[Intradermal metal deposits in an industrial worker]. / Intradermale Metalleinsprengungen bei einem Industriearbeiter.

CASE REPORT: We describe a 75-year-old patient with asymptomatic grey pigmentation on the face and fingers. He had worked over two decades in cutting high-voltage cables. The cutting procedures were performed without a face shield or protection gloves. The patient presented with gray punctate lesions beside some homogeneously stained zones. DIAGNOSTICS: Histologically, the deposits presented in the dermal tissue in a horse-shoe, oval, or splinter-like shape. The foreign body material was embedded by a few CD68-positive macrophages. Undyed histological sections were investigated via two-dimensional micro-synchrotron X-ray fluorescence analysis (SRXRF). The deposits were identified as zinc (Zn), copper (Cu), nickel (Ni), and strontium (Sr), which were strongly associated with maximal sulfur (S) concentrations. This association is presumably induced by complex binding between thiol groups and metal ions such as Zn, Ni etc. However, the iron (Fe) distribution patterns were quite different to those of Zn, Cu, or Ni. These heavy metals are major components of the metal wires that the patient worked with in his profession. CONCLUSION: To the best of our knowledge, this is the first case report in the dermatological literature of intradermal metal deposits identified via SRXRF analysis.

Merkel cell carcinoma: mitoses, expression of Ki-67 and bcl-2 correlate with disease progression.

BACKGROUND: There are conflicting data on markers of disease progression and outcome of Merkel cell carcinoma. OBJECTIVE: We suggest to review histological and various immunohistochemical features of Merkel cell carcinoma specimens, in order to identify prognostic markers of clinical relevance. METHODS: We collected paraffin-embedded blocks from primary tumours from 26 patients diagnosed with Merkel cell carcinoma and determined the following: type and size of the tumour, number of mitoses, proliferation rate (Ki-67 antibody), (anti)-apoptosis rate (bcl-2, p53, p63 antibodies) and lymphatic vessel invasion (D2-40 antibody for podoplanin). Two authors blinded to clinical outcome, independently assessed and scored all samples. The findings were correlated with tumour progression, which was determined by local recurrence, lymph node- or distant metastases. RESULTS: During the average follow-up period of 63.4 months 12 (46%) patients had disease progression. Statistical analysis revealed Ki-67-staining (P = 0.005) as a marker of disease progression, high number of mitoses (P = 0.026) correlated with lymph node metastasis, while a tendency for increased Bcl-2 expression (P = 0.064) was found in patients with local recurrence. A higher number of invaded lymphatic capillaries showed a tendency in correlation with metastases (P = 0.072). CONCLUSION: The findings indicate that high numbers of mitoses, proliferation and survival of tumour cells as marked by Ki-67- and Bcl-2-staining, and infiltration of lymphatic vessels, might correlate with the biological behaviour of Merkel cell carcinoma.

[Leucocytoclastic eosinophilic vasculitis with Löffler syndrome]. / Leukozytoklastische eosinophile Vaskulitis bei eosinophilem Lungeninfiltrat "Löffler"

Histologically, leukocytoclastic vasculitis (LV) presents with neutrophilic granulocytes with leukocytoclasia and erythrocyte extravasation, associated with variable counts of lymphocytes, plasma cells and eosinophilic granulocytes. The association of a LV with eosinophilic granulocytes and eosinophilic pneumonia was first described by Chan et al. in 1982. Our case represents the second report in the literature of this rare disease: a 85 year old patient with LV and numerous eosinophilic granulocytes in association with intermittent blood eosinophilia and Löffler syndrome (eosinophilic pulmonary infiltrates). The recurrent episodes were treated successfully with oral corticosteroids.

Hereditary, focal, transgressive palmoplantar keratoderma with associated clinical findings: a new entity?

We report on a 24-year-old male originating from Yugoslavia with a focal, transgressive palmoplantar keratoderma presumably inherited as an autosomal recessive trait. Associated clinical findings were hyperkeratotic lichenoid papules on the knees and elbows, psoriasis-like lesions in the groins and on the scalp, a spotty or reticulate hyperpigmentation of the face, trunk and extremities and a partial alopecia of the left eyebrow and eyelashes. The patient's sister was affected by similar but less pronounced cutaneous changes. Although our case shares some similarities with other hereditary palmoplantar keratodermas there remain substantial differences. We therefore believe this case to represent a new entity.

Isolation and polymerase chain reaction typing of Borrelia afzelii from a skin lesion in a seronegative patient with generalized ulcerating bullous lichen sclerosus et atrophicus.

A 64-year-old woman presented with bullous and ulcerating lichen sclerosus et atrophicus (LSA) on the neck, trunk, genital and perigenital area and the extremities. Histology of lesional skin showed the typical manifestations of LSA; in one of the biopsies spirochaetes were detected by silver staining. Despite treatment with four courses of ceftriaxone with or without methylprednisone for up to 20 days, progression of LSA was only stopped for a maximum of 1 year. Spirochaetes were isolated from skin cultures obtained from enlarging LSA lesions. These spirochaetes were identified as Borrelia afzelii by sodium dodecyl sulphate--polyacrylamide gel electrophoresis and polymerase chain reaction (PCR) analyses. However, serology for B. burgdorferi sensu lato was repeatedly negative. After one further 28-day course of ceftriaxone the lesions stopped expanding and sclerosis of the skin was diminished. At this time cultures for spirochaetes and PCR of lesional skin for B. afzelii DNA remained negative. These findings suggest a pathogenetic role for B. afzelii in the development of LSA and a beneficial effect of appropriate antibiotic treatment.

Primary mucinous carcinoma of the scalp.

Primary cutaneous mucinous carcinoma (MC) is a rare epithelial neoplasm derived from the sweat glands. Herein, we report a case of MC located on the head. A 66-year-old woman underwent excision of a nodular tumor with a reddish brown surface on the left parietal region. Histopathology revealed a neoplasm extending from the reticular dermis into the subcutaneous fat. The tumor cell aggregates showed cribriform and solid lobules and were embedded in lakes of mucin, separated by thin, fibrous septae. Focally single neoplastic cells were arranged in an Indian-file pattern. The tumor cells displayed an eosinophilic cytoplasm, large basophilic nuclei and some discrete nuclear atypia. Vascular spaces, filled by densely packed erythrocytes between the septae, were also observed. We compared the mucinous component with the tumor cell and the stromal component by light microscopy. Analyzing the tumor by an image analysis system in Alcian-blue-stained serial sections, we found the averaged total tumor area measuring 99.7 mm(2). The area of the mucinous component measured 92.4 mm(2), that of the tumor cells 3.7 mm(2) and that of the stromal component 3.6 mm(2). The extensive checkup of the patient disclosed no evidence for a further malignant neoplasm. After excision of the tumor an adjuvant radiotherapy was performed. The patient was free of recurrence and metastatic spread of the mucinous carcinoma during a 4-year follow-up.

[Lichen striatus or Blaschkitis in the adults. Variations of the same entity?]. / Lichen striatus oder Blaschkitis des Erwachsenen. Variationen derselben Entität?

A 64-year-old woman presented with extensive erythematous papulosquamous lesions along the lines of Blaschko on the right half of the trunk and the right leg. The clinical and histological features led to the diagnosis of lichen striatus, a dermatosis which usually appears during childhood. Treatment with acitretin led to marked improvement. Lichen striatus and its differential diagnoses--particularly "blaschkitis of adulthood" as first described in 1990--are discussed.

Primary invasive signet-ring cell melanoma.

The histopathological variants of malignant melanoma include the common type (lentigo maligna, superficial spreading melanoma, nodular melanoma, acrolentiginous melanoma), spindle cell, desmoplastic, balloon cell, pleomorphic (fibrohistiocytic), myxoid, small cell melanoma and malignant blue nevus. Recently, signet-ring cell melanoma was introduced as an additional cytologic variant. We describe a 72-year-old patient with a primary signet-ring cell melanoma of the skin located on the upper arm. Histopathologic examination disclosed a melanocytic tumor extending from the epidermis to the deep reticular dermis. Numerous pleomorphic tumor cells showed large, intracellular vacuoles and oval to spindle-shaped nuclei at their periphery. Mitotic figures and multinucleated melanocytes were also observed. Some of the signet-ring cells exhibited cytoplasmatic periodic acid-Schiff (PAS)-positivity. Immunohistochemistry showed positive reaction of the tumor cells for S-100, HMB-45 protein and vimentin, confirming their melanocytic differentiation. Tumor cells were negative for cytokeratins, epithelial membrane antigen (EMA), and carcinoembryonic antigen (CEA). The signet-ring cell melanoma disclosed an invasion to Clark Level IV and tumor thickness of 2.2 mm. Signet-ring cell melanoma is a rare morphologic variant of melanoma. Its recognition is important for differentiation from other tumors featuring signet ring cells.

Venous thromboembolism at a young age in a brother and sister with coinheritance of homozygous 20210A/A prothrombin mutation and heterozygous 1691G/A factor V Leiden mutation.

We report on members of a Turkish thrombophilic family with coinheritance of the prothrombin mutation PT20210A and the factor V Leiden mutation. The 23-year-old propositus and his elder sister both had episodes of venous theomboembolism at a young age (23 years and 26 years, respectively) and are homozygous for the PT20210A mutation and heterozygous for the factor V Leiden mutation. The 51-year-old father is suffering from coronary heart disease and is heterozygous for both thrombophilic mutations. The asymptomatic 43-year-old mother is heterozygous for the PT20210A mutation, but without activated protein C resistance. Two other children, a 20-year-old girl who is homozygous for the PT20210A mutation and a 13-year-old boy who is heterozygous for the PT20210A mutation, are both free from activated protein C resistance and thrombosis. This report provides further evidence for an early onset of thromboembolic disorders in individuals with an homozygous state of the prothrombin variant 20210A/A and coinheritance of another thrombophilic mutation. Consensus guidelines are required for the treatment and prophylaxis of patients and subjects who remain asymptomatic with homozygous or more than one heterozygous genetic defect associated with thrombophilia.

Isolation of Borrelia afzelii from circumscribed scleroderma.

A 45-year-old man presented with circumscribed scleroderma (CS) on the extremities. Histology of lesional skin showed the typical manifestations of scleroderma including a perivascular and interstitial infiltrate of lymphocytes and plasma cells; in one of the biopsies spirochaetes could be detected. Despite treatment with penicillin, progression of CS was observed and spirochaetes were isolated from skin cultures obtained from active scleroderma lesions. These spirochaetes were identified as Borrelia afzelii by sodium dodecyl sulphate-polyacrylamide gel electrophoresis of outer surface proteins and polymerase chain reaction (PCR) analysis of their chromosome. After two courses with ceftriaxone the lesions stopped expanding and sclerosis of the skin was diminished. At this time cultures for spirochaetes and PCR of lesional skin for Borrelia afzelii DNA remained negative. The pathogenetic role of Borrelia afzelii in the development of CS is discussed.

Increased dermal angiogenesis after low-intensity laser therapy for a chronic radiation ulcer determined by a video measuring system.

Acute and chronic radiation-induced dermatitis can occur after high doses of ionizing radiation of the skin. We describe a patient with a long-lasting radiotherapy-induced ulcer that healed after low-intensity laser therapy. A video measuring system was used to determine the number of dermal vessels in the ulcer before and after laser treatment. We found a statistically significant increase in the number of dermal vessels after low-intensity laser therapy in both the central and marginal parts of the ulcer compared with its pretreatment status.

Generalized pustular psoriasis of pregnancy (impetigo herpetiformis).

A 17-year-old woman had a sudden eruption of pustules in her intertriginous areas as well as of erythematosquamous plaques on the scalp, elbows, palms and soles in the third trimester of her first pregnancy. Histopathological evaluation of a biopsy revealed typical changes of pustular psoriasis with parakeratosis and abscesses of neutrophils (Kogoj's spongiform pustules). The diagnosis of pustular psoriasis was established by the typical clinical and histopathological findings. Laboratory parameters showed a highly elevated blood sedimentation rate, hypoferric anemia and decreased albumin levels. Serum concentrations of parathormone and its metabolites were normal. After systemic treatment with glucocorticosteroids and antibiotics, the lesions improved but did not clear. After delivery of a healthy boy, therapy was switched to retinoid photochemotherapy with isotretinoin and PUVA that resulted in rapid and complete clearing of the eruption. The coincidence of plaque-type psoriasis and a pustular eruption as described previously in impetigo herpetiformis supports the view that this dermatosis of pregnancy is a variant of generalized pustular psoriasis.

Erythema migrans and serodiagnosis by enzyme immunoassay and immunoblot with three borrelia species.

There is wide divergence of opinion between physicians regarding the use of serological measures for the diagnosis and treatment of erythema migrans, the hallmark of Lyme borreliosis. We studied the outcome of an enzyme immunoassay and immunoblot (Western blot) used on the sera of patients who had suffered tick bite and erythema migrans, and had been subsequently treated with various antibiotics. Ninety-nine consecutive patients presenting with erythema migrans after tick bite were prospectively recruited at the outpatient department of two Vienna City hospitals and at the consultation office for Lyme borreliosis of the Institute of Hygiene. University Vienna. Blood samples were taken before antibiotic treatment and 3 and 6 months thereafter. Blood samples from 100 blood donors served as controls. Antibodies against Borrelia burgdorferi sensu lato were determined by enzyme immunoassay (IgG and IgM EIA) and by IgG immunoblot. The latter was performed with isolates of B. alzelii (H2) B. burgdorferi sensu stricto (Le) and B. garinii (W) from Austrian patients. The 4 interpretation criteria for immunoblot results were: A (3 bands out of 8), B (2 bands out of 9), C and D (1 band out of 6). In all patients, the erythema resolved within the treatment period. No complications secondary to the borrelia infection were registered. After treatment there was no significant change in titre, nor was there a difference in the immunoblot pattern between the first, second and third serum samples. Serum antibodies to B. burgdorferi were positive by EIA in 22.9% (IgG) and 2.5% (IgM). Immunoblot results offered by borrelia species and by the interpretation criteria, ranging between 8.3% (criterion A, strain Le) and 44.2% (criterion D, strain H2). By EIA, control samples were IgG and IgM positive in 5% and 1%, respectively. Positive immunoblot results with strain H2 were found in 9%, 13%, 18%, and 20% by the criteria A through D respectively. After antibiotic treatment of erythema migrans the immunological response appears to be abrogated. Thus, serological results are not supportive for the diagnosis of erythema migrans, not will they retrospectively prove successful antibiotic treatment of borrelia infection.

Pseudoporphyria cutanea tarda induced by furosemide in a patient undergoing peritoneal dialysis.

We report on a 32-year-old female patient undergoing peritoneal dialysis for diabetic nephropathy who developed blisters and cutaneous fragility on the dorsa of the hands. Histopathology of an early lesion showed a subepidermal cleft under a regular epidermis with single necrotic keratinocytes and normal dermal structures. In an advanced lesion, a subepidermal bulla and caterpillar bodies were found in the epidermis. Dermal vessel walls were normal and surrounded by a discrete lymphocytic infiltrate. Direct immunofluorescence revealed perivascular deposits of C3. Uroporphyrin and coproporphyrin levels of serum, urine and dialysate were repeatedly normal, confirming pseudoporphyria. The patient took furosemide (500 mg daily). Replacement of furosemide by ethacrynic acid led to spontaneous healing of the blisters. The patient was free of further lesions at the 1-year follow-up.

Functional reconstruction of the upper eyelid after excision of squamous cell carcinoma in xeroderma pigmentosum.

This paper describes a technique for excision of a squamous cell carcinoma of the upper eyelid and subsequent functional reconstruction of the lid in a patient with xeroderma pigmentosum. By applying the modified technique of temporalis muscle flap, the upper eyelid was reconstructed by a skin graft from non-sun-exposed skin and a muscle pocket of the temporalis muscle. For the mucosal layer of the upper eyelid, a transplant of oral mucosa was used. An effective functional and aesthetic outcome has been achieved by this technique.