Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0154059 (Esophagus)
 2,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma and is characterized clinically by an indolent course with slow progression. MF is limited to the skin with widespread distribution, however, extracutaneous involvement of MF occurs during the advanced stages of the disease. Esophageal involvement of MF is a rare event. In the present study, we describe the first documented case of CD8(+) MF with esophageal involvement that was endoscopically diagnosed antemortem. A 70-year-old male with a 15-year history of MF presented with difficulty in swallowing. Endoscopic examination revealed a tumorous lesion with ulceration in all regions of the esophagus. Esophagus biopsy demonstrated atypical lymphocytic infiltrates with ulceration. Immunohistochemically, these atypical lymphocytes were positive for CD3, CD8 and cytotoxic granules. Therefore, a diagnosis of CD8(+) MF involving the esophagus was made. Extracutaneous involvement of the esophagus in MF is extremely rare and the majority of previously reported cases have been diagnosed postmortem. Only two cases of MF with esophageal involvement endoscopically diagnosed antemortem have been previously reported and this is the first documented case of CD8(+) MF with esophageal involvement diagnosed by this method. Early detection of extracutaneous involvement of MF is important for accurate treatment and endoscopic examination is a useful tool for detection of this pathology.
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PMID:CD8(+) mycosis fungoides with esophageal involvement: A case report. 2325 97

Less is known about gastrointestinal (GI) involvement of primary skin diseases due to the difference in embryology, histology, microbiology and physiology between integument and alimentary tract. Oesophagus, following the oropharyngeal mucosa, is the most common GI segment affected by primary skin diseases, especially by eosinophilic oesophagitis, lichen planus and autoimmune bullous dermatoses like pemphigus vulgaris, mucosal membrane pemphigoid and epidermolysis bullosa acquisita. Eosinophilic oesophagitis is an emerging chronic atopic disease with oesophageal dysfunction as the typical presentation, and oesophageal narrowing, rings and stricture as late complications. Oesophageal lichen planus mainly involves the proximal to mid-oesophagus in elderly aged women with long-term oral mucosal lesions. In acute attack of pemphigus vulgaris, oesophageal involvement is not uncommon but often neglected and may cause sloughing oesophagitis (oesophagitis dissecans superficialis) with acute GI bleeding in rare cases. GI manifestation of hereditary bradykininergic angio-oedema with colicky acute abdomen mostly affects small intestine, usually in the absence of pruritus or urticaria, and is more severe and long-lasting than the acquired histaminergic form. Strong evidence supports association between inflammatory bowel disease, especially Crohn disease, and hidradenitis suppurativa/acne inversa. Patients with vitiligo need surveillance of autoimmune liver disease, autoimmune atrophic gastritis or coeliac disease when corresponding symptoms become suspect. Melanoma is the most common primary tumour metastatic to the GI tract, with small intestine predominantly targeted. Gastrointestinal involvement is not uncommon in disseminated mycosis fungoides. Extramammary Paget's disease is an intraepidermal adenocarcinoma of controversial origin, and a high association between the anogenital occurrence and colorectal adenocarcinoma has been reported. As GI tract is the largest organ system with multidimensional functions, dermatologists in daily practice should be aware of the gastrointestinal morbidities related to primary skin diseases for an early diagnosis and treatment.
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PMID:Gastrointestinal involvement of primary skin diseases. 3245 73