Pyoderma gangrenosum in refractory celiac disease: a case report.

BACKGROUND

Pyoderma gangrenosum is an inflammatory neutrophilic dermatosis characterized by painful cutaneous ulcerations and often associated with systemic inflammatory and neoplastic diseases. Here we report the first case of pyoderma gangrenosum in a patient with refractory celiac disease.

METHODS

A 52-year-old woman with a previously diagnosed refractory celiac disease resistant to steroids and immunosuppressive drugs presented to our hospital for a rapidly growing, painful inflammatory skin lesion of the left leg. Physical examination revealed a painful lesion with focal ulceration, necrosis and pus discharge with active inflammatory borders at the external part of the left leg. Histological evaluation of a skin biopsy and analysis of inflammatory cytokines and matrix-degrading proteases in lesional skin samples confirmed the clinical suspicion of pyoderma gangrenosum. Treatment with oral prednisone was rapidly followed by a complete healing of the skin lesion but no improvement of symptoms/signs of malabsorption.

CONCLUSIONS

Treatment of the patient with systemic steroids healed the skin lesion without improving the underlying refractory celiac disease. This observation raises the possibility that refractory celiac disease and pyoderma gangrenosum may be immunologically different.