Reiko Nogami

A case of progressive systemic sclerosis associated with sarcoidosis and esophageal adenocarcinoma.

A 50‐year‐old Japanese female with progressive systemic sclerosis (PSS, CREST syndrome) is reported. During treatment for PSS, she was diagnosed by clinical and laboratory findings as having sarcoidosis, which was confirmed by histological examination of the skin and lymph nodes in July of 1991. She complained of back pain in August of 1991. Reflux esophagitis and Barretts esophagus, found by endoscopy, progressed into a well‐differentiated tubular adenocarcinoma. This is a very rare case of PSS associated with sarcoidosis and esophageal adenocarcinoma developing from Barretts esophagus.

Febrile Ulceronecrotic Mucha‐Habermann's Disease

Febrile ulceronecrotic Mucha‐Habermanns disease (FUMH) was first described by Degos in 1966. In the literature, nine cases of FUMH have been reported in both children and adults. We report a 16‐year‐old boy with the febrile ulceronecrotic type. A review of the nine cases in the literature showed acute necrotic lesions, as well as rare complications such as fever, superinfected lesions and viral infection which are not as common in pityriasis lichenoides et varioliformis acuta. There is no definitive treatment, but systemic corticosteroid, methotrexate, antibiotics (tetracycline, erythromycin), aciclovir, and 4,4‐diaminodiphenyl sulfone (DDS) have been frequently used. The most common histologic feature is mononuclear perivascular infiltrates consisting of T lymphocytes. The etiology is not known, but a hypersensitivity reaction, possibly to an infectious agent, is suggested.

Complicating systemic amyloidosis in dystrophic epidermolysis bullosa, recessive type

&NA; An autopsy case of dystrophic epidermolysis bullosa, recessive type, complicated by systemic secondary amyloidosis is described. The patient had developed multiple bullous lesions and erosions from birth, followed by repeated infection. At autopsy, chronic persistent inflammation was observed in the skin and in various visceral organs, accompanied by systemic amyloidosis. By the peroxidase‐antiperoxidase (PAP) method, amyloid deposits stained positively for anti‐AA‐ protein antiserum. In the present case, we concluded that the systemic amyloidosis was of the AA type, and developed secondarily to the chronic persistent inflammation in the prolonged course of dystrophic epidermolysis bullosa, recessive type.

A Case of CD8+ T Cell Lymphoma Occurring during Treatment for In situ Malignant Melanoma of the Palate

A 53‐year‐old Japanese male noticed pigmented lesions on his right upper gingiva and hard palate in February of 1986. Histological examination revealed in situ malignant melanoma. Chemotherapy, β‐interferon, and oral BCG were given. However, tumors subsequently developed in the nasal cavity in March of 1989. The patient died in April of 1990 after developing Garcins syndrome and the syndrome of inappropriate secretion of antidiuretic hormone (SIADH). Autopsy revealed aggressively infiltrating, whitish tumor masses invading the hard palate, the nasal cavity, the paranasal sinuses, the base of the skull, cranial nerves I‐X, and the pituitary body, as well as severe necrosis of the soft palate. However, there was no evidence of malignant melanoma. Instead, these oval tumor cells had atypical nuclei and scanty cytoplasm. They contained no melanin granules, were negative for S‐100 protein, and were also negative for various melanoma‐associated antigens. They were positive for CD2, CD3, and CD8 by avidin‐biotin‐peroxidase complex immunohistochemistry. It was concluded that the patient had CD8+ non‐Hodgkins malignant lymphoma (diffuse, large cell type) of the nasopharyngeal region, which was preceded by in situ malignant melanoma of the palate.

BCC-associated amyloidosis with a peculiar pattern of deposition.

An 83‐year‐old Japanese woman with lepromatous leprosy had been treated in a leprosarium. More than 10 years ago, she developed a dome‐like brown tumor on the dorsum of the nose which showed the histology of basal cell carcinoma. The lesion was a well bordered, rounded tumor with tumor cell nests on its periphery extending toward the center as cords of tumor nest and with amyloid depositions between the cords. The tumor nests and amyloid lessened toward the center of the tumor, being replaced by collagen fibers. Amyloidosis showing this peculiar pattern of transition was reported with a review of published cases.

Glycosaminoglycan Content in the Media of Cultured Dermal Fibroblasts Derived from Burn Scar and Normal Skin

The glycosaminoglycan (GAG) content of the extracellular matrix of burn scar in humans has been reported to differ from that of normal skin (1, 2). In order to investigate whether the GAG content altered as a result of functional changes in fibroblasts, the GAG content was determined in culture media of fibroblasts derived from growing burn scar, mature scar, and normal skin tissue. No statistical differences were observed in the population doubling‐times of scar and normal skin. Mature scar showed significantly higher values for all the concentrations of uronic acid, hexosamine, and sulfate measured in the glycosaminoglycan, as compared with normal skin values, and the concentrations from growing scar were slightly higher than those for normal skin. The above results may suggest an increase in glycosaminoglycan sulfate synthesis following the hyperplasia of the matrix in burn scar tissue.

Scleroderma-like changes in a patient with insulin-dependent diabetes mellitus.

A 38‐year‐old Japanese woman with diabetes showed sclerosis of the hands, a short sublingual frenulum, and hypomotility of the esophagus. She had been on insulin therapy for insulin‐dependent diabetes mellitus since the age of 15. For the previous one year, the proximal interphalangeal joints had become painful and swollen with sclerosis of the hands. Although these findings are also seen in PSS, this case was assumed to be due to diabetes mellitus. The patient had a high serum level of HbAlc, which may reflect a poor control of diabetes and non‐enzymatic glycosylation of collagen fibers, resulting in an accumulation of collagen in the dermis.

A Case of Dermatomyositis Associated with Pseudomyxoma Peritonei Originating from Mucinous Adenocarcinoma of the Appendix

A 69‐year‐old Japanese woman with erythema, severe edema on the face, Gottrons papules and poikiloderma was diagnosed as having dermatomyositis. She also noticed muscle weakness in her extremities, although her electromyogram showed neurogenic patterns. Her levels of CA19‐9 and CEA were elevated and a CT of her abdomen revealed a giant, multilobular, cystic lesion in the pelvis. This tumor was diagnosed as pseudomyxoma peritonei originating from a mucinous adenocarcinoma of the appendix.

Squamous‐cell Carcinoma Arising in Solar Keratosis of a Leprosy Patient: Relationship with Positive Skin Smear Examination

A 70‐year‐old Japanese female with lepromatous leprosy developed squamous‐cell carcinoma within a long standing area showing solar keratoses on her head. Alopecia had been present at least for the preceding 34 years on her forehead and frontal area of the head, and solar keratoses had existed at least for the previous 9 years. Skin smear examination for acid‐fast bacilli which had continually been negative for the previous 2 years (during which 8 examinations were done) became positive. We assume that there may be some relationship between the onset of the tumor and the positive skin smear examination, although the exact connection remains unclarified.

Two Cases of Genital Paget's Carcinoma. An Assessment of the Management for Aged Patients with Paget's Carcinoma.

外陰部Paget癌の2例を経験した。症例1は82歳の男性。陰茎周囲に腫瘤を形成しており, 皮膚·筋肉·骨·リンパ節·肝臓への浸潤転移を認めたため局所治療のみを施行した。症例2は93歳の男性。陰茎周囲から下腹部にかけて広範囲に色素斑と糜爛面を認めた。遠隔転移はなく広範囲切除術を施行した。外陰部Paget癌の治療についての考察を含めて報告した。