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Biomedical subjects

H Gollnick

Publications and source records attributed to H Gollnick.

At least 19 recordsLinked to original sources
PubMed1996

[Therapeutic experiences with extracorporeal photopheresis. Technical procedure, follow-up and clinical outcome in 31 skin diseases].

Extracorporeal photophoresis (ECP), a therapeutic modality that has been under investigation for some years, is based on separation of a leucocyte/lymphocyte-enriched cell fraction from the peripheral blood, extracorporeal treatment of the cells with 8-MOP/UVA and subsequent reinfusion of the cells in the patient. Its main effects seem to consist in changes to the immunologic behaviour of the photoinactivated/modulated cells. The immune response of the host is obviously stimulated by this treatment. ECP is normally performed for 4 h per day on 2 consecutive days every 4 weeks. The treatment is well tolerated and causes few side effects. In our department, 1210 ECP treatments were administered to 41 patients between 1990 and 1994 and a preliminary evaluation was performed. These patients included 21 with cutaneous T-cell lymphoma (CTCL), 10 with progressive systemic scleroderma, 4 with chronic graft-versus-host disease and 1 each with pemphigus vulgaris, epidermolysis bullosa acquisita, lupus erythematosus and cutaneous mucinosis. Patients with erythroderma and preserved immunocompetence achieved the best responses of all patients with CTCL. A treatment combining ECP with rIFN-alpha, PUVA and/or radiation was also successful in patients with tumour-stage CTCL and lymph node involvement. Progressive systemic scleroderma responded in more than 50% of our cases. Treatment results were impressive in 4 patients with chronic graft-versus-host disease presenting with sclerodermatous and lichenoid changes of the skin and mucous membranes. A clear improvement was also observed in the patient with pemphigus vulgaris refractory to standard therapies and in another patient with scleromyxoedema (Arndt-Gottron syndrome). The effectiveness of ECP seems to be quite well established in CTCL, but remains to be examined in autoimmune dermatoses. ECP is an attractive addition to the dermatological therapies available but our experience is still preliminary.

Adult↗
PubMed1996

Hypotrichosis, hair structure defects, hypercysteine hair and glucosuria: a new genetic syndrome?

Structural hair changes may be the expression of a genetic disorder affecting hair growth, part of a congenital syndrome with accompanying hair malformations, or a marker for an underlying metabolic disorder. We report a 22-month-old Turkish girl and her 10-month-old brother, whose scalp hair became fragile and sparse at about 6-7 months of age. Glucosuria, without diabetes or kidney disease, was detected 3-4 months later. Clinical examination revealed normal physical and mental development, and an analysis of plucked hairs showed dysplastic and broken hair shafts. Polarizing microscopy and scanning electron microscopic studies revealed torsion, and irregularities and impressions of the hair shaft, as seen in pili torti, trichorrhexis nodosa and pseudomonilethrix. Analysis of the amino-acid composition of the hair demonstrated a significant reduction of sulphonic cysteic acid and an elevated cysteine and lanthionine content in the girl, and elevated lanthionine levels in her brother. Electrophoretic analysis of the girl's hair proteins revealed a normal composition but a high extractability of hair proteins. The triad of hypotrichosis, structural hair-shaft defects and abnormal amino-acid composition, accompanied by glucosuria without diabetes, may represent a new genetic syndrome.

Amino Acids↗
PubMed1995

[Large cell anaplastic Ki-1 positive lymphoma of the skin. 5 personal cases and review of the literature].

Primary cutaneous large cell anaplastic non-Hodgkin lymphomas positive for Ki-1-antigen are rarely described. There are 100 published cases worldwide. Typically large cell anaplastic lymphomas have an inflammatory appearance, which often leads to false diagnosis and unsuccessful treatment with antibiotics. Histological examination reveals a highly malignant non-Hodgkin lymphoma. The tumour is composed of large pleomorphic lymphoid cells composed of T-cells in 80% of the cases and of B-cells in 10%. The immunological phenotype in the remaining 10% remains unclear. Crucial for the diagnosis is the expression of CD30 antigen in > 70% of the tumour cells. This article presents 5 cases of cutaneous Ki-1-positive lymphoma seen in our Berlin department during the last 10 years. In 4 patients the diagnosis was established in clinical stage I of cutaneous lymphoma without further manifestation; 1 patient had lymph node involvement and was in stage II. Total excision of the primary tumour in stage I with adjuvant polychemotherapy in stages II-IV led to complete remission in all cases. Long-term remissions were seen in case 1 (2 years) and in case 5 (1 year), whilst 2 patients showed local relapse, and 1 patient showed generalized lymphogenic and hematogenic metastasis. After repeated surgical removal or irradiation of the tumour and adjuvant polychemotherapy, further complete remission was achieved in 2 patients (up to now lasting 1 and 4 years). another patient has been in partial remission for the last 2 years. Our observations underline the high relapse rate of large cell anaplastic lymphoma.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1995

[Transformation of multiple basaliomas to squamous cell carcinomas in an HIV patient with Gorlin-Goltz syndrome].

We report on a 32-year-old male patient with Gorlin-Goltz syndrome, who presented with excessive numbers of superinfected basal cell carcinoma. This led us to suspect an underlying HIV infection, which was confirmed by ELISA and Western blotting. Laboratory investigation of the immunological state revealed severe immunosuppression with 267 CD4+ lymphocytes and a CD4/CD8 ratio of 0.3. The histological picture showed multiple basal cell carcinomas, some of them transforming into squamous cell carcinomas. We suspect that the excessive number and the unusual clinical and histological picture of the basal cell carcinomas in this patient were probably influenced by the underlying HIV infection.

AIDS-Related Opportunistic Infections↗
PubMed1995

[Generalized molluscum contagiosum in an African child with AIDS].

A 12-year-old girl from Zaire with AIDS (CDC: P2 D1) presented with a generalized molluscum contagiosum infection. She had suffered from systemic cryptococcosis and from cryptosporidiosis several months before admission. While molluscum contagiousum infection is usually a self-limiting disease in immunocompetent persons, a fulminant appearance and persistence of giant mollusca occurs with advanced immunodeficiency. Histological and immunohistological examinations showed a severe diminution of Langerhans and T cell populations that might enhance the dissemination of the infection. Molluscum-like lesions of cryptococci have been described, and cutaneous cryptococcosis is the main condition to be considered in the differential diagnosis. Further differential diagnoses should include American and African histoplasmosis, and the cutaneous manifestations of mycobacterial infections, of toxoplasmosis and of Pneumocystis carinii infection.

AIDS-Related Opportunistic Infections↗
PubMed1994

[Cutaneous drug reaction caused by suramin in 4 patients with metastatic prostate cancer].

Suramin has long been used in the treatment of onchocerciasis and trypanosomiasis. Recent investigations showed an antiproliferative effect of suramin on prostate carcinoma cell lines. Ongoing clinical trials have confirmed the effect of suramin on metastasizing prostate carcinoma. Five patients with metastatic prostate carcinoma resistant to classic hormonal therapy were treated with high-dose suramin. Four of the five developed a papulovesicular or maculopapular rash within 2-4 weeks of starting therapy. The rashes disappeared after discontinuation of suramin within 10 days. In tumour therapy, high dosages of suramin are used to achieve a serum level of 200-300 micrograms/ml. Therefore, a toxic reaction to suramin is suspected as the explanation for the skin rashes.

Aged↗
PubMed1994

[Multicentric reticulohistiocytosis and myelodysplastic syndrome].

Multicentric reticulohistiocytosis (MRH) is a rare disorder of skin and joints, and its aetiology is unknown. We describe a male patient who had first developed osteoblastic lesions of the skeleton in 1976 at the age of 59 years. Three years later, he presented with multiple papular and nodular skin lesions, predominantly on the limbs and upper trunk, and 4 years later he developed painful arthropathy. In 1988 skin examination showed multiple brownish-red papulo-nodules, some of which, at mechanically stressed areas, were ulcerated. The patient complained of pronounced arthrotic disorders with reduced joint mobility. Examination of biopsy specimens from active skin lesions demonstrated extensive numbers of macrophages with partial confluence to giant cells with typical ground-glass cytoplasm. Electron microscopy revealed phagocytosis of collagen-like-structures. Examination of bone marrow biopsy specimens revealed diffuse infiltration by histiocytic cells. The diagnosis made was therefore MRH. The patient was treated for several years with different therapeutic regimens, including azathioprin, dapsone, prednisolon, chlorambucil and cyclophosphamide, and complete remission of skin lesions and of bone marrow infiltration was observed. However, a myelodysplastic syndrome with refractory anaemia and ring sideroblasts developed, which is generally understood to be a preleukaemic condition. Myelodysplastic syndromes commonly develop between 3 and 10 years after the start of a therapy with antineoplastic agents.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1994

Ulerythema ophryogenes and keratosis pilaris in a child with monosomy 18p.

We report a 13-year-old boy with deletion of the short arm of chromosome 18 and follicular, partially inflammatory, keratotic papules of the eyebrows, foreskin, and cheeks (ulerythema ophryogenes) as well as the shoulders, upper back, upper arms, and thighs (keratosis pilaris), initially diagnosed as atopic dermatitis. Over 100 patients with this genetic defect have been reported, and the 18p- syndrome is considered one of the most frequently occurring deletion syndromes. However, ulerythema ophryogenes and keratosis pilaris have not been described in any of these patients, although the association of the latter with other genetic abnormalities is well known. Keratosis pilaris is a relatively common genodermatosis of ectodermal origin, frequently occurring with ichthyosis or atopy; concomitance with ulerythema ophryogenes has also been reported. The association of chromosome 18p deletion defect and ulerythema ophryogenes may be helpful in future attempts to localize the gene defect responsible for follicular genokeratoses.

Child↗
PubMed1994

Progressive differentiation of human sebocytes in vitro is characterized by increasing cell size and altering antigen expression and is regulated by culture duration and retinoids.

Increasing cell size, lipid accumulation, and altered antigen expression are features of sebaceous differentiation in vivo. Enhanced lipid synthesis with progressive differentiation is also present in cultured human sebocytes. This study was conducted to investigate the evolution of cell size and antigen expression of human sebocytes with progressive differentiation in vitro. Subconfluent human sebocyte cultures were examined for sebocyte differentiation evaluated on cytocentrifuge preparations by light microscopy and classified in stages according to morphological criteria described for sebocytes in vivo. Rates of 5.1 +/- 2.2% undifferentiated sebocytes, 29.2 +/- 4.9% early differentiated, 20.7 +/- 4.1% advanced differentiated, 37.6 +/- 6.4% fully differentiated, and 5.9 +/- 1.9% mature sebocytes were calculated in secondary cultures. The size of cultured sebocytes measured by computer-assisted planimetry significantly increased with progressive differentiation up to 4-5.5 times. The low rates of mature sebocytes and the only moderate increase of their size with progressive differentiation indicate an incomplete terminal differentiation in vitro. Sebocytes were subsequently stained with a series of monoclonal antibodies (mAb) to determine antigen expression using the alkaline phosphatase anti-alkaline phosphatase technique. The number of sebocytes labeled with the anti-keratin mAb CK8.12 and KL1, and the mAb 34D11 (82 kD protein) increased with progressive differentiation; significant differences were found after comparing early and advanced differentiated sebocytes. Sebocytes were positively stained with the anti-keratin mAb 6B10 (K 4), RPN1162 (K 7), CK13 (K 13), RPN1165 (K 19), CK8.60, and the mAb 115F5 (MAM-6c), OM-1 (sebaceous gland antigen), and 24F10 (basic polypeptides) only at late-stage differentiation. The expression of keratins 4, 7, 13, and 19 was confirmed by gel electrophoresis and immunoblotting. The data obtained were used to study the effects of the duration of cultivation and of the retinoids isotretinoin and tretinoin on sebocyte differentiation in vitro. Subcultivation of sebocytes upregulated, and treatment with isotretinoin but not with tretinoin downregulated labeling with mAb which recognize indicating progressive and late-stage differentiation.

Antigens, Differentiation↗
PubMed1994

Further evidence for involvement of both cell mediated and humoral immunity in generalized vitiligo.

Immunohistochemical and immunoserological evidence supports the involvement of both cell-mediated and humoral mechanisms in the pathogenesis of melanocyte destruction in vitiligo. Punch biopsies from depigmented vitiliginous skin (VS), normal-looking pigmented skin (PS), and marginal skin (MS) from patients with generalized vitiligo (n = 15) were labeled with K 1.2.58, OKM1 (CD11b), Leu 11b (CD16), Leu 19 (CD56), IFN-gamma receptor, IL-2 receptor (CD25), IgG, IgM, C3c, and C3d MoAbs. In addition, in vitro effects of vitiligo sera (n = 13) on human newborn melanocytes (HMel) under different culture conditions were studied. The immunohistochemical findings showed absence of K 1.2.58+ epidermal melanocytes in VS and abnormal morphology in MS. In these areas, a few CD11b+ cells in the dermis and epidermis could be detected but no significant numbers of CD16+ or CD56+ cells were seen among the mononuclear cellular infiltrate. IL-2 and IFN-gamma receptors were clearly expressed by the cellular infiltrate. No significant deposition of complement or immunoglobulin was seen. The addition of vitiligo sera to HMel cultures induced a significant cellular proliferation. The stimulation of cell proliferation occurred regardless whether the sera were added alone or when preheated (56 degrees C for 1 hr) and then supplemented with a complement source (P < 0.01 at 2%, P < 0.001 at 10%, and P < 0.01 at 20% for sera alone) (P > 0.05 at 2%, P < 0.05 at 10%, and P < 0.01 at 20% for decomplemented sera plus complement).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1994

Successful treatment of chronic graft-versus-host disease with extracorporeal photopheresis.

A 43-year-old woman who had undergone allogeneic bone marrow transplant for chronic myeloid leukemia developed chronic GVHD. Despite cyclosporine A and low-dose glucocorticoids and later concomittent PUVA-therapy, chronic GVHD with severe sclerodermatous manifestations including joint contracture and liver damage progressed. The Karnofsky performance score dropped to under 50%. Extracorporeal photopheresis (ECP) instead of PUVA-therapy was started. To our knowledge, we here report the first case of successful treatment of extensive chronic GVHD with ECP.

Adult↗
PubMed1993

Comparison of Borrelia burgdorferi ultrasonicate and whole B, burgdorferi cells as a stimulus for T-cell proliferation and GM-CSF secretion in vitro.

Peripheral blood mononuclear cells (PBMC) from five patients with confirmed Lyme borreliosis (LB) and from seven seronegative healthy subjects were incubated with ultrasonicated sterile-filtrated B. burgdorferi and with whole live Borrelia burgdorferi cells (strain RT1). PBMC culture conditions were 1 x 10(5) PBMC/well/ 10% autologous plasma (AP) and 2 x 10(5) PBMC/well/ 10% pooled human AB-serum (ABS). Recall antigens and Pokeweed mitogen (PWM) served as specific and nonspecific control stimuli. When whole B. burgdorferi cells were used as antigen, both the borreliosis patients (borr) and the healthy subjects (contr) reacted with an elevated stimulation index (SI) and secretion of granulocyte-macrophage colony stimulating factor (GM-CSF) in vitro (SI borr. = 4; SI contr. = 3.7. GM-CSF borr. = 3.1 U/ml; GM-CSF contr. = 5.4 U/ml). A significant difference was observed when the B. burgdorferi sonicate was used. In this case, only the borreliosis patients reacted with an elevated PBMC proliferation and GM-CSF secretion (SI borr. = 6.1; SI contr. = 1.4. GM-CSF borr. = 7.6 U/ml; GM-CSF contr. = 0.3 U/ml) (p < 0.001).

Adult↗
PubMed1993

Further evidence for Borrelia burgdorferi infection in morphea and lichen sclerosus et atrophicus confirmed by DNA amplification.

We present further evidence in support of the notion that Borrelia burgdorferi is possibly involved in the pathogenesis of morphea and lichen sclerosus et atrophicus (LSA). Running a nested polymerase chain reaction (PCR) with a primer set specific for the flagellin gene of B. burgdorferi enabled us to demonstrate the presence of Borrelia DNA in skin biopsies of patients with morphea (nine of nine) of LSA (six of six). Biopsy specimens obtained from patients with erythema chronicum migrans (two patients, four of four samples) and acrodermatitis chronica atrophicans (one patient, one of one sample) also showed positive PCR results. By contrast, there was no amplification of Borrelia DNA in control biopsies either from patients with chronic eczema (three of three) or psoriasis (two of two) or from normal skin (three of three). Antibodies directed against B. burgdorferi were only detected in the serum of patients with erythema chronicum migrans (two of two) and acrodermatitis chronica atrophicans (one of one) but were not present in cases of morphea (five of five), LSA (three of three), or in control subjects (three of three). These data suggest that B. burgdorferi may play a role in the pathogenesis of both morphea and LSA. Furthermore, we conclude that PCR analysis provides an important diagnostic tool, even in seronegative Borrelia infections.

Adolescent↗
PubMed1993

Aquagenic pruritus as a presenting symptom of polycythemia vera.

A female patient was presented because of a prickling sensation that appeared shortly after warm water contact. Examination revealed no abnormality, but water exposure was followed by pruritus without any visible skin changes. Blood and bone marrow examinations revealed abnormalities typical of polycythemia vera. Skin biopsy before and after warm water challenge showed increased numbers of mononuclear cells in the papillary dermis and epidermis particularly after water exposure. Phlebotomy was associated with prompt cessation of pruritus.

Biopsy↗
PubMed1993

[Nevoid acquired perforating dermatosis of vellus hair follicles--a new entity? Case report with immunohistochemical studies].

We report on a 45-year-old male patient with a 15-years history of partly excoriated and inflamed lesions of verruccous-papular character, arranged in a linear naevoid fashion on the extensor side of the right arm and shoulder. The lesions were not related to a dermatoma but seemed to be located in the Blaschko's lines. On histology an epidermal invagination was found, conspicuously arranged over proliferating vellus hair follicles, which were increased in number as in a hamartoma. The rete ridges were directed towards early anagens of the vellus hairs with consecutive crateriform invagination of the epidermis with parakeratosis and basophilic debris on the innermost layer of the invagination. Serial sections allowed demonstration of incomplete and complete perforation with accumulation of neutrophils. Immunohistochemistry revealed marked staining of basal cells of the proliferating epidermal keratinocytes with cytokeratins 14 and 18 within the lesion. No association with diabetes or renal insufficiency and no familial background could be found.

Adult↗
PubMed1993

[Bacillary epithelioid angiomatosis in advanced HIV infection].

A patient with advanced HIV infection developed multiple angiomatous papules and nodules on the upper chest within a few days. At first sight the lesions resembled disseminated Kaposi's sarcoma; the differential diagnosis, however, included eruptive haemangiomas and pyogenic granulomas. Such distinct clinical characteristics as the collarette-like desquamation at the borders of the tumours led to the suspicion of bacillary epithelioid angiomatosis in HIV infection, which was then confirmed by histology and ultrastructural demonstration of bacillary colonies within the lesions. Under systemic antibiotic treatment, marked regression of the lesions was quickly observed within 1 week and complete regression occurred after 4 weeks. It is important to consider bacillary angiomatosis in HIV infection in the differential diagnosis of Kaposi's sarcoma, and it is a separate entity in the form of angioproliferation caused by bacteria.

AIDS-Related Opportunistic Infections↗