Fatal Orbital Pyoderma Gangrenosum with Central Nervous System Involvement

  • Sofia Lopes Serviço de Dermatologia e Venereologia, Centro Hospitalar São João, Porto, Portugal http://orcid.org/0000-0003-2263-3709
  • Júlia Vide Serviço de Dermatologia e Venereologia, Centro Hospitalar São João, Porto, Portugal
  • Sofia Magina Serviço de Dermatologia e Venereologia, Centro Hospitalar São João, Porto, Portugal
  • Ana Paula Cunha Serviço de Dermatologia e Venereologia, Centro Hospitalar São João, Porto, Portugal
  • Filomena Azevedo Serviço de Dermatologia e Venereologia, Centro Hospitalar São João, Porto, Portugal
Keywords: Central Nervous System Diseases, Orbital Diseases, Pyoderma Gangrenosum

Abstract

Pyoderma gangrenosum is a chronic inflammatory disease characterized by the development of a painful deep ulcer with undermined borders. Head and neck are rarely affected regions of the body and also usually associated with a worse prognosis. Corticosteroids are the mainstay of treatment although available options are not specific nor completely effective in pyoderma gangrenosum. We report the case of a 46-year-old patient with an aggressive orbital pyoderma gangrenosum with progressive extension to the central nervous system and insufficient response to treatment, ultimately leading to patient’s death.

Downloads

Download data is not yet available.

References

Ruocco E, Sangiuliano S, Gravina AG, Miranda A, Nicoletti

G. Pyoderma gangrenosum: an updated review.

J Eur Acad Dermatol Venereol. 2009; 23:1008-17. doi:10.1111/j.1468-3083.2009.03199.x.

Bryan CS. Fatal pyoderma gangrenosum with pathergy

after coronary artery bypass grafting. Tex Heart Inst J.

; 39:894-7.

Partridge ACR, Bai JW, Rosen CF, Walsh SR, Gulliver WP,

Fleming P. Effectiveness of systemic treatments for pyoderma

gangrenosum: a systematic review of observational

studies and clinical trials. Br J Dermatol. 2018;

:290-5. doi: 10.1111/bjd.16485.

Braswell SF, Kostopoulos TC, Ortega-Loayza AG. Pathophysiology

of pyoderma gangrenosum (PG): an updated

review. J Am Acad Dermatol. 2015; 73:691-8. doi:

1016/j.jaad.2015.06.021.

Mantovani L, Zauli S, Sarno O, Querzoli P, Corazza M,

Virgili A. Treatment of a relapsing facial pyoderma gangrenosum

(malignant pyoderma). Int J Dermatol. 2013;

:753-6. doi: 10.1111/j.1365-4632.2012.05755.x.

Leiphart PA, Lam CC, Foulke GT. Suppression of pathergy

in pyoderma gangrenosum with infliximab allowing

for successful tendon debridement. JAAD Case Rep.

; 4:98-100. doi: 10.1016/j.jdcr.2017.08.009.

Huang B, Melmed GY, Shih DQ. Facial ulceration in a

patient with Crohn's disease. Gastroenterology. 2012;

:1071, 1258. doi: 10.1053/j.gastro.2011.09.032.

Lana MA, Moreira PR, Neves LB. Wall-eyed bilateral

internuclear ophthalmoplegia (Webino syndrome) and

myelopathy in pyoderma gangrenosum. Arq Neuropsiquiatr.

;48:497-501.

Chanson P, Timsit J, Kujas M, Violante A, Guillausseau

PJ, Derome PJ, et al. Pituitary granuloma and pyoderma

gangrenosum. J Endocrinol Invest. 1990;13:677-81.

Ahn C, Negus D, Huang W. Pyoderma gangrenosum:

a review of pathogenesis and treatment. Expert

Rev Clin Immunol. 2018; 14:225-33. doi:

1080/1744666X.2018.1438269.

Ambooken B, Khader A, Muhammed K, Rajan U, Snigdha

O. Malignant pyoderma gangrenosum eroding the

parotid gland successfully treated with dexamethasone

pulse therapy. Int J Dermatol. 2014; 53:1536-8. doi:

1111/ijd.12519.

Sehgal R, Resnick JM, Al-Hilli A, Mehta N, Conway T,

Stratman EJ. Nasal septal and mucosal disease associated

with pyoderma gangrenosum in a cocaine

user. JAAD Case Rep. 2017; 3:284-7. doi: 10.1016/j.

jdcr.2017.05.004.

Maverakis E, Ma C, Shinkai K, Fiorentino D, Callen JP,

Wollina U, et al. Diagnostic Criteria of Ulcerative Pyoderma

Gangrenosum: A Delphi Consensus of International

Experts. JAMA Dermatol. 2018;154:461-6. doi:

1001/jamadermatol.2017.5980.

Published
2018-12-27
How to Cite
Lopes, S., Vide, J., Magina, S., Cunha, A. P., & Azevedo, F. (2018). Fatal Orbital Pyoderma Gangrenosum with Central Nervous System Involvement. Journal of the Portuguese Society of Dermatology and Venereology, 76(4), 435-438. https://doi.org/10.29021/spdv.76.4.952
Section
Case Reports