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Kaohsiung Journal of Medical Sciences (2013) xx, 1e3

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LETTER TO THE EDITOR

Pyoderma gangrenosum: Intractable leg ulcersin Sjogren’s syndrome*

Dear Editor,

I am writing with regard to a patient with pyoderma gan-grenosum; a rare skin disease. This 52-year-old woman hadmultiple wounds with poor healing over her right lower legand ankle for 3 years after a traffic accident. The woundswere initially small, abrasive, and shallow, and subse-quently progressed to become a deep ulcer with somenecrotic tissue (Fig. 1A and B). She did not have diabetes,hypertension, or hyperlipidemia. A blood test revealed aplatelet count of 40,000/mL, white blood cell count of4300/mL, hemoglobin level of 10.5 g/dL, and C-reactiveprotein level of 1.06 mg/L. A bone marrow study showedreactive marrow to peripheral destruction, and an immu-nological study revealed that she was negative for rheu-matoid factor, positive for Ro, La, and double-stranded DNAantibodies, and had an antinuclear antibody ratio of1:1280. The levels of C3 and C4 were modestly lower at70 mg/dL and 14 mg/dL, respectively. She also complainedof dry eyes and mouth. A subsequent Schirmer’s test waspositive, and salivary scintigraphy showed delayed uptake,suggesting moderate sialoadenitis over both parotid glandsand the right submandibular gland. Based on the abovefindings, Sjogren’s syndrome was diagnosed.

A skin biopsy revealed inflammatory cells with neutro-philic and lymphoplasmacytic infiltration, however, therewas no evidence of thrombosis or vasculitis (Fig. 1C and D).These features were compatible with pyodermagangrenosum.

Pyoderma gangrenosum is often associated with under-lying diseases and is a rare ulcerative skin disease [1e3]. Itis defined as an inflammatory, reactive, noninfective, non-neoplastic skin disease, and is associated with anti-phospholipid syndrome, systemic lupus erythematous,

* Conflicts of interest: All authors declare no conflicts ofinterest.

Please cite this article in press as: Tsai Y-H, et al., Pyoderma gangrJournal of Medical Sciences (2013), http://dx.doi.org/10.1016/j.kjms

1607-551X/$36 Copyright ª 2013, Kaohsiung Medical University. Publishhttp://dx.doi.org/10.1016/j.kjms.2013.11.005

rheumatoid arthritis, and inflammatory bowel disease [2].However, Sjogren’s syndrome as the underlying morbidityhas rarely been reported in the literature [4].

There are several variants of pyoderma gangrenosumincluding ulcerative, pustular, bullous, and vegetativetypes, of which the ulcerative type is the most common [1].The skin biopsy of this patient demonstrated neutrophil andlymphoplasma cell infiltration with necrotic surroundings,compatible with pyoderma gangrenosum.

The blood, immunological, and imaging studies showedthat the patient had primary Sjogren’s syndrome. Systemiclupus erythematous and Sjogren’s syndrome have severaloverlapping features in the early stage of the diseasecourse. However, our patient only had three features oflupus, namely antinuclear antibody, double-stranded DNAantibody, and thrombocytopenia, which did not fulfill thecriteria for systemic lupus erythematous.

The cause of the intractable leg wounds was importantin our patient. According to her C-reactive protein level,white cell count, and clinical course, an infection wasless likely. In addition, the disease course, clinical data,and pathological report impressed an inflammatoryautoimmune disease, which met the diagnosis of Sjog-ren’s syndrome. Therefore, mini-pulse therapy with120 mg/day methylprednisolone was given for 3 consec-utive days. The prednisolone dose was then maintainedat 15 mg twice daily, and 200 mg/day hydroxy-chloroquine, 50 mg/day azathioprine, and 200 mg/daydapsone were added [3,5]. Her wounds healed progres-sively (Fig. 1E and F), and the platelet count improved to140,000/mL.

In summary, this case highlights the successful treat-ment of a patient with pyoderma gangrenosum with Sjog-ren’s syndrome. We used mini-pulse steroids as theinduction therapy, and then maintained this with a com-bination of dapsone [5] and azathioprine with tapering ofprednisolone to a lower dose [3].

enosum: Intractable leg ulcers in Sjogren’s syndrome, Kaohsiung.2013.11.005

ed by Elsevier Taiwan LLC. All rights reserved.

Figure 1. (A) Initial picture, wound with deep ulcer and some necrotic tissue. (B) Initial picture lateral view, wound with deepulcer and some necrotic tissue. (C) Post-treatment. (C,D) Pathology, inflammation with neutrophilic and lymphoplasmacyticinfiltration. (E) Post-treatment. (F) Post-treatment, lateral view.

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References

[1] Powell FC, Su WP, Perry HO. Pyoderma gangrenosum: classi-fication and management. J Am Acad Dermatol 1996;34:395e412.

Please cite this article in press as: Tsai Y-H, et al., Pyoderma gangrJournal of Medical Sciences (2013), http://dx.doi.org/10.1016/j.kjms

[2] Powell FC, O’Kane M. Management of pyoderma gangrenosum.Dermatol Clin 2002;20:347e55.

[3] Miller J, Yentzer BA, Clark A, Jorizzo JL, Feldman SR. Pyo-derma gangrenosum: a review and update on new therapies. JAm Acad Dermatol 2010;62:646e54.

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[4] Ravic-Nikolic A, Milicic V, Ristic G, Jovovic-Dagovic B. Pyo-derma gangrenosum associated with Sjogren syndrome. Eur JDermatol 2009;19:392e3.

[5] Teasley LA, Foster CS, Baltatzis S. Sclerokeratitis andfacial skin lesions: a case report of pyoderma gan-grenosum and its response to dapsone therapy. Cornea2007;26:215e9.

Yi-Hsuan TsaiChiung-Tang Huang

Department of Internal Medicine, Kaohsiung MedicalUniversity Hospital, Kaohsiung Medical University,

Kaohsiung, Taiwan

Please cite this article in press as: Tsai Y-H, et al., Pyoderma gangrJournal of Medical Sciences (2013), http://dx.doi.org/10.1016/j.kjms

Chee-Yin ChiaDepartment of Pathology, Kaohsiung Medical University

Hospital, Kaohsiung, Taiwan

Chen-Ching Wu*Department of Internal Medicine, Kaohsiung Medical

University Hospital, Kaohsiung Medical University,Kaohsiung, Taiwan

*Corresponding author. Department of Internal Medicine,Kaohsiung Medical University Hospital, Kaohsiung Medical

University, Number 100, Tzyou 1st Road, Kaohsiung807, Taiwan.

E-mail address: randymanwu@gmail.com

enosum: Intractable leg ulcers in Sjogren’s syndrome, Kaohsiung.2013.11.005