1/35. Severe multisystemic hypersensitivity reaction to carbamazepine including dyserythropoietic anemia.OBJECTIVE: To report a case of multisystemic hypersensitivity reaction to carbamazepine. CASE SUMMARY: An 81-year-old white man was admitted to our hospital because of fever, morbilliform pruritic rash, and jaundice. Fifty days before admission he had taken carbamazepine 200 mg p.o. tid because of seizures. During the first few days following admission, a maculopapular rash progressed to generalized erythroderma with subsequent extensive skin exfoliation. After discontinuing carbamazepine the fever disappeared within 72 hours and hepatic function tests returned to normal within four days. Moreover, after admission the hemoglobin values gradually fell to 6.7 g/100 mL. A bone marrow aspirate showed hypercellularity with marked dyserythropoietic abnormalities, and the bone marrow biopsy showed large and diffused infiltration due to the presence of a low-grade small lymphocytic lymphoma. No specific therapy for the lymphoma was undertaken. The biochemical follow-up showed a total improvement of hemoglobin values. Eight months after drug discontinuation, the patient was asymptomatic; peripheral blood cell count and hemoglobin concentrations were persistently normal. DISCUSSION: To the best of our knowledge, this is the first published case report implicating carbamazepine as the cause of anemia associated with bone marrow hypercellularity and dyserythropoietic changes, instead of hypocellularity and reduction of erythroid precursors. An interesting point raised by our observation is the possible relation between carbamazepine intake and actual lymphoproliferative disease. The development of non-Hodgkin's lymphoma following carbamazepine treatment has been reported, with regression after the drug was discontinued. However, in our case, a bone marrow biopsy repeated eight months after drug discontinuation confirmed the diagnosis of low-grade lymphoma. CONCLUSIONS: This case report describes a severe multisystemic reaction, characterized by generalized erythroderma; and renal, hepatic, and bone marrow failure in a patient who started carbamazepine therapy 50 days beforehand.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
2/35. Chronic eosinophilic leukaemia presenting with erythroderma, mild eosinophilia and hyper-IgE: clinical, immunological and cytogenetic features and therapeutic approach. A case report.A 23-year-old, white male metallurgist presented with pruritic erythematous maculo-papules over the trunk and upper limbs and 6 months later developed erythroderma, eosinophilia and multi-organ dysfunction. A diagnosis of chronic eosinophilic leukaemia was made on the basis of myeloproliferative involvement of both peripheral blood and bone marrow, associated with eosinophilic differentiation and a t(5;12)(q33;p13) translocation. The initial therapeutic approach was interferon alfa-2b plus cytosine arabinoside, for 13 months, followed by hydroxyurea plus vincristine. There was improvement of skin lesions, disappearance of eosinophilia and decrease of serum immunoglobulin e, towards normal values.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
3/35. Erythrodermia to pseudoephedrine in a patient with contact allergy to phenylephrine.BACKGROUND: phenylephrine and pseudoephedrine are sympathomimetic drugs belonging to the phenylamine family. Adverse cutaneous effects associated with these drugs have been reported but, in view of their frequent use, appear to be rare. The very close chemical structures of these drugs could explain potential cross-reactions among them but the results reported in the literature are controversial. CASE REPORT: An 18-year-old woman developed blepharoconjunctivitis after application of phenylephrine and tropicamide eye drops. Four years after this reaction, she took 1 tablet of Narine (pseudoephedrine and loratadine) and 3-4 hours later developed a generalized erythrodermic reaction. Cutaneous biopsy revealed hydropic changes in the basal layer and, in the dermis, moderate edema with slight perivascular lymphocyte and eosinophil infiltrates. patch tests with European standard series, commercial eye drops, tropicamide, phenylephrine, pseudoephedrine and other sympathomimetic agents were applied to the patient's back. After 47 and 96 hours, only the patches with pseudoephedrine and phenylephrine were positive. CONCLUSIONS: We believe that our patient has presented two different reactions with different clinical outcome and histopathology, which are unlikely to be due to cross-reactivity between the drugs involved. We have found no similar coincidences reported int the literature.- - - - - - - - - - ranking = 227.48388182813keywords = family (Clic here for more details about this article) |
4/35. Sezary's syndrome: a cytogenetic, cytophotometric and autoradiographic study.Cytophotometric, cytogenetic, and autoradiographic studies were performed in cells of three patients suffering from clinically diagnosed Sezary's syndrome with erythroderma and the presence of abnormal lymphoid cells in the peripheral blood, skin, bone marrow and lymph-nodes. Feulgen dna cytophotometry of cells in the peripheral blood and skin lesions showed marked aneuploidy and tetraploidy. Multiple translocations were identified with a G-banding technique. The chromosomal abnormalities varied widely between the patients, but C and D group chromosomes were more frequently involved than others. All breakpoints of the translocations were localised in the centromeric region. autoradiography of blood and skin samples revealed many labelled cells in the skin and a lower number in the blood, indicating cell proliferation in the skin. It is concluded that the pathological cells occurring in the sezary syndrome are abnormal lymphoid cells with a tendency to proliferate in the dermis. The variability observed between and in the patients is in all probability due to a difference in the degree of dedifferentiation.- - - - - - - - - - ranking = 2keywords = life (Clic here for more details about this article) |
5/35. erythrokeratodermia variabilis. A family study.erythrokeratodermia variabilis is a rare genodermatosis; American authors have reported only four previous cases. It had been a problem to obtain a large pedigree for clinical investigation. We studied a family with 12 involved members in five generations. Symmetrically distributed migratory patches and scaling plaques are characteristic and were found to involute with a combination of keratolytic agents and topical steroids. Exacerbations of these patches and plaques were noted in our female patients during such high estrogen states as pregnancy or oral contraceptive usage.- - - - - - - - - - ranking = 1137.4194091406keywords = family (Clic here for more details about this article) |
6/35. Sezary's syndrome and generalized plane xanthoma.The first case of Sezary's syndrome associated with generalized plane xanthoma is reported, thereby extending the association of lymphoreticular proliferative disorders with plane xanthomatosis. The association of Sezary's syndrome with plane xanthomatosis may be an in vivo example of defective cell regulation involving the major cellular components of the immune response.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
7/35. erythrokeratodermia variabilis in a Jewish Kurdish family.A Jewish family, originating from Kurdistan and presenting erythrokeratodermia variabilis in three consecutive generations, is described. The major features were the variable age of onset (from early infancy to 6 years) and the distinctive cutaneous lesions with demarcated erythematous hyperkeratotic plaques with irregular borders. The affected members had mild to severe expressions of the disease. The skin lesions were not influenced by puberty, pregnancy or old age. None of the patients had lesions of the palms and soles or abnormal neurological signs.- - - - - - - - - - ranking = 1137.4194091406keywords = family (Clic here for more details about this article) |
8/35. Deleterious mutations in SPINK5 in a patient with congenital ichthyosiform erythroderma: molecular testing as a helpful diagnostic tool for netherton syndrome.The congenital erythrodermas represent a heterogeneous group of inherited and acquired disorders often accompanied by systemic infections, impaired epidermal barrier function and concomitant life-threatening fluid and electrolyte imbalance. In the present report, we describe a patient who was considered to have congenital ichthyosiform erythroderma for 26 years until molecular testing led to the correct diagnosis of netherton syndrome.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
9/35. Lethal congenital erythroderma: a newly recognised genetic disorder.We report 4 patients and their extended families comprising 17 cases, all of whom had congenital exfoliative erythroderma resistant to treatment, associated with failure to thrive and hypoalbuminaemia. All died in the first year of life. This condition appears to be inherited in an autosomal recessive manner and the underlying defect remains unknown.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
10/35. Exfoliative dermatitis. Erythroderma can be a sign of a significant underlying disorder.64-year-old man presented with a 3-week history of a diffuse, pruritic rash that had started on his trunk and then spread to his entire cutaneous surface, including the palms of his hands and soles of his feet. physical examination revealed widespread fine scaling and diffuse erythema. Generalized lymphadenopathy was noted. No fever, hair loss, onycholysis, or nail shedding was detected. The patient had neither a personal history of skin disorders or, specifically, atopic eczema or psoriasis nor a family history of eczema or psoriasis. He also had no history of malignancy and was taking no medications. The patient's complete blood cell count with differential was unremarkable. He was treated with moisturizers, topical corticosteroids, and antihistamines and was advised to avoid possible irritants. One week later, the patient returned because of a worsening of his erythroderma. He also reported malaise and chills. Three 4-mm biopsy specimens were obtained from representative areas (ie, back, arm, and abdomen), and a 2-week course of oral corticosteroids was prescribed. The erythroderma greatly improved but worsened shortly after the steroid dose was tapered. The specimens showed psoriasiform hyperplasia with features suggestive of psoriasis vulgaris. The patient was treated with 25 mg of oral acitretin once a day. His erythroderma slowly resolved over 6 months, at which time the acitretin dose was tapered. The patient reported no recurrence of the erythroderma.- - - - - - - - - - ranking = 227.48388182813keywords = family (Clic here for more details about this article) |
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