Search tips
Search criteria 


Logo of jaadcrGuide for AuthorsAbout this journalExplore this journalJAAD Case Reports
JAAD Case Rep. 2017 March; 3(2): 124–126.
Published online 2017 March 12. doi:  10.1016/j.jdcr.2016.12.006
PMCID: PMC5348594

A case of primarily facial pyoderma gangrenosum associated with Takayasu arteritis

Ken Okamura, MD, PhD,a,* Takayuki Konno, MD, PhD,a Kosuke Onami, MD,a Mariko Nikaido, MD,a Naoko Okazaki, MD,a Yuko Abe, MD, PhD,a Masahiro Hayashi, MD, PhD,a Yoriko Yaguchi, MD,a Hiroko Sato, MD,b Tsuneo Konta, MD, PhD,b and Tamio Suzuki, MD, PhDa


Pyoderma gangrenosum (PG) is a type of neutrophilic dermatosis that shows noninfectious ulcers characterized by neutrophil infiltration of the skin.1 PG is frequently associated with systemic diseases such as inflammatory bowel disease, rheumatoid arthritis, and hematologic disorders.2 In Japan, PG is also a well-known complication of Takayasu arteritis (TA), yet only a few anecdotal reports are recorded worldwide.3, 4 Typically, patients with PG show skin lesions on the lower extremities; however, the PG lesions associated with TA are known to be more widespread than those without TA.3 Ujiie et al3 reported that 42.9% of the PG patients with TA (15 of 35) had lesions on the face and neck. We report a case of PG associated with TA that primarily affected the face and had been misdiagnosed as a facial skin infection.

Case report

The patient was a 37-year-old Japanese man who had TA diagnosed 4 years before the skin manifestation. When he was 33 years old, he presented with left cervical pain followed by dizziness, a fainting episode, and fever that failed to respond to antimicrobial agents. Fluorodeoxyglucose-positron emission tomography/computed tomography and computed tomography imaging confirmed TA. Subsequently, the patient was prescribed systemic corticosteroids and immunosuppressive agents. Four years later he developed multiple pustules and subcutaneous abscesses within an erythematous plaque in the left buccal region. Facial skin infection was diagnosed at an outlying facility, and he was prescribed antimicrobial agents for approximately 6 months. However, his skin lesion increased and spread to the other cheek, trunk, and lower legs, after which he visited our institution. The major symptoms associated with TA were well controlled with oral betamethasone (1 mg/d) and tacrolimus (3 mg/d). Irregular-shaped reddish scarring plaques with some pustules and subcutaneous abscesses were present on each of his cheeks and on the lower jaw (Fig 1, A). In addition, a crusted ulcerative plaque with erythema was observed on the extensor surface of his right lower leg (Fig 1, B). Histopathologic examination of a biopsy section taken from the facial lesion found mild acanthosis, dilated capillaries in the papillary dermis, partial invagination of the epithelial component, and dense infiltrates of inflammatory cells in the dermis, most of which were neutrophils (Fig 2). Multiple bacterial cultures from facial pustules and abscesses showed no evidence of bacterial infection. Serologic tests were negative for rheumatoid factor and showed absence of a monoclonal gammopathy. Complete colonoscopy found no evidence of inflammatory bowel disease. We diagnosed him with PG. After increasing the dose of systemic corticosteroid dose to 50 mg/d of prednisolone the purulent inflammation of the facial plaque rapidly resolved, and his leg ulcer epithelized within a month. However, rose-pink scars with telangiectasia persisted on his face (Fig 3). The prednisolone dose was gradually decreased while ensuring that it did not cause flare up of the PG.

Fig 1
Clinical findings of the PG patient associated with TA. A, Irregular-shaped reddish scarring plaques with some pustules or subcutaneous abscess on the left cheek and lower jaw. B, Crusted ulcerative lesion with erythema on the extensor surfaces of the ...
Fig 2
Histopathologic findings. Magnification of the rectangle in the low-power field image (inset) is shown in the center. A skin biopsy from the facial lesion found mild acanthosis, dilation of capillaries in the papillary dermis, partial invagination of ...
Fig 3
A clinical image after treatment with high-dose corticosteroids. Rose-pink scar with telangiectasia persisted on his face.


The case report presented here describes a male patient who had TA followed by PG, which had been misdiagnosed as a skin infection for 6 months.

TA is a rare, systemic vasculitis that predominantly involves the aorta and its branches; cutaneous lesions occur in up to 28% of patients.5 Although the pathogenesis of TA remains unknown, upregulated proinflammatory cytokines, such as interleukin (IL)-6, IL-8, IL-18, IL-23, and tumor necrosis factor-α in patients with TA has been shown.6 A very recent genomewide association study also found some susceptible genes associated with immunoregulatory pathways, including IL6.7 On the other hand, significant upregulation of IL-6 and IL-8 has been reported in PG.8 Overexpression of IL-8, IL-17, IL-23, and tumor necrosis factor-α has also been found in PG lesions.2

Considering the previously reported cytokine profiles in patients with TA and PG, the co-existence of the 2 diseases seems to be reasonable. Currently, the patient has facial scars. Early diagnosis and treatment is prudent to prevent this untoward complication.


Funding sources: None.

Conflicts of interest: None declared.


1. Steele R.B., Nugent W.H., Braswell S.F., Frisch S., Ferrell J., Ortega-Loayza A.G. Pyoderma gangrenosum and pregnancy: an example of abnormal inflammation and challenging treatment. Br J Dermatol. 2016;174:77–87. [PubMed]
2. Braswell S.F., Kostopoulos T.C., Ortega-Loayza A.G. Pathophysiology of pyoderma gangrenosum (PG): an updated review. J Am Acad Dermatol. 2015;73:691–698. [PubMed]
3. Ujiie H., Sawamura D., Yokota K., Nishie W., Shichinohe R., Shimizu H. Pyoderma gangrenosum associated with Takayasu's arteritis. Clin Exp Dermatol. 2004;29:357–359. [PubMed]
4. Ghosn S., Malek J., Shbaklo Z., Matta M., Uthman I. Takayasu disease presenting as malignant pyoderma gangrenosum in a child with relapsing polychondritis. J Am Acad Dermatol. 2008;59:S84–S87. [PubMed]
5. Pascual-Lopez M., Hernandez-Nunez A., Aragues-Montanes M., Dauden E., Fraga J., Garcia-Diez A. Takayasu's disease with cutaneous involvement. Dermatology. 2004;208:10–15. [PubMed]
6. Pan L.L., Du J., Gao N. IL-9-producing Th9 cells may participate in pathogenesis of Takayasu's arteritis. Clin Rheumatol. 2016;35:3031–3036. [PubMed]
7. Renauer P.A., Saruhan-Direskeneli G., Coit P. Identification of Susceptibility Loci in IL6, RPS9/LILRB3, and an Intergenic Locus on Chromosome 21q22 in Takayasu Arteritis in a Genome-Wide Association Study. Arthritis Rheumatol. 2015;67:1361–1368. [PubMed]
8. Kawakami T., Yamazaki M., Soma Y. Reduction of interleukin-6, interleukin-8, and anti-phosphatidylserine-prothrombin complex antibody by granulocyte and monocyte adsorption apheresis in a patient with pyoderma gangrenosum and ulcerative colitis. Am J Gastroenterol. 2009;104:2363–2364. [PubMed]

Articles from JAAD Case Reports are provided here courtesy of Elsevier