Cornelus J.G. Sanders

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

Summary 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.

Clinical Staging and Prognostic Factors in Folliculotropic Mycosis Fungoides

IMPORTANCE Large case series suggest that patients with folliculotropic mycosis fungoides (FMF) have a worse prognosis than patients with classic mycosis fungoides (MF). However, recent studies described a subgroup of patients with FMF with a more favorable prognosis. Distinction between indolent and aggressive FMF may have important therapeutic consequences but is hampered by the inability of the current tumor-node-metastasis-blood (TNMB) staging system to classify patients with FMF in a clinically meaningful way. OBJECTIVE To differentiate between indolent and aggressive FMF using clinicopathological criteria and to define prognostic factors in patients with FMF. DESIGN, SETTING, AND PARTICIPANTS In this prospective cohort study, we followed 203 patients with FMF, included in the Dutch Cutaneous Lymphoma Registry between October 1985 and May 2014 at a tertiary referral center hosting the Dutch Cutaneous Lymphoma Registry. Overall, 220 patients with FMF had been registered, but 17 patients with incomplete follow-up data or a history of classic MF were excluded. MAIN OUTCOMES AND MEASURES Main outcomes included clinical and histological characteristics, disease progression, and survival. Prognostic factors were investigated using Cox proportional hazard regression analysis. Distinction between early plaque-stage FMF and advanced plaque-stage FMF was made by a blinded review of skin biopsy specimens from patients presenting with plaques. RESULTS In a cohort of 147 men and 56 women (median [range] age, 59 [15-93] years), patients with histologically early plaque-stage FMF had a very similar overall survival (OS) rate to patients with only patches and/or follicular papules (10-year OS, 71% vs 80%), while the survival rate of patients with histologically advanced plaque-stage FMF was almost identical to that of patients presenting with tumors (10-year OS, 25% vs 27%). Subsequently, 3 clinical subgroups with significantly different survival data were distinguished: early skin-limited FMF (group A; n = 84; 5-year and 10-year OS, 92% and 72%); advanced skin-limited FMF (group B; n = 102; 5-year and 10-year OS, 55% and 28%); and FMF presenting with extracutaneous disease (group C; n = 17; 5-year and 10-year OS, 23% and 2%). Age at diagnosis, large cell transformation and secondary bacterial infection were independent risk factors for disease progression and/or poor survival. CONCLUSIONS AND RELEVANCE The results of this study provide useful criteria to differentiate between indolent and aggressive FMF and confirm the existence of a subgroup of FMF with a favorable prognosis.

Photodynamic therapy in a patient with Darier's disease

Patients with supernumerary nipples may also have associated anomalies. A wide range of concomitant associations has been described, mainly urogenital disorders, particularly in males. 6 Moreover, familiar antecedence of alcoholism as predisposing factor and the systematic urologic investigation in cases of polythelia/polymastia still remains controversial. In our case there was no urinary symptoms, but an accurated clinical evaluation in patients with supernumerary nipples is recommended. 1,2

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

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.

Drug rash with eosinophilia and systemic symptoms caused by the dietary supplement diindolylmethane

Drug rash with eosinophilia and systemic symptoms (DRESS) is a severe adverse drug reaction that is potentially life threatening with a mortality rate up to 10%. It is characterized by a variable combination of severe skin eruption, fever, lymphocyte activation (enlarged lymph nodes and atypical lymphocytes), eosinophilia, and internal organ involvement of which the liver is the most common affected organ. Compared with other cutaneous drug reactions, DRESS is characterized by a late onset, usually 2-8 weeks after the initiation of the drug (mainly antiepileptic drugs, allopurinol, and antibiotics) therapy, and a longer duration of symptoms despite discontinuation of the culprit drug. For the first time, we report a case of DRESS that is caused by a dietary supplement. Our patient reacted to diindolylmethane (DIM), which is derived from cruciferous vegetables such as broccoli, cauliflower, cabbage, kale, and Brussels sprouts. It is also an investigational drug being used in treating conditions related to human papilloma virus infection, such as cervical intraepithelial neoplasia and recurrent respiratory papillomatosis, and it is considered a potential chemopreventive agent. The current frequency of DIM consumption is unknown. Previous studies on the safety of DIM showed no severe adverse reactions.

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

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.