Complete remission of skin lesions in a patient with subcorneal pustular dermatosis (Sneddon-Wilkinson disease) treated with antimyeloma therapy: association with disappearance of M-protein.

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Complete remission of skin lesions in a patient with subcorneal pustular dermatosis (Sneddon-Wilkinson disease) treated with antimyeloma therapy: association with disappearance of M-protein.

Br J Dermatol. 2017 May;176(5):1341-1344

Authors: von dem Borne PA, Jonkman MF, van Doorn R

Abstract
Subcorneal pustular dermatosis (SPD), or Sneddon-Wilkinson disease, is a rare pustular skin disease that follows a chronic relapsing course. A well-known association exists between SPD and IgA monoclonal gammopathy of undetermined significance (MGUS), which exists in up to 40% of cases. SPD has also been observed in patients with IgA myeloma. In SPD, direct and indirect immunofluorescence studies do not reveal in vivo bound IgA to the epithelial cell surface, in contrast to IgA pemphigus, which has similar clinicopathological features. Here we describe the case of a male patient with SPD and a concurrent IgA MGUS who had been treated with dapsone for 20 years with frequent relapses. Following development of multiple myeloma, the patient was treated with intensive antimyeloma treatment consisting of high-dose melphalan with autologous stem cell transplantation. This resulted in a complete remission of the myeloma with disappearance of the M-protein. In addition, a sustained remission of SPD was achieved without further treatment. Twenty-eight months after melphalan therapy the M-protein reappeared in the serum, and 2 months later SPD reappeared with histopathologically proven skin lesions at predilection sites. Presence and absence of skin lesions was found to correlate with the presence and absence of the M-protein in the serum. This is the first report of antimyeloma therapy inducing a long-lasting remission in SPD. The findings in this patient strongly suggest a causal role for circulating IgA antibodies in the pathogenesis of SPD. Antimyeloma treatment should be considered in patients with IgA MGUS-associated SPD refractory to other therapies.

PMID: 27516004 [PubMed – indexed for MEDLINE]

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Topical Gentamicin for the Treatment of Genetic Skin Diseases.

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Topical Gentamicin for the Treatment of Genetic Skin Diseases.

J Invest Dermatol. 2018 Apr;138(4):731-734

Authors: Pasmooij AMG

Abstract
Clinical application of topical gentamicin is a worthwhile option to investigate further for Nagashima-type palmoplantar keratosis and other genetic skin diseases caused by nonsense mutations. It is especially interesting to study gentamicin 1B because it may be more efficacious than other gentamicin components. Topical gentamicin has an acceptable safety profile, although prospective tracking of antibiotic resistance is warranted.

PMID: 29579455 [PubMed – in process]

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Image Gallery: Paraneoplastic pemphigus and follicular dendritic cell sarcoma.

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Image Gallery: Paraneoplastic pemphigus and follicular dendritic cell sarcoma.

Br J Dermatol. 2018 Feb;178(2):e146

Authors: Jonkman MF, Pas HH

PMID: 29441554 [PubMed – in process]

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Comparing patch test results of methylchloroisothiazolinone/methylisothiazolinone tested with both TRUE Test® and 100?ppm using investigator-loaded chambers.

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Comparing patch test results of methylchloroisothiazolinone/methylisothiazolinone tested with both TRUE Test® and 100?ppm using investigator-loaded chambers.

Contact Dermatitis. 2018 Feb;78(2):159-161

Authors: Dittmar D, Schuttelaar ML

PMID: 29341185 [PubMed – in process]

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Laboratory diagnosis of pemphigus: direct immunofluorescence remains the gold standard.

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Laboratory diagnosis of pemphigus: direct immunofluorescence remains the gold standard.

Br J Dermatol. 2016 Jul;175(1):185-6

Authors: Giurdanella F, Diercks GF, Jonkman MF, Pas HH

PMID: 26798993 [PubMed – indexed for MEDLINE]

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Stevens Johnson syndrome/toxic epidermal necrolysis-overlap, induced by lacosamide.

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Stevens Johnson syndrome/toxic epidermal necrolysis-overlap, induced by lacosamide.

Eur J Dermatol. 2016 Apr 01;26(2):185-6

Authors: Kardaun SH, Vos BJ, Chandran NS

PMID: 27017922 [PubMed – indexed for MEDLINE]

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Two decades of p-phenylenediamine and toluene-2,5-diamine patch testing – focus on co-sensitizations in the European baseline series and cross-reactions with chemically related substances.

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Two decades of p-phenylenediamine and toluene-2,5-diamine patch testing – focus on co-sensitizations in the European baseline series and cross-reactions with chemically related substances.

Contact Dermatitis. 2017 Feb;76(2):81-88

Authors: Vogel TA, Heijnen RW, Coenraads PJ, Schuttelaar MA

Abstract
BACKGROUND: Cross-reactions and co-sensitizations are of great importance in understanding contact allergy and exposure sources.
OBJECTIVES: To investigate common cross-reactions and co-sensitizations in p-phenylenediamine (PPD)-sensitized and toluene-2,5-diamine (TDA)-sensitized individuals.
METHODS: From our patch test population, 8036 patients patch tested with the European baseline series were extracted. Readings had to be performed at least on day 3 according to ICDRG guidelines.
RESULTS: Two hundred and fifty-one patients were sensitized to PPD and/or TDA; 231 patients were sensitized to PPD, and 109 to TDA. Significant differences were observed regarding the strengths of patch test reactions to PPD and number of cross-reactions. For TDA, a difference was found between all reaction strengths, except between + and ++ strengths. PPD-sensitized individuals were more likely to be sensitized to carba mix, cobalt chloride, colophonium, p-tert-butyl phenolformaldehyde resin, paraben mix, and methylisothiazolinone. TDA-sensitized individuals were more often sensitized to carba mix.
CONCLUSIONS: Cross-reactivity was commonly found among individuals sensitized to PPD or TDA, and was strongly related to the strength of the patch test reaction. Regarding co-sensitizations, a frequently appearing or common exposure source could not be determined. However, modification of the allergen by, for example, the skin microbiota may have caused the formation of molecules that are, for the human immune system, indistinguishable from PPD.

PMID: 27555055 [PubMed – indexed for MEDLINE]

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Immunology and genetics of tumour necrosis factor in allergic contact dermatitis.

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Immunology and genetics of tumour necrosis factor in allergic contact dermatitis.

Contact Dermatitis. 2017 Mar 16;:

Authors: Dittmar D, Schuttelaar ML

Abstract
During the sensitization phase of allergic contact dermatitis, the proinflammatory cytokine tumour necrosis factor (TNF) plays an important role by promoting epidermal Langerhans cell migration to draining lymph nodes. It also plays a role during the elicitation phase. The TNF gene (TNF) is located within the major histocompatibility complex region. Many single-nucleotide variants exist in the promoter region of TNF, and these may either increase or decrease mRNA transcription and therefore lead to higher or lower levels of TNF. The most extensively studied single-nucleotide variant of TNF is a base pair substitution in the promoter region at location -308 relative to the transcription start site (rs1800629, TNF -308G>A), which is believed to increase transcription and lead to higher TNF levels. The role of TNF in allergic contact dermatitis and the functionality of TNF -308G>A are reviewed in this article. The association between genetic variants and disease can be studied in a case-control design. Only a few case-control studies investigating the association between TNF -308G>A and allergic contact dermatitis have been published, with contradictory results. These are reviewed critically, and suggestions for future case-control studies on this topic are given.

PMID: 28300283 [PubMed – as supplied by publisher]

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Acute localised exanthematous pustulosis: What are the diagnostic criteria?

Acute localised exanthematous pustulosis: What are the diagnostic criteria?

Australas J Dermatol. 2017 Feb;58(1):74

Authors: Kardaun SH

PMID: 28195325 [PubMed – in process]

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The Association between Hidradenitis Suppurativa and Crohn’s Disease: in Search of the Missing Pathogenic Link.

The Association between Hidradenitis Suppurativa and Crohn’s Disease: in Search of the Missing Pathogenic Link.

J Invest Dermatol. 2016 Sep;136(9):1747-8

Authors: van der Zee HH, Horvath B, Jemec GB, Prens EP

Abstract
Hidradenitis suppurativa is a chronic, autoinflammatory skin disease. Shalom et al. demonstrate in a large cross-sectional study an association between Crohn’s disease and hidradenitis suppurativa, but not with ulcerative colitis. This association supports the hypothesis that a similar pathogenic mechanism contributes to both diseases, providing new possibilities for functional studies and therapy development.

PMID: 27542293 [PubMed – in process]

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Hidradenitis suppurativa: A disease of infundibular epidermis, rather than pilosebaceous units?

Hidradenitis suppurativa: A disease of infundibular epidermis, rather than pilosebaceous units?

Br J Dermatol. 2016 Aug 20;

Authors: Janse IC, Blok JL, Diercks GF, Horváth B, Jonkman MF

Abstract
although the exact pathogenesis of hidradenitis suppurativa (HS) is unknown, it is established that it is primarily an infundibulitis.(1,2) We consider infundibulum as epidermis and not as hair follicle according to Ackerman, as the type of epithelial tissue of infundibular epidermis is indistinguishable from that of interfollicular epidermis.(1) This article is protected by copyright. All rights reserved.

PMID: 27542326 [PubMed – as supplied by publisher]

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Complement in bullous pemphigoid: results from a large observational study.

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Complement in bullous pemphigoid: results from a large observational study.

Br J Dermatol. 2016 Jun 25;

Authors: Romeijn TR, Jonkman MF, Knoppers C, Pas HH, Diercks GF

Abstract
Bullous pemphigoid (BP) is a subepidermal autoimmune blistering disease characterized by auto-antibodies directed against the hemidesmosomal proteins 180-kDa bullous pemphigoid antigen 2 (BP180, BPAG2) and/or 230-kDa bullous pemphigoid antigen 1 (BP230, BPAG1).(1) It is thought that complement activation plays an important role in the pathogenesis of BP.(1) However, the role of complement in the formation of subepidermal blisters has been disputed and complement independent mechanisms of blister formation have been described.(2-7) However, interpretation of these data are limited since almost all studies were based on experimental pemphigoid models of mice (2,3) and human keratinocytes. (4-6) Studies assessing the role of complement in a large population of BP patients are lacking. Using a large database comprising a period of 13 years (2002-2015) we had the unique opportunity to evaluate the relation of complement with various clinical and diagnostic parameters in a large cohort of BP patients. This article is protected by copyright. All rights reserved.

PMID: 27344014 [PubMed – as supplied by publisher]

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Contact allergy in Indonesian patients with foot eczema attributed to shoes.

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Contact allergy in Indonesian patients with foot eczema attributed to shoes.

J Eur Acad Dermatol Venereol. 2015 Aug;29(8):1582-9

Authors: Febriana SA, Soebono H, Coenraads PJ, Schuttelaar ML

Abstract
BACKGROUND: Shoe dermatitis is a form of contact dermatitis resulting from exposure to shoes. Allergens and types of shoes responsible may vary depending on manufacturing techniques, climatic conditions and indigenous traditions. This study focuses primarily on as yet unexplored shoe dermatitis cases in Indonesia.
OBJECTIVE: To determine the prevalence of shoe dermatitis in the Dermatology outpatient clinic, Sardjito University Hospital, Yogyakarta, Indonesia over a period of 3 years and to identify the responsible allergens.
METHODS: All patients meeting screening criteria for possible shoe contact dermatitis were patch tested with the European baseline series, shoe series and additional series based on earlier studies of Indonesian leather and shoe manufacturers; some were also patch tested with their own shoe materials and shoe extracts.
RESULTS: Sixty-four (7.1%) of 903 patients with foot skin disorders were diagnosed with shoe dermatitis. Twenty-five (52.1%) of 48 patch-tested patients showed positive reactions to one or more allergens related to footwear. Sixteen patients were patch tested with their own shoe materials; 11 showed positive reactions. The most frequent relevant sensitizers were rubber allergens followed by preservatives, shoe adhesives and leather materials.
CONCLUSION: Shoe dermatitis is common in Indonesia. Using three series of patch tests, we identified responsible allergens and patterns of sensitization in Indonesian shoe dermatitis patients.

PMID: 25640221 [PubMed – indexed for MEDLINE]

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Current practice in treatment approach for bullous pemphigoid: comparison between national surveys from the Netherlands and the UK.

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Current practice in treatment approach for bullous pemphigoid: comparison between national surveys from the Netherlands and the UK.

Clin Exp Dermatol. 2016 Mar 4;

Authors: Meijer JM, Jonkman MF, Wojnarowska F, Wiliams HC, Kirtschig G

Abstract
Treatment approaches for bullous pemphigoid (BP), the most common autoimmune skin blistering disease, are largely based on national and international guidelines. We conducted a national survey among dermatologists in the Netherlands to explore the current treatment of BP, and compared the results with those of a previously published survey from the UK. Almost all responders in the Netherlands (n = 175) used very potent topical corticosteroids, both as monotherapy and as adjunctive therapy. In contrast to UK dermatologists, the majority recommended whole-body application rather than local application to lesions. Systemic antibiotics were used by > 70% of responders. Half of the responders in the Netherlands considered systemic steroids the first-choice treatment, with the majority also using adjunctive therapy as a routine. Despite many similarities in treatment approach between the two countries, these surveys provide an important insight into the gap between actual and recommended practice at a country level in relation to the best external evidence.

PMID: 26940484 [PubMed – as supplied by publisher]

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Blistering disease: insight from the hemidesmosome and other components of the dermal-epidermal junction.

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Blistering disease: insight from the hemidesmosome and other components of the dermal-epidermal junction.

Cell Tissue Res. 2015 Jun;360(3):545-69

Authors: Turcan I, Jonkman MF

Abstract
The hemidesmosome is a specialized transmembrane complex that mediates the binding of epithelial cells to the underlying basement membrane. In the skin, this multiprotein structure can be regarded as the chief adhesion unit at the site of the dermal-epidermal junction. Focal adhesions are additional specialized attachment structures located between hemidesmosomes. The integrity of the skin relies on well-assembled and functional hemidesmosomes and focal adhesions (also known as integrin adhesomes). However, if these adhesion structures are impaired, e.g., as a result of circulating autoantibodies or inherited genetic mutations, the mechanical strength of the skin is compromised, leading to blistering and/or tissue inflammation. A particular clinical presentation emerges subject to the molecule that is targeted. None of these junctional complexes are simply compounds of adhesion molecules; they also play a significant role in signalling pathways involved in the differentiation and migration of epithelial cells such as during wound healing and in tumour invasion. We summarize current knowledge about hereditary and acquired blistering diseases emerging from pathologies of the hemidesmosome and its neighbouring proteins as components of the dermal-epidermal junction.

PMID: 25502077 [PubMed – indexed for MEDLINE]

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