Infundibulocystic basal cell carcinoma of the eyelid in basal cell nevus syndrome.

PURPOSE: To describe the histopathologic findings in a series of eyelid basal cell carcinomas removed from patients with basal cell nevus syndrome. METHODS: Retrospective case series of 5 patients with basal cell nevus syndrome identified from our oculoplastics service. The systemic and ophthalmic features were reviewed, and a retrospective histopathologic analysis of all available previously excised eyelid lesions was performed. The pertinent published literature on basal cell nevus syndrome and eyelid basal cell carcinoma was reviewed. RESULTS: A total of 26 eyelid lesions were examined histopathologically. Twenty-three of these lesions were basal cell carcinomas. The infundibulocystic variant of basal cell carcinoma was identified most commonly (57%). CONCLUSIONS: Eyelid basal cell carcinomas in patients with basal cell nevus syndrome were commonly of the infundibulocystic variety in our series. Infundibulocystic basal cell carcinomas, which can be clinically indistinguishable from the more common forms, are thought to be less aggressive than other types of basal cell carcinoma and are a reassuring histopathologic diagnosis. It is important for the ophthalmologist and pathologist to be aware of infundibulocystic basal cell carcinomas, as they are more common in patients with basal cell nevus syndrome and may be a clue to the diagnosis of this autosomal dominant cancer-predisposition syndrome or other associated syndromes. To our knowledge, this variant of basal cell carcinoma has not been previously discussed in the ophthalmic literature.

Lupus miliaris disseminatus faciei of the eyelids: report of two cases.

Lupus miliaris disseminatus faciei is a rare chronic, asymptomatic papular eruption, most commonly affecting the central face, including the eyelids. Our 2 patients presented with the typical clinical findings of small papular lesions in which histopathologic examinations disclosed granulomatous inflammation with necrosis associated with inflamed hair follicles. Except for its description as acne agminata by Duke-Elder in 1974, to the authors' knowledge, this condition has not been reported previously in the ophthalmic literature.

Reliability of Histopathology for the Early Recognition of Fibrosis in Traction Alopecia: Correlation with Clinical Severity.

Traction alopecia (TA) is hair loss caused by prolonged pulling or repetitive tension on scalp hair; it belongs to the biphasic group of primary alopecia. It is non-scarring, typically with preservation of follicular stem cells and the potential for regrowth of early lesions especially if traction hairstyles are stopped. However, the alopecia may become permanent (scarring) and fail to respond to treatment if the traction is excessive and prolonged. Hence, the ability to detect fibrosis early in these lesions could predict patients who respond to treatment. Histopathological diagnosis based on scalp biopsies has been used as a gold standard to delineate various forms of non-scarring alopecia and to differentiate them from scarring ones. However, due to potential discrepant reporting as a result of the type of biopsy, method of sectioning, and site of biopsy, histopathology often tends to be unreliable for the early recognition of fibrosis in TA. In this study, 45 patients were assessed using the marginal TA severity scoring system, and their biopsies (both longitudinal and transverse sections) were systematically assessed by three dermatopathologists, the aim being to correlate histopathological findings with clinical staging. Intraclass correlation coefficients were used to determine the level of agreement between the assessors. We found poor agreement of the identification and grading of perifollicular and interfollicular fibrosis (0.55 [0.23-0.75] and 0.01 [2.20-0.41], respectively), and no correlation could be drawn with the clinical severity score. Better methods of diagnosis are needed for grading and for recognition of early fibrosis in TA.

Multiple cutaneous and uterine leiomyomas associated with gastric GIST.

BACKGROUND: There are a number of reports documenting familial cases of leiomyomatosis cutis associated with uterine leiomyomata. However, to our knowledge, the association of gastrointestinal stromal tumour (GIST) with this entity has not as yet been reported. We report an interesting case of cutaneous leiomyomatosis, metachronous uterine leiomyomata, and a gastric GIST in a 43-year-old woman. OBSERVATION: The patient had previously undergone two separate uterine myomectomies at ages 25 and 26 years, respectively, followed by a hysterectomy at 27 years. At 36 years she underwent partial gastrectomy for excision of GIST and this was followed by the development of extensive, symptomatic cutaneous leiomyomata at 43 years. In the report, we have documented histological, immunohistochemical and clinical observations and furthermore report on the therapeutic measures undertaken. CONCLUSION: We report an interesting association of cutaneous leiomyomatosis, uterine leiomyomas and GIST.