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Letter to the Editor

Editor, We read with interest the case report published by von Bernuth et al. (1). We are following a patient whose clinical presentation is quite similar to that of the patient described by them. Our patient, now a 20-year-old young man, started suffering from recurrent oral aphthous ulcers at the age of 16 years. At the age of 17, he also developed oral candidiasis and an endoscopy revealed Candida esophagitis and esophageal ulcers. Treatment with fluconazole was initiated and maintained due to frequent relapses of the oral candidiasis. Nevertheless, this treatment did not eliminate the frequent appearance of the major oral aphthous ulcers. Due to several episodes of rectal bleeding, he also underwent a colonoscopy with biopsy which did not reveal any pathological findings. Slit lamp examination of the eyes was normal. A pathergy test was negative. Laboratory work up revealed normal white blood cell counts and normal percentages and numbers of peripheral B and total T (CD3 ) lymphocytes but low numbers of CD4 + lymphocytes (200–400/mm) with a reversed CD4 / CD8 + ratio. The percentage of cd T lymphocytes was 7.6%. HIV status was negative. Serum immunoglobulin levels were normal to high with protective levels of antibodies against tetanus and diphtheria toxoids, poliovirus, and measles, mumps and rubella viruses. A delayed-type hypersensitivity skin test with Candida antigen was positive. In vitro lymphocyte responses to mitogens (ConA, PHA and PWM) were mildly reduced and in vitro lymphocyte response to Candida antigen was normal. ADA and PNP deficiencies were excluded. Neutrophil function tests (chemotaxis, bactericidal activity and superoxide generation) were normal. No autoantibodies were found and serum levels of FT4, TSH, cortisol and PTH were all normal. Our patient resembles the patient described by von Bernuth et al. (1) as both patients have a combination of idiopathic CD4 + lymphocytopenia, recurrent aphthous ulcers, persistent oral candidiasis and candida esophagitis with ulcers. One differential diagnosis which was not mentioned by von Bernuth et al. (1) is Behçet’s disease (BD). Recurrent major aphthous ulcers are common in patients with BD and are associated with a more severe, repeated and prolonged oral disease compared with recurrent minor aphthous ulcers (2). It was also found that the first manifestations of BD in children are almost exclusively in the form of oral ulcers (3). We could not make the diagnosis of BD in our patient as no other required criteria for diagnosis of the disease were observed throughout the follow-up period. He also tested negative for HLA-B51, which is associated with BD in Israel (4, 5). One case of idiopathic CD4 + T lymphocytopenia associated with Behçet’s-like syndrome was published recently. The adult patient described had recurrent oral and genital ulcers, oral and esophageal candidiasis, pansinusitis, and tested positive for HLA-B51 (6). Of interest are the findings of increased numbers of peripheral T cells bearing the cd T-cell receptor in the patient described by von Bernuth et al. (1). Increased numbers of cd T cells have been demonstrated in patients with BD (7–9). Some of these cells were sensitized to the 65 kDa heat shock proteins (8). In BD patients, it was found that the cd T cells were in an activated stage whereas the ab+ T cells were not activated compared with controls (9). Gamma-delta T cells play major roles in mucosal immunity acting as the first line of defense (10). Gamma-delta T cells can also modulate epithelial cell growth (11) and recognize structures presented by microorganisms as well as by stressed cells but not normal cells (12). Whether this is the immunological link that plays a direct role in the pathogenesis of a new syndrome of idiopathic CD4 + lymphocytopenia with recurrent aphthous ulcers and oral and esophageal candidiasis , or just the result Pediatr Allergy Immunol 2003: 14: 338–339