Veronica Bonciolini

Celiac disease and dermatologic manifestations: many skin clue to unfold gluten-sensitive enteropathy.

Cutaneous manifestations of intestinal diseases are increasingly reported both in the adult and in the children, and this association cannot longer be considered a simple random. Besides the well-known association between celiac disease (CD) and dermatitis herpetiformis (DH), considered as the cutaneous manifestation of gluten-dependent enteropathy, is more frequently reported also the association with other mucocutaneous diseases. Among these there are both autoimmune, allergic, and inflammatory diseases, but also a more heterogeneous group called miscellaneous. The knowledge about pathogenic, epidemiological, clinical, and diagnostic aspects of CD is increasing in recent years as well as those about DH, but some aspects still remain to be defined, in particular the possible pathogenetic mechanisms involved in the association between both CD and DH and CD and other immunological skin diseases. The aim of this paper is to describe the skin diseases frequently associated with CD, distinguishing them from those which have a relationship probably just coincidental.

The last word on the so-called ‘Rowell’s syndrome’?

To date, 71 patients having the so-called ‘Rowell’s syndrome’ (RS) have been reported in the literature. However, most of them did not show all the clinical and serological features first described by Rowell and co-workers in 1963. Moreover, since then, subacute cutaneous lupus erythematosus (SCLE) has been identified and the diagnostic criteria as well as the clinical features of erythema multiforme (EM) defined. Accordingly several authors have questioned the existence of RS over the past years. In the present paper, the main clinical, histopathological and immunopathological features of both SCLE and EM are described and all of the cases of RS reported in the literature are also reviewed in depth. A real association between discoid LE and EM was present only in a minority of cases and could be considered a mere coincidence. As for other associations, e.g. those between CLE and lichen planus or psoriasis, the coexistence of CLE and EM does not justify the framing of a separate syndrome as suggested by Rowell et al.

Oral Curcumin (Meriva) Is Effective as an Adjuvant Treatment and Is Able to Reduce IL-22 Serum Levels in Patients with Psoriasis Vulgaris

Curcumin is a complementary therapy that may be helpful for the treatment of psoriasis due to its anti-inflammatory, antiangiogenic, antioxidant, and antiproliferative effects. In the present study we performed a randomized, double-blind, placebo-controlled clinical trial to assess the effectiveness of a bioavailable oral curcumin in the treatment of psoriasis. Sixty-three patients with mild-to-moderate psoriasis vulgaris (PASI < 10) were randomly divided into two groups treated with topical steroids and Meriva, a commercially available lecithin based delivery system of curcumin, at 2 g per day (arm 1), or with topical steroids alone (arm 2), both for 12 weeks. At the beginning (T0) and at the end of the therapy (T12), clinical assessment and immunoenzymatic analysis of the serum levels of IL-17 and IL-22 were performed. At T12, both groups achieved a significant reduction of PASI values that, however, was higher in patients treated with both topical steroids and oral curcumin than in patients treated only with topical steroids. Moreover, IL-22 serum levels were significantly reduced in patients treated with oral curcumin. In conclusion, curcumin was demonstrated to be effective as an adjuvant therapy for the treatment of psoriasis vulgaris and to significantly reduce serum levels of IL-22.

Dermatitis Herpetiformis: From the Genetics to the Development of Skin Lesions

Dermatitis herpetiformis (DH) is a rare autoimmune disease linked to gluten sensitivity with a chronic-relapsing course. It is currently considered to be the specific cutaneous manifestation of celiac disease (CD). Both conditions are mediated by the IgA class of autoantibodies, and the diagnosis of DH is dependent on the detection of granular deposits of IgA in the skin. There is an underlying genetic predisposition to the development of DH, but environmental factors are also important. This paper describes these different factors and discusses the known mechanism that lead to the development of skin lesions.

Newly Described Clinical and Immunopathological Feature of Dermatitis Herpetiformis

Dermatitis herpetiformis (DH) is an inflammatory cutaneous disease with typical histopathological and immunopathological findings clinically characterized by intensely pruritic polymorphic lesions with a chronic-relapsing course. In addition to classic clinical manifestations of DH, atypical variants are more and more frequently reported and histological and immunological are added to them, whereas the impact on quality of life of patients with DH is increasingly important to a certain diagnosis. The aim of this paper is to describe all the possible clinical, histological, and immunological variants of DH in order to facilitate the diagnosis of a rare disease and, therefore, little known.

Cutaneous Manifestations of Non-Celiac Gluten Sensitivity: Clinical Histological and Immunopathological Features

Background: The dermatological manifestations associated with intestinal diseases are becoming more frequent, especially now when new clinical entities, such as Non-Celiac Gluten Sensitivity (NCGS), are identified. The existence of this new entity is still debated. However, many patients with diagnosed NCGS that present intestinal manifestations have skin lesions that need appropriate characterization. Methods: We involved 17 patients affected by NCGS with non-specific cutaneous manifestations who got much better after a gluten free diet. For a histopathological and immunopathological evaluation, two skin samples from each patient and their clinical data were collected. Results: The median age of the 17 enrolled patients affected by NCGS was 36 years and 76% of them were females. On the extensor surfaces of upper and lower limbs in particular, they all presented very itchy dermatological manifestations morphologically similar to eczema, psoriasis or dermatitis herpetiformis. This similarity was also confirmed histologically, but the immunopathological analysis showed the prevalence of deposits of C3 along the dermo-epidermal junction with a microgranular/granular pattern (82%). Conclusions: The exact characterization of new clinical entities such as Cutaneous Gluten Sensitivity and NCGS is an important objective both for diagnostic and therapeutic purposes, since these are patients who actually benefit from a GFD (Gluten Free Diet) and who do not adopt it only for fashion.

Skin manifestations of celiac disease: not always dermatitis herpetiformis

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Mucosal psoriasis: a new insight toward a systemic inflammatory disease

Psoriasis is a chronic disease of the immune system affecting the skin, usually in the form of thick, red, scaly, and inflammatory patches. It affects both sexes and occurs in about 1–3% of the general population. Mucosae are also affected in a reasonable number of patients. In the past, the presence of specific oral manifestations in psoriasis has been the subject of controversy. In recent years, the literature has provided more evidence for defining psoriasis as a systemic disease. For many years mucosal psoriasis lesions were not considered part of the disease; at the moment, more studies are necessary to define the degree of mucosal involvement in psoriasis. Notwithstanding, the cause of this disorder remains largely unknown. The prevalence of psoriasis lesions in the mucosae is highly variable, being between 2 and 30%. Data are heterogeneous because often lesions are asymptomatic and patients do not report them. There is another reason for underestimating mucosal involvement in psoriasis, which is linked to the fact that a histological distinction between psoriasis and some common inflammatory entities of the oral mucosa cannot be made, or at least not in the early phase. Psoriasis affects several mucosae; mainly oral but also genital, ocular, and gastrointestinal (Table 1). The most involved areas are the tongue, cheeks, and gums. In general, mucosal lesions are associated with skin lesions, but some patients only present mucosal involvement at the beginning. In general, oral lesions in psoriasis can be divided into two major categories. The first one includes mucosal psoriasis lesions with a standard histopathological pattern of psoriasis, such as edema and acute inflammatory infiltrate in the epithelium and mixed in the lamina propria with neutrophils and lymphocytes, organized neutrophils ‘‘microabscesses’’ (Fig. 1). Some patients

Rowell syndrome: does it exist?

We read with interest the clinical case reported by Champagne et al. entitled ‘Severe Rowell syndrome associated with oral terbinafine’, recently published in Clinical and Experimental Dermatology. In our opinion, the described case seems to be a typical case of drug-induced subacute cutaneous lupus erythematosus (SCLE), caused by the terbinafine the patient was taking, rather than a case of Rowell syndrome (RS). In fact, at least four of the diagnostic criteria that discriminate drug-induced SCLE from idiopathic SCLE, reported by Marzano et al. in 2011, were present, including the widespread presentation and the occurrence of bullous, targetoid and vasculitic lesions. Other features, such as the slow healing of the lesions upon discontinuation of the drug and their recurrence after terbinafine re-introduction, strongly support our hypothesis. We consider that the proposed diagnosis of RS in this case is incorrect. Moreover, we believe that the diagnostic criteria for RS, previously published by Zeitouni et al., are totally inadequate for RS, which could be considered a true ‘ghost syndrome’. We published a review of all the putative RS cases reported in the literature up to 2011, and pointed out in that review that two different conditions have been reported under the classification of RS, namely: (i) patients with an association of annular/polycyclic SCLE and erythema multiforme (EM) like lesions (as in the case reported by Champagne et al.) and (ii) in a minority of cases, a mere coincidental association between discoid LE and EM. As for other associations, for example those between cutaneous (C)LE and lichen planus or psoriasis, we consider that the coexistence of CLE and EM does not justify the framing of a separate syndrome as originally suggested by Rowell et al.

Granular IgA Deposits in the Skin of Patients with Coeliac Disease: Is it Always Dermatitis Herpetiformis?

Coeliac disease is an immune-mediated enteropathy driven by gluten, which can be associated with dermatitis herpetiformis. The presence of granular IgA deposits, detected by direct immunofluorescence, is the hallmark of dermatitis herpetiformis; nevertheless, IgA deposits have also been demonstrated in healthy skin of patients with coeliac disease. The main objective of this study was to investigate whether IgA deposits could be found in the skin of patients with coeliac disease who have non-dermatitis herpetiformis inflammatory skin diseases. Direct immunofluorescence was performed on perilesional skin biopsies of 6 patients with coeliac disease with non-dermatitis herpetiformis inflammatory skin diseases and, as control, on 12 non-coeliac patients with inflammatory skin diseases. IgA deposits were found in all of the patients with coeliac disease, but were absent in the control group. In conclusion, IgA deposits may be considered an immunopathological marker for coeliac disease; therefore, patients with coeliac disease showing skin manifestations with positive direct immunofluorescence should be investigated carefully in order to make a differential diagnosis between dermatitis herpetiformis and other non-dermatitis herpetiformis inflammatory skin diseases.