Urticating Hashimoto-Pritzker Langerhans cell histiocytosis.

Red-brown papules developing during the neonatal period may present a diagnostic dilemma. The Darier sign has been a reliable feature in the diagnosis of mastocytosis. However, the cutaneous infiltrate of Hashimoto-Pritzker Langerhans cell histiocytosis (LCH) may contain a large number of mast cells, leading to confusion both clinically and histologically. We report an infant who developed red-brown papules of Hashimoto-Pritzker LCH during the neonatal period and presented with a positive Darier sign and acute urticaria.

Silicone auricular prosthesis.

Skin cancer surgery of the ear may result in a large defect of skin and cartilage requiring a staged flap procedure for reconstruction. An alternative to surgical reconstruction is the creation of a silicone auricular prosthesis. These prostheses provide a cost-effective and cosmetically acceptable means of camouflage for patients who decline or postpone surgical reconstruction. The process of creating a silicone auricular prosthesis is discussed.

An analysis of the late effects of radiotherapy in pediatric central nervous system tumors.

PURPOSE: The purpose of this study is to assess the long term results of all pediatric patients diagnosed with central nervous system (CNS) tumors treated with external beam radiotherapy either primarily or postoperatively. MATERIALS: Forty-seven pediatric patients with CNS tumors were treated between 1978 and 1989 with external beam radiotherapy to customized fields using cobalt 60, 4, 6, or 18 MV photons. Daily doses ranged from 1.5 to 2.0 Gray; total doses from 35 to 66 Gray. Ages ranged from one month to 18 years of age, with an average age of 8.2 years. Thirty-nine patients survived more than 12 months (range 36 to 178 months; median 62 months) and were included for analysis. Ten of 39 (25.6%) patients experienced Grade I to II complications and six patients had (15.7%) Grade III to IV complications for an overall incidence of 41%. The influence of field size, radiation dose, chemotherapy, age, and extent of surgical resection upon long-term complications were studied. CONCLUSION: Radiation therapy for pediatric CNS malignancies is associated with long-term complications. Significant long-term complications can be limited to an acceptable level of approximately 15%.

Retrospective analysis of patients with primary fallopian tube carcinoma treated at the University of Louisville.

Medical records of patients diagnosed with primary fallopian tube carcinoma between 1979 and 1989 were reviewed. Twenty-six patients were eligible; 8 patients were excluded after pathologic review, leaving 18 patients included in the study for this analysis. The median and mean age were 61 and 59 years, respectively, with a range of 39-80 years. There were three Stage I, five Stage II, seven Stage III, and three Stage IV patients. The most common presenting symptoms were abdominal/pelvic pain, abdominal distension, and vaginal discharge/bleeding. The primary site of the lesion was determined to be the right tube in 44% of the cases, the left tube in 39% of the patients, bilateral lesions in 11% of the patients, and indeterminate in 6%. Histologic grade was poorly differentiated (Grade III) in 13 patients, moderately differentiated (Grade II) in 4 patients, and well differentiated (Grade I) in one. No patient was correctly diagnosed preoperatively. Survival at 5 years of the entire group was 35% with a 3 year minimum followup. Corresponding disease free survival was 30%. Mean and median survival times were 74 and 37 months, respectively. The range of survival times was from 1 to 120 months. All Stage I patients, 80% (4/5) of Stage II, and 29% (2/7) of Stage III patients are alive without disease. None (0/3) of the Stage IV patients are alive. Treatment regimens consisted of intraperitoneal P-32, external beam radiotherapy, and/or chemotherapy. Radiotherapy was associated with a low incidence of treatment-related complications, the majority being gastrointestinal related. There was one chemotherapy-related death. These patients and their treatment outcomes add to the data base of numerous previous reports on fallopian tube carcinoma. Stage I and II patients fared excellently with primary surgical and adjuvant therapy. While the prognosis of Stage III and IV patients is much worse, significant levels of long term survival can be achieved with aggressive treatment.

Perilesional linear atrophy and hypopigmentation after intralesional corticosteroid therapy. Report of two cases and review of the literature.

We report on the cases of two patients in whom linear perilesional hypopigmentation and atrophy developed after intralesional injection of corticosteroids for treatment of keloids. Evaluation of our patients and the previously reported cases showed that perilesional linear atrophy or hypopigmentation (or both) is a distinct complication after intralesional or intraarticular administration of corticosteroids and is probably due to lymphogenous spread of the steroid suspension.

Adult Kawasaki syndrome.

Kawasaki syndrome (KS) is an idiopathic, acute, febrile, exanthemous illness that primarily affects infants and children. We describe a 20-year-old black woman who fulfilled the clinical criteria for the diagnosis of KS and excluded other possible causes. In addition, we reviewed data on 21 patients with adult KS reported in the English literature and accepted ten cases as representing this syndrome. The epidemiologic, clinical, laboratory, and pathologic features of the 11 cases representing adult KS are discussed. Although the initial reports of adult KS in the United States may have actually represented toxic shock syndrome, the occurrence of KS in adults should be acknowledged.

Syringoma presenting as milia.

We present two unrelated patients with numerous infraocular milium-like lesions that histologically revealed syringoma with many overlying keratin cysts in the papillary dermis. A Fontana-Masson stain revealed no melanin-containing cells in the keratin cyst walls, suggesting that they originated from eccrine ductal structures. A classification of the clinical variants of syringoma is presented.

Bart's syndrome: microscopic, ultrastructural, and immunofluorescent mapping features.

Bart's syndrome has been clinically described as the association of congenital localized absence of skin (CLAS), epidermolysis bullosa (EB), oral mucosal lesions, and dystrophic nails. Transmission occurs through an autosomal dominant gene with complete penetrance but variable expression. It has been difficult to classify this type of EB because of lack of microscopic and ultrastructural studies on affected family members. This is the first report of microscopic, ultrastructural, and immunofluorescent mapping studies of an affected individual with the complete inherited syndrome initially described by Bart. This study is also the first to document the association of CLAS and dominant dystrophic epidermolysis bullosa by histology, electron microscopy, and immunofluorescent mapping. Our two patients and one other affected family member had diminution or absence of a specific basement membrane antigen as defined by immunofluorescence with a monoclonal antibody (KF-1) in perilesional skin.

Leukemia cutis mimicking stasis dermatitis.

A patient with chronic myelogenous leukemia (CML) in blast crisis experienced a peculiar painful eruption on his left lower extremity during the preterminal phase of his disease. The lesion was characterized by tender, edematous, purpuric infiltration which resembled stasis dermatitis clinically. Dermatopathologic examination demonstrated leukemia cutis.

Nodular (keloidal) scleroderma.

Two patients with progressive systemic sclerosis (PSS) developed keloidal-like nodules within areas of thickened skin. This extremely unusual event is most likely a keloidal response to the early inflammatory component of scleroderma in patients who are either genetically at risk for keloid formation or in areas of the skin that have a high predilection for keloid formation such as the chest.