Author(s) : Ken Futaki, Mayumi Komine, Satomi Hosoda, Miho Hirashima, Hideto Yokokura, Tomoko Yamada, Satoru Murata, Yasushi Matsuyama, Takao Nagashima, Hiroyuki Nara, Seiji Minota, Mamitaro Ohtsuki , Department of Dermatology, Jikei University School of Medicine, 3-19-18, Nishi-shinbashi, Minato-ku, Tokyo, Japan, Department of Dermatology, Jichi Medical University, 3311-1, Yakushiji, Shimotsuke-city, Tochigi, Japan, Department of Rheumatology, Jichi Medical University, 3311-1, Yakushiji, Shimotsuke-city, Tochigi, Japan. |
ARTICLE
Auteur(s) : Ken Futaki1, Mayumi Komine2,
Satomi Hosoda2, Miho Hirashima2, Hideto
Yokokura2, Tomoko Yamada2, Satoru
Murata2, Yasushi Matsuyama3, Takao
Nagashima3, Hiroyuki Nara3, Seiji
Minota3, Mamitaro Ohtsuki2
1Department of Dermatology, Jikei University
School of Medicine, 3-19-18, Nishi-shinbashi, Minato-ku,
Tokyo, Japan
2Department of Dermatology, Jichi Medical
University, 3311-1, Yakushiji, Shimotsuke-city, Tochigi, Japan
3Department of Rheumatology, Jichi Medical
University, 3311-1, Yakushiji, Shimotsuke-city, Tochigi, Japan
A 29-year-old woman was referred to the Dermatology department
in March 2007, by the internists for a plaque on the left side
of her neck (figure
1A). She had been suffering from an intermittent fever of
38 °C with a sore throat and cough since 2006. Pustules appeared on
the face and forearm, which spontaneously disappeared within a
month. She had noticed a red oval plaque with pustules on the left
side of her neck in February 2007, following an episode of
fever. Since the fever was resistant to several antibiotics, she
was admitted to our hospital. Blood cell counts, serum complement
level, and renal function were within normal limits, with slight
liver dysfunction and elevated CRP level and erythrocyte
sedimentation rate (ESR). Auto-antibodies, including anti-nuclear
antibody, anti-SS-A/SS-B antibody, and anti-neutrophil cytoplasmic
antibodies, were negative, ruling out autoimmune diseases such as
systemic lupus erythematosus. Examinations by upper
gastrointestinal endoscopy, colon fiber, computed tomography (CT),
Galium scintigraphy, and ultrasonography detected no abnormal
findings. Ophthalmological examination revealed no abnormal
findings, including uveitis.
A biopsy was taken from the lesion on her neck.
Histopathological examination demonstrated a dense infiltration of
neutrophils intermingled with lymphocytes, plasma cells, and
multinucleated histiocytes throughout the dermis (figure 1B). Non-bacterial
pustules appeared on the biopsy wound and the ulceration recurred.
Based on the above clinical and histopathological findings, the
diagnosis of PG, superficial variant, was made.
In April, the fever exacerbated, and painful erythematous
nodules and pustules appeared on the lower legs. A tuber was
noted on the left pharyngeal tonsil, suspected to be an oral lesion
of PG. Human leukocyte antigen (HLA) B52 was positive. We closely
examined CT images for the aortic lesions seen in TA, and a small
hypertrophic lesion on the right pulmonary artery wall was noted.
MRI revealed thickened arterial walls and MR angiography showed
irregular vessel walls with stenosis. Blood-flow scintigraphy of
the lung revealed a reduction of circulation in the right lung
(figure 1C).
These radiological findings, without evidence of other inflammatory
disorders such as collagen or Behcet’s disease, with fever and
elevated ESR in a young woman, and positive HLA B52, made us
diagnose this case as unusual TA, with the lesion limited only to
pulmonary artery. Predonisolone 30 mg/day was started, and the
fever and skin lesions immediately started to improve.
Discussion
TA, a rare disorder more frequently seen in Asian population than
in other races, is an inflammatory arteriopathy predominantly
involving the aorta and its main branches [1]. Of the 1990 criteria
for the classification of TA by the American College of
Rheumatology [2], only two were present in our case, making the
diagnosis difficult, while fulfilling the criteria of active TA
suggested by Kerr GS et al. [3]. Various skin lesions associated
with TA have been reported, including erythematous nodules with
necrotizing or granulomatous vasculitis, and PG [4]. PG is one of
the neutrophilic dermatoses frequently related to ulcerative
colitis, Crohn’s disease, rheumatoid arthritis, and, rarely, with
TA [5]. It mainly affects the lower extremities of middle-aged
patients, forming recalcitrant ulcers. Several cases of TA with PG
have been reported without any relation to clinical severity. Our
case is unique in that TA without typical symptoms was diagnosed
due to the diagnosis of PG. PG with TA shows certain characteristic
findings compared to those of PG without TA, i.e.; relatively young
onset in the 20’s, predominantly female patients, and skin lesions
in more disseminated areas, including the upper limbs, head and
neck [6], which were all relevant in our case. We would like to
emphasize that TA should be considered in a young female with fever
of unknown origin accompanied by skin lesions indicative of PG.
Acknowledgements
Conflict of interest: none. Financial support: none.
References
1 Sunderkötter C, Sindrilaru A. Clinical classification
of vasculitis. Eur J Dermatol 2006; 16: 114-24.
2 Arend WP, Michel BA, Bloch DA, Hunder GG,
Calabrese LH, Edworthy SM, et al. The American
College of Rheumatology 1990 criteria for the classification of
Takayasu arteritis. Arthritis Rheum 1990; 33: 1129-34.
3 Kerr GS, Hallahan CW, Giordano J,
Leavitt RY, Fauci AS, Rottem M, Hoffman GS.
Takayasu arteritis. Ann Intern Med 1994; 120: 919-29.
4 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: 10-5.
5 Crowson NA, Mihm Jr. MC, Magro C. Pyoderma
gangrenosum: a review. J Cutan Pathol 2003; 30: 97-107.
6 Ujiie H, Sawamura D, Yokota K, Nishie W,
Shichinohe R, Shimizu H. Pyoderma gangrenosum associated
with Takayasu’s arteritis. Clin Exp Dermatol 2004; 29: 357-9.
|
Printable version
Version PDF