Guidelines of care for Mohs micrographic surgery. American Academy of Dermatology.
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Biomedical subjects
Publications and source records attributed to M L Turner.
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We describe a patient with chronic granulomatous disease who presented with erythematous papular skin lesions on the chest, back, and arm. Examination of biopsy specimens from the lesions on the arm and back showed suppurative granulomata in association with acute and chronic inflammation. Histopathologic examination of a specimen from the lesion on the arm revealed fungal elements, and cultures yielded Microascus cinereus. The patient was treated with 2.5 g of intravenous amphotericin B, and the lesions resolved. We report what is, to our knowledge, the first case of invasive disease due solely to M. cinereus.
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BACKGROUND: Studies conducted in vitro and in animals suggest that cytokine signals to monocytes or macrophages by interferon gamma are important in the containment and clearance of disseminated nontuberculous mycobacterial infections. METHODS: We studied seven patients with refractory, disseminated nontuberculous mycobacterial infections who were not infected with the human immunodeficiency virus. Three patients were from a family predisposed to the development of Mycobacterium avium complex infections; four patients had idiopathic CD4+ T-lymphocytopenia. Their infections were culture- or biopsy-proved, involved at least two organ systems, and had been treated with the maximal tolerated medical therapy. Cellular proliferation, cytokine production, and phagocyte function were assessed in peripheral-blood cells. Interferon gamma was administered subcutaneously two or three times weekly in a dose of 25 to 50 micrograms per square meter of body-surface area in addition to antimycobacterial medications. Clinical effects were monitored by cultures, biopsies, radiographs, and in one patient a change in the need for paracentesis. RESULTS: In response to phytohemagglutinin, the production of interferon gamma by mononuclear cells from the patients was lower than in normal subjects (P < 0.001), whereas stimulation with ionomycin and phorbol myristate acetate led to normal production of interferon gamma in the patients. Within eight weeks of the start of interferon gamma therapy, all seven patients had marked clinical improvement, with abatement of fever, clearing of many lesions and quiescence of others, radiographic improvement, and a reduction in the need for paracentesis. CONCLUSIONS: Interferon gamma in combination with conventional therapy may be effective for some cases of refractory disseminated nontuberculous mycobacterial infection.
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BACKGROUND: Patients with Gianotti-Crosti syndrome (GCS) present with a distinctive self-limiting acral papular or papulovesicular eruption associated with an underlying viral illness. Gianotti-Crosti syndrome in patients infected with human immunodeficiency virus has not been previously reported. OBSERVATIONS: We report on two children infected with human immunodeficiency virus who had GCS. Both patients had clinical and histopathologic findings characteristic of GCS. The first patient had evidence of prior infection with cytomegalovirus, without evidence of active viral illness. The second patient had evidence of subclinical infection with cytomegalovirus, positive hepatitis C antibody, and active infection with Mycobacterium avium-intracellulare at the time the skin eruption began. CONCLUSIONS: We call attention to a previously unreported skin eruption, GCS, in the setting of human immunodeficiency virus infection and emphasize that determining the etiologic factors for human immunodeficiency virus-associated GCS will be difficult; such patients will probably have a variety of clinical and subclinical infections that complicate this issue.
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The medullary dorsal horn is a site of action of opiates in producing facial scratching. Extracts of plasma (0.4 microliter) from 4 patients with the pruritus of cholestasis induced facial scratching when microinjected into the medullary dorsal horn of monkeys. This extract-induced scratching could be abolished or prevented by administering the opioid receptor antagonist, naloxone. Neither saline nor an extract of plasma from a nonpruritic cholestatic patient induced scratching when similarly administered. We infer that plasma of patients with the pruritus of cholestasis contains a factor which induces pruritus by a central opioid receptor-mediated mechanism.
A prospective study was carried out to determine the prevalence of autonomic dysfunction in patients with lymphoma, and to assess the effect on this of chemotherapy. Twenty consecutive patients presenting with Hodgkin's disease, high-grade non-Hodgkin's lymphoma, or low-grade non-Hodgkin's lymphoma were studied. All had advanced disease, requiring combination chemotherapy which included the use of vinca alkaloids. Clinical assessment and standard cardiovascular autonomic function tests were carried out prior to and following completion of chemotherapy. Although no patients had clinical evidence of autonomic neuropathy at presentation, 16 (80%) had abnormal cardiovascular autonomic function tests. There was no correlation with the presence or absence of mediastinal disease. There was significant improvement in autonomic scores with treatment despite the use of drugs of known neurological toxicity. Some patients showed residual abnormalities of autonomic function despite disease resolution. We suggest that subclinical autonomic dysfunction is common in patients with lymphoma, and probably represents a paraneoplastic syndrome--the pathogenesis and prevalence of which deserve further study. This phenomenon may predispose patients with lymphoma to develop gastrointestinal and genitourinary dysfunction, or postural hypotension, and should be considered during the evaluation of the neurotoxicity of chemotherapy regimens.
Invasive aspergillosis is a disease of the immunosuppressed patient. We describe two patients with acute lymphoblastic leukaemia who attained complete remission, with partial or complete bone marrow recovery, but who went on to develop fatal invasive aspergillosis contemporaneous with recovery of neutrophil counts. Quantitative recovery of peripheral blood neutrophil counts does not guarantee control of Aspergillus infection, perhaps due to functional neutrophil deficiencies post-chemotherapy, and specific defensive strategies adopted by the organism itself.
The eosinophilia-myalgia syndrome (EMS) has been associated with ingestion of L-tryptophan (L-TRP) produced by a single manufacturer. Epidemiological data implicated 1,1'-ethylidenebis (L-tryptophan) (EBT) (peak 97 or peak E) as a possible etiologic agent. We showed previously that Lewis rats treated with the L-TRP implicated in EMS develop fasciitis and perimyositis similar to those seen in human EMS. We now report the pathology associated with the treatment of Lewis rats with synthetic EBT and/or L-TRP. All animals treated for 6 wk with case-associated L-TRP or EBT developed significant myofascial thickening, compared with animals in the vehicle control and control L-TRP groups. However, even those animals receiving the control L-TRP showed a mild but significant increase in the thickness of the myofascia, compared with vehicle-treated control animals. All animals except vehicle controls also exhibited significant pancreatic pathology, including fibrosis and acinar changes. Only animals treated with case-associated L-TRP for 6 wk showed evidence of immune activation with increased frequency of CD8, Ia, and IL-2 receptor-positive cells in the peripheral blood. Animals receiving L-TRP or EBT for < 6 wk did not show significant differences in myofascial thickness, although these animals did show pancreatic acinar changes. Although these results demonstrate for the first time the pathological effects of EBT, they do not rule out the possibility that other impurities in the EMS-case-associated L-TRP may also contribute to some of the features of EMS.
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Bone marrow transplant (BMT) recipients frequently develop rashes as a consequence of their disease, its treatment or because of a complication such as infection. These rashes are often clinically atypical, yet appropriate management is dependent upon correct diagnosis and therefore a skin biopsy is often performed. In a group of 101 consecutive BMT recipients, 25 patients had a total of 34 skin biopsies. A specific histopathological diagnosis was made in 65% (22/34), including graft vs. host disease (GVHD) (15 cases), infection (4 cases), drug reaction (1 case) and recurrent lymphoma (1 case). Therapy was changed following the biopsy in 77% (17/22) of these cases. In 35% (12/34) the histological changes were non-specific, however, in 10 of these cases GVHD had been suspected clinically and its exclusion was therefore useful. Skin biopsy is of considerable value in the diagnosis and subsequent management of BMT recipients who develop a rash.
The cost-effectiveness of two treatment strategies for vulvar vestibulitis syndrome (VVS) was compared. Our prospective study consisted of 55 patients with human papillomavirus (HPV)- and non-HPV-associated VVS of at least 6 months' duration treated with intralesional recombinant alpha interferon injections, followed by surgery for nonresponders and responders compared with a hypothetical model of surgery alone. The setting was a private-practice multispecialty center for vulvovaginal disorders. Improvement was defined by patients' subjective evaluation of change in the level of introital dyspareunia and the ability to have sexual relations and objective evidence of change in the degree of erythema and tenderness to touch within the vestibule. Statistical analyses of the overall probability of improvement, overall costs and the cost per patient treated were done. Twenty-seven (49%) of 55 patients treated with alpha interferon had substantial or partial improvement. Of the 28 (51%) who did not improve following alpha interferon, 19 elected to have surgery. Surgery resulted in substantial improvement in 84% of the patients and partial improvement in 11%. Statistical analysis comparing the group treated with alpha interferon (some of whom went to surgery with the hypothetical model of surgical treatment alone) showed significant cost-saving in the group first treated with alpha interferon. At the level of effectiveness achieved in this study, intralesional alpha interferon as a first choice in the treatment of VVS is cost-effective.