Orlando G. Rodman

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.

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.

Bart's Syndrome: Microscopic, Ultrastructural, and Immunofluorescent Mapping Features

Abstract: Barts 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 auto‐somal 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.

Squamous Cell Carcinoma Arising in Porokeratosis of Mibelli

A 54‐year‐old woman had a linear unilateral skin lesion since age 6. Neither her parents nor her five sihlings have similar lesions. Six months prior, an ulcer developed within the abnormal skin at the flexor aspect of the right ankle.

Porokeratosis plantaris discreta

A 22-year-old black male patient developed discrete, exquisitely painful bilateral plantar lesions. Histologic examination revealed a cutaneous groove filled by a keratin plug (cornoid lamella). There is focal absence of granular layer underlying this defect. The differential diagnosis of this lesion, familiar to podiatrists but relatively unknown to dermatologists, is discussed.

An Unusual Congenital Leiomyoma

Abstract: Cutaneous leiomyomas are rarely encountered at birth. In their usual form they appear as discrete dermal papules or nodules with a pink or brown discoloration of the overlying skin. We examined an infant with an unusual mass attached to the right heel at birth. Histologically, the lesion was a leiomyoma. It apparently represented a bizarre proliferative anomaly of smooth muscle occurring during fetal development.

Pigmentary demarcation lines associated with pregnancy

Pigmentary demarcation lines are borders of abrupt transition between more deeply pigmented skin and that of lighter pigmentation. Two patients developed these lines on the lower extremities during pregnancy.

Familial myxovascular fibromas

Multiple verrucous papules were observed in a partial kindred. Histopathologic changes are unique, compatible with myxovascular fibromas, and similar to those recently described.