As well as malignant melanoma and melanocytic nevus, nonmelanocytic pigmented epidermal tumors also contain benign symbiotic melanocytes and synthesize melanin. This paper reviewed the clinicopathologic features of the pigmented epidermal tumors of the skin. Symbiotic melanocytes, including both normal and pigment blockade melanocytes, were observed in these tumors. Pigmentation of the tumor depended on the symbiotic melanocytes, epidermal tumor cells containing melanin, necrosis masses, and melanophages within the tumor or the dermis. These symbiotic melanocytes derive from the peripheral epidermis or appendages of skin around the tumor. Many factors, including ultraviolet-B, cytokines, and hormones, promote melanocyte motility and are related to the histogenesis of the pigmented epidermal tumors.
A 48-year-old female patient with hyperthyroidism had been receiving propylthiouracil (PTU) for the last 13 years. She developed hemoptysis and dyspnea 4 years after the beginning of PTU therapy (300mg/day). She manifested hematuria, occult blood in stool and severe anemia. RA-test and anti-nuclear antibody (homogenous and speckled) were positive. Soon after, she developed sudden deafness. Episcleritis/scleritis had been present when she received PTU more than 150mg/day. She had received 300mg/day of PTU again for the previous 1.5years, and she developed fever of unknown origin, anemia, and intestinal bleeding. Hematuria was also recognized. Purpura and ecchymosis of lower extremities were significant. They showed leukocytoclastic vasculitis of arterioles, venules, capillaries, and small arteries. She complained of arthralgia and myalgia. Ten months later, she developed hemosputum and her MPO-ANCA was 406EU/ml. Withdrawal of PTU resolved all symptoms. Among the previously reported 45 cases of ANCA-associated vasculitis caused by anti-thyroid drugs, 44% manifested skin lesions. Most of the cases developed crescentic glomerulonephritis and/or pulmonary hemorrhage similar to microscopic polyangiitis. There are two types of vasculitis induced by PTU ; ANCA-associated vasculitis and immune complex-mediated cutaneous leukocytoclastic vasculitis. A common mechanism is speculated for the formation of ANCA and anti-nuclear antibody.
In patients with primary cutaneous malignant melanoma on an extremity, Sentinel lymph node biopsy (SLNB, lymphatic mapping and selective lymphadenectomy) has been widely adopted for the determination of the indications for elective lymph node dissection (ELND). However SLNB is rarely applied for patients with melanoma located on head and neck, because of the complicated and variable lymphatic drainage in this area. We experienced a successful SLNB in a 69-year-old woman with a black papule in a dark brown macule on her right lower eyelid diagnosed as lentigo maligna melanoma. Preoperative lymphatic mapping using a hand-held gamma probe after the injection of 99mTc-Sn colloid clearly showed a sentinel lymph node located only at the submandibular lesion, and intraopetative SLNB was easily carried out by the blue dye method. Our successful trial shows that it is possible to usefully apply SLNB for the evaluation of ELND for head-and-neck melanoma.
A total of 170 Japanese psoriatic patients treated with topical PUVA in our department from 1990 to 2000 were examined for cutaneous carcinogenesis. The mean number of PUVA treatments and average cumulative UVA doses were 196.3 and 445.3 J/cm2, respectively. Five patients with skin cancer and precancerous lesions were found : 2 patients with basalioma, 2 with actinic keratosis, and 1 with Bowen’s disease. No metastasis and recurrence were observed after resections. Four patients had received more than 400 cumulative treatments of topical PUVA. This report suggests that topical PUVA treatment is probably not highly carcinogenic in Japanese patients if we treat them with topical PUVA less than 400 times or 1, 000 J/cm2.
Questionnaires about Tsutsugamushi disease surveillance were sent to 105 dermatologists in Kumamoto prefecture at the end of January 2001, 69 (65.9%) responded. Nineteen (27.5%) dermatologists knew about the existence of the infectious disease surveillance system, and 18 (26.1%) knew that Tsutsugamushi disease is one of the notifiable diseases. In 2000, 19 cases of Tsutsugamushi disease were diagnosed by 12 dermatologists, but only 11 (57.9%) cases were reported. Only one dermatologist took the opportunity to see the data of the infectious disease surveillance on the website. Twenty-two people saw the surveillance data in newspapers or bulletins.
Case 1 was a 40-year-old man who developed prurigo-like papules on his extremities in February of 1988. Two years later, he noted that the skin above the bilateral temporal arteries was swollen and reddish without clinical signs. He continues to have peripheral eosinophilia (30~54%), high titer of IgE (20,529g/l), and thrombocytopenia (6.7×109/l). Case 2 was a 51-year-old man who noted prurigo-like papules on his legs in 1999. After a year, he also developed a large indurated erythema on his left thigh and the swollen and reddish skin above the bilateral temporal arteries without clinical signs. He has peripheral eosinophilia (19~54%) and a high titer of IgE (5,405~11,510g/l). The skin biopsies of Cases 1 and 2 both showed eosinophilic vasculitis within the deep dermis and subcutaneous tissue. The biopsies taken from the temporal region in both cases showed that the lumen was obstructed and the internal elastic lamina was broken by infiltrated eosinophils and neutrophils. In these cases, the clinical, serological, and pathological findings were unusual, because temporal arteritis usually has severe symptoms without skin lesions, hypereosinophilia, or a high titer of IgE. Therefore, we suggest that these cases be diagnosed as juvenile temporal arteritis with eosinophilia as proposed by Fujimoto M. et al.
The patient was a 69-year-old man afflicted with a malignant hemangioendothelioma (MHE) of the scalp. We tried to treat him using rIL-2 (total dose, Celeuk® 118,000,000 JRU, Imunace® 4,550, JRU), but he ultimately died of dyspnea caused by intra-thoracic metastasis of the MHE. In the course of treatment, pleurodesis with rIL-2 and bleomycin was effective in treating the intractable pneumohemothorax caused by the metastasis of the MHE. Although the efficacy of rIL-2 has been confirmed in localized MHE, there are no effective therapies for MHE metastasis. The application of pleurodesis seemed to significantly improve the patient’s QOL.
One family with mastocytosis was reported. A 5-year-old boy had systemic brown pigmented macules and papules. Of his five siblings, an elder sister, a younger sister, and a younger brother have similar lesions. Those lesions were urticated by rubbing (Darier’s sign). Histopathological observation revealed a dense infiltration of mast cells in the upper dermis as evidenced by metachromasia with toluidine blue staining. Therefore, we diagnosed this case as mastocytosis. Familial mastocytosis is very rare and only about 50 familial examples have been reported in the world literature. We reviewed five cases of familial mastocytosis reported in Japan from 1941 to 1981.