[Lichen planus]. / Lichen planus.

Lichen planus is an inflammatory disorder of the skin and/or mucosa. Immune dysregulation, infections, environmental and genetic factors play a role in its pathogenesis. Clinically, there are 6 important distinctive manifestations. The mucosal subtypes manifest inside the mouth, oesophagus, genitalia and - although less often - the nose, ear canal, tear duct and conjuctiva. The non-mucosal subtypes occur on the skin, scalp (hair follicles) and nails. Patients may suffer from several subtypes of lichen planus. Unfamiliarity with the different manifestations may lead to a delay in diagnosis and thus to insecurity and distress in patients. The advice to all healthcare providers is to ask patients with lichen planus about symptoms of all subtypes and clinically inspect the skin and mucosa, or to refer the patient to a dermatologist.

Summary of the Dutch S3-guidelines on the treatment of psoriasis 2011. Dutch Society of Dermatology and Venereology.

This document provides a summary of the Dutch S3-guidelines on the treatment of psoriasis. These guidelines were finalized in December 2011 and contain unique chapters on the treatment of psoriasis of the face and flexures, childhood psoriasis as well as the patient's perspective on treatment. They also cover the topical treatment of psoriasis, photo(chemo)therapy, conventional systemic therapy and biological therapy.

Granulomatous slack skin. Report of three patients with an updated review of the literature.

PURPOSE: Granulomatous slack skin (GSS) is a rare cutaneous disorder characterized clinically by the evolution of circumscribed erythematous lax skin masses, especially in the body folds, and histologically by a granulomatous T-cell infiltrate and loss of elastic fibers. GSS is often associated with preceding or subsequent lymphoproliferative malignancies, especially mycosis fungoides (MF) and Hodgkin's disease (HD). No effective treatment is known yet. Whether this entity is a benign disorder, a peculiar host reaction to a malignant lymphoma, a precursor of malignant lymphoma or an indolent cutaneous T-cell lymphoma (CTCL) in itself is still a matter of debate. PATIENTS AND METHODS: The results of the patients with GSS from the Netherlands are compared with the cases reported in the world literature. RESULTS: A female patient had had GSS for 8 years without developing a secondary malignancy. In a second female patient with a histologically confirmed diagnosis of MF, GSS developed 18 years later in the axillary and inguinal folds which had previously been affected by plaque-stage MF lesions. A third male patient with a 6-year history of erythematosquamous skin disease diagnosed as CTCL developed GSS. Moreover, granuloma formation was also found in a facial basal cell carcinoma, in a cervical lymph node and the spleen. Clonal rearrangements of the T-cell receptor beta genes were found in the 2 female patients; the male patient could not be tested. CONCLUSION: GSS is a rare clinicopathological entity. Only 34 patients have been described so far. The development of GSS within plaque MF lesions has not been reported before. Our third case developed very extensive skin lesions and showed a strong propensity to develop granulomas as compared to cases reported before. The presence of a clonal T-cell population was demonstrated in all cases tested. Our cases support the idea that GSS is a very rare and rather indolent type of CTCL. Apparently, the disease is associated with a peculiar immune response, characterized by granuloma formation and disappearance of elastic fibers resulting in the lax skin. The relationship between GSS and other preexisting or subsequent lymphoproliferative diseases (diagnosed in approximately 50% of the cases) warrants a life-long follow-up.

Occurrence of cutaneous and uveal melanoma in patients with uveal melanoma and their first degree relatives.

Uveal and cutaneous melanomas are rare tumours, but have been described to occur together in one patient or in members of the same family. A group of 109 consecutive uveal melanoma patients from one specialized ocular tumour clinic were investigated dermatologically. The patient's own history and medical data and the family history of skin or eye problems were recorded. A total of three cutaneous melanomas were found as a result of this study--two in ocular melanoma patients and one in a first-degree relative. Four patients had first-degree relatives with a skin melanoma (in three of these families dysplastic naevus syndrome was also found), and one patient had a first-degree relative with an uveal melanoma. To find cutaneous and uveal melanoma coexisting in two cases and cutaneous melanoma in first-degree relatives in four cases out of a total of 109 uveal melanoma patients seems more than a coincidence. A linking factor in three cases was the familial atypical multiple mole melanoma syndrome, suggesting a common genetic predisposition to both malignancies in these families. In our only family with familial uveal melanoma, cutaneous melanoma and atypical naevi did not occur. A different genetic mechanism for these cases is probable.

A comparative study of atypical and melanocytic naevi on the tropical island Curaçao and in The Netherlands.

A cross-sectional study was undertaken in 270 inhabitants of The Netherlands (moderate maritime climate, latitude 51 degrees 5' N-53 degrees 3' N) with skin types I-III and 757 inhabitants of the tropical island Curaçao (latitude 12 degrees 2' N-12 degrees 23' N). The latter group consisted of 282 White individuals (mostly Dutch immigrants) and 475 people with non-White skin types IV, V and VI. All participants underwent total skin examination and melanocytic naevi (MN) and clinically atypical naevi (AN) were noted. There was no statistical difference in the mean number of naevi > or = 2 mm or > or = 5 mm between the Curaçao and The Netherlands White groups, but the mean number of naevi > or = 2 mm and > or = 5 mm was significantly lower in the Curaçao non-White group. We found no obvious differences in mean naevus counts between genders and the various comparable age groups. Furthermore, we found no significant difference in mean crude and mean age-standardized prevalence of (one or more) AN between Whites in The Netherlands and on Curaçao. The Mantel-Haenszel prevalence ratio weighted for age and gender differences of Curaçao Whites vs non-Whites was 5.93 (95% confidence interval 3.9-12.0), demonstrating that AN are significantly less prevalent in darker skin types. In all groups, people with AN had significantly more MN than people without AN. We found a generation-dependent difference in the expression of AN among White inhabitants of The Netherlands and on Curaçao, with a higher prevalence of AN in the younger generation.

Are atypical nevi a risk factor for uveal melanoma? A case-control study.

Atypical nevi and other potential risk factors for uveal melanoma were studied in 109 uveal melanoma patients and 149 controls. Information concerning employment, medical history, drug use, family history of cancer, excess sun exposure, and blistering sunburn before and after the age of 15 was obtained. A total skin examination was performed and skin type, hair color, eye color, freckles, actinic damage, the total number of common acquired nevi, and the number of clinically atypical nevi were noted. More atypical nevi were found in uveal melanoma patients than in controls (age- and sex-adjusted odds ratio of 2.9 [95% confidence interval 1.2-6.3] for one or two atypical nevi versus none; odds ratio of 5.1 [95% CI 1.3-20.0] for three or more atypical nevi versus none). Light skin types and freckling also prevailed in uveal melanoma cases. In our study, atypical nevi are more common in uveal melanoma patients than in controls. Further studies will have to indicate whether risk factors comparable to those for cutaneous melanoma really exist for uveal melanoma.

Dysplastic nevi. Occurrence in first- and second-degree relatives of patients with 'sporadic' dysplastic nevus syndrome.

In this study a cross-sectional survey was undertaken among 156 living family members of 31 probands originally classified as having sporadic (histologically verified) dysplastic nevus syndrome (DNS). Seven (13.2%) of 53 parents had clinically recognizable DNS. Twenty-six (36.1%) of the 72 sibs showed dysplastic nevi. The diagnosis of DNS in family members was based on mainly clinical examination; in eight family members--those with only mild manifestation of DNS--a nevus was removed for histologic confirmation. After correction for pedigree size, we found that 60% of patients with "type A sporadic" DNS actually had one or more relatives with a DNS phenotype. Only 25% (8/30) of the probands were ultimately true sporadic cases without a DNS-affected first- or second-degree relative. In 15% (5/31) of the probands no conclusions concerning the type of DNS could be made because the pedigree size did not allow such a conclusion. We also found a higher prevalence of dysplastic nevi among the younger generation as compared with the older generation in our probands with DNS and their families as well as in a general population study of 400 individuals. This generation-dependent difference in expression of the DNS phenotype suggests that besides a genetic factor, other factors may play a role in the development of the characteristic phenotype.

Effect of indomethacin and prednisolone on the disruption of the blood-aqueous barrier after hyperthermia on the rabbit eye by focused ultrasound.

In 24 rabbits both eyes were treated in the perilimbic area with hyperthermia by focussed ultrasound at a frequency of 4.65 MHz. The intra-ocular pressure (IOP) decreased significantly in all eyes. The effect of pre-treatment with indomethacin and prednisolone on the inflammatory response induced by the insonification was assessed by determination of protein in the aqueous humour 2 h after ultrasound treatment. Local pre-treatment with indomethacin but not with prednisolone was followed by less increase in protein concentration of the aqueous humour as compared to placebo-treated controls. The IOP was not influenced significantly by the drug pre-treatments.

The effect of therapeutic ultrasound on the average of multiple intraocular pressures throughout the day in therapy-resistant glaucoma.

The effect of therapeutic ultrasound was evaluated in 17 patients with therapy-resistant glaucoma by comparing average intraocular pressure (IOP) curves measured before and 3-4 months after insonification. The insonification regimen was standardized; the medication was identical during both pressure curve measurements. The average IOP decrease was 44% +/- 24 SD (range, 73% decrease to 6% increase). In 82% of the patients the IOP decrease was more than 34%. There was no correlation between the percentage of IOP decrease and the average IOP before insonification (correlation coefficient, 0.21; P = 0.41).