LETTERS TO THE EDITOR

 

Crohn's disease and Sweet's syndrome: A debut together

Debut conjunto de enfermedad de Crohn y síndrome de Sweet

 

 

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Key words: Sweet's syndrome. Crohn's disease.

Palabras clave: Síndrome de Sweet. Enfermedad de Crohn.

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Dear Editor,

The association of Sweet's Syndrome (SS) and Crohn's disease (CD) is unusual, with less than 50 reported cases. We report a case in which these entities debut together.

 

Case report

A 32-year-old woman with a history of guttate psoriasis 5 years earlier, with good response to PUVA treatment, without recent episodes. Admitted for presenting diarrhea with 40 daily liquid stools, some with mucus, ongoing for one month. In the last 2 weeks, there was evidence of onset of pustule-like erythematous inflammatory lesions on plaques, on the arms and legs (Fig. 1A), associated with oral ulcer, fever and migratory arthralgias of large joints. Laboratory results showed leukocytosis with deviation to the left and CRP: 103 mg/L. Colonoscopy revealed patchy erythematous areas with superficial ulcers. Colonic biopsies confirmed the presence of severe transmural inflammatory infiltrate with eosinophils and evidence of an epithelioid granuloma. A skin biopsy showed the presence of neutrophilic infiltrates (Fig. 1B) and granulomas in the deep dermis (Fig. 1C), confirming the diagnosis of SS. She was treated with ciprofloxacin, metronidazole, salazopyrin and topical steroids, with good response.

 

 

Discussion

SS was first described in 1964 by Sweet, characterized by an abrupt onset of cutaneous lesions consisting of painful, erythematous plaques (commonly located on hands, arms, upper trunk, neck and face), accompanied by fever, leukocitosis with neutrophilia and an increase of the acute phase reactants. Occasionally oral sores can appear, and the articular involvement is common (37-51% of cases). Its characteristic histological pattern is the presence of a dense infiltrate of neutrophils located in the dermis, accompanied by edema, without vasculitis (1,2).

The association with CD is less common, was described for the first time by Kemmett in 1998. Colonic involvement is practically constant, with female predominance. SS appears concurrently with the episode of CD (75% of the cases), but only in one third of the patients present concurrent with a first episode of CD.

The treatment with steroids has shown to be very effective in the treatment of skin lesions which disappear without scarring. The association with metronidazole may have an additional effect. It is useful to remind the fact that some drugs used for the maintenance of remission in CD patients such as azathioprine, may induce SS, and this entity must be investigated in patients with dermal lesions before initiation of this treatment (3-8).

 

Aida Ortega-Alonso¹, Norberto López-Navarro², Elena Gallego³,
Carmen Lara¹ and Guillermo Alcaín-Martínez^1
1 UGC Digestive Diseases. Instituto de Biomedicina de Málaga (IBIMA).
Hospital Universitario Virgen de la Victoria. Universidad de Málaga. CIBERehd. Málaga, Spain.
² UGC Dermatology. Hospital Universitario Virgen de la Victoria. Málaga, Spain.
³ UGC Pathology. Hospital Universitario Virgen de la Victoria. Málaga, Spain

 

References

1. Catalan-Serra I, Martin-Moraleda L, Navarro-Lopez L, et al. Crohn's disease and Sweet's syndrome: An uncommon association. Rev Esp Enferm Dig 2010;102(5):331-7. DOI: 10.4321/S1130-01082010000500009.         [ Links ]

2. Fernandez-Torres RM, Castro S, Moreno A, et al. Subcutaneous histiocytoid sweet syndrome associated with Crohn disease in an adolescent. Case Rep Dermatol Med 2014;2014:954254. DOI: 10.1155/2014/954254.         [ Links ]

3. Perez Rodriguez MT, Martinez-Ares D, Martin-Granizo I, et al. Sweet's syndrome and erythema nodosum in a Crohn's disease patient. Med Clin (Barc) 2007;128:156-7.         [ Links ]

4. Vaz A, Kramer K, Kalish RA. Sweet's syndrome in association with Crohn's disease. Postgrad Med J 2000;76(901):713-4. DOI: 10.1136/pmj.76.901.713.         [ Links ]

5. Rappaport A, Shaked M, Landau M, et al. Sweet's syndrome in association with Crohn's disease: Report of a case and review of the literature. Dis Colon Rectum 2001;44(10):1526-9. DOI: 10.1007/BF02234611.         [ Links ]

6. Treton X, Joly F, Alves A, et al. Azathioprine-induced Sweet's syndrome in Crohn's disease. Inflamm Bowel Dis 2008;14(12):1757-8. DOI: 10.1002/ibd.20518        [ Links ]

7. Gonzalvez Perales JL, Tamarit Orti R, Ballester Fayos J, et al. A case of Sweet's syndrome associated with Crohn's disease. Gastroenterol Hepatol 1997;20(3):134-7.         [ Links ]

8. Burrows NP. Sweet's syndrome in association with Crohn's disease. Clin Exp Dermatol 1995;20(3):279-80. DOI: 10.1111/j.1365-2230.1995.tb01324.x.         [ Links ]