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The term 'sclerosing diseases of the skin' comprises specific dermatological entities, which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present consensus provides an update to the 2017 European Dermatology Forum Guidelines, focusing on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, updated strategies for the first‐ and advanced‐line therapy of sclerosing skin diseases are addressed in detail. Part 1 of this consensus provides clinicians with an overview of the diagnosis and treatment of localized scleroderma (morphea), and systemic sclerosis including overlap syndromes. [ABSTRACT FROM AUTHOR]

Abstract: Background: Dermatology Life Quality Index (DLQI) is the most common health‐related quality of life measure in dermatology that is widely used in treatment guidelines for psoriasis. Eight of the 10 questions of the DLQI offer a ‘not relevant’ response (NRR) option that is scored as the item had no impact on patients’ life at all. Objective: To explore the occurrence of NRRs on the DLQI in psoriasis patients and to examine the effect of several socio‐demographic and clinical factors on giving NRRs. Methods: Data were obtained from two cross‐sectional surveys among psoriasis patients at two academic dermatology clinics in Hungary. Health‐related quality of life was measured by employing DLQI and EQ‐5D‐3L, while disease severity was graded by Psoriasis Area and Severity Index (PASI). Multivariate logistic regression was applied to determine the predictors of providing NRRs. Results: Mean age of the 428 patients was 49 years, and 65% were males. Mean PASI, DLQI and EQ‐5D‐3L index scores were 8.4 ± 9.5, 6.8 ± 7.4 and 0.74 ± 0.28, respectively. Overall, 38.8% of the patients had at least one NRR: 19.6% (one), 11.5% (two), 5.1% (three) and 2.6% (more than three). Most NRRs occurred in sport, sexual difficulties and working/studying items of the DLQI (28.4%, 16.4% and 14.0%, respectively). Female gender (OR 1.65; 95% CI 1.04–2.61), older age (OR 1.05; 95% CI 1.03–1.07) and higher PASI score (OR 1.03; 95% CI 1.01–1.06) were associated with providing more NRRs, whereas highly educated patients (OR 0.34; 95% CI 0.16–0.72) and those with a full‐time job (OR 0.47; 95% CI 0.29–0.77) less frequently tended to tick NRRs. Conclusion: The high rate of psoriasis patients with NRRs, especially among women, less educated and elderly patients, indicates a content validity problem of the measure. A reconsideration of the use of the DLQI for medical and financial decision‐making in psoriasis patients is suggested. [ABSTRACT FROM AUTHOR]

Background The hormone sensitivity of melanoma and the role of 'classical' oestrogen receptor ( ER) α and β in tumour progression have been intensively studied with rather contradictory results. The presence of 'non-classical' G protein-coupled oestrogen receptor ( GPER) has not been investigated on human melanoma tissues. Objective To analyse the expression of GPER, ERα and ERβ in pregnancy-associated ( PAM) and in non-pregnancy-associated ( NPAM) melanomas in correlation with traditional prognostic markers and disease-free survival ( DFS). Methods Receptor protein levels were tested using immunohistochemistry in 81 formalin-fixed paraffin-embedded melanoma tissues. PAMs ( n = 38) were compared with age- and Breslow thickness-matched cases ( n = 43) including non-pregnant women ( NPAM-W) ( n = 22) and men ( NPAM-M) ( n = 21). The association between receptor expression and DFS was analysed by uni- and multivariate Cox proportional hazards regression. Results G protein-coupled oestrogen receptor was detected both in PAMs and NPAMs. In 39 of the 41 (95.1%) GPER-positive melanomas, GPER and ERβ were co-expressed. GPER/ ERβ-positive melanomas were significantly more common in PAM compared to NPAM ( P = 0.0001) with no significant difference between genders ( P = 0.4383). In PAMs, the distribution of GPER and ERβ was similar (78.4% vs. 81.6%; P = 0.8504), while in NPAM, ERβ was the representative ER (60.5% vs. 27.9%; P = 0.0010) without gender difference (59.1% vs. 61.9%). GPER-/ ERβ-positive melanomas were associated with lower Breslow thickness, lower mitotic rate and higher presence of peritumoral lymphocyte infiltration ( PLI) compared to GPER-/ ERβ-negative cases ( P = 0.0156, P = 0.0036 and P = 0.0001) predicting a better DFS ( HR = 0.785, 95% CI 0.582-1.058). Despite the significantly higher frequency of GPER and ERβ expression in PAM, no significant difference was found in DFS between PAM and NPAM. All but one case failed to show ERα expression. Conclusions The presence of GPER and its simultaneous expression with ERβ can serve as a new prognostic indicator in a significant subpopulation of melanoma patients. [ABSTRACT FROM AUTHOR]

Background Topical corticosteroids may be needed for treating skin conditions in pregnancy. Nevertheless, only limited data on the fetal effects of topical corticosteroids are available. Objective To update an evidence-based guideline on the safe use of topical corticosteroids in pregnancy. Methods A guideline subcommittee of the European Dermatology Forum updated the guideline by adding and appraising new evidence. Results The current best evidence from 14 observational studies with 1 601 515 study subjects found no significant associations between maternal use of topical corticosteroids of any potency and some adverse pregnancy outcomes including mode of delivery, birth defect, preterm delivery and fetal death. However, maternal use of potent/very potent topical corticosteroids, especially in large amounts, is associated with an increase in the risk of low birthweight. Conclusion Mild/moderate topical corticosteroids should be preferred to potent/very potent ones in pregnancy. The well-known topical side-effects of corticosteroids on the mother's side need to be considered as well. [ABSTRACT FROM AUTHOR]

Background Unrealistic expectations regarding treatments and clinical outcomes may lead to disappointment about therapy and sub-optimal compliance; nonetheless, these expectations have not been studied in psoriasis patients yet. Objective To evaluate psoriasis patients' subjective future expectations regarding health-related quality of life (HRQOL) and life expectancy, and to explore clinical features associated with under- or overestimating behaviour. Methods A cross-sectional questionnaire survey of consecutive adult patients with moderate to severe psoriasis was conducted. HRQOL expectations were recorded by applying the EQ-5D descriptive system for 6 months ahead and for future ages of 60, 70, 80 and 90 respectively. Results In total, 167 patients (71% males) were included in the analysis with mean age of 50.4 ± 12.4 years and mean EQ-5D score of 0.71 ± 0.30. Overall 65% had chronic plaque psoriasis, 35% nail psoriasis, 35% scalp involvement, 29% psoriatic arthritis, 9% inverse psoriasis and 5% palmoplantar psoriasis respectively (combinations occurred). Participants expected 0.1 ± 0.23 mean improvement in EQ-5D within 6 months ( P < 0.001) that achieves the minimum clinically important difference. Overall 37% expected improvement and 13% decline; however, 49% expected no changes in any of the five dimensions of EQ-5D within 6 months. Female gender, inverse or palmoplantar involvement and more severe psoriasis were likely associated with higher expectations. Patients at the initiation of their first biological at the time of the survey expected 0.18 ± 0.24 increase that seems to be realistic compared to the EQ-5D utility gain achieved in randomized controlled trials. Males expected by 2.7 ± 11.1 more, while females expected by 5.2 ± 9.3 less life years compared to the average statistical gender- and age-matched life expectancy ( P < 0.05). Patients who expected to be alive at ages of 60, 70, 80 and 90 scored their future EQ-5D at ages of 60 to 90: 0.59 ± 0.46, 0.48 ± 0.41, 0.42 ± 0.41 and 0.22 ± 0.47 respectively. Conclusion Our findings highlight the importance of exploring expectations that might help to increase patients' compliance. [ABSTRACT FROM AUTHOR]

Background Folliculotropic mycosis fungoides (FMF) represents a variant of MF characterized by hair follicle invasion of mature, CD4-positive small lymphoid cells with cerebriform nuclei. The disease displays resistance to standard treatment modalities and has an unfavourable course. Objective Clinical analysis of 17 patients with FMF collected between 2005 and 2012, investigation of tumour cells and involved hair follicle. Methods Re-evaluation of clinical data, wide panel immunohistochemistry investigation on paraffin-embedded biopsy material, T-cell receptor gene rearrangement analysis of the samples. Results Male and older age group predominance, frequent head-neck involvement, acneiform lesions, keratotic plugs, cysts, nodules, follicular papules, alopecia and classic mycosis fungoides-like plaques represented the main clinical characteristics. Treatment response showed a wide range from transient complete response to therapy resistance and death due to the disease. The pathological alterations: folliculotropism, mild epidermotropism, follicular plugging, mucinous degeneration of hair follicle, basaloid hyperplasia, syringotropism were similar to those observed previously. The first case of a CD8-positive folliculotropic mycosis fungoides - with unusual clinical presentation - is reported here. Nestin overexpression of mesenchymal cells of the isthmic and suprabulbar regions of hair follicle and the reappearance of dermal nestin-expressing cells were observed in association with immature dendritic cell hyperplasia. Altered CK19 expression was detected suggesting a potential role of follicular keratinocytes in the disease process. It was found that a proportion of neoplastic T cells constantly express programmed death-1 receptor in our patients contrary to classic mycosis fungoides. Conclusion The spectrum of the clinical manifestation and the course of folliculotropic mycosis fungoides are broad and differ from classic mycosis fungoides. Folliculotropic neoplastic T-cell proliferation is associated with activation of inflammatory reactive T- and B-lymphoid cells, mesenchymal cells and changes in the hair follicle. [ABSTRACT FROM AUTHOR]

Background Periocular contact dermatitis may appear as contact conjunctivitis, contact allergic and/or irritative eyelid and periorbital dermatitis, or a combination of these symptoms. The clinical symptoms may be induced by several environmental and therapeutic contact allergens. Objectives The aim of the present study was to map the eliciting contact allergens in 401 patients with periocular dermatitis (PD) by patch testing with environmental and ophthalmic contact allergens. Methods Following the methodics of international requirements, 401 patients were tested with contact allergens of the standard environmental series, 133 of 401 patients with the Brial ophthalmic basic and supplementary series as well. Results Contact hypersensitivity was detected in 34.4% of the patients. Highest prevalence was seen in cases of PD without other symptoms (51.18%), in patients of PD associated with ophthalmic complaints (OC; 30.4%), and PD associated with atopic dermatitis (AD; 27.9%). In the subgroup of PD associated with seborrhoea (S) and rosacea (R), contact hypersensitivity was confirmed in 17.6%. Most frequent sensitisers were nickel sulphate (in 8.9% of the tested 401 patients), fragrance mix I (4.5%), balsam of Peru (4.0%), paraphenylendiamine (PPD) (3.7%), and thiomersal (3.5%). By testing ophthalmic allergens, contact hypersensitivity was observed in nine patients (6.7% of the tested 133 patients). The most common confirmed ophthalmic allergens were cocamidopropyl betaine, idoxuridine, phenylephrine hydrochloride, Na chromoglycinate, and papaine. Limitations Patients with symptoms of PD were tested from 1996 to 2006. Conclusions The occurence of contact hypersensitivity in PD patients was in present study 34.4%. A relatively high occurence was seen in cases of PD without other symptoms, in PD + OC and in PD + AD patients. The predominance of environmental contact allergens was remarkable: most frequent sensitizers were nickel sulphate, fragrance mix I, balsam of Peru, thiomersal, and PPD. The prevalence of contact hypersensitivity to ophthalmic allergens did not exceed l.5%. Conflicts of interest None declared [ABSTRACT FROM AUTHOR]

Background Atopic dermatitis (AD) is a clinically well-defined, chronic-intermittant, genetically predisposed skin disease. The increasing number of adult cases observed in the last years has turned the attention to ascertaining factors eliciting skin symptoms. Studies have revealed numerous environmental components (e.g. contact and aeroallergens) that may play an important role in sustaining the symptoms. The aim of the study was to search for contact allergens and aeroallergens triggering the skin disease in adult AD patients. Methods Patients over 18 years from our Atopy Outpatient Department were included in the study. Diagnosis of AD was based on the clinical criteria of Hanifin and Rajka. The distribution of skin symptoms was characteristic for adult AD: hands, shoulders, neck, flexures, face and eyelids. The extremities and the trunk were less involved. The potentially provoking contact and aeroallergens were examined in symptom and drug-free period with atopy patch and epicutaneous tests (APT, ET), which were supplemented by in vitro allergy and Prick tests. The relevance of sensibilization was evaluated by the comparison of in vivo and in vitro test results, medical history and skin symptoms. Results A total number of 34 cases of adult AD (23 women and 11 men) were studied. Four of them were classified into the intrinsic group (IG; 12%), and 30 were classified into the extrinsic group (EG; 88%). The incidence of contact sensitization to environmental allergens was remarkable: 13 of the EG, 1 of the IG (14 of 34, 41%). In the IG, a late thiomersal positivity was detected without clinical relevance. In the EG, epicutaneous standard series late positivity was seen in 13 patients, in four of them with multiple sensitivity. The allergens causing positivity were nickel (6 of 13), thiomersal (3 of 13), mercury-amidochlorate (3 of 13), mercury-chloride (2 of 13), iodine chlorhyrdoxyquin (1 of 13), lanalcolum (1 of 13) and fragrance mix (1 of 13). Among the detected allergens, the following were relevant: lanalcolum (1 of 13: cosmetics), fragrance mix (1 of 13: cosmetics), nickel (1 of 13: metal objects), thiomersal (1 of 13: eyedrops). No immediate response was seen with APT. Relevant late positivity was shown with APT test in one patient in the IG (1 of 4) to Dermatophagoides pteronyssinus. We observed late positivity in 18 patients in the EG (18 of 30). Among the detected allergens, the following were clinically relevant: D. pteronyssinus and/or Dermatophagoides farinae (15 of 18), cat epithel (4 of 18), timothy pollen (1 of 18) and dog epithel (1 of 18). Furthermore, we examined the relevance of APT, specific immunoglobulin E (IgE) tests and Prick tests. We observed multiplex positivity by specific IgE tests, APT and Prick tests in 14 patients in EG. Sensitization to D. pteronyssinus and/or D. farinae (11 of 14) cat epithel (4 of 14), dog epithel (1 of 14) and timothy pollen (1 of 14) proved to be clinically relevant with the atopic skin symptoms and medical history. Conclusion The proportion of contact sensitization to environmental allergens in the 34 adult atopic patients was remarkable (14 of 34, 41%). Out of the verified contact allergens, nickel, fragrance mix, thiomersal and lanalcolum proved to be relevant. House dust mite and cat epithel proved to be the most common relevant aeroallergens. D. pteronyssinus and D. farinae sensibilization was high, particularly in patients with severe skin symptoms on the face, eyelids and hands. Pollens should be considered in patients with seasonal relapse of AD. Sensitization to animal epithel was usually indicated by the flare-up of skin symptoms upon contact with animals. The relevance of the eliciting effects of sensitization could easily be supported in most cases by the medical history and the distribution of skin symptoms. In some adult AD patients with long-lasting AD, the relevance of triggering factors is hard to determine. [ABSTRACT FROM AUTHOR]

Recently, epidermal transglutaminase (TG) has been identified within the papillary IgA granules in dermatitis herpetiformis (DH). Although IgA type autoantibodies to tissue and epidermal TGs are characteristic serological markers for DH, these antibodies do not bind to normal papillary skin structures.To test the possibility of IgA immune complex precipitation within the vessel walls as a first step in the pathogenesis of skin symptoms, we analysed immunoglobulin, complement, and epidermal TG deposition along the vascular system of DH skin.Punch biopsy specimen were taken from 116 DH patients’ skin, and evaluated for the presence of vascular immune precipitates by direct immunofluorescence. Skin samples from 16 DH patients were also studied for tissue and epidermal TGs.In 74 (64%) of the 116 DH skin studied, significant vascular staining accompanied the DH-specific granular IgA fluorescence (IgA and C3 in 39 patients; IgA alone in 18; IgA, C3 and IgM together in five; IgM alone in 12). In most cases (92%), the precipitates were detected in the small vessels of the papillary dermis; however, a subpapillary vascular fluorescence was also observed in a few patients (8%). Skin IgA colocalized with epidermal TG in the vessel walls and within the scattered papillary peri- and intervascular DH bodies. Tissue TG did not colocalize either with the immunoglobulins or with the complement precipitates of the dermis. Furthermore, we could not detect keratinocyte TG in the DH bodies nor in the vessel walls.These findings support possible immune complex precipitation in the vessel walls of DH skin and further confirm the significance of epidermal but not tissue TG in the pathomechanism of skin symptoms. [ABSTRACT FROM AUTHOR]

Presents letters to the editor. Comments on the therapeutic efficacy of tacrolimus; Discussion on localized scleroderma; Comments on skin morphology.

Rácz E, Csikós M, Kornsée Z, Horváth A, Kárpáti S. Identification of mutations in the ATP2A2 gene in patients with Darier's disease from Hungary. Mutation analysis in the ATP2A2 gene had been performed in eight Hungarian patients with Darier's disease (DD), to get more information about phenotype–genotype relations. All patients had moderate to severe skin symptoms. Polymerase chain reaction (PCR) amplification of the entire coding region of ATP2A2 was performed. Mutation detection strategies included heteroduplex scanning by conformation-sensitive gel electrophoresis (CSGE) and direct nucleotide sequencing. We found distinct, heterozygous mutations (five missense, one nonsense, one deletion, and one insertion), six of which were novel. In a 31-year-old DD woman with learning difficulties we disclosed a previously described missense mutation (D702N) in exon 15. A 44-year-old DD woman had a novel T insertion at nucleotide 559 in exon 7 of the ATP2A2 gene, which resulted in a premature termination codon (PTC) at codon 192. A woman, whose skin symptoms developed unusually late, at the age 50, had a new T deletion (1320delT) in exon 11 resulting in a PTC at codon 448. Our most severe case had a known missense mutation N39T, resulting in a non-conservative amino acid change at the upstream stalk region. Three new missense mutations (A161D, R164S, and Q790P) affected conservative regions of the SERCA2 protein within the activation (A)-domain and the M6 transmembrane region. A further new nonsense mutation (C909X) was detected in the M8 transmembrane domain. Our data suggest that differences in DD phenotypes are probably also related to factors different from the type of causative mutation. [ABSTRACT FROM AUTHOR]

Abstract: Congenital ichthyosiform erythroderma (CIE) belongs together with lamellar ichthyosis (LI) to the group of autosomal recessive congenital ichthyoses (ARCI). Mutations in the transglutaminase (TGase) 1 gene (TGM1) have been identified in several families with LI and in some families with CIE. We report a case of CIE with two new nonsense mutations: a C7780G transversion in exon 11 resulting in a premature stop codon at aminoacid residue Y503X and a C8533G transversion in exon 13 leading to a nonsense mutation at S669X. These mutations were also identified in a heterozygous pattern in the unaffected parents. These two termination-codons result in the translation of a truncated protein at the C-terminal end domain of the TGM 1 molecule. B.C1 monoclonal antibody failed to detect TGase 1 in the patient's skin sample, and TGase activity measured by monodansyl cadaverine-incorporation showed the reduced TGase activity at the distribution of TGase 1 in the epidermis. [ABSTRACT FROM AUTHOR]

A 76-year-old (male) developed pruritic, widespread papules and plaques in a symmetrical arrangement. The disease proved to be linear IgA dermatosis with circulating IgA type antibasement membrane autoantibodies binding to the epidermal side of the salt-split skin. [ABSTRACT FROM AUTHOR]

The X-linked dominant Conradi–Hünermann–Happle (CDPX2, MIM 302960) syndrome belongs to the rare, heterogeneous group of diseases called chondrodysplasia punctata. The disease has been connected recently with deficiency of 3β-hydroxysteroid-Δ8-Δ7-isomerase (also called emopamil-binding protein, EBP), catalysing an intermediate step in the conversion of lanosterol to cholesterol (1, 2). We report a case of CDPX2 with a new missense mutation (C→G 439) in exon 4, leading to a R147G aminoacid substitution in the EBP. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to the article regarding the side affect of the total skin electron beam irradiation therapy (TSEBT) which is the transient leukonychia.

The article discusses a study which examined the prevalence of cryofibrinogenaemia (CFE) in patients with dermatitis herpetiformis. Topics discussed include the formation of cryofibrinogen in cold plasma but not in serum, the development of acral purpuras in DH which may be partially attributed to CFE, and a reduction in cryofibrinogen level in dapsone-treated CFE patients without DH.

A letter to the editor is presented which discusses the case of a 24-year old woman who experience a rapid development of T-cell lymphoma in her tongue.

A letter to the editor is presented about the case of a 63-year-old woman who underwent methotrexate treatment of recurrent impetigo herpetiformis with hypothyroidism.

The term 'sclerosing diseases of the skin' comprises specific dermatological entities, which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present consensus provides an update to the 2017 European Dermatology Forum Guidelines, focusing on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, updated strategies for the first- and advanced-line therapy of sclerosing skin diseases are addressed in detail. Part 2 of this consensus provides clinicians with an overview of the diagnosis and treatment of scleromyxoedema and scleroedema (of Buschke)., (© 2024 The Authors. Journal of the European Academy of Dermatology and Venereology published by John Wiley & Sons Ltd on behalf of European Academy of Dermatology and Venereology.)

The term 'sclerosing diseases of the skin' comprises specific dermatological entities which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present guideline focuses on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, current strategies in the first- and advanced-line therapy of sclerosing skin diseases are addressed in detail. Part 2 of this guideline provides clinicians with an overview of the diagnosis and treatment of scleromyxedema, scleredema (of Buschke) and nephrogenic systemic sclerosis (nephrogenic fibrosing dermopathy)., (© 2017 European Academy of Dermatology and Venereology.)

The term 'sclerosing diseases of the skin' comprises specific dermatological entities, which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present guideline focuses on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, current strategies in the first- and advanced-line therapy of sclerosing skin diseases are addressed in detail. Part 1 of this guideline provides clinicians with an overview of the diagnosis and treatment of localized scleroderma (morphea), and systemic sclerosis including overlap syndromes of systemic sclerosis with diseases of the rheumatological spectrum., (© 2017 European Academy of Dermatology and Venereology.)