Primary cutaneous extranodal natural killer/T-cell lymphoma presenting as bilateral erythematous patches on the arms.

Natural killer/T-cell lymphoma is a rare, Epstein-Barr virus-associated type of cytotoxic lymphoma thatpresents mainly in the nasal cavity and its vicinity. Very few cases of primary cutaneous extranodal natural killer/T-cell lymphoma have been reported till date. All the previously reported cases of primary cutaneous extranodalnatural killer/T-cell lymphoma presented as lesions resembling cellulitis, subcutaneous nodules or ulcers. We report a rare case which presented as erythematous and purpuric round patches on the arms and was finally diagnosed as primary cutaneous extranodalnatural killer/T-cell lymphoma, following a skin biopsy. The atypical patchy lesions presented a diagnostic challenge. We herein describe this clinically novel atypical patch-like presentation of primary cutaneous extranodalnatural killer/T-cell lymphoma together with the key histopathologic features and highlights of the previously reported cases.

Hansen's disease (Leprosy): current and future pharmacotherapy and treatment of disease-related immunologic reactions.

Hansen's disease, also known as leprosy, remains an important public health problem throughout the world, including North America. The causative microbe in Hansen's disease is Mycobacterium leprae, an acid-fast organism that is difficult to grow in vitro. The nine-banded armadillo is the major animal reservoir in the United States. Manifestations of disease vary based on host immune response and can range from tuberculoid to lepromatous leprosy (paucibacillary to multibacillary disease). Hansen's disease typically affects the skin, nerves, and eyes, and patients may present with skin lesions, weakness, numbness, eye pain, or loss of vision. Definitive diagnosis is based on a combination of physical examination findings and skin biopsy and/or smear. Modern antibacterial therapy typically consists of combinations of dapsone and rifampin with or without clofazimine. Clofazimine is available only as an investigational drug through the National Hansen's Disease Program. Other options include moxifloxacin, ofloxacin, minocycline, and clarithromycin. Hansen's disease is associated with type 1 (reversal) and type 2 (erythema nodosum leprosum) immunologic reactions, during which the disease process appears to worsen dramatically. These reactions may occur at any time before, during, or after treatment. Antibacterial therapy should usually be continued during these reactions. Treatment options for these reactions differ based on clinical manifestations and include corticosteroids, thalidomide, pentoxiphylline, tumor necrosis factor inhibitors, and T cell inhibitors. Prompt diagnosis, antimicrobial therapy, and treatment of reactions dramatically reduce complications of the disease.

Reactions in borderline leprosy.

This is a retrospective study of 276 patients consisting of 157 active and 119 reactive patients of borderline leprosy. They were followed up for 10 years after sulphone monotherapy. The presenting symptoms were carefully examined from the records and systematically presented. Frequency of reactions was least in BT cases and most in BL cases. Risk factors of reaction appear to be the type of leprosy, multiplicity of lesions, high BI and, possibly, psychological stress. Biopsy of skin lesions was performed in all cases initially, and at the subsidence of the disease. Histological findings closely correlated with clinical classification. While all the cases showed clinical subsidence, histological subsidence was found in 200 (73%) cases, and the condition was static in 36 cases (13%). Immunological upgrading was seen in 110%, while 4% showed downgrading. Bacteriological status and lepromin reaction of active and reactive cases were compared. All these factors need to be taken into consideration for instituting prompt and proper treatment.

Development of giant reaction in response to PPD skin test in lepromatous leprosy patients.

The present study analyzes some clinical and immunological aspects of the giant reaction (GR) in lepromatous leprosy. Sixteen out of a total of 147 (10.9%) lepromatous patients developed the clinical features of GR upon the intradermal administration of PPD; most (14 of 16) GRs occurred in bacteriologically positive cases. GR precipitated an episode of erythema nodosum leprosum (ENL) in three patients. In addition, patients with GR showed enhanced in vitro response to PPD, by the lymphoproliferation test and interferon-gamma assay, as compared to either PPD-negative individuals or PPD-positive patients without GR. Therefore, cell-mediated-immune response to mycobacterial antigens is present in lepromatous patients with GR. It is suggested that the exacerbated in vivo response to PPD in lepromatous leprosy is the result of an increased immunoreactivity to the antigen, which well may be associated with the local and/or systemic release of cytokines [tumor necrosis factor-alpha (TNF alpha) and interferon-gamma (IFN gamma)] by the inflammatory cells. These episodes may, in fact, play an important role in determining the development of disabilities and reactional states, thereby interfering with the prognosis of leprosy disease.

Specific antigen and antibody to Mycobacterium leprae in the cryoprecipitate of a patient with Lucio phenomenon.

Using a sensitive and specific enzyme-linked immunosorbent assay (ELISA) assay we showed that the cryoglobulins of a patient with Lucio phenomenon contain phenolic glycolipid I antigen and a specific antibody.