| Abstract |
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Patient |
18-year-old student |
Duration |
2 years |
Distribution |
Oral cavity, trunk, groins and lower extremities |
| History and Physical Examination |
The patient is an 18-year-old student. His problem started with suppurative lesions on his groins nearly two years ago (Fig. 1). At that time, several courses of different types of antibiotics failed to settle the lesions. Hence, a biopsy taken which was compatible with suppurative hydradenitis. Appropriate medication therapy continued for another one month, but new lesions developed mainly on the shins which seemed more remarkable and larger than old one. Another skin biopsy was done and was compatible with pyoderma gangrenosum.
A through search for finding the possible underlying diseases was performed. Barium enema, GI endoscopy and abdominal CT scan all failed to show any significant finding.
The patient was admitted in the hospital and short course of systemic steroid started which seem to be effective initially, but unfortunately once tapering down started, his condition flared up and second course of systemic steroid were not effective as the first one. At this stage, a 15 day course of thalidomide was added to prednisolone which seemed to deteriorate the condition. Subsequently, higher doses of prednisolone with both cyclosporine and azathiprine resumed with good effect, but unfortunately once tapering down started his condition flared up again and new lesions appeared on his gingiva. Another biopsy taken from the gingival lesions (Fig. 2) which suggested the Wegener's granulomatosis. A c-ANCA test was highly positive ( >100).
The patient's disease was controlled with prednisolone, cellcept and hydroxychloroquine but once again the lesions flared up following lowering the dose of steroid. Hence, treatment with cyclophosphamide started in early June 2010 accompanied with prednisolone 20mg/d and azathiprine 75mg/d which after 45 days has had even worse effects than before (Fig. 3).
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| Images |
Figure 1
Figure 2
Figure 3
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| Laboratory Data |
P-ANCA (IF) negative
C-ANCA (IF) 1/1000 (N<1/10)
ANA 1/20 (N<1/20)
Anti Mpo IgE 11u/mol (N<12)
Anti PR3 IgG (Elisa) >300 U/ml (N<12)
Chest CT scan: normal
Abdominal CT scan normal
GI endoscopy and Barium enema normal |
| Histopathology |
Biopsy of the buccal mucosa
Section shows a mucosal lesion composed of hyperplastic parakeratinized stratified squamous epithelium with anastomosing rete ridges, exocytosis, and microabscess formation. The underlying connective tissue reveals vascular proliferation with plump endothelial cells, severe mixed inflammatory cells infiltration, leukocytoclastic vasculitis, few giant cells and extravasated RBCs and foci of bacterial colonization.
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| Diagnosis |
Pyoderma Gangrenosum in the setting of Wegener's Granulomatosis?
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| Questions |
What is unusual about this patient is its recalcitrancy to treatment and its lack of respiratory symptoms.
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| Discussion |
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| References |
1. Boudny C, Nievergelt H, Braathen LR, Simon D. Wegener's granulomatosis presenting as pyoderma gangrenosum. J Dtsch Dermatol Ges. 2008 Jun;6(6):477-9
2. Szõcs HI, Torma K, Petrovicz E, Hársing J, Fekete G, Kárpáti S, Horváth A. Wegener's granulomatosis presenting as pyoderma gangrenosum. Int J Dermatol. 2003 Nov;42(11):898-902.
3. Sinovich V, Snow J. Protracted superficial Wegener's granulomatosis. Australas J Dermatol . 2003 Aug;44(3):207
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| Keywords |
Pyoderma gangrenosum, wegener's granulomatosis,
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| Comments from Faculty and Members |
Yelva Lynfield M.D., Dermatologist, Mendota Heights, MN, USA on 22nd July 2010
Since pyoderma gangrenosum sometimes responds to dapsone, I wonder why this relatively non-toxic drug was not tried.
Dylan W.T Kuek M.D. , Malacca, Malaysia on 26th July 2010
This is a young male patient with suppurative lesions on his groins for the past 2 years. I wonder if he has any history of drug abuse such as cocaine due to drug-mediated immunmodulation. Risks screenings should be done .
Since serial courses of immunosuppressants and immunomodulators has been given to this patient with no clinical improvements, IVIG may be considered . Also, drug related side effects should not be missed including optic neuritis secondary to thalidomide.
Adel Aly M.D., Dermatologist, Cairo, Egypt on 26th July 2010
Methotrexate 0.3-1 mg /kg sc weekly together with steroid good for maintenance
Kaherine Liao M.D. Instructor in Rheumatology, Harvard Medical School, Boston, MA, USA on July 28th , 2010
Wegener's and pyoderma gangrenosum don't usually go together as you and your colleague pointed out which makes me wonder whether this is actually pyoderma. I asked one of our vasculitis guys here and he has not seen the two together. I found 2 cases in the lit of this. Sometimes underlying vasculitis can look like this (had a case of misdx w/ pyoderma for 2 yrs but was really medium vessel vasculitis). Did they get a good biopsy that went into the dermis so that vasculitis can be accurately assessed? The one biopsy report of the gingiva sounded non-sp to me- leukocytoclastic vasculitis and inflamm infiltrates. I also wonder whether there is any evidence of underlying inflammatory bowel disease in this patient (not that he needs another dx).
The +ANCA and PR3 are very convincing for Wegeners and it's good news that he does not have other systemic manifestations. A good biopsy would be very helpful. If it is clearly vasculitis driving these lesions then it may be helpful to review how aggressive they were with the previous treatments including endoxan. If it is not vasculitis, and this is pyoderma, then would first review how the meds were used, and if they were true failures. Reading through his chart, since he had been through so many meds, I wondered whether enough time was given for each med to assess for efficacy. As a last resort, I have seen anti-TNFs work on pyoderma but that is completely anecdotal and the pts usually had underlying IBD.
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