The sign of Leser-Trélat in a case of adenocarcinoma of the lung.

This is what we believe to be the first report of the sign of Leser-Trélat in association with occult adenocarcinoma of the lung. The sign of Leser-Trélat is proposed as a sign of possible occult malignancy, despite various suggestions to the contrary. Also, it is suggested that a tumor-produced humoral factor (eg, transforming growth factor-alpha [TGF-alpha]) could be responsible for both the acute eruption of the monomorphous seborrheic keratoses and the nearly concomitant development of acanthosis nigricans, which occurred in our case. The possible distinction between a hyperplastic and a neoplastic origin of various types of seborrheic keratosis is discussed in relation to this hypothetical humoral factor. In addition, we suggest a refinement of the definition of the sign of Leser-Trélat and discuss the use of "sign of Leser-Trélat" and "syndrome of Leser-Trélat" in relation to physical findings. All patients with the sign of Leser-Trélat should undergo a thorough evaluation for occult malignancy.

Generalized elastolysis (cutis laxa).

Generalized elastolysis is a rare and unique systemic disorder of connective tissue in which the elastic fibers suddenly become fragmented, disorganized and fewer in number with the resultant production of such entities as cutis laxa, emphysema, aortic aneurysms and bowel diverticula appearing in the organ system involved, that is, skin, lung, vasculature or gastrointestinal tract. Presented here are three cases that, illustrate the typical features of this condition. In addition, 14 more cases were retrieved from the literature and the information reviewed. Various etiologic factors relating to the synthesis and degradation of elastic tissue are discussed in light of recent findings in the biochemistry of connective tissue.

Immunohistochemical studies of the human cutaneous basement membrane-anchoring fibril complex.

Antiserum to the human cutaneous basement membrane-anchoring fibril complex specifically binds to the basement membrane zone of normal human skin as demonstrated by indirect immunofluorescence and, in addition, cross-reacts with normal human glomerulus. This antiserum does not prevent binding of pemphigoid antibody to its specific antigen; thus the antigen of bullous pemphigoid is unlikely to be a component of the cutaneous basement membrane (basal lamina).

The human cutaneous basement membrane--anchoring fibril complex: preparation and ultrastructure.

The human cutaneous basement membrane (CBM) has been isolated together with the anchoring fibrils (AF) of the dermis. These structures are removed from the dermis as a single complex, the CBM-AF complex. The epidermis is first removed from the dermis by immersion of whole, defatted skin in cold 2 n NaSCN for 5 to 6 hr. Subsequently, removal of the CBM-AF complex is effected by focusing high-intensity ultrasonic energy against the dermal surface. Purification of the preparation is achieved by low-speed sedimentation. All stages of the process were monitored by electron microscopy, which demonstrated that the morphology of the CBM-AF complex was well preserved and that this complex represents the principal mass component in the system. In addition, our report describes an ultrasound-induced variation in collagen fibril morphology, which we have called 'disordered collagen.' This altered type of fibril lacks periodicity or recognizable banding and has a frayed appearance. Finally, the experiments provide the basis for an analysis of the relative strengths of certain bonds at the dermal--epidermal junction. The strongest of these appears to be the bond between basement membrane and anchoring fibril.

Separation of epidermis from dermis with sodium thiocyanate.

After human skin is treated with 2 N sodium thiocyanate, epidermis is easily separated from dermis. The level of cleavage occurs at the lamina lucida of the basement membrane zone. Bullous pemphigoid antigen remains attached to the epidermis.

Coexistence of benign familial pemphigus and psoriasis vulgaris.

The treatment of psoriasis vulgaris in a 67-year-old woman was complicated by the presence of an acantholytic diathesis. The clinical and histologic evidence showed that the patient had an atypical phenotype of familial benign chronic pemphigus (FBCP) as well as psoriasis. Consideration of the possible effects of psoriasis and FBCP on each other may help explain some of the typical features noted in this patient.

Benign papular acantholytic dermatosis.

Seven male patients, aged 41 to 63, had benign papular acantholytic dermatosis. The patients complained of firm, intensely pruritic, 1- to 3-mm, reddish-brown or flesh-colored papules. The lesions were usually found on the upper part of the torso or on the neck. At least five of the seven patients reported that sunlight initiated or exacerbated the condition. The mean duration of the disorder exceeded two years. Histologically, the predominant lesion in all seven cases was acantholysis. Dyskeratosis was not a characteristic finding, being absent in five of the seven cases and minimally present in two. We will compare the features of benign papular acantholytic dermatosis with those of other papular acantholytic disorders.