Cases reported "Chronic Disease"

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1/39. Acral keratotic graft versus host disease simulating warts.

    A 55-year-old man experienced persistent oral graft versus host disease after receiving an HLA-matched allogeneic stem cell peripheral blood transplant for chronic myeloid leukaemia. Twelve months post-transplant, multiple keratotic lesions resembling warts developed on his fingers, on his palms and on the soles of his feet. skin biopsy showed a lichenoid reaction under a hyperplastic and hyperkeratotic epidermis lacking signs of papillomavirus infection. The lesions partially regressed when the patient's oral prednisone was increased. This instance of acral keratotic lesions may represent an unusual expression of chronic graft versus host disease; however, the the hydroxychloroquine which had been used for 8 months to control the patient's oral graft versus host disease cannot be excluded as a cofactor.
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2/39. Pustular psoriasis with a striking linear pattern.

    Pustular psoriasis has different clinicomorphologic forms such as generalized, localized (mainly palmoplantar or acral), and annular. There are also few cases with a linear distribution pattern. We report a case of pustular psoriasis with a striking linear distribution probably related to Blaschko's lines. We suggest that linear pattern should also be considered among the morphologic variants of pustular psoriasis.
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3/39. Myeloma-associated systemic amyloidosis presenting as chronic paronychia and palmodigital erythematous swelling and induration of the hands.

    Mucocutaneous involvement occurs predominantly in primary systemic amyloidosis as well as in myeloma-associated systemic amyloidosis. It is rarely observed in other types of amyloidoses. Signs of such involvement may aid in the early diagnosis of the disease process. Herein, we describe a 64-year-old white male patient with myeloma-associated systemic amyloidosis in whom the disease presented with unique cutaneous lesions consisting of chronic paronychia and palmodigital erythematous swelling and induration of the hands. Following weekly regimens with prednisone (20 mg/day) and melphalan (2 mg/day) administered every 16 weeks, almost complete resolution of the cutaneous lesions was observed after 1 year of therapy. Also, in response to chemotherapy, modest regression of the myelomatous bone lesions and complete resolution of the underlying gammopathy occurred.
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4/39. Chronic recurrent multifocal osteomyelitis associated with ulcerative colitis: a case report.

    Chronic recurrent multifocal osteomyelitis (CRMO) is a rare disease of bone first described by Giedion et al in 1972. It is associated with several pathologic processes including psoriasis, palmoplantar pustulosis, and SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, and osteitis). The only published association of CRMO and Crohn's disease was reported by Bognar et al in 1998. The authors describe the association of CRMO and ulcerative colitis (UC) in a 12-year-old girl. As far as the authors know, this is the first published report of CRMO associated with UC and the second of CRMO associated with inflammatory bowel diseases.
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5/39. Three-headed reversed palmaris longus muscle: a case report and review of the literature.

    The palmaris longus muscle (PLM) is extremely variable both in number and form. We present the first reported case of three-headed reversed PLM in a 36-year old woman. The relevant literature is reviewed. Variations of the muscles of the forearm and specifically the PLM are not uncommon. However, different rates are given for the types and agenesis of PLM. In one study, the incidence of agenesis was 12.8% and other anomalies were 9%. 15 cases of reversed PLM have been reported in the literature. In our case, the three-headed PLM was resected. The patient's symptoms resolved and at 1-year follow-up she remains symptom-free.
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6/39. The definition of trichophyton rubrum syndrome.

    Although chronic widespread dermatophyte infection is reported widely in the literature, neither a uniform nomenclature, nor even a clear definition of this syndrome have been established so far. Thus, we suggest trichophyton rubrum syndrome (TRS) for denomination and define the following obligatory clinical and mycological criteria for TRS. (A) skin lesions at the following four sites: (1) feet, often involving soles; (2) hands, often involving palms; (3) nails; and (4) at least one lesion in another location than (1) (2) or (3), except for groins. (B) Positive microscopic analyses of potassium hydroxide preparations of skin scrapings in all four locations. (C) Identification of trichophyton rubrum by cell culture at three of the four locations at least. For diagnosis of TRS the criteria (A) and (B) and (C) have to be fulfilled. This standardization is a prerequisite for further investigations of underlying mechanisms of this disease. The typical clinical pattern of TRS is illustrated by the presentation of two paradigmatic cases.
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7/39. Chronic tophaceous gout presenting as hyperpigmented nodules in the limbs of a patient with coexisting psoriasis.

    We describe a 53-year-old male renal transplant recipient with hypertension and triglyceridemia, who showed rare manifestations of gout presenting as brownish nodules on the arms and legs as well as chronic tophaceous gouty arthritis of the hands and feet mimicking rheumatoid arthritis, in association with subsequently developed psoriasis of the palms. In elderly Asian men, hypertension and renal insufficiency may be risk factors predisposing to the development of multiple hyperpigmented nodules of tophi in the more proximal extremities.
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8/39. Combined partial deficiencies of carnitine palmitoyltransferase II and mitochondrial complex I presenting as increased serum creatine kinase level.

    Increased serum creatine kinase level is a marker of neuromuscular disorders. When combined with exercise intolerance, muscle cramps, fatigue, myoglobinuria, or muscle weakness, metabolic myopathies of a variety of causes should be considered. We encountered an adolescent male with a persistently high serum creatine kinase level and chronic fatigue who was found to have combined partial defects of carnitine palmitoyltransferase II and mitochondrial complex I. Metabolic myopathy may present with chronic fatigue and a persistently high serum creatine kinase level but without muscle weakness and may be attributable to combined enzyme defects.
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9/39. Cases from the Osler Medical Service at Johns Hopkins University.

    A 37-year-old woman presented with increasing abdominal pain and jaundice. Six weeks before admission, she developed persistent diarrhea and jaundice of the skin. She also bruised easily, and her gums bled. In the subsequent weeks, her appetite decreased, she was fatigued, and she had nausea, vomiting, and abdominal distension. She had a history of drinking 1 quart of vodka every day for 20 years, with brief periods of abstinence; she stopped consuming alcohol 11 days before admission because it no longer provided symptomatic relief. Her past medical history was also notable for depression, including a suicide attempt 4 years earlier. She did not smoke, use illicit drugs, or have unprotected sexual intercourse. She had received no blood transfusions and had not traveled recently. She took no medications, except for occasional ibuprofen.On physical examination, she was thin and deeply jaundiced, and she trembled and responded slowly to questions. She was afebrile but tachypneic, and she had orthostatic hypotension. Her HEENT examination was notable for scleral and sublingual icterus, as well as crusted blood on her gums and teeth. The jugular veins were flat. The cardiac examination revealed tachycardia (heart rate, 103 beats per minute) without murmurs, rubs, or gallops. The abdomen was nontender and protuberant, with hypoactive bowel sounds; the spleen was not palpable, and there was no fluid wave or caput medusae. The liver percussed to 18 cm, with a smooth edge extending 10 cm below the costal margin. She had cutaneous telangiectases on her chest and bilateral palmar erythema. There was no peripheral edema. The neurologic examination was notable for asterixis. Her stool was guaiac positive. Laboratory studies revealed the following values: hematocrit, 21.2%; white blood cells, 17,310/mm(3); ammonia, 42 micromol/L; serum creatinine, 3.9 mg/dL; serum urea nitrogen, 70 mg/dL; albumin, 2.1 g/dL; total bilirubin, 26.8 mg/dL; alanine aminotransferase, 14 U/L; aspartate aminotransferase, 77 U/L; alkaline phosphatase, 138 U/L; prothrombin time, 103 seconds (international normalized ratio, 10.6); and urinary sodium, <5 mg/dL. urinalysis revealed an elevated specific gravity and numerous muddy granular casts. hepatitis a, B, and C serologies were negative. On abdominal ultrasound examination, there was no ascites, and the liver was echogenic. The portal and hepatic veins were patent, and the hepatic arteries were normal. The spleen measured 14 cm.What is the diagnosis?
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10/39. Palmar-plantar keratoderma of Unna Thost associated with atopic dermatitis: an underrecognized entity?

    We report six cases of palmar-plantar keratoderma of Unna Thost (PPKUT) associated with atopic dermatitis. All had typical features of PPKUT with diffuse, yellowish thickening on the palms and soles with a well-defined erythematous rim of demarcation on the sides associated with palmar-plantar hyperhidrosis. The changes were obvious since birth or arose during early life, and were persistent. We believe that the association between the two disorders is not coincidental but an underrecognized entity that may shed light on the underlying pathogenesis of these two conditions.
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