Prospective evaluation of follow-up in melanoma patients in Germany - results of a multicentre and longitudinal study.

BACKGROUND: Patient numbers requiring long-term melanoma surveillance are constantly rising. Surveillance is costly and guideline recommendations vary substantially. METHODS: In this German nationwide study, information on surveillance and treatment of patients diagnosed with melanoma and melanoma in situ (MMis) between April and June 2008 was prospectively collected over four years. Additionally, patient self-report questionnaires were evaluated to assess anxiety, depression, health-related quality of life, socio-demographic information and use of disease specific health information sources at year 4 after primary diagnosis. RESULTS: Complete data was available for 668 patients from 67 centres, of whom 96.0% were in regular melanoma surveillance. In year 3-4 of surveillance, only 55.6% of locoregionary metastases were detected during surveillance visits. Only 33.3% were self-detected by the patient even though 69.4% were documented as being clinically visible or palpable. Costs of 4year surveillance of 550 patients without tumour recurrence (stage I-IIC and MMis) accumulated to 228,155.75 €. Guideline-adherence for follow-up frequency, lymph node ultrasound, S100 serum level tests and diagnostic imaging recommendations was approximately 60% in year 3-4 of surveillance. Multivariate regression analysis showed that certain patient/tumour characteristics and regional differences were significantly associated with guideline deviations. The percentage of patients who exceeded published cut-off scores indicating clinically relevant symptoms of anxiety and depression were significantly increased. Patients frequently reported lack of psychosocial support and education but ascribed great importance to these. CONCLUSIONS: We recommend further reduction of melanoma follow-up in low-risk melanoma patients and improvement of psycho-social support and patient education for all melanoma patients.

[Sinushistiocytosis with massive lymphadenopathy (Rosai-Dorfman disease)]. / Sinushistiozytose mit massiver Lymphadenopathie (Rosai-Dorfman-Erkrankung).

Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare, painless lymphoproliferative disorder of unknown origin with a usually benign course. About 40% of the patients show an extranodal involvement with skin being the most common site in 27% of these patients. We describe a patient with widespread disease involving the respiratory tract, kidneys and skin. Histopathology revealed the characteristic features of SHML with emperipolesis and immunohistochemical positivity of histiocytes for S100 and macrophage-associated antigens.

[DRGs in dermatology. A critical assessment]. / DRGs in der Dermatologie. Eine kritische Standortbestimmung.

The obligatory implementation of the DRG system in 2004 represents a fundamental change in German hospital financing. Since the first version of G-DRG catalogue was published, the final adaptation of the system to German clinical reality has consumed much time and energy. This adjustment has been very challenging for dermatology for a variety of reasons. A DRG evaluation project conducted by the German Society of Dermatology made possible improvements in the G-DRG catalogue 2004 from the perspective of dermatology. The hospitals now have to adjust to the new situation and to find their position in this system. A high coding quality is of greatest importance. When developing their clinical priorities, hospitals should not make the mistake of pursuing the temptations presented by the latest version of the DRG catalogue. Strategies of the practical use of the DRG system in hospitals are reviewed.

[Quality assessment of dermatology in the G-DRG system 2004]. / Abbildungsqualität der Dermatologie im G-DRG-System 2004.

BACKGROUND: Since January 2004, all German hospitals have been obliged to operate with a new hospital funding system based on DRGs. For the DRG system to serve as a fair basis for reimbursement requires that the dermatologic services be adequately covered in the classification system. The German Dermatologic Society (DDG) in cooperation with the DRG-Research Group of the University Hospital Muenster carried out a DRG evaluation study. Based on these results, suggestions for the adjustment of the G-DRG system were proposed by the DDG in the G-DRG adaptation round for 2004. METHODS: Based on data of the DRG evaluation project (14,555 dermatological cases from 19 hospitals) the homogeneity in the G-DRG system 2004 was examined and compared with the quality of depiction in the G-DRG version 1.0. RESULTS: The correlation between expenditure and case mix index in the hospitals improved in the G-DRG system 2004. Most proposals submitted by the German Dermatologic Society for the adaptation into the G-DRG system 2004 were accepted. Some fundamental problems such as reimbursement of high cost drugs and special services, as well as the reimbursement of high and low outliers, were only marginally addressed. CONCLUSIONS: The G-DRG system 2004 will need to be continuously adapted in the field of dermatology. Based on this work, the German Dermatologic Society has made suggestions to be adapted in the G-DRG system 2005 and submitted them to the German DRG Institute.

Subcutaneous curettage vs. injection of botulinum toxin A for treatment of axillary hyperhidrosis.

BACKGROUND: Axillary hyperhidrosis is a functional non-inflammatory abnormality of the eccrine sweat glands. The cause of genuine hyperhidrosis is unknown and, therefore, no specific corrective therapy is available and conservative treatment often fails. Subcutaneous sweat gland curettage of the axillae is one of the proven surgical modalities. Local injection of botulinum toxin A (BT-A) is a promising new conservative approach. OBJECTIVE: The purpose of this study was to compare the efficacy of subcutaneous curettage vs. injection of BT-A in axillary hyperhidrosis. METHODS: A total of 113 patients (36.3% males, 63.7% females) suffering from genuine axillary hyperhidrosis were treated by either subcutaneous curettage (n = 90) or local injection of BT-A (n = 23). Median follow-up period was 23.5 months. Questionnaires were handed out to patients for a subjective assessment of symptoms before treatment, 6 months after the procedure, and at the time of last follow-up. The patients were asked to rate the amount of axillary sweating based on a score ranging from 1 (no axillary secretion) to 6 (maximum axillary secretion). The subjective scores of sweating at rest, at high temperatures, under physical stress, under emotional stress and after spicy meals were assessed. RESULTS: The patients' subjective assessments of the overall outcome after subcutaneous curettage were 'very good' in 36.4%, 'good' in 29.9% and 'satisfactory' in 16.9%. The subjective score of axillary sweating at rest was reduced to 40.0% after 6 months, and finally to 45.7% at the end of follow-up (median: 28.2 months). Patients treated by BT-A injection assessed outcome as 'very good' in 39.1%, 'good' in 21.7% and 'satisfactory' in 8.7%. Sweating at rest was reduced to 48.5% after 6 months, and finally to 68.8% at the end of follow-up (median: 16.1 months). The mean duration of the antiperspiration effect of BT-A was 7.6 months (median: 7 months), but there were two cases of long durations, i.e. 14 and 18 months. CONCLUSIONS: Subcutaneous curettage and injection of BT-A both present major advantages compared with earlier methods. Subcutaneous curettage offers the same permanent efficacy but far fewer side-effects than sympathectomy, and less scarring than local excisional procedures, respectively. Of the conservative approaches BT-A is by far the most efficacious. Patients should be informed of the advantages and disadvantages of both methods.

[Pigmented pheochromocytoma. Case report with immunohistochemical and electron microscopic characterization]. / Pigmentiertes Phäochromozytom. Fallbericht mit immunhistochemischer und elektronenmikroskopischer Charakterisierung.

We present a case of pigmented adrenal paraganglioma in a 39-year-old female patient with associated neurofibromatosis type 1 (NF1). Histology showed features typical for phaeochromocytomas except for varying amounts of brown pigment within the cytoplasm of tumour cells, which proved to be melanin by histochemical and ultrastructural analysis. The occurrence of melanin is believed to reflect the origin of this neoplasm from multipotent cells of the neural crest. Pigmented phaeochromocytoma has to be taken in consideration in the differential diagnosis of pigmented neoplasms, especially in the adrenal gland, where it has to be discriminated from pigmented cortical adenoma (so-called black adenoma) and primary malignant melanoma.

[Clinical and histopathological characteristics of early Leser-Trélat syndrome]. / Klinische und histopathologische Charakteristika des initialen Leser-Trélat-Syndroms.

Leser-Trélat-syndrome is characterized as the eruptive appearance of multiple seborrheic keratoses in association with underlying malignant disease. A 73 year old female patient with a metastatic adenocarcinoma of the colon presented with this paraneoplastic change. The rapid appearance of solitary seborrheic keratoses with associated inflammation, seen clinically and histologically, may be an early sign of Leser-Trélat-syndrome. The recognition of this inflammatory component as an early sign may contribute to the prompt diagnosis of this paraneoplasia, even before the eruption of numerous seborrheic keratoses.

Linear atrophoderma of Moulin.

We report a typical case of linear atrophoderma of Moulin that represents a distinct clinical entity. A 17-year-old woman presented with hyperpigmented and atrophic band-like skin lesions measuring 3-5 cm in breadth on the right side of her trunk and on the right buttock, in an arrangement following the system of Blaschko's lines. The skin lesions had a normal texture and showed no signs of inflammation, lilac ring, erythema, induration, sclerosis or depigmentation. Routine laboratory data were normal. Antinuclear antibodies and anti-Scl70-antibodies were negative. Histopathologically, a moderate diffuse hyperpigmentation within the lower epidermis, a focal vacuolar degeneration of the basal layer, and a few dermal perivascular lymphocytes accompanied by signs of pigment incontinence were noted. Intravenous penicillin G was administered for 14 days in a dosage of 10 x 10(6) IU twice daily. This treatment was repeated after 3 and 9 months. No effect was noted. Linear atrophoderma of Moulin takes a chronic course without progression or regression. The disease leads to significant cosmetic impairment and this may cause emotional stress. Due to its relationship to idiopathic atrophoderma of Pierini-Pasini and linear scleroderma, treatment with intravenous penicillin was tried. In our case it had no effect.

Long-term results of wide surgical excision in 106 patients with hidradenitis suppurativa.

BACKGROUND: Hidradenitis suppurativa (acne inversa) is a chronic recurrent disorder characterized by abscessing inflammation, fistulating sinus tracts, and scarring. Predilection sites are the intertriginal regions. The severe course of the disease demands an early and curative treatment. OBJECTIVE: The aim of this study was to review the effect of radical surgical excision concerning cure rate and potential complications within a large group of patients. METHODS: We analyzed data for 106 patients suffering from hidradenitis suppurativa treated during the period 1980-1998. The mean duration of the disease was 7 years. In about 90% of the cases, two or more sites were affected. Inguinal (70.8%) and axillary regions (61.3%) were most commonly involved. All patients were treated by radical wide excision using intraoperative marking of sinus tracts with methylviolet solution. The method of reconstruction depended on the size and location of the defect. Median postoperative follow-up time was 36 months. RESULTS: The overall complication rate was 17.8%. Most of these were minor complications such as suture dehiscence, postoperative bleeding, and hematoma. Wound infection occurred in only 3.7% of patients. The rate of recurrence within the operated fields was 2.5%. There was no relation between the surgical method of reconstruction and the rate of recurrence. Recurrence was related to the severity of the disorder. CONCLUSION: Our results confirm early radical excision as the treatment of choice for hidradenitis suppurativa. Using intraoperative color-marking of sinus tracts, the recurrence rate is minimal. The method of reconstruction has no influence on recurrence and should be chosen with respect to the size and location of the excised area.

Botulinum toxin for treatment of craniofacial hyperhidrosis.

The effect of botulinum toxin A (BTX) was studied on 12 patients with idiopathic craniofacial hyperhidrosis. After confirming the diagnosis by Minor's iodine starch test we first treated one-half of the forehead with an injection of 2.5-4 ng BTX (Dysport) equidistantly intracutaneously. After 4 weeks we assessed the efficacy by another Minor's iodine starch test and then treated the other half. Another 4 weeks later a standardized telephone interview was carried out. After 1-7 days the craniofacial sweating in the area injected had completely ceased in 11 patients and was mildly reduced in the remaining one. The efficacy was confirmed by repeated Minor's iodine starch tests. Mild weakness of frowning was the only side effect, lasting 1-12 weeks and completely resolving in all patients. Although sweating has not yet recurred in most patients at follow-up periods up to 27 months, one patient had a relapse 9 months after treatment. Following reports on palmar and axillary hyperhidrosis and gustatory sweating (Frey's syndrome) this is apparently the first report on the use of BTX in the treatment of idiopathic craniofacial hyperhidrosis. BTX seems a promising new treatment for localized hyperhidrosis.

[Congenital melanocytic nevi]. / Kongenitale melanozytäre Nävi.

Congenital melanocytic nevi are benign skin tumours with a population-based prevalence of 1-6%. They run an increased life-time risk of transformation into malignant melanoma. Additionally, they can be associated with an involvement of the leptomeninges (neurocutaneous melanosis), and may cause considerable cosmetic and psychic problems. In contrast to the past, the risk of congenital melanocytic nevi developing into melanoma is now regarded as well-established. Thus, excision is recommended whenever possible. Large lesions which do not allow complete excision should be treated by dermabrasion within the first weeks of life.

[Restrictive dermopathy]. / Restriktive Dermopathiey.

Restrictive dermopathy is a rare, fatal, autosomal recessive, congenital skin disease. Rigidity of translucent thin skin, which is thus highly vulnerable and tears, spontaneously causes intra-uterine fetal akinesia or hypokinesia deformation sequence (FADS), characteristic dysmorphic facies with fixed open mouth in O position, and generalized joint contractures (arthrogryposis). Polyhydramnios and pulmonary hypoplasia are distinctive manifestations, leading to respiratory insufficiency and premature delivery at about 31 weeks of gestation. We report on a case of a prematurely born infant who presented with the typical morphological features and describe the light- and electron-microscopical findings as described in the literature.

Cigarette smoking as a triggering factor of hidradenitis suppurativa.

BACKGROUND: Hidradenitis suppurativa is a chronic inflammatory skin disease involving the axillary, inguinal and anogenital regions and sometimes, in addition, the submammary or sacral areas. The etiology of this condition is unknown. OBJECTIVE: A matched-pair case-control study was performed to evaluate the influence of smoking habits on the manifestation of this disease. METHODS: Patients who had received surgical treatment for hidradenitis suppurativa in two dermatological centers completed a questionnaire dealing with family history, course of the disease and smoking habits. To form a randomized matched-pair control group, an equal number of patients admitted for various other skin diseases such as atopic dermatitis, varicose veins, skin tattoos, alopecia areata or melanoma was matched for sex and age and evaluated for smoking habits. Statistical analysis was performed by use of several chi2 tests in a cross-table setting. Moreover, a comparison to the expected smoking prevalence in Germany based on national statistics was performed. RESULTS: Out of 84 patients treated for hidradenitis suppurativa, 63 subjects (27 men, 36 women) completed the questionnaire. The rate of active cigarette smokers was 88.9% (56 patients), whereas 4 subjects (6.4%) had never smoked. 3 patients (4.8%) stated to be ex-smokers, but 2 of these had quit smoking only recently and after onset of the disease. The rate of smokers in the matched-pair control group was 46%. The significantly higher proportion of active smokers among patients with hidradenitis suppurativa can be expressed by an odds ratio of 9.4, the calculated 95% confidence interval was 3.7-23.7 (p < 0.001). The expected smoking prevalence in Germany was 26.7% according to national statistics. 73% of our patients had no family history of hidradenitis suppurativa whereas 27% reported at least one affected first-degree relative. CONCLUSION: From the exceedingly high rate of smokers among patients with this condition we conclude that cigarette smoking is a major triggering factor of hidradenitis suppurativa. Remarkably, the disease can be categorized as a smoking sequel that is neither of vascular nor neoplastic nature. Because familial occurrence was rather rarely reported, and because an environmental factor in the form of cigarette smoking appears to be of crucial importance to trigger the disease, we assume that the genetic basis of hidradenitis suppurativa is polygenic rather than mendelian. Smoking cessation should be encouraged particularly in patients with hidradenitis suppurativa although it is unknown whether this improves the course of the disease.

Dermabrasion of congenital nevocellular nevi: experience in 215 patients.

BACKGROUND: The indication for surgical treatment of congenital nevocellular nevi results from aesthetic-cosmetic consideration as well as from the increased risk of melanomatous transformation. OBJECTIVE: We evaluated the outcome after dermabrasion of congenital nevi of different sizes and treated at different ages. METHODS: 215 patients treated by dermabrasion during the years 1979-1995 were examined at a median interval of 24 months postoperatively. RESULTS: No postoperative development of malignant melanoma arising from the congenital nevus was seen in any of the patients during the time of follow-up. No serious long-term complications were seen. Hypertrophic scars were seen within parts of the operation field in 14.6%, but in those the cosmetic result was still satisfactory. Permanent reduction of pigmentation to 0-20% as compared with the preoperative status was achieved if treatment was performed within the newborn period. In case of large and giant nevi, permanent removal of pigmentation was better than in small or medium-sized nevi. CONCLUSION: Dermabrasion proved to be an adequate modality for removal of pigmentation in the therapy of large and giant congenital nevocellular nevi when assessed within 2 years following the procedure. Early treatment is crucial for permanent removal of pigmentation. Long-term effects remain to be adequately monitored.

Malignant melanoma of the vulva. Report of 89 patients.

BACKGROUND: Rates of melanoma have increased worldwide over the last few decades. Currently, this rate of increase is greater for melanoma than for any other cancer in the U.S. Approximately 3% of all melanomas diagnosed in women are located within the genital tract, predominantly affecting the vulva. Overall, melanomas of the vulva account for 2-10% of all malignancies of the female external genitalia. Due to the rarity of this disease, treatment recommendations do not exist. METHODS: This retrospective study was designed to evaluate the significance of clinical and pathologic features for survival among 89 patients examined for malignant melanoma at 5 hospitals in Germany from 1978 to 1991. A complete workup based on age, initial symptoms, tumor localization, presence of ulceration, postoperative stage, surgical procedure, and survival, was performed. RESULTS: The overall 5-year survival rate of 36.7% confirms the poor prognosis of this disease. Definitive treatment concepts require a standardized treatment of patients with malignant melanoma of the vulva; however, because of the rarity of vulvar melanomas, prospective studies are difficult to perform. CONCLUSIONS: Parameters such as age, Breslow's thickness of invasion, Clark's level of invasion, lymph node involvement, anatomic site, and postoperative stage are prognostic factors for survival. Surgery should be performed in accordance with the accepted standards for cutaneous melanoma.