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Pyoderma gangrenosum in refractory celiac disease: a case report
© Sedda et al.; licensee BioMed Central Ltd. 2013
Received: 7 August 2013
Accepted: 22 November 2013
Published: 27 November 2013
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.
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.
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.
Pyoderma gangrenosum (PG) is an inflammatory neutrophilic dermatosis characterized by painful cutaneous ulcerations persisting for more than 4 weeks . Although accurate epidemiological data on PG are missing, the general incidence has been estimated to be between 3 and 10 per million per year, with a peak of incidence between the ages of 20 to 50 years [2–6]. Women are affected more frequently than men. Based on clinical presentation, PG can be differentiated in four major types: ulcerative, pustular, bullous and vegetative [3–5]. The diagnosis of PG requires the exclusion of other disorders, which can manifest with cutaneous ulceration (e.g. infections, vascular diseases, malignancies), and is based on clinical history, histopathological findings and response to therapy. Histopathological analysis of skin biopsies is useful to exclude other pathologies which clinically mimic PG rather than establishing a diagnosis of PG per se [3, 4, 7]. PG occurs most commonly on the lower legs with preference for the pretibial area or on peristomal areas. Other sites of involvement include breast, hand, trunk, head and neck. Moreover PG can have extracutaneous manifestations, such as involvement of upper airway mucosa, genital mucosa and eye, spleen infiltrates, neutrophilic myositis, sterile pulmonary neutrophilic infiltrates and sterile cortical osteolysis. It has been however suggested that the diagnosis of PG should be questioned when the legs or peristomal areas are not involved [3–5, 7].
Although PG can manifest in individuals apparently healthy, it is frequently associated with inflammatory or neoplastic systemic diseases, such as inflammatory bowel diseases, rheumatic disorders, leukemia and myelodysplastic syndrome [3–5]. Furthermore, an association with such diseases in the context of skin ulcerations with crater-like holes and cribiform scarring helps make the final diagnosis of PG [3–5], thus highlighting the diagnostic relevance of identifying diseases which might be associated to PG.
Here we describe a case of PG complicating the natural history of a woman with refractory celiac disease (RCD), a form of celiac disease (CD) characterized by symptoms/signs of malabsorption and villous atrophy unresponsive to a strict gluten-free diet (GFD) .
Cytokine and MMP transcripts in lesional and non-lesional skin of one refractory celiac disease patient
RNA transcript relative expression
CD is an immune-mediated enteropathy triggered by the ingestion of gluten in genetically-susceptible individuals . The clinical spectrum of CD is wide, ranging from asymptomatic presentations to symptomatic cases with either classical intestinal (e.g. abdominal pain, chronic diarrhea, weight loss) or non-classical extraintestinal (e.g. anemia, osteoporosis) features . CD can be associated with cutaneous manifestations, which can improve following exclusion of gluten from the diet. The most common CD-associated skin pathologies are dermatitis herpetiformis, which is characterized by itchy, chronic, papulo-vesicular eruption, and psoriasis .
This is the first case to show a possible association between RCD and PG. Interestingly, PG responded rapidly to steroids while no simultaneous improvement of the underlying RCD was seen. This raises the possibility that RCD and PG are immunologically different disorders. We feel it is fair to conclude that physicians should consider this putative association so that appropriate medical therapy can be started early on.
“Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal”.
This work received support from the “Fondazione Umberto di Mario” Onlus, by “FC Fondazione Celiachia Onlus” and Giuliani Spa, Milan, Italy.
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- The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-230X/13/162/prepub
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