Metastatic umbilical carcinoma: the Sister Joseph's nodule.

Metastatic umbilical carcinoma has been referred to by generations of physicians as Sister Joseph's nodule. Though not common, this characteristic lesion is important to recognize and properly evaluate. We present a case of an eighty-two-year-old woman with a Sister Joseph's nodule due to an unknown primary carcinoma, and we review the diagnostic and prognostic features of umbilical metastases.

Mandibulofacial dysostosis (Treacher Collins syndrome): a case report.

Mandibulofacial dysostosis, also known as Treacher Collins syndrome, is a rare congenital anomaly that must be identified in infancy to prevent irrevocable developmental impairment. Information is sparse in the current medical literature concerning this rare syndrome. This article reports a case of Treacher Collins syndrome with the presence of a scarring alopecia and acne keloidalis nuchae, which are possibly coincidental symptoms, but have not been previously described clinically in this malady.

Genital benign chronic pemphigus (Hailey-Hailey disease) presenting as condylomas.

BACKGROUND: Genital lesion sof benign chronic pemphigus (FBCP)(Hailey-Hailey disease) may present as verrucous papules. Genital warts and papular acantholytic dyskeratosis of the genitalia may be considered in the differential diagnosis. OBJECTIVE: Our purpose was to describe the clinical characteristics and histologic features of verrucous anogenital FBCP. METHODS: Six patients, five women and one man, with verrucous anogenital lesions of FBCP, initially diagnosed as warts, were examined and biopsy specimens were evaluated histologically. RESULTS: The lesions were located exclusively in the perineal and perianal regions with axillary involvement in one case. Family history was negative in three of the six cases. Histologic examination confirmed the diagnosis of FBCP but was significant for the absence of crusts and the presence of only minimal inflammation. CONCLUSION: Our cases and the literature are reviewed with the conclusion that all verrucoid genital lesions with the histologic characteristics of Hailey-Hailey disease may represent FBCP.

Split-thickness skin grafting in extensive ulcerative sarcoidosis.

A 64-year-old white male had extensive ulcerative cutaneous sarcoidosis of the lower extremities. After stabilization with methotrexate and prednisone therapy, the ulcers were debrided and covered with split-thickness skin grafts. An 80% graft acceptance was maintained 2 months later. Successful grafting of ulcerative sarcoidosis has not been reported previously.

Histopathologic changes in condylomata acuminata after application of podophyllum.

The histologic changes of podophyllin-treated condylomata acuminata were studied at 24-hour, 48-hour, and 7-day intervals. The 24-hour specimens had scattered individual atypical cells, whereas 48-hour specimens demonstrated small focal areas of atypia. Seven-day specimens exhibited very mild atypia of cells. All treated specimens had inflammation-necrosis ranging from scattered inflammatory cells to degeneration of the squamous epithelium.

Emotional eccrine sweating. A heritable disorder.

A family with hereditary emotional hyperhidrosis is described. The inheritance pattern is autosomal dominant. A simple quantitative palmar sweat test was used to objectively confirm historical data. Of two family members tested, both had a marked decrease in palmar sweat secretion during administration of diltiazem, a calcium-channel blocker. Additional studies in a large group of patients are needed to extend this observation.

Oral hairy leukoplakia. A distinctive marker of human T-cell lymphotropic virus type III (HTLV-III) infection.

Oral hairy leukoplakia (HL) is a newly described lesion occurring principally on the lateral borders of the tongue in immunosuppressed homosexual men infected with human T-cell lymphotropic virus type III (HTLV-III). Clinically, HL appears as a slightly raised, poorly demarcated lesion with a corrugated or "hairy" surface. Histologically, the lesion is characterized by keratin projections on the surface (which often resemble hairs), parakeratosis, and acanthosis. In addition, large pale-staining cells with pyknotic nuclei are seen in the upper stratum malpighii, which appear similar to the koilocytes described in uterine condylomata. Candida organisms are frequently observed on the lesion surface. Little, if any, subepithelial inflammation is present. Human papillomavirus and Epstein-Barr virus have been identified in biopsy specimens from lesions of oral HL. The association of this lesion in patients with HTLV-III infection has been established. We saw a patient with HTLV-III infection and HL, in whom the immunochemical and ultrastructural findings revealed the presence of a mixed viral infection. Because oral HL may be of diagnostic value as an early indicator of HTLV-III infection, awareness of its characteristic clinical, histologic, immunochemical, and ultrastructural features is important.

Pigmentary demarcation lines: a population survey.

An examination of 380 patients was accomplished in order to determine the frequency of occurrence of pigmentary demarcation lines in an unselected population. Both white and black patients were included in the study, and there was an equal sex distribution. Separation of data by age groups enabled us to determine that the age of onset is in early childhood in the majority of cases. Seventy-nine percent of black female adults have at least one type of pigmentary demarcation line, with types A and B being present in over 50% of the cases. Seventy-five percent of black male patients had at least one pigmentary demarcation line, with type C being most prevalent. Fifteen percent of white female patients had one pigmentary demarcation line. Finally, it was determined that seven of fifty black women (14%) had the new appearance of type B lines with pregnancy.

Fibrofolliculomas, trichodiscomas, and acrochordons: the Birt-Hogg-Dubé syndrome.

The Birt-Hogg-Dubé syndrome is a triad of cutaneous lesions including multiple fibrofolliculomas, trichodiscomas, and acrochordons. The inheritance is autosomal dominant in nature. We present a family of three with multiple firm, skin-colored papules in whom the three types of lesions are documented. In addition, we discuss the clinical differential diagnosis of multiple firm, skin-colored papules.

Multiple hamartoma syndrome (Cowden's disease) associated with non-Hodgkin's lymphoma.

A 70-year-old woman with the multiple hamartoma syndrome is described. Diagnosis was based on the clinical presentation and histopathologic examination of cutaneous trichilemmomas. The case is reported to document the association of Cowden's disease with non-Hodgkin's lymphoma. This is the first known report of the occurrence of trichilemmomas in the sacral area. The literature concerning the association of the multiple hamartoma syndrome with malignancy is reviewed.

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.