Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In recent years, especially with the advent of acquired immunodeficiency syndrome, new skin disorders associated with systemic disease have been described in the literature. Eosinophilic folliculitis and pruritic papules of human immunodeficiency virus (HIV) infection are clinically similar lesions that respond to phototherapy. Bacillary angiomatosis, another HIV-related skin disease that is caused by a pleomorphic gram-negative organism, resembles Kaposi's sarcoma clinically but is curable if treated early with antibiotics. Toxic strep syndrome, a scarlatiniform, desquamative eruption associated with hypotension, fever and multiorgan system dysfunction, is caused by group A streptococcal soft tissue infection. Paraneoplastic pemphigus, a recently characterized autoimmune vesicular eruption, produces painful mucocutaneous ulcerations in patients with an occult neoplasm, such as chronic lymphocytic leukemia or malignant lymphoma.
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PMID:New cutaneous manifestations of systemic diseases. 765 17

Paraneoplastic pemphigus (PNP) is a rare multisystem, autoimmune disease with prominent mucocutaneous manifestations, occurring most commonly in association with haematological malignancies. It is characterised by the presence of circulating autoantibodies against epithelial adhesion proteins. We report a 46-year-old woman with common variable immunodeficiency who developed paraneoplastic pemphigus after receiving radiotherapy for relapsed non-Hodgkin's lymphoma. Flaccid bullae covering approximately 70% of the skin, painful oropharyngeal ulceration and periocular erosions were prominent clinical features. Despite supportive treatment and attempts at disease control using high-dose corticosteroids and cyclophosphamide, the patient became increasingly debilitated, developed septic shock secondary to Pseudomonas aeruginosa septicaemia on two occasions and died of respiratory failure 6 weeks after presentation. We highlight the need to be aware of (PNP) and to perform appropriate immunological investigations. In addition, we emphasise the importance of a multidisciplinary approach to the management of such patients.
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PMID:Paraneoplastic pemphigus occuring after radiotherapy for relapsed non-Hodgkin's lymphoma in a patient with common variable immunodeficiency. 1275 Jul 36