Ceaseless Oral Ulcerative Manifestations ^†

On April 2016, a 24-year-old male patient came to our facilities for multiple mouth ulcers involving the tongue borders and the cheek mucosa (Figure 1). Patient didn’t refer any systemic disease, but several episodes of diarrhea for a month; he was also diagnosed of incontinent cardia associated with hypersecretory antral gastropathy, HP negative. Blood test was slightly positive for anti-BP180 (20 U/mL—n.v. < 9) and negative for coeliac antibodies. Patient did not want to undergo biopsy because of the summer period but, because of the symptoms, he requested a therapy. Clobetasol propionate ointment and 3 mg Betamethasone tablets once a day were prescribed for a month with improvement in symptoms. While tapering, symptoms re-occurred. Few months later, after therapy suspension, patient underwent a triple biopsy of the lesions. New blood test was negative for pemphigoid and an altered CD4/CD8 ratio (0.9 vs. 1.4–1.9 n.v.) became evident. Report from biopsy was an epitheliotropic CD4+ lymphoproliferative disorder without clear monoclonality; a diagnosis of suspected lymphomatoid papulosis, without signs of cutaneous lesions and spontaneous remission, was hypothesized. [1]. The fecal calprotectin was 10 times higher (49,898 mg/Kg; n.v. 50 mg/Kg); although the biopsy of intestinal villi was negative, genetic typing of HLA locus DQ2/DQ8 [2], showed a predisposition for coeliac disease. Later, patient developed erythematous plaques involving the forearms and trunk, worsening after sun exposure. Abdomen MRI didn’t show any alteration. Blood testing for HIV, Treponema pallidum, EBV, ASCA, ANCA, ANA and ENA were all negative. A year later, patient reported asymmetrical arthralgia, first in elbows, wrists and shoulders with muscular pain in flexor muscles of the forearms. Ventricular extrasystoles have been diagnosed recently: Holter ECG reported 30051 monomorphic isolated ventricular extrasystoles. The patient was visited by numerous specialist physicians, e.g. Dermatologist, Cardiologist, Rheumatologist but he is still undiagnosed (Figure 2) and finds improvement for oral ulcers with topical corticosteroids and occasionally systemic cortison (1 mg). Seloken (beta blocker) and Dibase 50.000 UI are also administered.