Division of Dermatopathology, Department of Pathology, Massachusetts General Hospital, Harvard Medical School, and Harvard Vanguard Medical Associates, Boston, MA, USA
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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, it was found to be a 3 cm × 4 cm, raised, light and dark gray variegated lesion with fissures, crusted bloody scale, and necrosis. There 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 proliferation was associated with numerous eosinophils. Histological features and immunohistochemical phenotype, including expression of CD1a 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 several-year 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 visceral lesions, lytic bone lesions, or other signs of systemic involvement by Langerhans cell histiocytosis. The scrotal lesion was re-excised and the patient remains disease-free more than 1 year after the diagnosis.