Biomedical subjects
Publications and source records attributed to S A Büchner.
Minocycline is another possible alternative therapy for pemphigus vulgaris. We successfully treated a Jewish patient with oral pemphigus vulgaris with with minocycline and nicotinamide. Minocycline may be a useful second-line drug for therapy- resistant cases of pemphigus vulgaris.
Darier disease is an autosomal dominant disorder which may occasionally become manifest in a segmental form. Two clinical phenotypes with a different genetic background have been elaborated in recent years. More than 50 patients with isolated linear disease expression have been documented. In this phenotype the skin outside the segmental affection is absolutely normal. Such a phenotype is explained by a postzygotic mutation with somatic mosaicism which was labeled as type 1 manifestation of segmental forms in autosomal skin disorders. A patient with classical type 1 segmental Darier disease is presented. On the other hand, only 3 patients with Darier disease showing a segmental manifestation in combination with a diffuse distribution have so far been observed. These cases correspond to the recently described type 2 manifestation of segmental forms of autosomal dominant disorders. We describe a fourth patient with type 2 segmental Darier disease. The genetic explanation of such a phenotype is possible with the assumption that a germline mutation for the disease exists but, in addition, a postzygotic mutation is needed resulting in loss of heterozygosity. Hence, in a circumscribed region a homozygous or hemizygous state of the mutation is apparent which can explain the enhanced severity of the segmental manifestation.
Onchomycosis is the most common nail disease, accounting for approximately 30% of all cutaneous fungal infections. The treatment approach needs to take into account the location and extent of onychomycosis, sensitivity of drug to fungal organism, adverse-effects profile, dosage schedule, duration of therapy, concomitant medical conditions, and concurrent medications. To confirm the diagnosis, it is important to correctly select the appropriate site for specimen collection used for both direct microscopy and fungal culture. Topical antifungal agents may be considered for the treatment of early onychomycosis, in the absence of nail matrix involvement. The newer generation of oral antifungal agents for the treatment of onychomycosis are terbinafine, itraconazole and fluconazole. These drugs used alone, or in combination with topical antifungals, are providing the basis for effective treatment of onychomycosis in a large proportion of patients.
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BACKGROUND: Intolerance to cow's milk protein is frequently seen in children and rarely in adults. Non-IgE-mediated hypersensitivity to cow's milk protein has been suspected in children based on in vitro evidence. Food intolerance may play a pathogenetic role in some cases of Melkersson-Rosenthal syndrome, which is often of unknown origin. OBJECTIVE: We describe an adult female patient who developed a Melkersson-Rosenthal syndrome and at the same time was found to have in vivo and in vitro evidence of a delayed-type hypersensitivity to cow's milk protein. METHODS: Allergic and immunologic examinations, including skin prick tests, patch tests, serology, lymphocyte transformation tests and biopsies were performed. RESULTS: A positive patch test to alpha-lactalbumin, a positive lymphocyte transformation test to whole cow's milk and an immunohistology with infiltration of CD4+ and CD8+ T cells were found. CONCLUSIONS: There is a possible pathogenetic relation between delayed-type hypersensitivity to cow's milk protein and Melkersson-Rosenthal syndrome in the patient presented.
We report the case of a 13-year-old boy with vitiligo who developed lichen planus involving both vitiliginous skin and normally pigmented skin. Biopsy of lichen planus surrounded by a depigmented halo revealed a dermal infiltrate composed of CD3 + T cells and macrophages. The association of these skin diseases may indicate a pathogenetic relationship.
We report a 31-year-old renal transplant patient treated with cyclosporin A who developed an unusual sebaceous gland hyperplasia accompanied by a disseminated follicular spiny hyperkeratosis. Those alterations were most evident on his face and limbs. In some locations hairy hyperkeratosis with authentic hair neogenesis was found. The histology of these alterations showed a marked hyperkeratosis of the hair follicles with formation of hair-like spikes either alone or in connection with hairs. The presence of true hairs distinguishes our case from the morphologically similar disseminated spiked hyperkeratosis and other spiny keratinization disorders. Scanning electron microscopy helped to demonstrate the hair-like structure of these keratoses. Some of the sebaceous glands showed cystic widening of their lumina, which were filled with abundant amorphous eosinophilic material, a finding similar to earlier observations. Our case demonstrates that these skin alterations should be classified as side effects of cyclosporin A and that they are apparently dose-dependent.
BACKGROUND: Granuloma annulare is a chronic inflammatory disease of connective tissue, manifested clinically by small, firm nodules. It is observed only rarely around the eyes. PATIENTS AND METHODS: We present four patients aged 33 to 66 years with papulomatous lesions of the eyelids. The lesions were mildly or not symptomatic. Biopsies were taken. There was no association with any systemic inflammatory disease. RESULTS: Histology was characteristic for granuloma annulare in all four cases. The clinical course was benign. CONCLUSION: When faced with papulomatous lesions of the eyelids, the ophthalmologist may consider a diagnosis of granuloma annulare in patients of any age. While granuloma annulare is generally observed in young persons, one of our patients was aged 66 years.
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In a female patient with increasing redness of the hands and face, proteins of Borrelia burgdorferi were detected in a biopsy of the ear by DNA amplification. Although acrodermatitis chronica atrophicans has been documented to be caused by B. burgdorferi, this is the first case with proven spirochetal presence in the skin of the head. After 2 weeks of intravenous therapy with ceftriaxone marked improvement of discoloration of the skin was noted.
Uncombable hair syndrome refers to a clinical disorder characterized by scalp hairs arranged in bundles in all directions that resist to brush and comb. Several entities may lead to spun-glass hair. As a rule the syndrome becomes obvious during the first years of life. The hair is normal in quantity, and increased fragility is not a common feature. The hair is often dry with silvery blond color. Under the light microscope the hairs may appear normal. Scanning electron microscopy shows a characteristic triangular, kidney- or heat-shaped diameter with typical longitudinal canalicular deformation. We present a 9-year-old girl with the typical clinical features of pili trianguli et canaliculi. Investigation by scanning electron microscopy confirmed the diagnosis. In addition the girl had enamel defects of the teeth and nail abnormalities that classify for a subtype of ectodermal dysplasia.
Cryosurgery is being increasingly considered as the treatment of choice for a number of malignant skin tumors. Recent advances made in the field of cryobiology have broadened our knowledge of the practical aspects of cryosurgery. Tissue damage from freezing depends on both, freeze and thaw rates. In treating skin cancers, a rapid freezing and a slow thawing should be used. Repeating the freeze-thaw cycle increases the cold injury. By measuring the lateral spread of freeze on the surface, we can estimate the depth of the frozen tissue. The depth of the tissue ice ball approximates the lateral spread of freeze. Liquid nitrogen is the cryogen of choice for dermatological cryosurgery of malignant skin lesions. Cryosurgery has proven to be a successful form of treatment for basal cell carcinoma, small squamous cell carcinoma and precancerous lesions of the skin. The overall cure rates obtained by cryosurgery compare favorably with those obtained by other treatment modalities. Cryosurgery is a simple, safe and effective method of treatment and has a definite place in the management of many malignant tumors of the skin.
BACKGROUND: Focal dermal hypoplasia (FDH) is a rare ectomesodermal dysplasia syndrome characterized by cutaneous, skeletal, dental, ocular, and soft-tissue defects. An X-linked dominance with death of male subjects has been assumed as the mode of inheritance. Only a few cases of FDH have been reported in male subjects. A 68-year-old man had typical skin lesions of FDH. Clinical, histologic, and immunohistochemical features are presented. OBSERVATIONS: The cutaneous abnormalities consisted of atrophic hyperpigmented and hypopigmented macules and erythematous, slightly raised lesions showing a highly characteristic linear distribution. Other abnormalities, including syndactyly, apocrine hidrocystoma of eyelids, and bilateral cholesteatoma were observed. Only one case of FDH in association with an apocrine hidrocystoma has been reported previously. Consistent findings microscopically in the erythematous lesions were areas of scar formation with subepidermal bulla overlying the neodermis. A strongly positive immunohistochemical reaction for vimentin, fibronectin, and collagen type III was observed in the scar tissue. No collagen type IV was detected in the basement membrane zone of the epidermis covering the lesion. CONCLUSION: It has been proposed that fibroblastic abnormalities may lead to an alteration of collagen synthesis in FDH, although confirmation of this hypothesis was unavailable. Our findings suggest that production of collagen type IV may be delayed in FDH resulting from a fibroblastic defect.
We report a patient who developed erythematous indurated plaques with alopecia on the face and multiple well-demarcated infiltrated scaling lesions on the trunk and extremities. Skin biopsy showed a mucinous degeneration of the follicular outer root sheaths and sebaceous glands consistent with the diagnosis of follicular mucinosis. A histologic examination of a specimen from the left forearm showed microscopic changes of mycosis fungoides. The present case confirms the need of multiple skin biopsies in the evaluation of patients with follicular mucinosis.
Cryosurgery with liquid nitrogen is commonly used to treat benign and malignant skin tumours. One of the crucial factors which influences the freezing and thawing rate of the target cells is the response of the microcirculation to cold stimulus. In our experiment, laser Doppler flowmetry was used to monitor blood flow during cryosurgical treatment of basal cell carcinomas. Outside the frozen tissue hemisphere, blood flow, as measured by laser Doppler flowmetry, increased almost instantaneously and remained on a high level during thawing. Baseline blood flow at the contralateral anatomical site remained stable. Pharmacological or physical modification of the cutaneous microcirculation before therapy may influence freezing and thawing times, and therefore the outcome with regard to healing, cosmetic appearance and the recurrence rate of basal cell carcinomas.
A review of the spectrum of dermatologic findings related to human immunodeficiency virus infection is presented. Oral candidiasis, seborrheic dermatitis, oral hairy leukoplakia, herpes simplex infections and molluscum contagiosum were among the most frequently encountered dermatologic disorders among the HIV-seropositive individuals. The recognition of cutaneous manifestations is of particular importance for the early diagnosis and prognosis of HIV-infection.
The most common disease of the pilosebaceous follicle is acne vulgaris. The primary detectable pathologic defect in acne is abnormal keratinization of the follicular epithelium, resulting in a retention hyperkeratosis. The primary lesion produced by this process, the comedo, ist the precursor of most other acne lesions. These include inflammatory papules, pustules and nodules. Treatment principles are directed against known pathogenic factors. The major topical modalities that are currently being used include tretinoin, benzoyl peroxide and topical antibiotics. Benzoyl peroxide is a powerful antibacterial agent. Topical erythromycin and clindamycin appear to have equivalent efficacy. A major treatment advance has been heralded by the use of oral 13-cis retinoic acid (isotretinoin) in the management of patients with severe nodulocystic acne, acne conglobata and acne fulminans. Isotretinoin is teratogenic and should not be given to women of childbearing age.