[Deeper skin tones rarely depicted in Dutch dermatology textbooks]. / Donkere huid zelden afgebeeld in Nederlandse dermatologieboeken.

OBJECTIVE: Within diverse populations such as in the Netherlands, medical education must prepare students to diagnose skin conditions on a broad range of skin tones. To develop the visual pattern recognition skills to do so, medical students need exposure to skin conditions on deeper skin tones. The purpose of this study is to assess the inclusion of images of brown skin in Dutch dermatology textbooks. DESIGN: Observational study. METHOD: Two large Dutch student textbook web shops were searched for dermatology textbooks, and all available general dermatology textbooks explicitly aimed at medical students were selected. All images of skin were examined and divided into the categories 'light skin', 'light to medium brown skin', 'medium to deep brown skin', 'deep to very deep brown skin', and 'indeterminate'. RESULTS: Five textbooks, with a total of 2060 images of skin, were examined. 87.6% of images showed light skin, 7.0% showed light to medium brown skin, 2.9% showed medium to deep brown skin, and 0.5% showed deep to very deep brown skin. 2.0% was categorized as 'indeterminate'. CONCLUSION: Dutch dermatology textbooks currently include only small percentages of images of brown skin. Unfamiliarity with disease presentation on deeper skin tones can lead to delayed diagnosis and worse outcomes in Black and Brown patients. Future textbooks should include images of different skin tones, including deeper ones, for every skin condition.

Frequency and prognosis of associated malignancies in 504 patients with lymphomatoid papulosis.

BACKGROUND: Lymphomatoid papulosis (LyP) can be associated with other haematological malignancies (HM), but reported percentages vary from 20% to over 50%. OBJECTIVE: To evaluate the frequency and prognostic significance of associated HM and non-HM in LyP patients. METHODS: In this multicentre cohort study, the complete Dutch LyP population was included from the Dutch Cutaneous Lymphoma Registry between 1985 and 2018. Clinical and histopathological information was retrieved from every individual patient. RESULTS: After a median follow-up of 120 months (range, 6-585), an associated HM was observed in 78/504 (15.5%) patients. Most common associated HM were mycosis fungoides (MF; n = 31) and anaplastic large-cell lymphoma (ALCL; n = 29), while 19 patients had another HM of B-cell (n = 14) or myeloid origin (n = 5). Even after a 25-year follow-up period, percentages of associated HM did not exceed 20%. Thirty-nine of 465 patients (8.4%) without a prior or concurrent associated HM developed an associated HM during follow-up, after a median of 68 months (range of 3-286 months). Nine of 78 patients died of associated HM, including 6/22 patients developing extracutaneous ALCL, while all patients with associated MF or skin-limited ALCL had an excellent prognosis. Compared with the general population, LyP patients showed an increased risk (relative risk, 2.8; 95% confidence intervals, 2.4-3.3) for non-HM, in particular cutaneous squamous cell carcinoma, melanoma and intestinal/lung/bladder cancer. CONCLUSIONS: An associated HM was reported in 15.5% of the LyP patients, particularly MF and ALCL. Although the frequency of associated HM is lower than suggested and the prognosis of most patients with associated HM is excellent, a small subgroup will develop aggressive disease, in particular extracutaneous ALCL. Furthermore, LyP patients have a higher risk of developing other malignancies. Clinicians should be aware of these risks, and LyP patients require close monitoring.

Evaluation of treatment results in multifocal primary cutaneous anaplastic large cell lymphoma: report of the Dutch Cutaneous Lymphoma Group.

BACKGROUND: There is no consensus on the treatment of multifocal primary cutaneous anaplastic large cell lymphoma (C-ALCL). Radiotherapy (RT) and methotrexate (MTX) are the current treatment options, but their efficacy is unknown. Recently, targeted therapies showed promising results in C-ALCL, and may therefore be an attractive first choice of treatment. OBJECTIVES: To assess the efficacy of conventional treatment strategies for patients with multifocal C-ALCL, and to define which patients may require novel targeted therapies. METHODS: In this multicentre study, treatment was evaluated in patients initially presenting (n = 24) or relapsing with multifocal C-ALCL (n = 17; 23 relapses). Distinction was made between patients with five or less lesions (n = 36) and more than five lesions (n = 11). RESULTS: Treatments most commonly used were RT (n = 21), systemic chemotherapy (n = 9) and low-dose MTX (n = 7) with complete response rates of 100%, 78% and 43%, respectively, and an overall response rate of 100%, 100% and 57%, respectively. Four patients showed complete spontaneous regression. In total, 16 of 24 patients (67%) first presenting with multifocal C-ALCL relapsed, including all five patients initially treated with CHOP (cyclophosphamide, hydroxydaunorubicin, oncovin and prednisone). Compared with patients presenting with two to five skin lesions, patients presenting with more than five lesions had a higher chance of developing extracutaneous relapse (56% vs. 20%) and more often died of lymphoma (44% vs. 7%). CONCLUSIONS: Patients with five or less lesions should be treated with low-dose RT (2 × 4 Gy). Maintenance low-dose MTX (20 mg weekly) is a suitable option in patients with more than five lesions. Targeted therapies may be considered in rare patients who are refractory to MTX or patients developing extracutaneous disease.

Recommendations for treatment in folliculotropic mycosis fungoides: report of the Dutch Cutaneous Lymphoma Group.

BACKGROUND: Folliculotropic mycosis fungoides (FMF) is an aggressive variant of mycosis fungoides (MF) and generally less responsive to standard skin-directed therapies (SDTs). Recent studies distinguished indolent (early-stage FMF) and more aggressive (advanced-stage FMF) subgroups. The optimal treatment for both subgroups remains to be defined. OBJECTIVES: To evaluate initial treatment results in patients with early- and advanced-stage FMF. METHODS: A study was undertaken of 203 patients (84 early-stage, 102 advanced-stage, 17 extracutaneous FMF) included in the Dutch Cutaneous Lymphoma Registry between 1985 and 2014. Type and results of initial treatment were retrieved from the Dutch Registry. Main outcomes were complete remission (CR); sustained complete remission; partial remission (PR), > 50% improvement; and overall response (OR; CR + PR). RESULTS: Patients with early-stage FMF were treated with nonaggressive SDTs in 67 of 84 cases resulting, respectively, in CR and OR of 28% and 83% for monotherapy topical steroids, 0% and 83% for ultraviolet B (UVB), and 30% and 88% for psoralen plus ultraviolet A (PUVA). In patients with advanced-stage FMF these SDTs were less effective (combined CR and OR 10% and 52%, respectively). In patients with advanced-stage FMF local radiotherapy (CR 63%; OR 100%), total skin electron beam irradiation (CR 59%; OR 100%) and PUVA combined with local radiotherapy (CR 5%, OR 75%) were most effective. CONCLUSIONS: The results of the present study demonstrate that not all patients with FMF should be treated aggressively. Patients with early-stage FMF may benefit very well from standard SDTs also used in early-stage classic MF and have an excellent prognosis.

[A neonate with peculiarly arranged skin abnormalities]. / Een baby met bijzonder gerangschikte huidafwijkingen.

A tropical doctor reviewed a female Malawian neonate with a vesicular rash and linear hyperpigmentation that followed the lines of Blaschko. This distribution is typical for incontinentia pigmenti, a congenital X-linked dermatosis, which usually resolves spontaneously in adolescence.

[The practice guideline 'Bacterial skin infections' (first revision) from the Dutch College of General Practitioners; a response from the perspective of dermatology]. / De standaard 'Bacteriële huidinfecties' (eerste herziening) van her Nederlands Huisartsen Genootschap; reactie vanuit de dermatologie.

We reviewed the first revision of the clinical practice guidelines on the management of bacterial skin infections developed by the Dutch College of General Practitioners. Bacterial skin infections are subdivided into superficial and deep infections; the former are often treated locally while the latter may require systemic antibiotics or surgical intervention. The rate of infection with methicillin-resistant Staphylococcus aureus (MRSA) in the community is relatively low in The Netherlands, but the guideline provides recommendations, such as the restricted use of mupirocin ointment, to facilitate future MRSA control measures. Clinical distinction between erysipelas and cellulitis is often impossible; therefore, the term cellulitis is used throughout the guideline and refers to both Staphylococcus aureus and Streptococcus pyogenes infections of the skin and subcutaneous tissue. The first line of therapy for cellulitis remains a small spectrum, beta-lactamase resistant penicillin, such as flucloxacillin for 10 days. There are no conclusive studies on the prevention of recurrent cellulitis, so recommendations are based on expert opinion and pathophysiological considerations. The lack of rigorous and controlled studies often precludes making clear evidence-based recommendations. However, this guideline succeeds remarkably well in combining the available evidence and formulating sound practical management advice for bacterial skin infections in primary care in The Netherlands. It deserves widespread implementation among general practitioners.

Therapeutic strategies for epidemic Kaposi's sarcoma.

Kaposi's sarcoma (KS) remains the most commonly diagnosed malignancy in HIV-infected patients, and is one of the AIDS-defining diagnoses. Several different therapeutic options are available, but the optimal therapy is still unclear. The incidence of KS has sharply declined since highly active antiretroviral therapy (HAART) became widely available, making HAART indispensable in the treatment of epidemic KS. HAART can be given alone or in combination with systemic and local therapy. Systemic therapy can be given in disseminated, progressive or symptomatic disease. Treatment options are interferon-alpha and chemotherapy including pegylated-liposomal anthracyclines and paclitaxel. For local disease, radiotherapy, intralesional chemotherapy or cryotherapy may be used. In resource-limited settings, intravenous vincristine, oral etoposide or intramuscular bleomycin may be feasible options. Other therapies, such as angiogenesis inhibitors, are under investigation in clinical trials.

Primary cutaneous marginal zone B-cell lymphoma: clinical and therapeutic features in 50 cases.

BACKGROUND: Primary cutaneous marginal zone B-cell lymphoma (PCMZL) is a low-grade B-cell lymphoma that originates in the skin, with no evidence of extracutaneous disease. Studies focusing on the optimal treatment of PCMZL have not been published thus far. We describe 50 patients with PCMZL to further characterize clinical characteristics and outcome and, in particular, to evaluate our current therapeutic approach. OBSERVATIONS: The majority of the patients (36/50 [72%]) presented with multifocal skin lesions, and 14 patients (28%) presented with solitary or localized lesions. The initial treatment of patients with solitary lesions consisted of radiotherapy or excision, whereas patients with multifocal lesions received a variety of initial treatments, most commonly radiotherapy and chlorambucil therapy. Cutaneous relapses developed in 19 (48%) of 40 patients who had complete remission and were more common in patients with multifocal disease. After a median period of follow-up of 36 months, 2 patients developed extracutaneous disease, but none of the patients died of lymphoma. CONCLUSIONS: Patients with PCMZL who have solitary lesions can be treated effectively with radiotherapy or excision. For patients with PCMZL who have multifocal lesions, chlorambucil therapy and radiotherapy are suitable therapeutic options. In case of cutaneous relapses, the beneficial effects of treatment should carefully be weighed against the potential adverse effects.

[Women with pelvic complaints and atypical varicose veins, varicose veins of the vulva and insufficiency of the pelvic veins; treatment with embolisation]. / Vrouwen met onderbuikklachten en met atypische varices, vulvavarices en insufficiënte bekkenvenen; behandeling door embolisatie.

In three women, aged 34, 52 and 30 years, respectively, who suffered from chronic pelvic symptoms such as a heavy feeling and abdominal pain, atypical varicose veins were observed on the medial side of the upper thighs together with varicose veins of the vulva; these are indicators of insufficiency of the pelvic veins. The symptoms were reduced following embolisation of the insufficient pelvic veins. These symptoms are also known as the pelvic congestive syndrome. The exact correlation between insufficiency of the pelvic veins and the complaints mentioned above has not yet been determined, but it seems that in most patients the symptoms diminish or disappear following embolisation of the insufficient pelvic veins. When patients complain of chronic pelvic pain of unknown aetiology, one should look for atypical varices and vulval varices and consider pelvic-vein insufficiency as a possible cause.

Photosensitivity in patients with lupus erythematosus: a clinical and photobiological study of 100 patients using a prolonged phototest protocol.

BACKGROUND: There is a clear relationship between ultraviolet (UV) radiation (UVR) and the clinical manifestations of patients with lupus erythematosus (LE). Cutaneous lesions are induced or exacerbated by exposure to UVR. Of patients with LE, 24-83% are reported to be photosensitive to UVR. LE tumidus appears to be the most photosensitive subtype of LE, followed by subacute cutaneous LE (SCLE). In general, the history of patients with LE correlates poorly with the presence or absence of photosensitivity, due to a delayed time interval between UV exposure and exacerbation of skin lesions. Phototesting using artificial UVR and visible light is a reliable way of diagnosing photosensitivity. OBJECTIVES: To investigate the photoreactivity of patients with various subtypes of LE using an individualized phototest protocol. The results of phototests were correlated with the history of photosensitivity, the subtype of LE, the presence of autoantibodies and the use of anti-inflammatory medication by these patients. METHODS: Phototesting with UVA, UVB and visible light was performed in 100 patients with LE. The diagnosis of LE was established both on clinical examination and skin histology. Serological studies were also performed in all patients. The phototests were performed on large skin areas of the forearm or trunk; the first dose was twice the minimal erythema dose and the dosage was increased according to the individual reactions of the patients at the test sites. Follow-up of skin reactions at the test sites was performed for up to 2 months. Histological examination of the photoprovoked skin lesions was carried out in 57 patients. RESULTS: Of the 100 patients included (81 women and 19 men; mean age 41 years, range 17-79), 46 had chronic discoid LE, 30 SCLE and 24 systemic LE. An abnormal reaction to UVR and visible light was found in 93% of our patients with LE. No clinical or histological evidence at the phototest sites of polymorphic light eruption was found. There was no correlation between photosensitivity and LE subtype, presence of autoantibodies or medical history. Concomitant use of anti-inflammatory medication seemed to exert only minimal influence on the results of phototesting. CONCLUSIONS: When using an extended phototesting protocol, almost all patients with LE in this study showed clinical and histological evidence of aberrant photosensitivity. Therefore, patients with LE should receive thorough advice and instruction on photoprotective measures, regardless of their history, LE subtype or presence of autoantibodies.

[Increase in rate of resistance to fusidic acid among Staphylococcus aureus isolates from patients admitted with atopic dermatitis]. / Toename van resistentie tegen fusidinezuur van Staphylococcus aureus-isolaten bij opgenomen patiënten met constitutioneel eczeem.

To determine whether there has been an increase in the incidence of resistance to fusidic acid among Staphylococcus aureus isolates in the Netherlands, a retrospective study was carried out. The resistance pattern of S. aureus isolates from patients with atopic dermatitis at the Dermatology inpatient department of the University Medical Centre Utrecht was determined during the period 1995-2001. The rate of resistance increased from 9.7% to 23.4% during this period, whereas the rate of resistance of S. aureus to methicillin remained stable at around 0.5%. Prolonged topical use of fusidic acid is probably the main cause for the increase in fusidin resistance. Therefore it is advised to limit the use of fusidic acid for infected dermatitis to short periods of about two weeks, and only after sensitivity of the strain to fusidin has been confirmed.