Cases reported "Dermatitis, Exfoliative"

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1/14. Type III and type IV hypersensitivity reactions due to mitomycin C.

    A 71-year-old man developed an exfoliative dermatitis of the palms of the hands and soles of the feet, and a generalized itch, during treatment with intravesical instillations of mitomycin C for an undifferentiated carcinoma of the bladder. patch tests with mitomycin C 0.03%, 0.1% and 0.3% aq. were positive. Because of the serious consequences of this finding, the patient was retested with mitomycin C in pet. (same concentrations), a more stable preparation. This showed clear positive reactions. During this last series of patch tests, he developed palpable purpura on the legs. We postulated that this reaction was an immune-complex-mediated reaction, caused by the 2nd series of patch tests with mitomycin C. To prove this, we performed histopathological and immunofluorescence investigations, and these showed the reaction to be consistent with Henoch-Schonlein-type purpura. We therefore conclude that this patient developed systemic reactions to mitomycin C, characterized by an eczematous dermatitis as well as purpuric reactions. The intravesical installations with mitomycin C have been stopped. The patient's skin problems (the purpura as well as the eczema) have completely resolved and have not recurred.
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2/14. A case of acute graft-versus-host disease following autologous peripheral blood stem cell transplantation.

    A 42-year-old woman developed severe erythema with exfoliative scaling on the bilateral palms and soles and erosive dermatitis on the axillae and groin eight days after an autologous peripheral blood stem cell transplantation for the treatment of non-Hodgkin's lymphoma. She also developed exanthema; however she did not show intestinal, hepatic, or renal involvement. The skin biopsy revealed characteristic apoptotic cell death of the epidermis with eosinophilic necrosis, and she was diagnosed with acute graft-versus-host disease (GVHD). The cutaneous lesions responded to topical corticosteroid treatments and improved within a month without systemic immunosuppressing therapies. The cutaneous GVH reaction did not recur. However, she was treated with an intermittent thrombocyte transfusion because of persistent thrombocytopenia. On day 130, she developed intestinal pneumonia and died due to respiratory dysfunction. Unlike an allo-bone marrow graft, GVHD after an autologous stem cell transplantation is not common. Even for an autologous transplantation, GVH may develop with less characteristic clinical manifestations.
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3/14. Keratosis lichenoides chronica: report of a case developing after erythroderma.

    A 66-year-old male presented with keratosis lichenoides chronica after a presumed drug-induced erythroderma. After resolution of the erythroderma, slightly scaly erythematous and violaceous papules in a reticular arrangement over the trunk and limbs developed in association with hoarseness, palmoplantar keratoderma, onycholysis and subungual keratosis. histology from a lichenoid lesion showed pseudo-epitheliomatous hyperplasia, hyperorthokeratosis, parakeratosis, dyskeratosis, neutrophil exocytosis and focal vacuolar degeneration of the basal layer of the epidermis. There was a band-like chronic inflammatory infiltrate in the upper dermis. The skin improved with prednisone 40 mg/day for 15 days, leaving atrophic hypopigmented scars. A diagnosis of keratosis lichenoides chronica was made.
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4/14. Erythrodermic cutaneous T-cell lymphoma with disseminated pustulosis. Production of high levels of interleukin-8 by tumour cells.

    Interleukin (IL) -8 is a neutrophil chemoattractant cytokine with proinflammatory and growth-promoting activities, which is involved in the pathogenesis of several inflammatory diseases. It is found in high amounts in lesional biopsies of pustular diseases such as psoriasis and palmoplantar pustulosis. We report a 50-year-old woman with a 10-year history of erythroderma with disseminated pustulosis. skin biopsies showed an epidermotropic infiltrate composed of atypical CD4 CD8 lymphocytes with numerous admixed neutrophils. Peripheral blood flow cytometric analysis revealed a major clonal subset of CD3 CD4 CD8 T-cell receptor Vbeta22 atypical lymphocytes. bone marrow biopsy, lymph node biopsy and computed thoracoabdominal tomography were normal. Serologies for human T-cell lymphotropic virus type I and human immunodeficiency virus were negative. Our patient's status deteriorated despite topical (nitrogen mustard, psoralen plus ultraviolet A) and systemic (interferon, methotrexate, multiagent chemotherapy) treatments, and she finally died. We showed that our patient's peripheral blood lymphocytes (PBL) spontaneously produced high amounts of IL-8. In contrast, PBL of patients with classical sezary syndrome produced lower amounts of IL-8. The production of IL-8 by tumour T cells could explain this unusual clinical and histopathological presentation of cutaneous T-cell lymphoma as disseminated pustulosis.
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5/14. sarcoidosis in a child, presenting as an erythroderma with keratotic spines and palmar pits.

    A case of sarcoidosis with generalized erythroderma, exfoliation and micropapule formation in a 6-year-old boy is presented. Follicular spiny keratoses, resembling those seen in pityriasis rubra pilaris, and palmo-plantar pitting were among the other extraordinary features in this case. The epidermis overlying the widespread sarcoid granulomata showed parakeratosis, lymphocytic infiltration and degeneration of the basal layer.
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6/14. Unusual cutaneous manifestations of sarcoidosis.

    The diagnosis of sarcoidosis is best established when there is evidence of granulomatous involvement in two or more organs. Three patients with unusual cutaneous manifestations of sarcoidosis, including palmar and follicular lesions, nonscarring alopecia, and widespread plaque or erythrodermic sarcoidosis, are reported. In all three patients the skin biopsy was important in establishing the diagnosis.
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7/14. 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.
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8/14. A new type of erythrokeratoderma.

    We describe a Dutch man suffering from a previously undescribed erythrokeratoderma associated with palmoplantar keratoderma and circular constrictions of the fingers. No mutations were identified in the genes encoding loricrin, connexin 26, 30, 30.3, 31 and 31.1, and ARS/complex B. There are some similarities between the disorder described here and other palmoplantar keratodermas and erythrokeratodermas, but assignment to a particular disease category is not possible. Hence we propose that we have delineated a novel type of keratoderma.
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9/14. 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.
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10/14. Psoriatic erythroderma associated with enalapril.

    A 59-year-old man with a 35-year personal and positive family history of psoriasis was admitted to our department for treatment of psoriatic erythroderma. The patient had commenced therapy with enalapril 10 mg b.i.d. for the treatment of hypertension approximately 6 weeks before hospitalization. Five weeks after the initiation of enalapril, his psoriasis began to flare, and for a period of about 1 week it reached the extent of erythroderma. The patient did not associate the psoriatic flare with other factors such as infections, trauma, or stress. The patient presented with diffuse erythema and pronounced desquamation covering his entire trunk, scalp, and extremities (Figure). Nearly 100% of the body surface area was involved. The palms and soles were also affected, displaying erythema, hyperkeratosis, and painful fissures. The nails showed pits, oil spots, and subungual hyperkeratosis. The patient also had psoriatic arthritis affecting the interphalangeal joints of his fingers. Laboratory tests revealed an elevated erythrocyte sedimentation rate, an elevated creatinine level of 180 mmol/L, a blood urea nitrogen level of 10.8 mmol/L, and a uric acid level of 716 mmol/L. urinalysis showed proteinuria of 1.5 g/24 h. The patient's renal condition was diagnosed as chronic tubulointerstitial nephritis, most probably related to his dermatologic disease. allopurinol and dietary measures were recommended. Following treatment with methotrexate and replacement of enalapril therapy, the erythema and scaling gradually subsided and became confined to his pre-eruptive chronic plaques (approximately 5% of body surface area). Rechallenge with enalapril was not performed.
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