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The treatment of the painful osteomyelitis in patients with SAPHO syndrome (Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis) is often a problem. A 53-year-old woman had experienced palmo-plantar pustular skin lesions for four years, and in the past two years complained about progressive breath-and movement-dependent pain of the sternum. On examination she had extensive palmoplantar pustules and a painful swelling in the area of the right sternoclavicular joint. The three-phase bone scintigraphy showed a strong focal enrichment in the right sternoclavicular joint and at the transition from the manubrium to the corpus sterni suggesting active osteo-chondritis. Initially prednisolone and ibuprofen were administered, but only the skin changes regressed. The strong sternal pain decreased only after infusion of 4 mg zoledronic acid over three days. In a follow-up examination after five months the patient was still free of pain. The bisphosphonates inhibit osteoclastic activity and lead to long-lasting improvement of osteo-articular complaints in the SAPHO syndrome.

Steatocystoma multiplex is a rare,almost exclusively autosomal dominant disease of the sebaceous glands. The sites of predilection are the sternal region, upper back, axillae and proximal parts of the extremities. A 78-year-old woman developed multiple yellowish nodules only on the face over a few months. Histology revealed large cysts with a thin wall and connection to sebaceous glands. Immunohistochemical analysis showed the inner epithelial layer of the cysts was positive with calretinin.With curettage and CO2 laser therapy, a good cosmetic result was achieved.This is the first case report in the German literature of a rare sporadic form of steatocystoma multiplex with late onset and exclusive localization in the face.

History and Clinical Findings: A 48-year-old obese man presented with thickening, coarseness and hyperpigmentation of the skin, especially of the intertriginous areas, papillomatous to verrucous lesions of the lips and buccal oral mucosa, and hyperkeratosis of the palms ("tripe palms") and soles. He was obese, reported sleep apnea and had a history of hyperuricemia, mixed hyperlipidemia and previous myocardial infarction. He was on a maintenance dose of a proton pump inhibitor for chronic gastro-esophageal reflux., Examinations: Immunohistochemical studies of the skin lesion revealed increased epidermal immunoreactivity for the melanocortin-1-receptor. Increased levels of tumor markers CA 19-9 (141100 U/ml), CA 72-4 (755 U/ml) and CEA (189 ng/ml) were found in the serum. Gastroscopic findings were suspicious of adenocarcinoma of the stomach: it was classified histologically as a signet-ring cell, non-mucinous adenocarcinoma. At the time of diagnosis the tumor had already metastasized to perigastric and peripancreatic lymph nodes with peritoneal carcinosis., Treatment and Course: Since a curative resection was impossible a gastrojejunostomy was carried out. After this the patient received several courses of chemotherapy according to different schemes. Serum tumor marker levels and cutaneous signs regressed several times., Conclusions: Marked acanthosis nigricans -- especially when associated with further cutaneous markers of malignancy, e.g. mucocutaneous papillomatosis or so-called tripe palms -- calls for thorough search for malignant tumor, also if metabolic or endocrinological abnormalities co-exist. A pathogenetic role of a-melanocyte-stimulating hormone in the development of the skin changes is suggested.

Hemophagocytic lymphohistiocytosis is a rare and potentially fatal syndrome associated with a variety of genetic, malignant, autoimmune, or infectious conditions. The importance of cutaneous presentations of this syndrome has only recently been brought forward. We report the first case of Epstein-Barr-virus-associated hemophagocytic lymphohistiocytosis presenting with papulonodular and granulomatous skin lesions. A girl of African origin developed several umbilicated papules on her extremities and face at the age of 18 months. She was born in Germany, had never visited Africa, and was otherwise healthy. Over the next 5 months the lesions progressed in size and number and became hyperkeratotic. Histopathologic analysis of early lesions revealed a superficial lympho- and plasmacellular dermatitis with some features of panniculitis. Later biopsy specimens from nodular lesions showed the formation of tuberculoid granulomas in the deep dermis. At the age of 23 months she became severely ill, rapidly developing high fever, hepatosplenomegaly, icterus, pancytopenia, and ascites. On the basis of bone marrow and lymph node biopsies, the diagnosis of hemophagocytic lymphohistiocytosis was established. However, this phenomenon could not be detected in any of the skin specimens. An active Epstein-Barr virus infection was diagnosed by polymerase chain reaction in blood, lymphoid tissue, and skin. Despite chemotherapy with etoposide and cortisone, the girl expired 14 days after clinical onset of her systemic disease.

Background: Anogenital dermatoses (AGD) are common and often very distressing. Clinically it is often unclear if allergic contact dermatitis or irritant dermatitis is involved. In order to optimize therapy and prophylaxis, it is essential to identify relevant allergens or irritants., Patients and Methods: Data of the Information Network of Departments of Dermatology (IVDK, data center in Göttingen) collected between 1999 and 2003 were analyzed. The anogenital area was involved in 1 168 patients with suspected allergic contact dermatitis. Clinical variables and patch test results were statistically compared with the remaining IVDK patch test population, the latter standardized for age and sex., Results: Allergic contact dermatitis had been suspected prior to patch testing in 39.5 %, while in 24.6 % this diagnosis was eventually confirmed. Irritant contact dermatitis was diagnosed in 11.8 %. Other diagnoses, included balanitis, lichen sclerosus et atrophicus and herpes genitalis. Positive reactions to cinchocaine (6.6 %), bufexamac (3.5 %) and benzocaine (2.4 %) were observed significantly more often among patients with anogenital dermatitis. Among those in whom co-factors were considered important (n = 422), wetness (38.4 %), occlusion (30.3 %), mechanical strain (4.7 %) and heat (3.6 %) were mentioned as irritation factors., Conclusion: Because of the significantly higher frequency of sensitization to cinchocaine, benzocaine and bufexamac in patients with anogenital dermatitis, these ingredients should be used only with caution. According to the literature, ingredients of toiletries, cosmetics and contraceptives of any kind seem to cause allergic contact dermatitis rarely although there are several case reports. Comprehensive patch test including the standard series plus major sensitizers such as cinchocaine, benzocaine and bufexamac, and in particular patients' own skin care products, is recommended.

Unlabelled: A new fragrance mix (FM II), with 6 frequently used chemicals not present in the currently used fragrance mix (FM I), was evaluated in 6 dermatological centres in Europe, as previously reported. In this publication, test results with the individual constituents and after repeated open application test (ROAT) of FM II are described. Furthermore, cosmetic products which had caused a contact dermatitis in patients were analysed for the presence of the individual constituents. In 1701 patients, the individual constituents of the medium (14%) and the highest (28%) concentration of FM II were simultaneously applied with the new mix at 3 concentrations (break-down testing for the lowest concentration of FM II (2.8%) was performed only if the mix was positive). ROAT was performed with the concentration of the FM II which had produced a positive or doubtful (+ or ?+) patch test reaction. Patients' products were analysed for the 6 target compounds by gas chromatography-mass spectrometry (GC-MS)., Results: 50 patients (2.9%) showed a positive reaction to 14% FM II and 70 patients (4.1%) to 28% FM II. 24/50 (48%) produced a positive reaction to 1 or more of the individual constituents of 14% FM II and 38/70 (54.3%) to 28% FM II, respectively. If doubtful reactions to individual constituents are included, the break-down testing was positive in 74% and 70%, respectively. Patients with a positive reaction to 14% FM II showed a higher rate of reactions to the individual constituent of the 28% FM II: 36/50 (72%). Positive reactions to individual constituents in patients negative to FM II were exceedingly rare. If doubtful reactions are regarded as negative, the sensitivity, specificity, positive predictive value and negative predictive value for the medium concentration of FM II towards at least 1 individual constituent was 92.3% (exact 95% confidence interval 74.9-99.1%), 98.4% (97.7-99.0%), 48% (33.7-62.6%) and 99.9% (99.6-"100.0%), respectively. For the high concentration, the figures were very similar. The frequency of positive reactions to the individual constituents in descending order was the same for both FM II concentrations: hydroxyisohexyl 3-cyclohexene carboxaldehyde (Lyral) > citral > farnesol > citronellol > alpha-hexyl-cinnamic aldehyde (AHCA). No unequivocally positive reaction to coumarin was observed. Lyral) was the dominant individual constituent, with positive reactions in 36% of patients reacting to 14% FM II and 37.1% to 28% FM II. 5/11 patients developed a positive ROAT after a median of 7 days (range 2-10). The 5 patients with a doubtful or negative reaction to 28% FM II were all ROAT negative except 1. There were 7 patients with a certain fragrance history and a positive reaction to either 28% or 14% FM II but a negative reaction to FM I. Analysis with GC-MS in a total of 24 products obtained from 12 patients showed at least 1-5 individual constituents per product: Lyral (79.2%), citronellol (87.5%), AHCA (58.3%), citral (50%) and coumarin (50%). The patients were patch test positive to Lyral, citral and AHCA. In conclusion, patients with a certain fragrance history and a negative reaction to FM I can be identified by FM II. Testing with individual constituents is positive in about 50% of cases reacting to either 14% or 28% FM II.

History and Clinical Findings: A 75-year-old woman took aceclofenac (100 mg/day) for 15 days for long-standing arthritis. Starting with erythema of the face she developed multiple target lesions with central bullae on her neck, chest, back, including the palmoplantar region. In several areas the lesions became confluent and showed wide detachment. The mucous membranes of the eyes, mouth and genitalia also showed marked inflammation, most prominent were large erosions in the mouth. The clinical course culminated in an overall skin loss of about 15 % of the total body surface area., Diagnosis, Treatment and Course: Treatment with topical and systemic corticosteroids as well as antiseptics resulted in complete clearing of the cutaneous lesions within 4 weeks. Various analgetics, particularly the closely related diclofenac, were tolerated without any side-effects after oral intake. Re-exposition with aceclofenac was rejected due to safety reasons., Conclusion: This is the first report of a potentially life-threatening adverse cutaneous drug reaction after taking with aceclofenac.

It is mandatory to establish a firm diagnosis before starting systemic antimycotic therapy because of potential side effects and relatively high therapy costs. Direct microscopy and fungal culture are the most widely employed diagnostic tools. Not infrequently, a strong clinical suspicion cannot be proven by even repeated direct microscopic examinations and fungal cultures. In these cases histologic examination is a simple alternative. We identified 32 cases of histopathologically proven onychomycosis; of the 29 where a fungal culture was performed, only 14 (48%) were positive. Direct microscopy was performed only in 12 cases since most had repeatedly negative direct microscopy results before being referred to us; 5 cases (42%) were positive. Histopathology for onychomycosis has several advantages in addition to its sensitivity--particularly in difficult to diagnose cases: lack of the danger of contamination, a permanent preparation with the possibility to demonstrate fungal invasion of the nail organ and to identify other or concomitant nail disorders, simplicity of the procedure; quicker results as compared to fungal culture. Exact specification of the fungus is not possible with histology. Even though histological examination is not mentioned in the current guidelines for onychomycosis of the German Dermatological Society, it should be kept in mind as a simple but sensitive diagnostic tool.

Linear IgA disease is a rare autoimmune bullous skin disorder that may be idiopathic or drug-induced. The mechanisms of loss of self-tolerance to target antigens of the dermo-epidermal junction are unclear and may be variable. Regardless of the etiology, the clinical presentation is quite heterogeneous. Frozen sections of a blister roof are helpful in early diagnosis to rule out full-thickness epidermal necrosis or subcorneal separation as seen in toxic epidermal necrosis or staphylococcal scaled skin syndrome, respectively. Drug-induced cases of linear IgA disease typically resolve quickly once the causative agent is withdrawn. Vancomycin is the best-documented insiting drug in the literature. The significance of further associations such as malignancy or different autoimmune diseases is uncertain. Review of medication exposures after diagnosis is crucial in identifying possible offending agents. We present a patient with vancomycin-induced linear IgA disease in whom the drug was immediately discontinued because of the characteristic clinical presentation and rapid histopathological examination. The development of new blisters stopped within 24 hours and the skin healed within 2 weeks.

Contact allergy to the industrial preservative group of Biobans has been reported in individual cases. We have performed a prospective study on 408 metalworkers who were tested with 6 different Biobans, formaldehyde and 5 other preservatives. Furthermore, retesting was performed after a time interval of 6 months to 4 years. The test results were also compared with those of the multicentric data provided by the Information Network of Departments of Dermatology in Germany and Austria (IVDK), particularly with regard to cross-reactivity between the various Biobans and formaldehyde. In 408 metalworkers 14 (3.4%) showed positive reactions to Biobans (2.2% P 1487, 2.0% CS 1246, 1.5% CS 1135), the majority being + reactions. On retesting 10 patients, the reactions were confirmed in only 2 cases (P 1487 and CS 1135). 4 patients showed positive reactions to 3 Biobans (P 1487, CS 1135, CS 1246), while 9 patients had only solitary sensitizations. Concomitant sensitization to formaldehyde was present in 5 out of 10 patients, being reproducible in 3 of them. In the IVDK data, sensitization prevalence in 3400 patients ranged from 1.8% (P 1487) to 0.8% (CS 1246). A high proportion of doubtful or irritant reactions as well as weak + reactions was observed. Cross-reactivity between the various Biobans was low, moderate agreement being calculated for CS 1135 and CS 1246 (kappa coefficient 0.43). The consensus pattern between formaldehyde and the tested Biobans was even lower. In conclusion, patch test reactions to Biobans are not rare among metalworkers but are often weak and not reproducible. Simultaneous reactivity to various Biobans and formaldehyde may be due to cross-reactivity or cosensitization from common exposure.

Pyodermatitis-pyostomatitis vegetans is a rare, polymorphous inflammatory disorder of the skin and oral mucosa first described by Hallopeau in 1898. On the skin papules, pustules and reddish brown annular vegetating plaques develop, most frequently in the intertriginous areas. In the mouth, yellowish flat ulcerations arise, typically in the shape of "snail tracks". The association with inflammatory bowel disease is very common. An unusual case with a chronic relapsing course of 2 decades is presented. Gastrointestinal inflammation was absent. Prednisolone in high and medium doses suppressed most lesions. Various attempts with other drugs (dapsone, isotretinoin, azathioprine) to reduce the corticosteroid dose failed. This is the first report of the successful treatment of pyodermatitis-pyostomatitis vegetans with cyclosporin A, which proved to be highly effective in this regard. The unknown aetiopathology of pyodermatitis-pyostomatitis vegetans is discussed.