Fatal cardiovascular disease and cutis laxa following acute febrile neutrophilic dermatosis

doi:10.1016/S0022-3476(83)80529-0

The Journal of Pediatrics

Volume 102, Issue 2, February 1983, Pages 243-248

https://doi.org/10.1016/S0022-3476(83)80529-0 Get rights and content

Acute neutrophilic dermatosis (Sweet syndrome) is a benign self-limited disease in adults. A child with apparent evolution of acute neutrophilic dermatosis to postinflammatory cutis laxa and elastolysis then developed fatal vascular involvement. One other patient with postinflammatory cutis laxa with aortic regurgitation and sudden fatal unrecognized occlusive coronary arterial disease is discussed. If cardiovascular symptoms or signs develop during the course of Sweet syndrome or postinflammatory cutis laxa, a thorough investigation is warranted to rule out potentially fatal coronary arterial disease. Coronary bypass surgery may be the only effective treatment for the severely fibrosed proximal coronary arterial system.

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Cited by (79)

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Citation Excerpt :
Second-line therapies in children include indomethacin, cyclosporine, and dapsone.7 Dapsone is thought to be less effective in MASS50; however, it may be useful in treating evolving cutis laxa, as discussed previously.42,51 The addition of intravenous immunoglobulin (IVIG) to dapsone has been used in immunodeficient children who do not respond well to standard treatments.50
Sweet heart: A case of pregnancy-associated acute febrile neutrophilic dermatosis with myopericarditis
2015, JAAD Case Reports
Citation Excerpt :
Cardiac sequelae are extremely rare.7,8 Cases of acquired cutis laxa leading to cardiac complications have been observed months to years after the initial presentation of Sweet syndrome in the pediatric population.9-11 There is only one previous case report, to our knowledge, of myopericarditis in an adult with Sweet syndrome.12
Pyoderma gangrenosum and neutrophilic aortitis preceding the development of rheumatoid arthritis
2015, Annales de Dermatologie et de Venereologie
Nous rapportons un cas de pyoderma gangrenosum (PG) associé à une aortite neutrophilique compliquée d’une dissection aortique ascendante, et suivi de l’apparition d’une polyarthrite rhumatoïde (PR).
Une femme de 79 ans était hospitalisée fin 2009 pour un PG végétant. Un traitement par corticothérapie générale, puis colchicine et dermocorticoïdes, permettait une cicatrisation complète des lésions. Au cours de l’hospitalisation, la patiente présentait une dissection de l’aorte ascendante, traitée avec succès par une intervention chirurgicale en urgence. L’examen anatomopathologique de la pièce opératoire trouvait une aortite neutrophilique diffuse. Les diagnostics de maladie de Takayasu et de lupus étaient écartés. La tomodensitométrie (TDM) thoracique objectivait un syndrome interstitiel pulmonaire. Il existait une discrète lymphocytose dans le liquide bronchiolo-alvéolaire, sans germe pathogène isolé. Les lésions cutanées récidivaient trois, puis quatre ans plus tard, associées à l’apparition d’une PR. Une aggravation de la pneumopathie était mise en évidence par TDM en 2013, avant d’observer une stabilisation en 2014. Le PG n’a plus récidivé par la suite.
À notre connaissance, aucune autre observation d’aortite neutrophilique associée avec un PG ou une PR n’a été publiée à ce jour. Tandis que dans la littérature émerge le concept de « systémicité » des dermatoses neutrophiliques, la présence d’une localisation cutanée et aortique de la pathologie neutrophilique chez une même patiente conforte cette approche.
We report a case of pyoderma gangrenosum (PG) associated with a complication comprising ascending aortic dissection (neutrophilic aortitis) in a setting of rheumatoid arthritis (RA).
A 79-year-old female patient was hospitalized in late 2009 for vegetating PG. Treatment with general steroids followed by colchicine and topical steroids resulted in complete healing of skin lesions. During hospitalization, the patient presented dissection of the ascending part of the aorta, for which emergency surgery proved effective. Histological examination of the excised tissue revealed diffuse neutrophilic aortitis. Diagnoses of Takayashu's disease and of lupus were ruled out. A chest CT scan showed interstitial lung disease with mild lymphocytosis in the bronchoalveolar fluid, but with no isolated pathogenic organisms. Relapse of skin lesions occurred 3 and 4 years later, associated with the development of RA, and worsening of the interstitial lung disease was noted in a scan carried out it in 2013, following which stabilization was observed in April 2014. There was no recurrence of the PG lesions.
To our knowledge, no other cases involving association of neutrophilic aortitis with PG and RA has been published to date. The literature describes the emergence of the concept of systemic neutrophilic dermatoses, and this notion is reinforced by the presence of a cutaneous and aortic site of the neutrophilic disease in a single patient.
Neutrophilic dermatoses as systemic diseases
2014, Clinics in Dermatology
Neutrophilic dermatoses (ND) are inflammatory skin conditions characterized by a sterile infiltrate of normal polymorphonuclear leukocytes. The main clinical forms of ND include Sweet syndrome, pyoderma gangrenosum, erythema elevatum diutinum, subcorneal pustular dermatosis, and their atypical or transitional forms. ND are often idiopathic, but they may be associated with myeloid hematologic malignancies (Sweet syndrome), inflammatory bowel disease or rheumatoid arthritis (pyoderma gangrenosum), and monoclonal gammopathies (erythema elevatum diutinum, subcorneal pustular dermatosis). The possible infiltration of internal organs with neutrophils during the setting of ND underlies the concept of a neutrophilic systemic disease. ND may be seen as a polygenic autoinflammatory syndrome due to their frequent association with other autoinflammatory disorders (monogenic or polygenic) and the recent published efficacy of interleukin-1 blocking therapies in their management.
Hurwitz Clinical Pediatric Dermatology, Fouth Edition
2011, Hurwitz Clinical Pediatric Dermatology, Fouth Edition

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^*: Supported by Grant HL 07605 from the National Institutes ofHealth, National Heart, Lung and Blood Institute.

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Original articleFatal cardiovascular disease and cutis laxa following acute febrile neutrophilic dermatosis*

Am J Cardiol

J Pediatr

Hereditary disorders of fibrous tissue

Heart disease in infants, children and adolescents

Occlusive fibroelastosis of coronary arteries in the newborn

Circulation

Are infantile periarteritis nodosa with coronary artery involvement and fatal mucocutaneous lymph node syndrome the same? Comparison of 20 patientsfrom North America with patients from Hawaii and Japan

Pediatrics

Original article
Fatal cardiovascular disease and cutis laxa following acute febrile neutrophilic dermatosis*