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Biomedical subjects

G Bonsmann

Publications and source records attributed to G Bonsmann.

At least 37 records · Page 2Linked to original sources
PubMed1997

Neutrophilic hidradenitis induced by chemotherapy involves eccrine and apocrine glands.

Neutrophilic eccrine hidradenitis is a self-limited inflammatory dermatosis primarily induced by chemotherapeutic agents. We report the case of a 43-year-old patient treated with cytarabine, daunorubicin, and thioguanine for acute myelogenous leukemia who developed painful, red nodules in both axillae on the third day of chemotherapy. The lesions healed spontaneously without sequelae and reappeared once when chemotherapy was readministered. Histologic examination and immunohistochemical staining for carcinoembryonic antigen (CEA), epithelial membrane antigen (EMA), and S100 revealed necrosis of eccrine and apocrine glands. As a secondary event, neutrophils, histiocytes, and lymphocytes of T and B cell types were infiltrating the glandular coils. Electron microscopic examination confirmed the presence of severe cellular degeneration of the secretory epithelia and coiled sweat ducts. Ultrastructural features and absence of labeling with a nick-end labeling technique were consistent with a non-apoptotic mode of cell death. Our findings strongly suggest a cytotoxic effect of chemotherapeutic agents as accumulated in the secretory epithelia of sweat glands. Distal ducts and myoepithelial cells remained intact and may account for rapid regeneration of the glandular structures after discontinuation of chemotherapy. In view of the involvement of both eccrine and apocrine glands, we suggest the term neutrophilic hidradenitis, which is part of the spectrum of drug-associated sweat gland reactions.

Adult↗
PubMed1996

[Papillomatosis confluens et reticularis. Successful therapy with minocycline].

Confluent and reticulated papillomatosis (CRP) is a rare dermatosis of unknown aetiology. Recent electron microscopic studies suggest that CRP is a disorder of keratinisation. In our case we could not confirm the previously reported ultrastructural findings. CRP is generally resistant to therapy. We treated a 19-year-old patient with typical CRP with oral minocycline. Within a few weeks the eruption resolved completely. A mild relapse 7 months later responded promptly to a repeated course of minocycline. Twelve months after discontinuation of therapy there is no evidence of recurrence. In CRP minocycline should be preferred to systemic retinoid therapy because of its minor side effects.

Adult↗
PubMed1996

[Molluscum contagiosum-like papules within the scope of disseminated infection with Mycobacterium avium complex in an AIDS patient].

A 38-year-old HIV-positive man had several attacks of high fever associated with extensive perspiration over a 10-week period. Simultaneously, he developed molluscum contagiosum-like papules and an erythematous plaque on the face, ulcerated papules on both shoulders and buttocks and subcutaneous nodules on the arms. Histological examination of biopsy specimens revealed a diffuse, histiocytic infiltrate with abundant rod-shaped bacteria. Mycobacterium avium complex was cultured from the tissue and Mycobacterium avium complex DNA was detected by the polymerase chain reaction. The diagnosis of disseminated disease was additionally confirmed by culturing Mycobacterium avium complex from blood, sputum and stool. The skin lesions healed completely within 10 weeks by a multiagent as the patient was treated with a drug therapy. We describe the differential diagnosis, diagnostic procedures and therapy of disseminated infection with Mycobacterium avium complex.

AIDS-Related Opportunistic Infections↗
PubMed1996

Mycosis fungoides presenting as an acquired ichthyosis.

A 25-year-old patient with an acquired ichthyosis-like condition is described. Histologic and molecular biologic examination of the fine, whitish, scaling skin lesions suggested the diagnosis of mycosis fungoides. Typical lesions of mycosis fungoides, such as patches, plaques, or tumors, were not present. To the best of our knowledge, this is the first case of mycosis fungoides presenting solely with the clinical features of acquired ichthyosis. The sudden appearance of ichthyosiform skin changes in adults must be taken seriously and the underlying cause must be evaluated. A skin biopsy should be performed in all cases of acquired ichthyosis to rule out mycosis fungoides.

Adult↗
PubMed1995

[Scarring psoriatic alopecia].

Temporary hair loss is well accepted as a possible result of psoriatic plaques. On the other hand, it is still a controversial issue whether psoriasis can cause scarring alopecia. The following report presents a 38-year-old woman who had been suffering from progressive hair loss from chronic psoriatic plaques of the scalp for some years. Clinical examination revealed an area of scarring alopecia in association with typical features of psoriasis. Histology showed a cord-like fibrosis replacing former hair follicles, a perivascular lymphohistiocytic infiltrate in the upper dermis occasionally invading the follicular epithelium, and characteristic features of psoriasis of the scalp. The clinical course and the lack of evidence for any other causes of scarring alopecia suggest an aetiopathogenetic link between psoriasis and scarring alopecia. Knowledge of this relationship appears to be of practical significance, since efficient antipsoriatic therapy can stop hair loss and thus may be able to prevent scarring.

Adult↗
PubMed1995

[Life threatening angioedema caused by acquired C1 inhibitor deficiency associated with paraproteinemia and livedo racemosa].

A 61-year-old patient with life-threatening angioneurotic oedema was found to have an acquired C1-inhibitor (C1-INH) deficiency. In addition to lowered serum levels of C1-INH (both protein concentration and enzymatic activity), C2, C4 and CH50, which are characteristic for the hereditary form of angioneurotic oedema, markedly lowered C1q was found, which is typical for the acquired form. There were no antibodies against C1-INH. Repeated thorough examination disclosed no neoplasm, though the presence of neoplasm has often been reported to be associated with the acquired C1-INH deficiency. However, the patient showed persistent paraproteinaemia and paraproteinuria and developed livedo reticularis. Treatment with danazol resulted in a rise of the complement fraction levels and cessation of angioneurotic oedema. Paraproteinaemia and livedo reticularis persisted unchanged.

Angioedema↗
PubMed1995

Normolipemic papular xanthomatosis in erythrodermic atopic dermatitis.

We describe papular xanthomatosis that progressively developed in a patient with long-standing erythrodermic atopic dermatitis and normal lipid metabolism and without an associated systemic disease. Light microscopy showed a lobulated aggregate of sometimes foamy histiocytes. Ultrastructurally, these histiocytes contained lipid inclusions and lacked features of Langerhans or epithelioid cells. Other granulomatous skin diseases such as tuberculosis, sarcoidosis, or foreign body granuloma were excluded by histologic study, polarizing microscopic examination, electron microscopy, and microbiologic investigations. Nevertheless, these xanthomas showed an antigen expression pattern similar to that found in noninfectious granulomas (CD1a-, MS-1-, CD11c+, MRP-8/-14+, 25F9+, RM 3/1+/-, CD36(+), indicating that normolipemic papular xanthomatosis may be reactive process and should not be included among the true cutaneous non-Langerhans cell histiocytoses.

Adolescent↗
PubMed1995

Chronic urticaria, arthralgia, raised erythrocyte sedimentation rate and IgG paraproteinaemia: a variant of Schnitzler's syndrome?

Schnitzler's syndrome is a distinct disease entity characterized by the association of chronic urticaria, intermittent fever, arthralgia, elevated erythrocyte sedimentation rate and IgM macroglobulinaemia. We report a patient with the same symptoms, but a monoclonal IgG instead of IgM gammopathy. Histological examination of the urticarial lesions showed signs of mild leucocytoclastic vasculitis. Except for the different class of the monoclonal immunoglobulin, the clinical symptoms, laboratory findings and histology in this patient were identical with those in classical Schnitzler's syndrome. IgG and IgM paraproteins may be equivalent with regard to the putative pathophysiology of the disease process in Schnitzler's syndrome. We therefore suggest that the spectrum of Schnitzler's syndrome is expanded to include patients with chronic urticaria and monoclonal IgG gammopathy, as a closely related variant.

Arthralgia↗
PubMed1994

A unique non-Langerhans cell histiocytosis with some features of generalized eruptive histiocytoma.

A symmetric eruption of hundreds of coalescent small red macules and a few slightly elevated papules sparing the flexures was observed in a 73-year-old man. Light microscopic examination showed loose aggregates of small and large histiocytic cells. Electron microscopy showed an absence of Langerhans cell granules and lipid droplets. Features shared with generalized eruptive histiocytoma were the symmetry of the eruption sparing the flexures, the blue-red coloration, and the absence of lipid-containing foam cells and multinucleated giant cells. However, the primary occurrence of macules rather than papules or nodules, the tendency of the macules to coalesce, and the dimorphic histiocytoid infiltrate are not found in generalized eruptive histiocytoma. Nevertheless, immunohistochemistry confirmed that this unique condition is a form of MS-1+ cutaneous non-Langerhans cell histiocytosis.

Aged↗
PubMed1993

Immunohistochemical comparison of cutaneous histiocytoses and related skin disorders: diagnostic and histogenetic relevance of MS-1 high molecular weight protein expression.

Twenty-nine cases of Langerhans cell histiocytosis (LCH), non-Langerhans cell histiocytoses (N-LCH), non-infectious granulomas, and fibroblast-related lesions were examined with a panel of monoclonal and polyclonal antibodies on freshly frozen tissue sections to characterize the macrophage phenotype of N-LCH syndromes. MS-1 high molecular weight extracellular protein, specific for sinusoidal endothelial cells and dendritic perivascular macrophages in normal human organs, was expressed by N-LCH cells but was not found in LCH cells, epithelioid cells in sarcoidosis, or palisading histiocytes in granuloma annulare. The subcellular location of MS-1 protein, i.e., cytoplasmic vs. peripheral/extracellular, allowed discrimination of small and large (foamy or multinucleated) N-LCH cells. MS-1-positive cells, which were found intermingled in cellular dermatofibromas but not in fibrous dermatofibromas, differed from MS-1-positive N-LCH cells by their dendritic morphology, and thus rather resembled their normal dermal counterparts. A preserved functional relationship of these two MS-1-positive cell types was indicated by the fact that N-LCH and cellular dermatofibromas were the only lesions found to be highly vascularized. As expected, CD1a showed high specificity for LCH, while CD34 was predominantly expressed by fibroblast-related lesions; in cellular dermatofibromas, CD34 and MS-1 expression partially overlapped. The other antigens tested showed non-specific or overlapping patterns of expression. In conclusion, assessment of MS-1 protein expression (in addition to assessment of CD1a and CD34) promises to be of diagnostic value in the discrimination of N-LCH from related skin disorders, and it may indicate a common differentiative pathway for most N-LCH disease entities.

Antibodies, Monoclonal↗
PubMed1993

[Dyshidrosiform bullous pemphigoid].

Dyshydrosiform bullous pemphigoid is a clinical variant of bullous pemphigoid in which the lesions appear primarily on palms and soles. Knowledge of this unusual manifestation of bullous pemphigoid may be of practical relevance, since it has to be included in the differential diagnosis of blistering palmoplantar dermatoses. The correct diagnosis is confirmed by histological examination and immunofluorescence, which reveal all the characteristic hallmarks of bullous pemphigoid. Three patients with dyshydrosiform bullous pemphigoid are presented; clinical and histological aspects, findings on immunofluorescence, differential diagnosis and therapy are discussed.

Aged↗
PubMed1993

[Incontinentia pigmenti in a male infant].

Bloch-Sulzberger incontinentia pigmenti (IP) is a rare X-linked neuroectodermal syndrome. Over 97% of the patients are female. We report on a male baby who developed blisters in linear groups or bands shortly after birth. When the child was 3 months old the blisters were followed by verrucous papules, which cleared after 1 year leaving areas of brownish grey hyperpigmentation. In addition to the skin involvement, our patient showed central motor dysfunction on the right side of the body and also dental and ocular anomalies. Both parents were in good health. Chromosome analysis yielded a normal karyotype (46, XY). The genes for coagulation factor VIII and biglycan in the Xq28 region were not deleted. The presence of the disease in this male infant may be due to an early somatic mutation or a half-chromatid mutation. A further possibility is mosaic expression of an unstable premutation. This model offers a good explanation for the reports in the literature of transmission of the disease from mother to son.

Diagnosis, Differential↗
PubMed1993

[The Köbner phenomenon in Duhring dermatitis herpetiformis].

We report on four female patients with confirmed dermatitis herpetiformis DH and Köbner phenomenon in identical localizations. All four had typical bilateral lesions in a band-like arrangement along the straps of their brassieres, and two patients also had additional lesions in other sites prone to pressure. Obviously, the Köbner phenomenon is not as rare in DH as would be expected in view of a single previous case report.

Adult↗
PubMed1993

[Dexamethasone-cyclophosphamide pulse therapy in bullous autoimmune dermatoses].

The problem in the treatment of bullous autoimmune dermatoses with long-term corticosteroids is that the high doses cause side-effects. An alternative form of therapy with high-dose dexamethasone-cyclophosphamide pulses was used to treat 20 patients between 33 and 86 years: 7 patients had bullous pemphigoid, 6 pemphigus vulgaris, 5 pemphigus foliaceus, and 2 cicatricial pemphigoid. On each of days 1-3 100 mg dexamethasone was administered i.v. and on day 1, 500 mg cyclophosphamide i.v. In the therapy interval between the pulses 50 mg cyclophosphamide per day. Initially the pulses were repeated every 2 weeks and later at 10-week intervals. After 6 months of this regimen 13 patients were symptom-free, 4 had improved, and 3 showed no change. The efficacy of treatment was equal in newly diagnosed and previously treated cases. (Side-effects were leucopenia (n = 3), myalgia and arthralgia (n = 2), taste disturbance (n = 2), diffuse hair loss (n = 2), thrombophlebitis (n = 1), herpes zoster (n = 1) and a delayed-type hypersensitivity reaction to mesna. Owing to the rather short follow-up, no conclusions on long-term side-effects of this therapy are possible. However, dexamethasone-cyclophosphamide pulse therapy appears so far to be a good alternative to the standard continuous corticosteroid treatment.

Adult↗
PubMed1992

[Cervix cancer in HPV16-associated Bowenoid papulosis].

In a 38-year-old female patient with bowenoid papulosis of the anogenital region and an extensive carcinoma in situ of the cervix uteri, HPV16 DNA was found in the biopsy specimens and HPV16/18 DNA was detected in the cervical swab. The known coincidence of both these diseases indicates the necessity for careful regular gynaecological check-ups of patients with bowenoid papulosis.

Adult↗
PubMed1992

Generalized lichenoid juvenile xanthogranuloma.

A generalized eruption of small, flat and shiny papules was observed in an otherwise healthy 6-month-old baby. The light and electron microscopical and immunohistological features of the self-healing lesions were consistent with juvenile xanthogranuloma and suggested an atypical lichenoid variant of this non-Langerhans cell histiocytosis.

Female↗