Esophageal involvement is frequent in lichen planus: study in 32 patients with suggestion of clinicopathologic diagnostic criteria and therapeutic implications.

OBJECTIVE: Lichen planus (LP) is a classic skin disease that can involve the skin, hair, and nails, as well as the oral and genital mucosa. Histopathology is characterized by a T-lymphocytic, lichenoid, and interface dermatitis. Multiple case reports and small case series have shown that LP can involve the esophagus. However, the diagnostic criteria, incidence, and best treatment options remain uncertain. This study aimed to refine the diagnostic criteria, estimate prevalence, and present an outlook on treatment options to prevent long-term sequelae. PATIENTS AND METHODS: Thirty-two consecutive patients with LP of the skin, hair, nails, oral mucosa, and/or genital mucosa underwent a comprehensive clinicopathologic assessment. Esophagogastroduodenoscopy was performed, and biopsies were evaluated histologically, immunohistochemically, and by direct immunofluorescence. Patients diagnosed with esophageal lichen planus (ELP) were followed up prospectively where possible. RESULTS: In total, 20 of 32 patients had ELP. Ten of these 20 patients were classified as having proven ELP, with clear-cut endoscopically visible lesions; the other 10 were classified as having probable ELP. Eight of 10 patients with proven ELP were started on new or additional therapy because of esophageal findings. Treatment with a topical budesonide formulation or systemic corticosteroids was successful in most patients with proven ELP and reversed functional esophageal stenosis. CONCLUSION: ELP can be found in more than 50% of patients with proven mucocutaneous LP when clinical and pathologic findings are correlated carefully. Topical or systemic corticosteroids are the first-line therapy for ELP. Timely medical therapy seems to prevent scarring stenosis of the esophagus.

Fatal bleeding due to a heparin-like anticoagulant in a 37-year-old woman suffering from systemic mastocytosis.

A 37-year-old female patient with systemic mastocytosis who was admitted with severe unexplained bleeding symptoms is studied. Laboratory procedures established the diagnosis of a patient-derived-heparin-like anticoagulant as a very rare hemostatic abnormality predisposing to bleeding. The patient died from refractory disease despite therapy with protamine, initiation of chemotherapy, and supportive measures. The case illustrates the clinical presentation and diagnosis of heparin-like anticoagulants. Etiology, pathophysiology, and therapeutic options are discussed.