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A full-term 5-month-old breast-fed female infant presented with a 2-month history of diarrhea and a 1 1/2-month history of an acrodermatitis enteropathica-like skin eruption. Her serum zinc level and the maternal milk zinc level were markedly reduced. All symptoms disappeared promptly with oral zinc therapy, after which the patient's serum zinc level returned to normal.
BACKGROUND: Recent reports have implicated Borrelia burgdorferi infection as a possible cause of localized scleroderma (LS). OBJECTIVE: Our purpose was to describe the clinical, histologic, and immunopathologic features of patients with LS who had serum antibodies to B. burgdorferi. METHODS: Ten patients were examined clinically and by routine microscopy. Biopsy specimens from seven patients were studied immunohistochemically with monoclonal antibodies. The proliferative response of peripheral blood mononuclear cells to B. burgdorferi was investigated in seven patients by lymphocyte proliferation assay. RESULTS: Seven patients had plaque-type morphea, and three patients had linear scleroderma. Two patients had a history of previous erythema migrans. One patient had coexistent acrodermatitis chronica atrophicans, and in two patients lichen sclerosus et atrophicus was observed. Histologically, a prominent inflammatory phase with sclerosis of the connective tissue was shown in all patients. Immunohistochemical studies revealed that the inflammatory infiltrates consisted of both B and T lymphocytes, predominantly of the CD4+ subset. All 10 patients had strongly elevated serum antibodies to B. burgdorferi. Patients with LS showed significantly elevated lymphoproliferative responses to B. burgdorferi when compared with healthy control subjects. CONCLUSION: Our findings suggest that some cases of LS are linked to Borrelia infection.
Shortly after the introduction of sulfa drugs, sulfapyridine was found to have unique therapeutic properties, unrelated to antibacterial activity. Later, sulfones were found to share the same properties. The disorders initially improved were dermatitis herpetiformis, pyoderma gangrenosum, subcorneal pustular dermatosis, acrodermatitis continua, impetigo herpetiformis and ulcerative colitis. They were also sometimes helpful in many other disorders. They are effective in select disorders characterized by edema followed by granulocytic inflammation or edema followed by vesicle or bullae formation. The sulfones work in low doses in leprosy and their mode of action is not fully understood. Several pieces of experimental information are available. It is proposed that these drugs are entering or influencing the protein moiety of glycosaminoglycans and decreasing tissue viscosity. This decreased tissue viscosity prevents edema and dilution of tissue fluid and decreases acute inflammation and vesicle and bullae formation.
Current laboratory diagnosis of Lyme borreliosis relies on tests for the detection of antibodies to Borrelia burgdorferi with known limitations. By using a simple extraction procedure for urine samples, B. burgdorferi DNA was amplified by a nested PCR with primers that target the specific part of the flagellin gene. To control possible inhibition of the enzyme (polymerase), a special assay using the same primers was developed. We examined 403 urine samples from 185 patients with skin manifestations of Lyme borreliosis. Before treatment, B. burgdorferi DNA was detected in 88 of 97 patients with Lyme borreliosis. After treatment, all but seven patients became nonreactive. Six of these seven persons suffered from intermittent migratory arthralgias or myalgias, and one from acrodermatitis chronica atrophicans. Two of 49 control patients with various dermatologic disorders and none out of 22 presumably healthy persons were reactive in the PCR. In addition to urine, breast milk from two lactating women with erythema migrans was tested and also found reactive. Borrelia burgdorferi DNA can be detected with high sensitivity (91%) by a nested PCR in urine of patients with Lyme borreliosis. In addition, this test can be a reliable marker for the efficacy of treatment.
In 1986-1988 there were 123 patients with positive serology for Lyme borreliosis out of 4000 sera referred to the Department of Bacteriology and Immunology, University of Helsinki. Of the 63 patients with positive serology in 1986-1987 20 showed a predominant involvement of the nervous system, 18 complained of joint symptoms and 11 patients merely showed a skin involvement including 8 patients with erythema chronicum migrans (ECM) and 3 patients with acrodermatitis chronica atrophicans (ACA). Two of the patients had unspecific general symptoms and in 5 patients the type of involvement remained unknown. The serology was considered to be falsely positive in 2 patients with tuberculous meningitis, in one with syphilis and in another with recurrent fever.
Several extrahepatic manifestations are associated with chronic HBV infection, many with significant morbidity and mortality. The cause of these extrahepatic manifestations is generally believed to be immune mediated. PAN is a rare, but serious, systemic complication of chronic HBV affecting the small- and medium-sized vessels. PAN is seen more frequently in North American and European patients and rarely in Asian patients. PAN ultimately involves multiple organ systems, some with devastating consequences, though the hepatic manifestations are often more mild. The optimal treatment of HBV-associated PAN is thought to include a combination of antiviral and immunosuppressive therapies. HBV-associated GN occurs mainly in children, predominantly males, in HBV endemic areas of the world, but is only occasionally reported in the United States. In children, GN is usually self-limited with only rare progression to renal failure. In adults, the natural disease course of GN may be more relentless, slowly progressing to renal failure. Immunosuppressive therapy in HBV-related GN is not recommended, but antiviral therapy with alpha-interferon has shown promise. The serum-sickness like "arthritis-dermatitis" prodrome is seen in approximately one third of patients acquiring HBV. The joint and skin manifestations are varied, but the syndrome spontaneously resolves at the onset of clinical hepatitis with few significant sequelae. Occasionally, arthritis following the acute prodromal infection may persist; however, joint destruction is rare. The association between HBV and mixed essential cryoglobulinemia remains controversial; but a triad of purpura, arthralgias, and weakness, which can progress to nephritis, pulmonary disease, and generalized vasculitis, has characterized the syndrome. Finally, skin manifestations of HBV infection typically present as palpable purpura. Though papular acrodermatitis of childhood has been reported to be caused by chronic HBV, this association remains controversial.
The aim of the present study was to determine the susceptibility of Borrelia afzelii strains to antibiotics, and to test the hypothesis that persistence of borrelia in skin, after therapy, is a consequence of resistance to the antibiotic used for treatment. Ten B. afzelii strains isolated from skin of seven adult patients (two with acrodermatitis chronica atrophicans, five with erythema migrans) were studied. In three patients B. afzelii was isolated from erythema migrans lesion before antibiotic therapy and 2-3 months after treatment with cefuroxime axetil (two patients) or with ceftriaxone (one patient). MICs and MBCs for amoxicillin, azithromycin, ceftriaxone, cefuroxime, doxycycline and amikacin were measured. There was total resistance to amikacin but isolates were susceptible to all other antibiotics except one isolate that was resistant to cefuroxime, MIC > 4 mg/L. Comparison of MBC values after 3 and 6 weeks' incubation revealed comparable results for azithromycin and ceftriaxone while for amoxicillin, cefuroxime and doxycycline, some differences were found. In one of the patients from whom there were borrelia isolated before and after treatment with cefuroxime axetil, both isolates were resistant to cefuroxime. In the other two patients, the paired isolates were susceptible to the antibiotic used for therapy.
The immuno-pathogenetic mechanisms underlying chronic Lyme neuroborreliosis are mainly unknown. Human Borrelia burgdorferi (Bb) infection is associated with Bb-specific secretion of interferon-gamma (IFN-gamma), which may be important for the elimination of Bb, but this may also cause tissue injury. In order to increase the understanding of the pathogenic mechanisms in chronic neuroborreliosis, we investigated which cell types that secrete IFN-gamma. Blood mononuclear cells from 13 patients with neuroborreliosis and/or acrodermatitis chronicum atrophicans were stimulated with Bb antigen and the phenotypes of the induced IFN-gamma-secreting cells were analyzed with three different approaches. Cells expressing CD8 or TCRgammadelta, which both have cytolytic properties, were the main phenotypes of IFN-gamma-secreting cells, indicating that tissue injury in chronic neuroborreliosis may be mediated by cytotoxic cells.
Dominant types of viral hepatitis are presently A, B, and C with prophylactic immunization available only for A and B. Hepatitis B and C and human immunodeficiency virus (HIV) infection constitute a worldwide scourge and treatment is far from satisfactory. Each produces severe oxidative stress (OS) and secondary cellular damage of varying severity and, as in toxic hepatitis, progression and regression are dependent on redox balance between oxidation and antioxidation. Experimental and clinical studies suggest that xenobiotics and co-infections exert cumulative, detrimental effects on their pathogeneses and further deplete antioxidants. It is proposed therefore that in the clinical management of these infections and especially in their early stages, considerable benefit should accrue from antioxidant repletion at dosages substantially above recommended daily allowances (RDAs) in conjunction with a nutritious high protein diet. Because plasma zinc and selenium concentrations are very low, their replenishment by high dosages is urgent and mandatory particularly in advanced HIV infections bordering on acrodermatitis enteropathica. Also recommended is their long-term continuance at high normal levels.
In addition to classic organ system involvement, Lyme disease may be characterized by myositis, liver and spleen involvement, and atypical cutaneous manifestations. Myositis is characteristically localized near an involved joint or localized neuropathy. Nuclear imaging with gallium-67 may be useful for detection. Myositis responds to treatment with intravenous or oral antibiotics. Patients with erythema migrans have been observed to have liver function test abnormalities in the absence of symptomatic hepatitis. Splenomegaly has been noted infrequently in patients with Lyme disease. Chronic cutaneous manifestations of Lyme disease--including erythema migrans, acrodermatitis chronica atrophicans, and lymphadenosis benigna cutis--have been observed more frequently in Europe than in the United States. It appears that they are caused primarily by the Borrelia afzelii genomic group of Borrelia burgdorferi, which has been found exclusively in Europe.
Clinical recognition of the role of trace elements in human nutrition is increasing. Heretofore, many clinicians felt that the development of deficiency states was not likely except in very extreme conditions because the presence of trace elements in nature was so ubiquitous. The increased use of total parenteral nutrition seems to have made this viewpoint untenable. The recognition of additional genetic diseases of trace element metabolism such as Menkes' kinky hair syndrome and acrodermatitis enteropathica has also served to focus clinical attention on trace elements. As time passes, no doubt, additional diseases of trace element metabolism will be recognized.
Lyme borreliosis is the most common tick-transmitted disease in the northern hemisphere and is caused by spirochaetes of the Borrelia burgdorferi species complex. A complete presentation of the disease is an extremely unusual observation in which a skin lesion results from a tick bite and is followed by heart and nervous system involvement, and later on by arthritis. Late involvement of eye, nervous system, joints, and skin can also occur. The only sign that enables a reliable clinical diagnosis of Lyme borreliosis is erythema migrans. Other features of some diagnostic value are earlobe lymphocytoma, meningoradiculoneuritis (Garin-Bujadoux-Bannwarth syndrome), and acrodermatitis chronica atrophicans. The many other symptoms and signs have little diagnostic value. Microbial or serological confirmation of borrelial infection is needed for all manifestations of the disease except for typical early skin lesions. However, even erythema migrans might not be pathognomonic for Lyme borreliosis, especially in the southern part of the USA where there is no microbiological evidence for infection with the agent. Treatment with antibiotics is beneficial for all stages of Lyme borreliosis, but is most successful early in the course of the illness. Prevention relies mainly on avoiding exposure to tick bites but there is some interest in chemoprophylaxis and also in vaccine development following initial disappointments.
A low molecular weight zinc binding compound from human milk has been purified by ultrafiltration, gel filtration, and ion-exchange chromatography. Evidence is provided that this compound is citrate. A higher amount of citrate-bound zinc was found in human milk than in cow's milk. It is suggested that the therapeutic value of human milk for patients with the genetic disorder of zinc metabolism acrodermatitis enteropathica (AE) derives from a greater content of bioavailable zinc citrate in human than in cow's milk.
In order to evaluate the virtual advantage of proving borrelial infection in dermatoses clinically diagnosed as erythema (chronicum) migrans (ECM), acrodermatitis chronica atrophicans (ACA) or lymphadenosis cutis benigna (LCB) by means of histological, cultural or serological trials, skin and serum samples obtained from altogether 99 patients suffering from these dermatoses were examined. Serum and--to some extent--skin specimens gained from healthy individuals (n = 36), patients with not tick bite associated disorders of skin, internal organs and nervous system (n = 121) were used as controls. In addition, serum specimens from patients enduring circumscribed scleroderma (morphea), n = 31, and sera of Bavarian forest workers (n = 211) were proven for the presence of borrelial antibodies. Using an indirect immunofluorescence assay with borrelial strains, propagated in inbred mice, as antigens, elevated IgM- and/or IgG antibody titers were found in 51 (84%) out of 61 ECM-, in 17 (100%) ACA- and in 2 (33%) out of 6 LCB-sera. Antibody titers decreased significantly after adequate antibiotic therapy. The groups in comparison yielded spirochetal serum antibodies as follows: Healthy individuals and patients with various diseases 7 out of 157 (4.5%), morphea patients 7 out of 31 (23%) and forest workers 71 out of 211 (34%). Borrelia burgdorferi was cultivated from 6 out of 32 skin and from 1 blood specimen obtained from ECM patients and from 1 out of 5 ACA skin specimens. Borreliae could also be detected in the blood of 3 out of 11 thymusaplastic nude mice after intraperitoneal implantation of ECM-tissue. More frequently than borreliae spindle-shaped bacteria resembling fusobacterium sp. were detected not only by in vitro cultivation of ECM and ACA skin samples, but also in the blood of thymusaplastic mice after implantation of ECM-tissue. By Warthin-Starry silver strain borrelia-like structures were detected in 9 out of 28 ECM, in 3 out of 12 ACA and in 1 out of 5 LCB skin specimens. Therefore in our experience serological examinations turned out to be the most productive diagnostic tool.
Crohn's disease is a chronic inflammatory bowel disease which may have distinctive mucocutaneous manifestations. Included in this group are perianal, peristomal, and perifistular ulceration, as well as granulomatous cutaneous inflammation separate from gastrointestinal tract openings (metastatic Crohn's disease). In the oral cavity, both ulcerations and granulomatous nodules may occur. Malabsorption of nutrients may lead to several changes, including an acrodermatitis enteropathica-like syndrome secondary to zinc deficiency. Patients with Crohn's disease may also have pyoderma gangrenosum, erythema nodosum, cutaneous vasculitis, and other less specific changes.
The ability to define subpopulations of immunologically competent lymphocytes has permitted an enhanced understanding of the interaction between functionally distinct components of the immune system. T cells can provide help in antibody formation or they may suppress antibody production. Abnormal immunoregulatory mechanisms have been demonstrated in the hyperimmunoglobulin E-recurrent infection syndrome. This disorder is associated with a marked elevation of IgE and specific elevations of IgE antibodies directed toward staphylococcal antigens. Abnormal T cell regulation of immune responses has been demonstrated. Graft-versus-host disease (GVHD) occurs in an immunodeficient patient who has received an infusion of immunocompetent cells. The diagnosis of graft-versus-host (GVH) reaction may be complicated by the protean manifestations of the disorder. The acute form, consisting of a maculopapular rash, fever, and diarrhea, may be confused with acute infection or drug reaction. Chronic GVHD has been incorrectly diagnosed as histiocytosis X, acrodermatitis enteropathica, or scleroderma. Utilizing chromosome markers and/or identification of histocompatibility antigens, the presence of circulating lymphocytes from donor immunocompetent cells (blood transfusion, maternal source) can be documented. The development of sensitive technics for identifying cells can establish a precise diagnosis. Certain immunodeficiency disorders can be identified by biochemical means. Biotin-dependent multiple carboxylase enzyme deficiency is associated with a chronic dermatitis, alopecia, ataxia, and secondary infection of the skin with Candida. The disorder responds promptly to the administration of biotin with correction of dermatologic, neurologic, and immunologic abnormalities.
Several recently recognized canine dermatoses are described. Syndromes discussed include superficial necrolytic dermatitis, Malasezzia dermatitis, sebaceous adenitis, nodular dermatofibrosis in German shepherd dogs, solar-induced dermatitis, and acrodermatitis.
Zinc is important in a multitude of biological functions, including regulation of the immune response, modulation of keratogenesis and wound healing, maintenance of normal reproductive function, and acuity of taste and smell. Zinc-responsive dermatosis is an uncommon disease of dogs resulting from either an absolute or relative deficiency in zinc. Dermatological lesions are characterized by erythema, alopecia, scales, and crusts that primarily affect the head. Two forms of the disease exist: a familial form affecting Alaskan Malamutes and Siberian Huskies and a form that affects growing puppies fed zinc-deficient or oversupplemented diets. The history, clinical signs, and skin biopsy results are typically diagnostic. Life-long zinc supplementation is usually necessary in the familial form of the disease, although dietary correction alone may be curative in the second form. Lethal acrodermatitis is a rare inherited disorder of Bull Terriers that does not respond to zinc supplementation and is invariably fatal.