The patient was a 77-year-old male former smoker, with history of several basal cell carcinomas (BCCs) in sun-protected areas around the waistline, who presented with another small ulceration on the anterior right upp...
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The patient was a 77-year-old male former smoker, with history of several basal cell carcinomas (BCCs) in sun-protected areas around the waistline, who presented with another small ulceration on the anterior right upper scrotum near the base of the penis. BCC was suspected clinically and the lesion was treated with cryosurgery. The tumor recurred, became raised, and began to bleed. On examination, itwas found to be a 3 cm ×4 cm, raised, light and dark gray variegated lesion with fissures, crusted bloody scale, and *** were no other penile, testicular, or scrotal lesions or masses. There was no regional lymphadenopathy. An excisional biopsy was performed. It showed nodular BCC surrounded by a cellular proliferation of round histiocytic cells with convoluted, lobulated and reniform nuclei and abundant cytoplasm (Fig. 1). The histiocytic proliferationwas associatedwith numerous eosinophils. Histological features and immunohistochemical phenotype, including expression of CD1 a and S-100 antigens by histiocytic cells, were all consistent with Langerhans cell histiocytosis. As expected, BCC expressed keratin. The patient had no history of exposure to ionizing radiation, chemotherapy, immunosuppressive medications, prior lymphoma or other malignancy. However, he spent 4 years on a ship loading coal into the furnace of a steam engine, during which he slept in adjacent quarters that were covered with coal dust. Additionally, he had a severalyear history of occupational skin exposure to machine oil, oil refinery waste, sulfur waste, hydraulic fluid, and asbestos. He also reported a history of nude sunbathing His current medical problems include pleural asbestosis, poorly controlled type II diabetes, and congestive heart failure. His past dermatologic history includes intertrigo, onychomycosis, and an unknown skin lesion removed from the face 40 years ago. Recent clinical follow-up and imaging studies, including chest X-ray and bone scan, showed no evidence of visce
We analysed data of 96 infants (under 1 year of age) with haemophagocytic lymp hohistiocytosis (HLH) from the registry of an HLH study conducted during 1986-2 002 in Japan. The cases were classified into five groups. ...
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We analysed data of 96 infants (under 1 year of age) with haemophagocytic lymp hohistiocytosis (HLH) from the registry of an HLH study conducted during 1986-2 002 in Japan. The cases were classified into five groups. The diagnosis of famil ial HLH (FHL) as group 1 (n =27) wasmade with positive family history and/or rec ent molecular test for perforin and Munc13-4 mutations. Neonatal enterovirus-o r herpes simplex virus-associated HLH as group 2a (n =7), Epstein-Barr virus- associated HLH (n =12) as group 2b, adenovirus-or cytomegalovirus-associated H LH as group 3 (n =9)were mostly diagnosed by viral isolation or by the detection of viral genome. Juvenile rheumatoid arthritis-associated macrophage activatio n syndrome was classified as group 4 (n =4) and the remaining without known trig gers as group 5 (n =37). The peak onset age was 1-2 months for group 1, 1-2 we eks for group 2a, 12 months for group 2b, none for group 3, 9 months for group 4 and 2 months for group 5. Future novel diagnostic measures are required to defi ne the precise nature of HLH in group 5. Conclusion:These data may provide usefu l information for neonatologists/ paediatricians in the differential diagnosis o f haemophagocytic lymphohistiocytosis in early infancy.
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