Joseph McGuire

Isotretinoin treatment of Darier's disease

A total of 104 patients with Dariers disease were treated with 13- cis -retinoic acid (isotretinoin) at nineteen medical centers. Clinical evaluation by both physicians and patients indicated that the drug is very effective in alleviating Dariers disease but does not produce prolonged remissions.

Treatment of burns with skin substitutes

More than 2 million persons sustain thermal injuries in the United States annually (Monafo and Crabtree, 1985) and more than 10,000 burn victims die (Collini and Kealey, 1989). The principal factors affecting mortality are the total area burned and the area of third degree (full thickness) burns (Tompkins et al., 1985) with wound sepsis being the leading cause of mortality. Early aggressive excision and immediate covering of the wounds improve survival (Herndon and Parks, 1986). Various biological and synthetic substrates have been employed to replace the injured skin. Most of these provide a permeability barrier which substitutes for the epidermal function of the lost skin. An ideal skin replacement should also provide a substitute for dermis, which provides both support and stability for the epidermal replacement and prevents wound contraction. The dermal and epidermal replacement should be firmly integrated by a complete basement membrane zone (BMZ).

Cutaneous angiosarcoma as a second malignancy in a lymphedematous leg in a Hodgkin's disease survivor

Angiosarcoma is a rare and highly malignant neoplasm. Cutaneous angiosarcoma not associated with postmastectomy lymphedema is rare. There are only eight reported cases of development of cutaneous angiosarcoma in a lymphedematous extremity in a patient with preexisting malignancy other than breast cancer. This is the first reported case of development of cutaneous angiosarcoma in a lymphedematous extremity in a Hodgkins disease survivor. A review of cutaneous angiosarcoma associated with lymphedema and a discussion of the pathogenesis of angiosarcoma are included.

EPITHELIAL DIFFERENTIATION IN THE ABSENCE OF EXTRACELLULAR MATRIX

SummaryTo investigate the regulation of epithelial differentiation, normal human epidermal keratinocytes were cultured floating on the surface of culture medium without attachment to a solid substrate. Keratinocytes spread out on the surface of the medium, proliferated and differentiated either into several flat lacy sheets 1 to 3 cells thick (on medium containing 0.15 mM calcium) or formed one single aggregate of cells from 5 to 15 cells in thickness on medium containing 1.15 mM calcium. The cell aggregates demonstrated a pattern of ordered epithelial differentiation. Levels of progressive differentiation resembling the structure of normal human epidermis were identified by light microscopy, immunohistochemistry, and electron microscopy. Differentiation proceeded from cells at the air side toward cells at the medium side with basal appearing cells on the air side and keratinocytes expressing filaggrin and involucrin on the side toward the medium. These results demonstrate that organized epithelial differentiation can occur in the absence of extracellular matrix. In contrast with other air-liquid interface cultures, epithelial differentiation in the absence of extracellular matrix progresses from air towards medium.