Diletta Bonciani

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.

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.

Hailey-Hailey disease treated with methotrexate.

BACKGROUND Hailey-Hailey disease (HHD) is a chronic, recurrent blistering disorder characterized clinically by erosions occurring primarily in intertriginous regions and histologically by suprabasal acantholysis. MAIN OBSERVATIONS We report a long standing case of HHD initially unresponsive to cyclosporin, multiple topical and systemic steroids. Good response was achieved with methotrexate 7,5 mg weekly for 16 week, intramuscularly, and topical steroids as needed. CONCLUSION In conclusion, we suggest that methotrexate could be considered a therapeutic option for the treatment of HHD and in particular as a maintaining therapy to control the disease flares.

A study of fractional CO2 laser resurfacing: the best fluences through a clinical, histological, and ultrastructural evaluation

Background  Fractional resurfacing is a laser treatment modality to create numerous microscopic thermal injury zones of controlled width, depth, and density that are surrounded by a reservoir of spared epidermal and dermal tissue, allowing rapid repair of laser‐induced thermal injury.

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.

Why is Kikuchi-Fujimoto disease misleading?

Background  Kikuchi–Fujimoto disease (KFD) or histiocytic necrotizing lymphadenitis is a rare, benign, autoimmune condition characterized by lymphadenopathy, fever, and neutropenia. KFD has also been frequently reported in association with systemic lupus erythematosus (SLE).

Gluten-free diet in patients with dermatitis herpetiformis: not only a matter of skin.

I n their prospective study in a recent issue of the Archives, Qureshi et al provide convincing evidence that intake of nonlight beer increases the risk of developing psoriasis. Since there are several other factors known to negatively influence psoriasis, such as obesity and smoking, it seems reasonable to counsel patients on lifestyle modification, as proposed by Shelling and Kirshner in a “Practice Gaps” article in the same issue of the Archives. However, the effectiveness of such counseling is somewhat questionable because of personality traits rendering patients with psoriasis susceptible to addictive behavior concerning alcohol and food intake. In a comparative study of personality traits in patients with psoriasis, atopic dermatitis, and urticaria, our research team showed important differences in selfregulation, competence, and coping behavior. In contrast to patients with atopic dermatitis and urticaria, patients with psoriasis showed low scores for intrinsic positive affect and high scores for impulsive behavior. In these patients, alcohol may function as source of positive affect. Faced with negative life events or stressful situations, patients with psoriasis showed deficits in managing the situation appropriately and tended to avoid negative outcomes because of an overall lack of action-oriented behavior. Because the risk behavior in patients with psoriasis is so closely linked to personality structure, an early and specific preventive intervention seems desirable to enhance self-efficacy and self-motivation, reduce helplessness, and aid in the development of coping strategies to enable the patient with psoriasis to generate intrinsic positive affect. Such psychotherapeutically oriented motivational counseling should help patients with psoriasis to overcome state orientation, depressive mood states, and nonadaptive coping strategies.

Etanercept therapy in a hepatitis B virus (HBV)-positive psoriatic patient developing a monoclonal gammopathy of undetermined significance

Hepatitis B is a common viral infection that may be challenging because of the potential complications associated with immunosuppression in chronically infected individuals. Moreover, viral hepatitis B is often present in psoriatic patients. A particular focus on patient management, screening recommendations, and a prophylactic therapy are mandatory in these patients. As a general rule, the presence of chronic hepatitis B virus (HBV) infection is recognized as a major contraindication in the use of anti-tumor necrosis factor (TNF) agents. Reports in the literature concerning the safety of TNF-alpha inhibitors in the setting of chronic viral infections and, in particular, of HBV are limited. We report our experience with etanercept in the management of a patient affected by psoriasis vulgaris and concurrent chronic hepatitis B who developed, upon etanercept treatment, a monoclonal gammopathy of undetermined significance (MGUS).

Docetaxel-induced-like subacute cutaneous lupus erythematosus

A 57-year-old man was diagnosed as having a gastric adenocarcinoma, and underwent chemotherapy with 5-fluorouracil and cisplatin. Docetaxel was added as a second-line therapy 4 months later, because of metastases detected in the lung and liver. At the end of the first cycle of docetaxel, the patient developed asymptomatic, violaceous, infiltrated, partially scaling lesions on the dorsal surface of both hands (Fig. 1a) followed by the occurrence of multiple annular, erythematous plaques on his trunk (Fig. 1b). Skin biopsies were taken from lesions on the trunk and on the left hand, and histological examination identified interface dermatitis in both (Fig. 2a,b). Laboratory investigations revealed mild anaemia together with neutropenia and lymphopenia. Thrombocytopenia was absent. Erythrocyte sedimentation rate was 93 mm/h (normal range 0–20 mm/h), while C-reactive protein, rheumatoid factor and complement factors were normal. Serological analysis was positive for Ro/SS-A [7.4 (antibody index; normal range <1 AI)] La/ SS-B (3.8 AI) and antinuclear (1 : 320) antibodies. Direct immunofluorescence (DIF) of the biopsy from the hand lesion revealed granular deposits of IgG, IgM and C3 at the dermoepidermal junction (Fig. 2c). In agreement with the oncologist, docetaxel was discontinued and replaced by capecitabine. The cutaneous lesions improved in the following weeks but, to obtain complete remission, hydroxychloroquine (200 mg/day) was added. After 3 weeks, the skin lesions had resolved and there was no recurrence in the subsequent 9-month follow-up period (Fig. 1c,d). Given the clinical and histopathological data, the trunk lesions were diagnosed as subacute cutaneous lupus erythematosus (SCLE) induced by docetaxel. This was based on the temporal link between drug exposure and onset of skin lesions, which resolved after drug discontinuation. Serological results were consistent with a drug-induced SCLE (DI-SCLE). Moreover, docetaxel has been recently suggested to induce SCLE in immunogenetically predisposed patients by stabilizing microtubules and affecting Ro/SS-A antigen (Ro52) expression. By contrast, the diagnosis of the hand lesions is more questionable. Their appearance resembled dermatomyositis, especially with regard to the localization and the violaceous colour, but no papules or Gottron sign were seen, and no joint or muscle involvement was found. Both electromyography and serology tests were negative. Moreover, the trunk lesions were not consistent with dermatomyositis. Another possible differential diagnosis could be palmoplantar erythrodysaesthesia (PPE) syndrome, which has been reported as a common side effect of docetaxel. However, we excluded PPE because the hand lesions did not involve the palms or affect the feet, and were asymptomatic. Moreover, although the histopathological examination was compatible with PPE, DIF in PPE is usually negative, whereas in our case we found several immunological deposits. Therefore, our final diagnosis for the hand lesions was of an atypical manifestation of SCLE induced by docetaxel, based on (i) the histopathological examina-

A case of rupioid psoriasis exacerbated by systemic glucocorticosteroids

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