Galeone, Massimiliano MD; Scarfì, Federica MD; Arunachalam, Meena MD; Bruscino, Nicola MD; Difonzo, Elisa Margherita PhD
From the Division of Dermatology, Department of Critical Care Medicine and Surgery, University of Florence, Florence, Italy
Correspondence: Massimiliano Galeone, MD, Division of Dermatology, Department of Critical Care Medicine and Surgery, University of Florence, Piazza Indipendenza, 11-50129 Florence, Italy. E-mail address: email@example.com.
Received for publication September 29, 2011, and accepted December 20, 2011.
We report a case of a 30-year-old woman with vegetative lesions in the perianal area to highlight the importance of considering perianal Crohn disease in the differential diagnosis of a resistant condylomata acuminata that does not improve after treatment.
A 30-year-old 4-month pregnant woman was referred to our outpatient service because of a 3-year history of vegetative lesions in the perianal area that was accompanied by constipation and pain during defecation. The patient denied having the following risk factors for sexually transmitted disease: unprotected sex before marriage, multiple sexual partners, and previous history of anogenital warts. A previous diagnosis of condylomata acuminata led to treatment with 4 cycles of electrodesiccation over the past 3 years. These lesions relapsed a few months later. Ten years before clinical presentation, the patient was diagnosed with ileal Crohn disease (CD) and was treated with systemic corticosteroids and sulphasalazine, which were ineffective. The patient subsequently underwent a 20-cm ileoectomy along with ileocecal valve removal. Six years before clinical presentation, CD extended to involve the descending colon, the sigmoid colon, the rectum, and the anal canal. A perianal fistula that extended from the anal canal to the perianal skin was also present, which had not been surgically corrected.
On physical examination, raised red-violet lesions with a bright red surrounding were evident along the intergluteal cleft and extended from the anal sphincter to the entire perineum. The lesions had a papillomatous appearance and a parenchymal consistency (Fig. 1A). Two 5-mm punch biopsies, to the right and the left side of the anus, were performed, and histologic examination revealed noncaseating granulomas in the deep dermis composed mainly of epithelioid cells and multinucleated giant cells, surrounded by a mixed infiltrate of histiocytes, lymphocytes, plasma cells, and a scarce amount of eosinophils (Fig. 1B). On this basis, we confirmed the presence of perianal CD (PCD), which we suspected on clinical examination.
CD is associated with a great variety of cutaneous manifestations, which can be classified into nonspecific (pyoderma gangrenosum, erythema nodosum, erythema multiforme, palmar erythema, vasculitis) and specific, according to the absence or presence of typical granulomas.1,2
Perianal lesions, one of the most common extraintestinal manifestations of CD, can be documented in one-third of patients with intestinal disease, particularly in those with colon involvement.3–8 They occur concurrently or may precede the intestinal manifestations by a few months or even years.5
The severity of cutaneous involvement is not correlated with the intestinal disease activity. PCD includes fissures, fistulas, and abscesses. Papillomatous vegetations are rarely observed in this condition6–8 and, when present, may resemble condylomata acuminata or a wide variety of cutaneous granulomatous conditions such as sarcoidosis, deep fungal infections, and mycobacterial infections.9
PCD does not usually improve with conventional treatment and/or surgical removal of the intestinal lesions, as witnessed in the present clinical case.3 Several drugs have been attempted but have yielded unsatisfactory results. Among those most utilized are metronidazole, prednisone, cyclosporine, and biologic agents, such as infliximab and adalimumab. Topical treatments include typically tacrolimus, corticosteroids, and antibiotics.2,4,7 Because our patient was pregnant at the moment of clinical presentation, systemic treatment was not possible. Thus, topical corticosteroids (methylprednisolone 0.1% cream) was used and resulted in a partial improvement of the lesions. After giving birth, the patient will initiate systemic therapy at her next follow-up visit.
Papillomatous, vegetative lesions, as previously mentioned, are one of the least common manifestations of PCD and are, thereby, not always given adequate attention. The failure to diagnose PCD occurs more often in highly specialized medical environments, where physicians essentially tend to limit themselves to their field or are trained to follow algorithms without considering the disease as a whole. In such cases, a close collaboration between specialists is necessary for accurate diagnoses and treatment. The present clinical case highlights that despite a positive clinical history of CD, these lesions can be easily misdiagnosed as condylomata acuminata even for periods as long as 3 years.
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