George T. Nahass

Extracorporeal Photochemotherapy for Drug-Resistant Pemphigus Vulgaris

Excerpt The extracorporeal exposure of pathogenic peripheral blood leukocytes to the natural compound 8-methoxypsoralen (8-MOP) and ultraviolet A radiation has provided great benefit in certain adv...

Antiphospholipid antibodies and the antiphospholipid antibody syndrome

The antiphospholipid antibody syndrome is a multiple-system disorder characterized by persistently elevated antiphospholipid antibodies and/or arterial or venous thrombosis, thrombocytopenia, or recurrent spontaneous abortion. Anticardiolipin antibodies and the lupus anticoagulant are different classes of antiphospholipid antibodies associated with this disorder. Cutaneous manifestations are common and may be the presenting sign of the underlying disease. This article reviews the clinical manifestations, laboratory assays, histopathologic features, and treatment of the antiphospholipid antibody syndrome.

Acute radiodermatitis after radiofrequency catheter ablation

Radiofrequency (RF) catheter ablation is used in the treatment of a variety of arrhythmias. This report describes the development of acute radiodermatitis after two prolonged RF catheter ablation procedures for supraventricular tachycardia. It also reviews the characteristics and treatment of radiation-induced skin reactions.

Pityriasis rubra pilaris and HIV infection

Many cutaneous diseases have been reported in patients with human immunodeficiency virus infection. We report two patients with pityriasis rubra pilaris and human immunodeficiency virus infection. In one patient, the onset of pityriasis rubra pilaris preceded the discovery of human immunodeficiency virus infection. In the second patient, the onset of pityriasis rubra pilaris occurred shortly after the patient tested positive for human immunodeficiency virus infection. Both patients had a severe form of pityriasis rubra pilaris, and both had a minimal therapeutic response to etretinate.

Basal cell carcinoma of the scrotum: Report of three cases and review of the literature

BACKGROUND Although basal cell carcinoma (BCC) is the most common human malignancy, only 21 cases involving the scrotum have been previously reported. OBJECTIVE Our purpose is to describe three additional cases of scrotal BCC and review the literature summarizing the clinical features and identifying any predisposing factors. METHODS We retrospectively reviewed 21 cases of scrotal BCC and described three new cases. Polymerase chain reaction (PCR) was used to detect human papillomavirus (HPV) DNA in our biopsy specimens. RESULTS Scrotal BCCs present as persistent ulcerations or plaques without identifiable predisposing factors. Lymphatic, pulmonary, or skin metastases were present in 3 of 24 cases (13%) resulting in death in one case. PCR did not detect HPV DNA in our three cases. CONCLUSION Scrotal BCC rarely occurs and should be considered in the diagnosis of a persistent scrotal ulcer or plaque. Metastatic disease may be more common than with other BCCs and wide local excision or Mohs micrographic surgery may be the most appropriate initial therapeutic approach.

Pemphigus vulgaris treated with photopheresis.

A 31-year-old man had a 4-year history of severe pemphigus vulgaris confirmed by histologic examination and direct and indirect immunofluorescence. His disease had been resistant to maximal doses and varying combinations of prednisone, oral gold, cyclosporine,methotrexate, azathioprine, pulse steroids, and pulse cyclophosphamide with methylprednisolone. Many adverse effects complicated his medical therapy, including renal insufficiency, hypertension, and a cushingoid appearance. At the time of initiation of photopheresis the patient had pemphigus lesions on 70% of his body surface (Fig. I). His treatment regimen included intramuscular methotrexate 20 mg weekly, azathioprine 100 mg daily, and oral prednisone 240 mg daily. He had been taking these medications for I month after failing to respond to monthly (eight) pulses of cyclophosphamide and methylprednisolone. Photopheresis was started with treatments on two consecutive days every 2 weeks. By the seventh cycle new blister formation had ceased and treatment frequency was reduced to every 3 weeks. His medications were slowly tapered with a greater than 80% reduction in his daily prednisone, discontinuation of methotrexate, and maintenance of azathioprine and gold at pretreatment doses. The patient tolerated photopheresis without complications and eventually all skin lesions healed with the exception of a few crusted erosionson his face (Fig. 2). Although the patient had one episode of a disseminated cutaneous herpetic infection that required intravenous acyclovir, he had had

Herpes simplex virus DNA in occult lesions: Demonstration by the polymerase chain reaction

Herpes simplex virus DNA was identified by the polymerase chain reaction with primers specific for the herpes DNA polymerase gene. A herpes-specific amplimer was detected in two of three cases in which the clinical and histologic features were inconclusive.

Merkel cells and prurigo nodularis

BACKGROUND Increased numbers of dermal nerves have been demonstrated in prurigo nodularis and have been theoretically linked to the intense pruritus. We hypothesized that the neuronal proliferation in prurigo nodularis might be associated with an increased density of Merkel cells because they are also a component of the neurocutaneous system. METHODS We examined skin biopsy specimens from 20 cases of prurigo nodularis for Merkel cells with the use of a standard immunohistochemical assay (avidin-biotin-peroxidase complex system) with an antibody to cytokeratin 8 (CAM 5.2). Six cases of lichen simplex chronicus were examined as controls. RESULTS Merkel cells were present in the interfollicular area of the basal cell layer in 15 (75%) of 20 prurigo nodularis cases and in one (17%) of six cases of lichen simplex chronicus. CONCLUSION Merkel cells are increased in number in prurigo nodularis and may be a component of the neurocutaneous abnormality associated with this disorder.