelle M.; Lowenstein, Stefan; Johnson, Kelly A.; Babik, Jennifer; Haag, Carter; Keller, Jesse J.; Ortega Loayza, Alex; Cohen, Jarish; McCalmont, Timothy H.; Demer, Addison M.; Mansh, Matthew D.; Hylwa, Sara A.; Liu, Jing; Shinkai, Kanade.

In: JAMA Dermatology, Vol. 155, No. 1, 01.01.2019, p. 79-84.

Research output: Contribution to journalArticle

Sanchez, IM, Lowenstein, S, Johnson, KA, Babik, J, Haag, C, Keller, JJ, Ortega Loayza, A, Cohen, J, McCalmont, TH, Demer, AM, Mansh, MD, Hylwa, SA, Liu, J & Shinkai, K 2019, 'Clinical Features of Neutrophilic Dermatosis Variants Resembling Necrotizing Fasciitis', JAMA Dermatology, vol. 155, no. 1, pp. 79-84. https://doi.org/10.1001/jamadermatol.2018.3890
Sanchez IM, Lowenstein S, Johnson KA, Babik J, Haag C, Keller JJ et al. Clinical Features of Neutrophilic Dermatosis Variants Resembling Necrotizing Fasciitis. JAMA Dermatology. 2019 Jan 1;155(1):79-84. https://doi.org/10.1001/jamadermatol.2018.3890
Sanchez, Isabelle M. ; Lowenstein, Stefan ; Johnson, Kelly A. ; Babik, Jennifer ; Haag, Carter ; Keller, Jesse J. ; Ortega Loayza, Alex ; Cohen, Jarish ; McCalmont, Timothy H. ; Demer, Addison M. ; Mansh, Matthew D. ; Hylwa, Sara A. ; Liu, Jing ; Shinkai, Kanade. / Clinical Features of Neutrophilic Dermatosis Variants Resembling Necrotizing Fasciitis. In: JAMA Dermatology. 2019 ; Vol. 155, No. 1. pp. 79-84.
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abstract = "Importance: Pyoderma gangrenosum and necrotizing Sweet syndrome are diagnostically challenging variants of neutrophilic dermatosis that can clinically mimic the cutaneous and systemic features of necrotizing fasciitis. Improved characterization of these rare variants is needed, as improper diagnosis may lead to inappropriate or delayed treatment and the potential for morbidity. Objective: To determine the characteristics of necrotizing neutrophilic dermatosis to improve diagnostic accuracy and distinguish from infection. Design, Setting, and Participants: A case series of patients with necrotizing neutrophilic dermatosis treated at 3 academic hospitals (University of California San Francisco, Oregon Health and Science University, and University of Minnesota) from January 1, 2015, to December 31, 2017, was performed along with a literature review of related articles published between January 1, 1980, and December 31, 2017. Data were obtained from medical records as well as Medline and Embase databases. All patients had signs resembling necrotizing infection and had a final diagnosis of pyoderma gangrenosum with systemic features or necrotizing Sweet syndrome. Patients were excluded if a diagnosis other than neutrophilic dermatosis was made, if key clinical information was missing, and if reported in a non-English language. Main Outcomes and Measures: Description of key characteristics of necrotizing neutrophilic dermatosis. Results: Overall, 54 patients with necrotizing neutrophilic dermatosis were included, of which 40 had pyoderma gangrenosum with systemic features and 14 had necrotizing Sweet syndrome. Of the 54 patients, 29 (54{\%}) were male and 25 (46{\%}) were female, with a mean (SD) age of 51 (19) years. Skin lesions commonly occurred on the lower (19 [35{\%}]) and upper (13 [24{\%}]) extremities and developed after a surgical procedure (22 [41{\%}]) or skin trauma (10 [19{\%}]). Shock was reported in 14 patients (26{\%}), and leukemoid reaction was seen in 15 patients (28{\%}). Of the patients with necrotizing neutrophilic dermatosis, 51 (94{\%}) were initially misdiagnosed as necrotizing fasciitis and subsequently received inappropriate treatment. Debridement was performed in 42 patients (78{\%}), with a mean (SD) of 2 (2 [range, 1-12]) debridements per patient. Four amputations (7{\%}) were performed. Forty-nine patients (91{\%}) received antibiotics when necrotizing neutrophilic dermatosis was misdiagnosed as an infection, and 50 patients (93{\%}) received systemic corticosteroids; all patients responded to immunosuppressants. Conclusions and Relevance: A complex spectrum of clinical findings of pyoderma gangrenosum and Sweet syndrome with prominent systemic inflammation exists that defines a new subset of neutrophilic dermatoses, termed necrotizing neutrophilic dermatoses; recognizing the difference between this variant and severe infection may prevent unnecessary surgical procedures and prolonged disease morbidity associated with a misdiagnosis and may expedite appropriate medical management.",
author = "Sanchez, {Isabelle M.} and Stefan Lowenstein and Johnson, {Kelly A.} and Jennifer Babik and Carter Haag and Keller, {Jesse J.} and {Ortega Loayza}, Alex and Jarish Cohen and McCalmont, {Timothy H.} and Demer, {Addison M.} and Mansh, {Matthew D.} and Hylwa, {Sara A.} and Jing Liu and Kanade Shinkai",
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T1 - Clinical Features of Neutrophilic Dermatosis Variants Resembling Necrotizing Fasciitis

AU - Sanchez, Isabelle M.

AU - Lowenstein, Stefan

AU - Johnson, Kelly A.

AU - Babik, Jennifer

AU - Haag, Carter

AU - Keller, Jesse J.

AU - Ortega Loayza, Alex

AU - Cohen, Jarish

AU - McCalmont, Timothy H.

AU - Demer, Addison M.

AU - Mansh, Matthew D.

AU - Hylwa, Sara A.

AU - Liu, Jing

AU - Shinkai, Kanade

PY - 2019/1/1

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N2 - Importance: Pyoderma gangrenosum and necrotizing Sweet syndrome are diagnostically challenging variants of neutrophilic dermatosis that can clinically mimic the cutaneous and systemic features of necrotizing fasciitis. Improved characterization of these rare variants is needed, as improper diagnosis may lead to inappropriate or delayed treatment and the potential for morbidity. Objective: To determine the characteristics of necrotizing neutrophilic dermatosis to improve diagnostic accuracy and distinguish from infection. Design, Setting, and Participants: A case series of patients with necrotizing neutrophilic dermatosis treated at 3 academic hospitals (University of California San Francisco, Oregon Health and Science University, and University of Minnesota) from January 1, 2015, to December 31, 2017, was performed along with a literature review of related articles published between January 1, 1980, and December 31, 2017. Data were obtained from medical records as well as Medline and Embase databases. All patients had signs resembling necrotizing infection and had a final diagnosis of pyoderma gangrenosum with systemic features or necrotizing Sweet syndrome. Patients were excluded if a diagnosis other than neutrophilic dermatosis was made, if key clinical information was missing, and if reported in a non-English language. Main Outcomes and Measures: Description of key characteristics of necrotizing neutrophilic dermatosis. Results: Overall, 54 patients with necrotizing neutrophilic dermatosis were included, of which 40 had pyoderma gangrenosum with systemic features and 14 had necrotizing Sweet syndrome. Of the 54 patients, 29 (54%) were male and 25 (46%) were female, with a mean (SD) age of 51 (19) years. Skin lesions commonly occurred on the lower (19 [35%]) and upper (13 [24%]) extremities and developed after a surgical procedure (22 [41%]) or skin trauma (10 [19%]). Shock was reported in 14 patients (26%), and leukemoid reaction was seen in 15 patients (28%). Of the patients with necrotizing neutrophilic dermatosis, 51 (94%) were initially misdiagnosed as necrotizing fasciitis and subsequently received inappropriate treatment. Debridement was performed in 42 patients (78%), with a mean (SD) of 2 (2 [range, 1-12]) debridements per patient. Four amputations (7%) were performed. Forty-nine patients (91%) received antibiotics when necrotizing neutrophilic dermatosis was misdiagnosed as an infection, and 50 patients (93%) received systemic corticosteroids; all patients responded to immunosuppressants. Conclusions and Relevance: A complex spectrum of clinical findings of pyoderma gangrenosum and Sweet syndrome with prominent systemic inflammation exists that defines a new subset of neutrophilic dermatoses, termed necrotizing neutrophilic dermatoses; recognizing the difference between this variant and severe infection may prevent unnecessary surgical procedures and prolonged disease morbidity associated with a misdiagnosis and may expedite appropriate medical management.

AB - Importance: Pyoderma gangrenosum and necrotizing Sweet syndrome are diagnostically challenging variants of neutrophilic dermatosis that can clinically mimic the cutaneous and systemic features of necrotizing fasciitis. Improved characterization of these rare variants is needed, as improper diagnosis may lead to inappropriate or delayed treatment and the potential for morbidity. Objective: To determine the characteristics of necrotizing neutrophilic dermatosis to improve diagnostic accuracy and distinguish from infection. Design, Setting, and Participants: A case series of patients with necrotizing neutrophilic dermatosis treated at 3 academic hospitals (University of California San Francisco, Oregon Health and Science University, and University of Minnesota) from January 1, 2015, to December 31, 2017, was performed along with a literature review of related articles published between January 1, 1980, and December 31, 2017. Data were obtained from medical records as well as Medline and Embase databases. All patients had signs resembling necrotizing infection and had a final diagnosis of pyoderma gangrenosum with systemic features or necrotizing Sweet syndrome. Patients were excluded if a diagnosis other than neutrophilic dermatosis was made, if key clinical information was missing, and if reported in a non-English language. Main Outcomes and Measures: Description of key characteristics of necrotizing neutrophilic dermatosis. Results: Overall, 54 patients with necrotizing neutrophilic dermatosis were included, of which 40 had pyoderma gangrenosum with systemic features and 14 had necrotizing Sweet syndrome. Of the 54 patients, 29 (54%) were male and 25 (46%) were female, with a mean (SD) age of 51 (19) years. Skin lesions commonly occurred on the lower (19 [35%]) and upper (13 [24%]) extremities and developed after a surgical procedure (22 [41%]) or skin trauma (10 [19%]). Shock was reported in 14 patients (26%), and leukemoid reaction was seen in 15 patients (28%). Of the patients with necrotizing neutrophilic dermatosis, 51 (94%) were initially misdiagnosed as necrotizing fasciitis and subsequently received inappropriate treatment. Debridement was performed in 42 patients (78%), with a mean (SD) of 2 (2 [range, 1-12]) debridements per patient. Four amputations (7%) were performed. Forty-nine patients (91%) received antibiotics when necrotizing neutrophilic dermatosis was misdiagnosed as an infection, and 50 patients (93%) received systemic corticosteroids; all patients responded to immunosuppressants. Conclusions and Relevance: A complex spectrum of clinical findings of pyoderma gangrenosum and Sweet syndrome with prominent systemic inflammation exists that defines a new subset of neutrophilic dermatoses, termed necrotizing neutrophilic dermatoses; recognizing the difference between this variant and severe infection may prevent unnecessary surgical procedures and prolonged disease morbidity associated with a misdiagnosis and may expedite appropriate medical management.

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