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Hemimaxillofacial dysplasia and segmental odontomaxillary dysplasia appear to be the same syndrome, having the common features of unilateral abnormalities of bone, teeth, gums, and skin. Oral manifestations are the hallmark of this condition. Those affected are generally recognized in childhood and may have partial anodontia, abnormal spacing of the teeth, delayed eruption, and gingival thickening of the affected segment. Reported cutaneous manifestations include facial asymmetry, Becker's nevus, "hairy nevus," lip hypopigmentation, discontinuity of the vermilion border, depression of the cheek, and erythema. The oral lesions do not appear to be progressive. We describe a child with features consistent with hemimaxillofacial dysplasia/segmental odontomaxillary dysplasia. Findings of a biopsy specimen from the cheek confirmed the presence of a Becker's nevus. Cutaneous findings reported in the previous 31 cases are reviewed and summarized. The acronym HATS (hemimaxillary enlargement, asymmetry of the face, tooth abnormalities, and skin findings) is introduced to reflect the spectrum of abnormalities in bone, teeth, and skin that may be seen in this developmental disorder.

Summary There is a relationship between sunlight and the development of melanocytic neoplasms. Because the incidence and excision of melanocytic neoplasms varies according to season, we sought to determine if dysplasia and/or intraepidermal melanocytic expression differed in a cohort of dysplastic naevi (DN) removed in January compared with a similar cohort removed in August. The DN were graded based on the degree of dysplasia, and the number of intraepidermal melanocytes were counted after immunohistochemical staining with HMB-45 and Melan-A. There was no seasonal difference in the grading of the dysplastic naevi in either season (P = 0.08). Comparing 85 cases from August and 86 from January, there was a larger number of Melan-A-positive melanocytes in the August samples (P

Pretreatment risk stratification of basal cell carcinoma (BCC) is largely based on histologic subtype reported from biopsy specimens.We sought to determine the degree of concordance between characteristics identified on biopsy specimen and excision and to determine if histologic characteristics other than subtype correlated with depth of invasion.Histologic specimens of 100 BCC biopsy specimens and corresponding excisions were reviewed. Anatomic site, histologic subtype, maximum depth of extension, contour of the lobules at the leading edge, elastosis characteristics, presence of necrosis, calcification, and ulceration were recorded. Concordance between biopsy specimens and their excisions with relation to depth of tumor lobules was analyzed.The concordance between the subtype of biopsy specimen and excision was 62%. Micronodular tumors had the greatest mean depth, followed by infiltrative, nodular, and superficial subtypes. Subtype reported from biopsy specimen (P = .0002) and excision (P.0001) correlated to depth and was superior to age, contours of excision specimens, the presence of necrosis, and the extent of excisional solar elastosis. Gender, anatomic site, contours of biopsy specimens, elastosis color, elastosis type, the presence of ulceration, and calcification did not correlate with depth.Selection bias is present as only standard excisions were included; BCCs treated by other methods were not examined.BCC subtype identified on biopsy specimen may not correlate with subtype identified on excision. Morphologic subtype has the highest correlation with depth and reporting should reflect the highest risk growth pattern if a biopsy specimen contains more than one pattern. Consideration should be given to reporting necrosis and degree of solar elastosis.

Birt-Hogg-Dube Syndrome is an autosomal dominant condition characterized by a triad of fibrofolliculomas, trichodiscomas, and acrochordons. Since the first description in 1977, many conditions have been described in association with its clinical triad. Recent epidemiological studies have shown a significant association between the occurrence of lesions in the fibrofolliculoma/trichodiscoma category with renal neoplasms and pneumothoracies. The BHD protein folliculin had recently been identified. The histological findings of the clinical lesions are distinctive. We report a patient with a history of melanoma who presented for routine surveillance. Facial lesions in the fibrofolliculoma/trichodiscoma category were identified. Diagnostic work-up revealed concomitant multinodular goiter, pulmonary cyst, and renal mass. The patient later developed pneumothorax. Clinical manifestations, histological findings, associations, management, and a review of the Birt-Hogg-Dube Syndrome are discussed.

background. Infantile digital fibroma (IDF) is a rare benign fibrous tumor of childhood that frequently recurs despite local excision. Conservative, nonsurgical management may result in regression and/or joint deformity. objective. To describe the histologic features of IDF and discuss a case excised using Mohs micrographic surgery (MMS). methods. Case report and review of the clinical, histologic, and ultrastructural features. results. Characteristic inclusion bodies of actin were identified with hematoxylin and eosin, Masson's trichrome, and rapid actin immunostain. The tumor was debulked and the majority was removed after one stage of MMS, except where the deep margin approached the joint space. The defect healed by secondary intention. At 2 years the patient had no recurrence or functional joint deformity. conclusion. MMS is a surgical treatment option for IDF.