Pyoderma gangrenosum associated with Sjögren syndrome

ARTICLE

Auteur(s) : Ana Ravic-Nikolic, Vesna Milicic, Gordana Ristic, Bojana Jovovic-Dagovic

Department of Dermatology, Clinical Center “Kragujevac”, Zmaj Jovina St. 30, 34000 Kragujevac, Serbia

A 61-year-old man with a two year history of Sjögren syndrome (SS) was admitted to the Department of Dermatology with a non-healing, painful ulcer on the posterior side of the left thigh. We observed a large ulcer, with an irregular shape, and purplish, necrotic periphery (figure 1A). The lesion started as a pustule more than five months previously, then increased in size and broke to form an ulcer. Laboratory investigations showed an elevated sedimentation rate and leucocytosis. Electrolytes, kidney and liver analyses, and a peripheral smear were normal. A stool test for occult blood was negative. VDRL was negative. Rheumatoid factor, C-reactive protein and ANA were positive. Chest radiography, abdomen ultrasound and colonoscopy were normal. A pathergy test was positive. The diagnosis of pyoderma gangrenosum (PG) was based primarily on clinical grounds. Consent was refused for a biopsy. We started treatment with methylprednisolone (120 mg/day) and sulfasalazine (2000 mg/day), but after two weeks the response was poor. Sulfasalazine was stopped, corticosteroid therapy was continued with dapsone (50 mg/day). After the first signs of improvement, methylprednisolone was gradually reduced while dapsone was increased to 60 mg/day. As local therapy, wound dressings were applied. The patient was released from hospital with 50 mg/day of oral methylprednisolone, 60 mg/day of dapsone and advice about wound care. The patient received regular follow up care in the Outpatient Department. After two months of dapsone, the ulcer healed leaving an atrophic scar (figure 1B).

Pyoderma gangrenosum is a rare neutrophilic dermatosis with unclear etiology, which usually occurs in the fourth and fifth decades of life but all ages can be affected [1]. PG was first described in 1930 [2]. Pathophysiology of the disease may be based on altered neutrophil chemotaxis [3]. The pathergy test is positive-direct trauma to the skin can induce a new lesion. Clinically, PG can manifest as: classic (deep ulceration most commonly localized on the legs or around stoma sites), atypical: occurs on genitalia and/or oral mucosa and: extracutaneous, which involves the lungs, liver, etc. [1]. Diagnosis is usually made by exclusion of other possible diseases. To rule out other diseases a complete patient history and physical and laboratory examinations should be conducted. Cultures of the ulcer for bacteria, mycobacteria, viruses and fungi are needed for exclusion of infections (ecthyma, impetigo, necrotizing fasciitis, syphilitic gumma, anthrax, chronic herpes simplex infection, tuberculosis gumma, deep mycosis, leishmaniasis). Doppler ultrasound may be performed in patients suspected of having vascular ulcers. Serum studies (VDRL, ANCA, ANA, antiphospholipid antibody test) are helpful in the exclusion of systemic disorders (systemic lupus erythematodes, rheumathoid arthritis, Sweet syndrome, Wegener’s granulomatosis, leucocytoclastic vasculitis) [1, 4]. Although nonspecific, the histopathology of PG is useful for exclusion of cutaneous carcinomas [1]. The initial lesion shows a deep suppurative inflammation with dense neutrophilic infiltrates. In at least 40% of cases, leukocytoclastic vasculitis is present [1]. Apart from local wound care, therapy consists of systemic therapy (corticosteroids, cyclosporine, cyclophosphamide, intravenous immune globin, dapsone, sulfasalazine) [5]. The prognosis of PG is good, but it depends on the underlying disease. Recurrence can occur in 46% [6].

We presented PG associated with SS. Ulcerative colitis, Crohn’s disease, leukemia, monoclonal gammapathies and various types of arthritis are associated with PG in about 50% of cases. Myelomas, lupus erythemotodes and Sjörgren’s syndrome are more rarely associated [1]. In our case, the diagnosis of PG was based on the patient’s history (the initial lesion was described as a bite reaction which increased to a form a painful ulcer), clinical findings (atypical non-healing ulcer), positive pathergy phenomenon and a good response to dapsone. Laboratory and physical examinations revealed no other underlying disease.

Acknowledgements

References

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