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Biomedical subjects

S Lautenschlager

Publications and source records attributed to S Lautenschlager.

At least 37 records · Page 2Linked to original sources

[Skin manifestations of graft-versus-host reaction following bone marrow transplantation].

We review the cutaneous manifestations of acute and chronic graft versus host disease (GvHD). Acute GvHD is characterized by initial itching, pain on pressure and erythema which begins on posterior auricular skin, palms and soles. The disease evolves into a typical but nonspecific maculopapular rash. Confluent rashes and follicular erythema may occur. Erosive oral lesions usually develop. The most severe variant of GvHD is toxic epidermal necrolysis, which often has a fatal outcome. The onset of chronic GvHD usually occurs more than 100 days after bone marrow transplantation and may be preceded by the acute form. The spectrum of skin changes includes lichenoid pruritic lesions with violaceous color and scleroderma-like skin involvement. Investigation of unknown rashes in these patients includes skin biopsy, which clearly differentiates leukocytoclastic vasculitis and erythema exsudativum multiforme with lymphocytic vasculitis from cutaneous manifestations of GvHD. Special stains may reveal bacteria and fungus in septicemic patients. The therapeutic options are discussed.

Acute Disease↗

[Correlation between chronic urticaria and depression/somatization disorder].

In a multicenter study, 100 patients with chronic urticaria were examined with a standardized personality test (Giessen test), a standardized symptom questionnaire (Giessener Beschwerdebogen) and a specially developed questionnaire concerning symptoms, history and behaviour during symptomatic periods. Almost one third of the patients showed elevated scores both for depression and for symptoms that are often associated with depression. It therefore seems worth-while examining such persons more specifically and possibly treating them by psychosomatic methods. The present results do not allow the classification of chronic urticaria as a somatization disorder. Since one-third of the patients had symptoms of depression, combined dermatological and psychosomatic approach may make it possible to offer them an appropriately targeted treatment.

Adolescent↗

Mutations of keratinocyte transglutaminase in lamellar ichthyosis.

Lamellar ichthyosis is a severe congenital skin disorder characterized by generalized large scales and variable redness. Affected individuals in three families exhibited drastically reduced keratinocyte transglutaminase (TGK) activity. In two of these families, expression of TGK transcripts was diminished or abnormal and no TGK protein was detected. Homozygous or compound heterozygous mutations of the TGK gene were identified in all families. These data suggest that defects in TGK cause lamellar ichthyosis and that intact cross-linkage of cornified cell envelopes is required for epidermal tissue homeostasis.

Base Sequence↗

Zosteriform metastases in melanoma.

Zosteriform metastasis is a rare form of tumor spread to the skin that most often arises from an internal carcinoma or a hematologic malignancy. We describe a 29-year-old woman with malignant melanoma of the back in whom zosteriform metastases developed along the fifth thoracic dermatome.

Adult↗

Palmoplantar keratoderma and associated syndromes.

This article focuses on the current state of knowledge concerning the characterization and classification of palmoplantar keratoderma and associated syndromes. In addition, therapeutic options are discussed. Exact diagnosis enables dermatologists to give patients accurate genetic counseling and may help to detect underlying defects or proneness to cancer. Furthermore, precise classification of this disease facilitates the use of the most efficient therapeutic modalities. Important criteria for the classification of palmoplantar keratoderma are the mode of transmission, age at onset, and distribution of the keratoderma. The disorder may be diffuse or focal; it may be restricted to the palms and soles or also involve the dorsal aspects of the hands and feet. Psoriatic-like lesions in other parts of the body may occur in certain variants. The association of other signs and symptoms may provide diagnostic clues. Ultrastructural investigation will show diagnostic features in some types of palmoplantar keratoderma, and biochemical analysis may be helpful in the classification of keratoderma. In the future, the most accurate diagnosis will be the identification of the genetic defect and its chromosomal localization.

Diagnosis, Differential↗

[Skin changes in HIV infections].

The spectrum of dermatologic findings related to human immunodeficiency virus includes a variety of cutaneous and mucocutaneous disorders. The most frequent diagnoses are oral candidiasis, seborrheic dermatitis, pyodermas and Kaposi's sarcoma. Distinctive skin lesions occur at various stages of HIV infection. Especially herpes zoster, seborrheic dermatitis and oral candidiasis may act as indicators, and their recognition is of particular importance for the early diagnosis of HIV infection and for the prevention of further opportunistic infections. In addition, some dermatologic findings as mollusca contagiosa and Kaposi's sarcoma occur mostly as late manifestations and may constitute a cutaneous correlate of advanced cellular immune deficiency.

AIDS-Related Opportunistic Infections↗

Lymphoproliferative responses to Borrelia burgdorferi in patients with erythema migrans, acrodermatitis chronica atrophicans, lymphadenosis benigna cutis, and morphea.

BACKGROUND AND DESIGN: Specific humoral and cell-mediated immune responses play an important role in the pathogenesis of Lyme borreliosis. Several previous studies demonstrated that a specific cellular immune response to Borrelia burgdorferi can occur independently of a diagnostic humoral response. Little is known about T-cell reactivities against B burgdorferi in early and late cutaneous manifestations of Lyme borreliosis. We studied the lymphoproliferative response of peripheral blood mononuclear cells to B burgdorferi antigen from 99 patients (25 with erythema migrans, 16 with acrodermatitis chronica atrophicans, 13 with lymphadenosis benigna cutis, and 45 with localized scleroderma) and 21 control subjects. The results are expressed as a stimulation index (SI) (mean count per minute of triplicate cultures with stimulant divided by mean count per minute without stimulant). The serum samples from all patients and control subjects were tested for antibodies to B burgdorferi by indirect immunofluorescence assay. RESULTS: The 21 healthy seronegative controls had an SI of 3.3 +/- 2.0 (mean +/- SD). Compared with that of control subjects, the SIs were significantly elevated in patients with erythema migrans (9.8 +/- 9.1), acrodermatitis chronica atrophicans (11.8 +/- 8.2), and lymphadenosis benigna cutis (7.2 +/- 6.2). The 45 patients with localized scleroderma had elevated proliferative responses, with an SI of 6.5 +/- 7.3, but these responses did not significantly differ from those of controls. Elevated titers of antibodies to B burgdorferi were present in six (24%) of 25 patients with erythema migrans, five (38%) of 13 patients with lymphadenosis benigna cutis, and 13 (29%) of 45 patients with localized scleroderma. All 16 patients with acrodermatitis chronica atrophicans had markedly elevated antibody titers. CONCLUSIONS: Our findings show that a significant lymphoproliferative response to B burgdorferi occurs in the majority of patients with cutaneous manifestations of Lyme borreliosis. The lymphocyte proliferation assay may be of diagnostic value in patients in whom Lyme borreliosis is strongly clinically suspected and who have nondiagnostic levels of antibodies against B burgdorferi.

Acrodermatitis↗

A new clinical disorder of twisted and rolled body hairs with multiple, large knots.

BACKGROUND: We present a new hair disorder characterized by an unusual twisting and matting of body hairs. This disorder may occur as an acquired or, possibly, an inherited trait. OBJECTIVE: Our purpose was to analyze the clinical features of three patients and report the changes revealed by light microscopy and scanning electron microscopy. METHODS: We examined two unrelated adults with acquired multiple large knots of body hairs and a newborn infant with a familial history of the same entity. RESULTS: In the two adults the hairs were rolled and knotted together, and twisting of body hairs occurred in areas where rubbing was frequent. In the familial type, which was suggestive of an autosomal dominant inheritance pattern, no mechanical explanation could be found. Light microscopy confirmed the clinical impression of twisted and felted hairs, and scanning electron microscopy revealed sticking of at least 20 hairs. No underlying skin disease or deeper process was found. CONCLUSION: Multiple twisted and rolled body hairs that may develop into multiple, large knots may appear as a minor variant of hair matting or felting. Scanning electron microscopy shows multiple hairs that originate from different hair follicles and roll and stick together centrally.

Aged↗

The petrified ear.

A case of an otherwise healthy 66-year-old male with bilateral symmetric ossification of his ears is described. The rare occurrence of petrified ears is emphasized, and possible etiologic factors are discussed. No other explanation than anamnestic prolonged exposure to hypothermia was found.

Aged↗

Natural history of the Naegeli-Franceschetti-Jadassohn syndrome and further delineation of its clinical manifestations.

BACKGROUND: The Naegeli-Franceschetti-Jadassohn (NFJ) syndrome is rare; only three families have been reported. OBJECTIVE: Our purpose was to determine the natural history of this ectodermal dysplasia and to delineate further its clinical manifestations. METHODS: We reexamined the original family with the NFJ syndrome 65 years after the first description. RESULTS: The pedigree includes 62 members with 14 affected patients. We examined the 10 living patients. Longitudinal analysis of the pedigree revealed that the reticulate pigmentation fades after puberty and may disappear completely in old age. Hypohidrosis, the main problem for the patients, remains constant. Teeth are always severely affected, leading to early total loss. All patients lack dermatoglyphics. Diffuse palmoplantar keratoderma may coexist with punctate keratoses that are sometimes accentuated in the creases or exhibit a linear pattern. Four patients had congenital malalignment of the great toenails, not described previously in association with the NFJ syndrome. CONCLUSION: The NFJ syndrome is an ectodermal dysplasia with numerous specific abnormalities.

Adult↗

Localized scleroderma associated with Borrelia burgdorferi infection. Clinical, histologic, and immunohistochemical observations.

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.

Adolescent↗