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Biomedical subjects

G Bonsmann

Publications and source records attributed to G Bonsmann.

At least 19 recordsLinked to original sources
PubMed2006

[Current diagnosis of cutaneous lupus erythematosus].

Cutaneous lupus erythematosus (CLE) is a disease with different subtypes and the new classification system includes acute CLE (ACLE), subacute CLE (SCLE), chronic CLE (CCLE), and intermittent CLE (ICLE). The broad spectrum of skin involvement and the possibility of systemic organ manifestations at the beginning and during the course of CLE require specific diagnostic procedures. Clinical assessment of the cutaneous manifestations is necessary along with a detailed patient's history. The diagnosis of CLE is confirmed by histopathology and immunofluorescence microscopy. Selective laboratory screening and additional diagnostic procedures depending on clinical symptoms are recommended. Photoprovocation tests can be performed to assess photosensitivity in patients with CLE and to support the diagnosis. Recently, a scoring system for the activity of the cutaneous manifestations in CLE has been developed and is now evaluated in several clinical studies. In this review, the classification and the characteristic clinical criteria of the different CLE subtypes as well as the current diagnostic possibilities are emphasized.

Diagnosis, Differential↗
PubMed2006

[Cutaneous lupus erythematosus. Part 1: clinical manifestations and classification].

Cutaneous lupus erythematosus (CLE) is a heterogenous disorder with a wide range of skin manifestations. Therefore, it has been difficult to develop a unifying concept for classifying CLE from the dermatologic perspective in the past. In 2004, the classification system was updated and includes now acute CLE (ACLE), subacute CLE (SCLE), chronic CLE (CCLE), and intermittent CLE (ICLE). Additional rarely described variants are not listed as separate entities but are included in the classical forms. Diagnosis of the different subtypes of CLE is made by considering genetic, clinical, histopathologic, and immunoserologic findings, with a systematic analysis of individual criteria. In the past years, the etiology and pathogenesis of CLE has been subject of intensive research and it has been shown by several groups that exogenous factors, such as ultraviolet light and drugs, can induce CLE. The first part of this review will enable the reader to identify the various clinical manifestations of CLE and to employ characteristic criteria to assess differential diagnostic considerations.

Biopsy↗
PubMed2006

[Cutaneous lupus erythematosus. Part 2: diagnostics and therapy].

The diagnosis of cutaneous lupus erythematosus (CLE) requires a specific diagnostic approach to identify subtypes, to address differential diagnostic considerations, and to rule out systemic organ involvement. In addition to a detailed patient's history and clinical evaluation of the skin, histopathologic and immunofluorescent examination of a skin biopsy as well as laboratory screening are recommended. Photoprovocation tests can be performed to confirm the diagnosis of CLE and to assess photosensitivity in these patients. Recently, a scoring system for the activity of the cutaneous manifestations in CLE has been developed and validated which involves anatomical areas and morphologic signs of the skin lesions. In all subtypes of CLE, antimalarials are still the treatment of choice. Advances in biotechnology have led to the development of several novel agents for the treatment of autoimmune diseases; however, controlled trials have not been performed in patients with CLE. Furthermore, there is need for specific immunointervention, especially for patients who fail to respond to standard therapies. The second part of this review will enable the reader to differentiate CLE from other diseases and to suggest specific diagnostic procedures and treatment approaches.

Antimalarials↗
PubMed2005

[Diagnostic and therapeutic recommendations for mucous membrane pemphigoid of the eye].

Mucous membrane pemphigoid (MMP) is often accompanied with a chronic cicatrizing conjunctivitis that may eventually lead to loss of vision. The study is intended to investigate the current scientific knowledge on the diagnosis of and therapy for MMP involving the eye. Previous studies published before December 2004 have been systematically reviewed for their level of evidence. Consequently, recommendations for patient management are provided.

Chronic Disease↗
PubMed2005

Management of leukocytoclastic vasculitis.

Leukocytoclastic vasculitis (LcV) is the most common form of vasculitis of the skin and usually results from deposition of immune complexes at the vessel wall. It presents in different forms and in association with different diseases. When IgA is the dominant immunoglobulin in immune complexes, systemic involvement is likely in both children and adults (Henoch-Schönlein purpura--HSP). LcV due to IgG- or IgM-containing immune complexes has less systemic involvement and a better prognosis than HSP. Other forms of LcV include cryoglobulinaemic, urticarial and ANCA-associated LcV as well as LcV associated with vasculopathy and coagulopathy in SCLE/SLE or in bacteraemia/sepsis. The aim of diagnostic guidelines is to determine the specific type and systemic involvement of LcV and to identify an underlying cause. Basic work-up should encompass history of drug intake and of preceding infections, biopsy with immunofluorescence, differential blood count, urine analysis and throat swabs. Therapy of immune complex LcV often does not require aggressive therapy due to a usually favourable course. It includes avoidance or treatment of eliciting agents and use of compression stockings to reduce purpura. There are no large prospective randomized controlled studies. Corticosteroids are indicated when there are signs of incipient skin necrosis. In chronic or relapsing LcV we suggest colchicine as a first-line and dapsone as a second-line therapy. Corticosteroids may reduce the incidence of severe renal insufficiency in children according to some studies, but there is no study showing such an effect in adults. Severe systemic vasculitis requires immunosuppressive strategies.

Humans↗
PubMed2004

[Leukocytoclastic vasculitis].

Leukocytoclastic vasculitis (LcV) is the most common form of cutaneous vasculitis. Often LcV results from deposition of immune complexes in the vascular wall. When IgA is the dominant immunoglobulin in these complexes, systemic involvement is likely (Henoch-Schönlein purpura), being more severe in adults. LcV in which immune complexes are composed of IgG or IgM are more often limited to the skin and may additionally show minor systemic involvement. In other forms of LcV additional pathophysiological factors play a role. LcV can also be a presenting or accompanying symptom of severe systemic ANCA-associated vasculitis. In some cases, LcV is a sign of bacteriemia. The aim of diagnostic procedures is to determine the specific type of vasculitis and degree of systemic involvement as well as possible causes. If no trigger or cause can be found, uncomplicated cases of LcV should be treated symptomatically. Corticosteroids are indicated at initial signs of necrosis or ulceration. Chronic recurrent LcV may respond to dapsone or colchicine. Severe systemic vasculitis requires immunosuppressive therapy.

Adolescent↗
PubMed2003

[Subcutaneous fat necrosis of the newborn].

Subcutaneous fat necrosis presents as red-to-bluish, indolent, well-circumscribed, subcutaneous nodules and plaques, unusually in healthy infants. Histologically, a lobular panniculitis with a mixed inflammatory infiltrate is seen. Characteristic crystalloid structures can be found within some of the lipocytes and, most prominently, within macrophages. This rarely diagnosed skin disease is harmless and self-limited, yet the associated hypercalcaemia has a high complication rate.

Fat Necrosis↗
PubMed2001

[Necrobiotic xanthogranuloma with skin and liver amyloidosis].

BACKGROUND: Necrobiotic xanthogranuloma is a largely unknown disease. Both the frequency of this disease and the involvement of internal organs have clearly been underestimated until now. CASE REPORT: A patient was admitted because of ulcerating, xanthomatous, subcutaneous nodules, scleritis and conjunctivitis. Laboratory studies revealed an elevated erythrocyte sedimentation rate, a monoclonal IgG-lambda protein, a pancytopenia and later a hypocomplementemia. Furthermore, a hepatosplenomegaly and esophageal varices were found. A skin biopsy specimen showed a granulomatous infiltrate consisting of lymphocytes, histiocytes, plasma cells, foam cells as well as Touton and bizarre-appearing foreign-body giant cells and cholesterol clefts typical of necrobiotic xanthogranuloma besides a deposit of amyloid. A liver biopsy sample disclosed an amyloidosis of the parenchyma, too. Neither a therapy with chlorambucil and prednisolone nor with interferon alpha-2a resulted in improvement. CONCLUSION: Apart from treatment of skin lesions and ophthalmic manifestations further investigations are necessary because necrobiotic xanthogranuloma can be associated with malignancy and can involve internal viscera like lungs, heart and liver.

Adult↗
PubMed2001

[Cyclic neutropenia. Detection of a mutation in the gene for neutrophil elastase (ELA2)].

BACKGROUND AND OBJECTIVE: Cyclic neutropenia is a rare congenital hematopoietic disease which occurs sporadically or as an autosomal dominantly inherited disorder. Recently, the locus for cyclic neutropenia was mapped to chromosome 19p13.3. Autosomal dominant and sporadic cyclic neutropenia are now attributable to mutations of the ELA2 gene encoding neutrophil elastase. PATIENTS/METHODS: Based on a case report we review the clinical picture and diagnostic criteria of cyclic neutropenia and report about the recent molecular biological findings and current treatment options. RESULTS: Serial blood cell counts revealed the characteristic oscillations of the circulating neutrophils with 21-day periodicity from near normal to extremely low levels. The neutrophil nadir lasted 3-5 days. The neutropenia was associated with fever, malaise, painful oral aphthous ulcers and lymphadenopathy. Mutational analysis of the patient's genomic DNA revealed a single base-pair transition (nt 4716, G-->A) in intron 4 of ELA2. Since the initiation of therapy with subcutaneous recombinant human granulocyte-stimulating colony factor (rHuG-CSF) 24 months ago, the patient has been free of symptoms. CONCLUSIONS: Despite the typical medical history with onset of the symptoms in early infancy the diagnosis of cyclic neutropenia was not established until the age of 9 years. A common reason for the delayed diagnosis of cyclic neutropenia may be unawareness of the rare but distinctive disorder and the practice of obtaining screening blood counts without leukocyte differentials. Patients with cyclic neutropenia should be enrolled to the Severe Chronic Neutropenia International Registry (SCNIR).

Base Pairing↗
PubMed2001

Terbinafine-induced subacute cutaneous lupus erythematosus.

BACKGROUND: Recently, the induction of subacute cutaneous lupus erythematosus (SCLE) and exacerbation of systemic lupus erythematosus by terbinafine have been reported. OBJECTIVE: We describe 4 cases of SCLE, one associated with chilblain lupus, which occurred during therapy with oral terbinafine for onychomycosis. METHODS: Of 21 consecutive patients with SCLE attending the outpatient dermatology department at Muenster University clinic during a 1-year period, 4 patients with terbinafine-induced SCLE were seen. Patients were examined fully and photographed; histologic findings as well as serologic and follow-up data were evaluated. RESULTS: In addition to high titers of antinuclear antibodies (ANA) with a homogeneous pattern, anti-Ro(SS-A) antibodies were present; in 3 of 4 women, anti-La(SS-B) antibodies were also found. All patients had anti-histone antibodies as in drug-induced lupus and showed the characteristic genetic association of SCLE with the HLA-B8,DR3 haplotype; moreover, in 2 cases, HLA-DR2 was also present. After discontinuation of terbinafine, ANA titers decreased; anti-histone antibodies also became undetectable within 4(1/2) months in 3 patients concomitant with subsidence of the SCLE eruption in all patients. CONCLUSION: Terbinafine is a drug that appears to infrequently induce SCLE with high titers of ANAs and anti-histone antibodies in genetically susceptible persons.

Administration, Oral↗
PubMed2000

Bilateral upper limb lymphoedema associated with psoriatic arthritis: a case report and review of the literature.

Lymphoedema is an unusual extra-articular feature of rheumatoid arthritis and has rarely been described in psoriatic arthritis. We report a 41-year-old man with psoriasis and psoriatic arthritis who developed bilateral lymphoedema of the upper extremities. Lymphoscintigraphy showed absent lymphatic drainage in the right arm and a subnormal increase in lymphatic flow under manual exertion in both arms. Colour Doppler ultrasound studies did not reveal venous or arterial abnormalities. Conservative management and therapy with cyclosporin (for worsening arthritis) resulted in partial resolution of the lymphoedema and improvement of flow parameters on the right side upon repeat lymphoscintigraphy.

Adult↗
PubMed1999

Disseminated superficial actinic porokeratosis: treatment with topical tacalcitol.

Disseminated superficial actinic porokeratosis is characterized by multiple, brown, annular, keratotic lesions that develop predominantly on the extensor surfaces of the legs and arms during the third and fourth decades of life. No ideal treatment of disseminated superficial actinic porokeratosis has been found. We describe a patient with this condition whose skin lesions responded to topical tacalcitol.

Administration, Topical↗
PubMed1999

Lipodystrophia centrifugalis abdominalis infantilis in a 4-year-old Caucasian girl: association with partial IgA deficiency and autoantibodies.

We report the third case of lipodystrophia centrifugalis abdominalis outside East Asia. A 4-year-old Caucasian girl developed an area of bluish erythema on the left side of the lower abdomen which spread centrifugally to the umbilical and inguinal areas with depression of the skin resulting from the loss of subcutaneous fat, surrounded by an erythematous border. This unusual skin disease was characterized by clinical and histological examination. Laboratory tests revealed a partial IgA deficiency, antinuclear antibodies and IgG antibodies against gliadin.

Abdomen↗
PubMed1999

A child with localized vulval pemphigoid and IgG autoantibodies targeting the C-terminus of collagen XVII/BP180.

Localized vulval pemphigoid of childhood (LVPC) has previously been reported in six girls. Clinical features and immunopathological data have suggested it to be a morphological variant of bullous pemphigoid. Epitope targets of the autoantibodies of these patients have not been defined in detail. We describe a 9-year-old girl with possible cicatricial LVPC and circulating IgG antibodies directed against native collagen XVII/BP180, its 120-kDa soluble ectodomain and against the C-terminus of collagen XVII/BP180. No reactivity was detected towards the NC16A domain of collagen XVII/BP180. Linear IgG and C3 deposits were found along the cutaneous basement membrane zone. On 1 mol/L salt-split skin, IgG autoantibodies were shown to bind to the epidermis, and the HLA type II allele DQB1*0301, a marker with significantly increased occurrence in patients with ocular and oral cicatricial pemphigoid, was identified in this patient. Our data suggest that LVPC is a variant of bullous pemphigoid in which direct immunofluorescence microscopy combined with immunoblot analysis can deliver valuable diagnostic information for differential diagnosis. However, differentiation between the scarring and non-scarring course of the disease cannot be made with the present diagnostic markers and therefore careful follow-up of patients with LVPC is required.

Autoantibodies↗
PubMed1999

Chronic intoxication by doxycycline use for more than 12 years.

We report the clinical case of a 12-years' intoxication by doxycycline. A patient with a depersonalization and derealization syndrome took 1 g doxycycline per day. In addition to hepatocellular necrosis with cholestasis, nephrotoxicity, leukopenia, anaemia and skin hyperpigmentation he suffered from hitherto unreported adverse cardiac events as intermittent supraventricular tachycardia and sporadic Wenckebach heart block. Despite a long period of self-medication these side-effects were reversible.

Adult↗
PubMed1997

Borrelia burgdorferi-associated primary cutaneous B cell lymphoma: complete clearing of skin lesions after antibiotic pulse therapy or intralesional injection of interferon alfa-2a.

We report two patients with low-grade malignant primary cutaneous B cell lymphoma in association with Borrelia burgdorferi infection. Extracutaneous manifestations were ruled out by standard staging procedures. Infection with Borrelia burgdorferi was confirmed by cultivation from lesional skin in both patients. In the first patient skin lesions cleared completely after pulse therapy with cefotaxime, whereas in the second patient antibiotic treatment failed. In this patient, however, skin lesions completely cleared after intralesional injection of interferon alfa-2a. Antibiotic treatment or intralesional injection of interferon alfa-2a should be considered as a first-line treatment of Borrelia burgdorferi-associated primary cutaneous B cell lymphoma before more aggressive conventional therapeutic modalities (e.g., radiation therapy) are applied.

Adult↗