Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0235108 (tense)
 2,176 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 69-year-old Chinese man presented in 2001 with a blistering eruption over the upper and lower limbs associated with oral ulceration for 1 month. He had stage IIIA follicular small cell cleaved non-Hodgkin's lymphoma diagnosed 5 years previously, and had received several lines of palliative chemotherapy, including two courses of chlorambucil, six cycles of cyclophosphamide, adriamycin, vincristine, and prednisolone (CHOP), and two four-cycle courses of rituximab, with disease stabilization at the time of presentation. Examination revealed erythematous, annular plaques with raised, urticarial borders studded with tense bullae and vesicles over the thighs. Some lesions were arciform and annular, with vesicles arranged in a ring at the border (Fig. 1). There was involvement of the feet with desquamation at the tips of the toes (Fig. 2). Severe erosions with hemorrhagic crusts on the lips, tongue, and buccal mucosa were seen. Herpes simplex virus serology was negative. A biopsy specimen from a vesicle on the left thigh showed suprabasal acantholysis (Fig. 3), some apoptotic keratinocytes (Fig. 4), satellite cell necrosis in the epidermis, and a superficial perivascular infiltrate of lymphocytes and eosinophils. Direct immunofluorescence showed intercellular immunoglobulin G (IgG) and C3 within the epidermis and along the basement membrane zone. Indirect immunofluorescence on monkey esophagus was positive for anti-intercellular antibody at a titre of 1/160 and positive on rat bladder at a titre of 1/80. A presumptive diagnosis of paraneoplastic pemphigus was made. This was later confirmed by the presence of antibodies against envoplakin (210 kDa), periplakin (190 kDa), and desmoglein 1 on immunoprecipitation studies. He was started on prednisolone 60 mg/day (1 mg/kg/day), with complete resolution of skin lesions within 1 week, but persistence of oral ulcers. Cyclophosphamide was added at a low dose of 1 mg/kg/day as he had baseline leukopenia. Cyclosporine was later added to a maximum of 4 mg/kg/day with only mild improvement of the oral lesions. He declined rituximab therapy. He died 2 months later from fulminant pneumonia.
 ...
PMID:Paraneoplastic pemphigus resembling linear IgA bullous dermatosis. 1696 19

Bullous pemphigoid (BP) has been associated with neurological disorders (NDs), which has led to the hypothesis that molecular mimicry exists between hemidesmosomal proteins and neuronal peptides. A 79-year-old hemiparetic woman presented with tense bullae affecting exclusively her right paretic upper limb for three months. Histopathology, taken from the perilesional area, revealed an inflammatory infiltrate with predominant eosinophils. IIF evidenced linear IgG deposition in the epidermal side of the cleavage. ELISA detected circulating anti-BP180 and anti-BP230 autoantibodies. Immunoblotting exhibited unspecific reactivity against the 190-kDa periplakin in normal human epidermal extract. The immunocompromised cutaneous district concept may explain the possible mechanism for the exclusive involvement of the autoimmune blistering disease in lymphedematous hemiparetic upper limb.
 ...
PMID:Bullous Pemphigoid Associated with Ischemic Cerebrovascular Accident and Dementia: Exclusive Blistering Lesions on the Upper Hemiparetic Limb. 2998 77