Post-Irradiation Morphea of the Breast in a Patient with Subacute Cutaneous Lupus Erythematosus: Case Report and a Literature Review.

The appearance of morphea after radiotherapy, especially in the context of breast cancer, is a rare but known phenomenon. The incidence of post-irradiation morphea (PIM) of the breast is approximately one in every 500 patients, a higher rate than morphea of any other etiology, which is three per 100,000 per year. PIM usually appears less than 1 year after irradiation (range 1 month to 32 years). The histological pattern of PIM is different from the one in post-irradiation fibrosis, which is a common side effect of radiotherapy and usually appears during the first 3 months after irradiation. Several theories have been proposed to explain the pathogenesis of PIM, probably caused by a disturbance of the cytokine pattern. The development of PIM in patients with autoimmune diseases has been described in the literature. To our knowledge, we report the first case of PIM in a patient with subacute cutaneous lupus erythematosus. We should therefore pay attention when looking at patients with PIM to search for an underlying autoimmune disease.

Primary Cutaneous Cryptococcosis due to Cryptococcus neoformans in an Immunocompetent Host Treated with Itraconazole and Drainage: Case Report and Review of the Literature.

Cryptococcus neoformans is an opportunistic germ, usually causing infections in immunocompromised patients. The main sources of infection with C. neoformans are excrement from birds, decomposing wood, fruit, and vegetables. Primary cutaneous cryptococcosis (PCC) is a clinical entity, differing from secondary cutaneous cryptococcosis and systematic infection. We report the case of an immunocompetent 60-year-old woman with PCC due to C. neoformans in her right thumb. She reported an accidental injury caused by a rose thorn while she was gardening. Clinical examination showed the presence of an erythematous ulcerated nodule with elevated borders, suppuration, and central necrosis. Skin histology examination showed cutaneous and subcutaneous fibrinoid necrosis with bleeding, abscess, neutrophil-rich cellular infiltration, and the presence of PAS-, Grocott- and mucin-positive spores. The mycological culture showed milky and creamy colonies of C. neoformans after 3 days. As there was no previous history of pulmonary cryptococcosis, we diagnosed PPC. We treated the patient surgically with accurate debridement of nonvital tissues in the right thumb. In addition, we started itraconazole treatment 100 mg twice daily for 6 months, which led to rapid clinical improvement without relapse. PCC is a rare infection that can present with quite unspecific clinical pictures including acneiform lesions, purpura, vesicles, nodules, abscesses, ulcers, granulomas, pustules, draining sinuses, and cellulitis. Prolonged systemic antifungal therapy is necessary in order to get a healing result without relapse. We summarize all the cases of PCC in immunocompetent patients published so far in the literature.

Topical Treatment of Psoriasis Vulgaris: The Swiss Treatment Pathway.

Topical treatment is crucial for the successful management of plaque psoriasis. Topicals are used either as a stand-alone therapy for mild psoriasis or else in combination with UV or systemic treatment for moderate-to-severe disease. For the choice of a suitable topical treatment, the formulation matters and not just the active substances. This expert opinion paper was developed via a non-structured consensus process by Swiss dermatologists in hospitals and private practices to illustrate the current treatment options to general practitioners and dermatologists in Switzerland. Defining treatment goals together with the patient is crucial and increases treatment adherence. Patients' personal preferences and pre-existing experiences should be considered and their satisfaction with treatment and outcome regularly assessed. During the induction phase of "classical" mild-to-moderate psoriasis, the fixed combination of topical calcipotriol (Cal) 50 µg/g and betamethasone dipropionate (BD) 0.5 mg/g once daily is frequently used for 4-8 weeks. During the maintenance phase, a twice weekly (proactive) management has proved to reduce the risk of relapse. Of the fixed combinations, Cal/BD aerosol foam is the most effective formulation. However, the individual choice of formulation should be based on a patient's preference and the location of the psoriatic plaques. Tailored recommendations are given for the topical management of specific areas (scalp, facial, intertriginous/genital, or palmoplantar lesions), certain symptoms (hyperkeratotic or hyperinflammatory forms) as well as during pregnancy or a period of breastfeeding. As concomitant basic therapy, several emollients are recommended. If topical treatment alone does not appear to be sufficient, the regimen should be escalated according to the Swiss S1-guideline for the systemic treatment of psoriasis.

Trichophyton violaceum: An Emerging Pathogen in Southern Switzerland.

BACKGROUND: Trichophyton violaceumis an anthropophilic dermatophyte, endemic in Africa and recently an emerging pathogen in Europe. OBJECTIVE: To perform a retrospective analysis of dermatophytoses due to T. violaceum between 2007 and 2018, in order to evaluate epidemiological trends in Southern Switzerland (Ticino). METHODS: We reviewed all medical records of patients affected by dermatophytosis due to T. violaceum between January 2007 and December 2018 in Bellinzona (Ticino, Switzerland). RESULTS: Dermatophytoses due to T. violaceumwas diagnosed in 44 patients, 33 of which were in the last 4 years. The most affected sex was female (25/44) and the most frequent diagnosis was tinea capitis (30/44). The majority of tinea capitis patients were children younger than 13 years of age (27/30). The main source of contagion were people from endemic areas, especially from Eritrea. CONCLUSION: In Southern Switzerland T. violaceumhas been rarely diagnosed before 2014. Its increased occurrence seems linked to increased migratory flows from Eritrea. Since it is responsible mainly for tinea capitis, with most of the time no clearly defined alopecia patches, it is important to consider it as a possible diagnosis when facing scalp scaling. Tinea capitis due to T. violaceum is a benign affection, but if left untreated it can spread epidemically, especially among children in schools and kindergartens. Mycological examination is then required, not only for a correct diagnosis and epidemiological data, but also for planning the appropriate treatment.

Vibrio parahaemolyticus Induced Cellulitis and Septic Shock after a Sea Beach Holiday in a Patient with Leg Ulcers.

Infections with Vibrio parahaemolyticus, a gram-negative bacterium found in salt water, are mainly gastrointestinal or cutaneous. The development of sepsis is not uncommon. We report the case of an 85-year-old patient who developed lower limb cellulitis caused by V. parahaemolyticus, originating from leg ulcers and complicated by septicaemia and septic shock, after a sea beach holiday. We discuss the epidemiology, pathogenesis, clinical manifestations and treatment of V. parahaemolyticus infections.

Emergency Consultations in Dermatology in a Secondary Referral Hospital in Southern Switzerland: A Prospective Cross-Sectional Analysis.

BACKGROUND/AIMS: The spectrum of dermatological emergencies is broad. Only a few studies have assessed the profile of dermatological conditions resulting in an emergency visit in a referral hospital. We sought to assess the conditions prompting an urgent dermatological visit and to compare the diagnoses with those made during the regular scheduled encounters. METHODS: We performed a cross-sectional study of all patients with a cutaneous problem attending our emergency consultation during a 7-month period. The study variables were gender, age, duration of symptoms, diagnosis, need for hospitalization and/or follow-up. We further evaluated patients attending scheduled visits to compare the demographic characteristics and diagnoses between the two groups. RESULTS: Six hundred fifty-two consecutive patients with an urgent dermatological consultation were included. Three hundred sixty (55.2%) were women and 292 (44.8%) were men. Infectious diseases (32.8%) as well as various forms of eczema (24.8%) constituted the most frequent causes for an emergency visit. Approximately 40% of emergency visits took place more than 1 week after the development of the cutaneous manifestations. The most frequent disorders seen in the 1,738 control patients included benign melanocytic and nonmelanocytic tumors (27.2%) and malignant skin lesions (11.5%). CONCLUSIONS: Our study indicates that the dermatological diagnoses in the emergency visits significantly differ from those of the routinely scheduled appointments. In a significant portion of patients, the use of an emergency consultation was not justified. This study provides support to the idea that a specific training is required to manage dermatological emergencies and that efforts should be made to reduce unjustified emergency visit use.

Current Perspectives on Erythema Multiforme.

Recognition and timely adequate treatment of erythema multiforme remain a major challenge. In this review, current diagnostic guidelines, potential pitfalls, and modern/novel treatment options are summarized with the aim to help clinicians with diagnostic and therapeutic decision-making. The diagnosis of erythema multiforme, that has an acute, self-limiting course, is based on its typical clinical picture of targetoid erythematous lesions with predominant acral localization as well as histological findings. Clinically, erythema multiforme can be differentiated into isolated cutaneous and combined mucocutaneous forms. Atypical erythema multiforme manifestations include lichenoid or granulomatous lesions as well as lesional infiltrates of T cell lymphoma and histiocytes. Herpes simplex virus infection being the most common cause, other infectious agents like-especially in children-Mycoplasma pneumoniae, hepatitis C virus, Coxsackie virus, and Epstein Barr virus may also trigger erythema multiforme. The second most frequently identified cause of erythema multiforme is drugs. In different studies, e.g., allopurinol, phenobarbital, phenytoin, valproic acid, antibacterial sulfonamides, penicillins, erythromycin, nitrofurantoin, tetracyclines, chlormezanone, acetylsalicylic acid, statins, as well as different TNF-α inhibitors such as adalimumab, infliximab, and etanercept were reported as possible implicated drugs. Recently, cases of erythema multiforme associated with vaccination, immunotherapy for melanoma, and even with topical drugs like imiquimod have been described. In patients with recurrent herpes simplex virus-associated erythema multiforme, the topical prophylactic treatment with acyclovir does not seem to prevent further episodes of erythema multiforme. In case of resistance to one virostatic drug, the switch to an alternative drug, and in patients non-responsive to virostatic agents, the use of dapsone as well as new treatment options, e.g., JAK-inhibitors or apremilast, might be considered.

Cutaneous Granulomatosis: a Comprehensive Review.

Cutaneous granulomatosis is a heterogeneous group of diseases, characterized by a skin inflammatory reaction triggered by a wide variety of stimuli, including infections, foreign bodies, malignancy, metabolites, and chemicals. From a pathogenic point of view, they are divided into non-infectious and infectious granulomas. Pathophysiological mechanisms are still poorly understood. Non-infectious granulomatous skin diseases include granuloma annulare, necrobiosis lipoidica, rheumatic nodules, foreign body granulomas, cutaneous sarcoidosis, and interstitial granulomatous dermatitis. Necrobiosis lipoidica is more frequent in diabetic patients. Infectious granulomas of the skin are caused by mycobacteria, in particular Mycobacterium tuberculosis or atypical mycobacteria; parasites, such as Leishmania; or fungi. Pathogenic mechanisms of M. tuberculosis-related granuloma are discussed. From a clinical point of view, it is useful to divide cutaneous granulomatosis into localized and more disseminated forms, although this distinction can be sometimes artificial. Three types of localized granulomatous lesions can be distinguished: palisaded granulomas (granuloma annulare, necrobiosis lipoidica, and rheumatoid nodules), foreign body granulomas, and infectious granulomas, which are generally associated with localized infections. Disseminated cutaneous granulomas can be divided into infectious, in particular tuberculosis, and non-infectious forms, among which sarcoidosis and interstitial granulomatous dermatitis. From a histological point of view, the common denominator is the presence of a granulomatous inflammatory infiltrate in the dermis and/or hypodermis; this infiltrate is mainly composed of macrophages grouped into nodules having a nodular, palisaded or interstitial architecture. Finally, we propose which diagnostic procedure should be performed when facing a patient with a suspected cutaneous granulomatosis.

Current Perspectives on Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are considered a delayed-type hypersensitivity reaction to drugs. They represent true medical emergencies and an early recognition and appropriate management is decisive for the survival. SJS/TEN manifest with an "influenza-like" prodromal phase (malaise, fever), followed by painful cutaneous and mucous membrane (ocular, oral, and genital) lesions, and other systemic symptoms. The difference between SJS, SJS/TEN overlap, and TEN is defined by the degree of skin detachment: SJS is defined as skin involvement of < 10%, TEN is defined as skin involvement of > 30%, and SJS/TEN overlap as 10-30% skin involvement. The diagnosis of different degrees of epidermal necrolysis is based on the clinical assessment in conjunction with the corresponding histopathology. The mortality rates for SJS and TEN have decreased in the last decades. Today, the severity-of-illness score for toxic epidermal necrolysis (SCORTEN) is available for SJS/TEN severity assessment. Drugs with a high risk of causing SJS/TEN are anti-infective sulfonamides, anti-epileptic drugs, non-steroidal anti-inflammatory drugs of the oxicam type, allopurinol, nevirapine, and chlormezanone. Besides conventional drugs, herbal remedies and new biologicals should be considered as causative agents. The increased risk of hypersensitivity reactions to certain drugs may be linked to specific HLA antigens. Our understanding of the pathogenesis of SJS/TEN has improved: drug-specific T cell-mediated cytotoxicity, genetic linkage with HLA- and non-HLA-genes, TCR restriction, and cytotoxicity mechanisms were clarified. However, many factors contributing to epidermal necrolysis still have to be identified, especially in virus-induced and autoimmune forms of epidermal necrolysis not related to drugs. In SJS/TEN, the most common complications are ocular, cutaneous, or renal. Nasopharyngeal, esophageal, and genital mucosal involvement with blisters, erosions as well as secondary development of strictures also play a role. However, in the acute phase, septicemia is a leading cause of morbidity and fatality. Pulmonary and hepatic involvement is frequent. The acute management of SJS/TEN requires a multidisciplinary approach. Immediate withdrawal of potentially causative drugs is mandatory. Prompt referral to an appropriate medical center for specific supportive treatment is of utmost importance. The most frequently used treatments for SJS/TEN are systemic corticosteroids, immunoglobulins, and cyclosporine A.

Cutaneous Manifestations of Dermatomyositis: a Comprehensive Review.

Dermatomyositis (DM) is an idiopathic inflammatory myopathy characterized by the presence of skin lesions and inflammation of skeletal muscles; however, this feature may be absent in amyopathic DM. DM is a rare disease, occurring at any age, and has two peaks of incidence: one in childhood between 5 and 15 years of age and one in adulthood between 40 and 60 years, with a female preponderance. DM has been associated with malignancy; therefore, every newly diagnosed patient should undergo screening investigations, but evidence-based guidelines on their extension are lacking. The etiopathogenesis is still unclear, but a range of factors such as genetic predisposition, environment triggers, and immune- and non-immune-mediated mechanisms play a role in the development of the disorder. Some dermatological findings and new discoveries on immunological mechanisms in DM, including the association of certain autoantibodies with cancer, can be helpful in clinical practice. Therapy should consider both skin and muscle or internal organ involvement. Photoprotection, including the use of antimalarial drugs, plays an important role in the therapeutic approach of skin involvement. Treatment of muscle and internal organ involvement is based on systemic corticosteroids, alone or in association with disease-modifying antirheumatic drugs. Intravenous immunoglobulins have been demonstrated to be effective as second-line therapy. Rituximab is an emerging treatment for difficult-to-treat DM, both in adults and children. In this review article, we discuss DM subforms, focusing on their dermatological manifestations that can be classified as pathognomonic, characteristic, compatible, less common, rare, recently-described, and non-specific skin lesions.

Evaluation of the National Skin Cancer Campaign: a Swiss experience of Euromelanoma.

AIMS OF THE STUDY: Skin cancer is a burden to healthcare and patients worldwide. The incidence of skin cancer has been rising during recent decades and this trend is expected to continue in the future. Numerous risk factors have been identified and prevention strategies developed. The Euromelanoma campaign is a pan-European skin cancer prevention programme, targeted to both primary and secondary prevention of malignant melanoma. The current study aimed to evaluate the results of the Swiss skin cancer screening day 2016. METHODS: A questionnaire was used to obtain data on characteristics and suspected skin cancers of all participants. Follow-up of patients with suspicious lesions was performed 3 to 6 months later. RESULTS: During the campaign, 2795 people were screened. Of the screened individuals, 157 participants (58% female, 42% male; mean age 58.8 years) underwent further evaluations; 6 cutaneous malignant melanomas, 21 basal cell carcinomas and 2 squamous cell carcinomas were detected. Detection rates were 0.21% for cutaneous melanoma, 0.75% for basal cell carcinoma and 0.07% for squamous cell carcinoma. CONCLUSIONS: Our study provides an up-to-date evaluation of the Swiss Euromelanoma campaign 2016. The results are mostly in line with data from other European studies. Considering the morbidity, mortality and financial and social impact of skin cancer, the capacity to raise awareness of risk factors, skin cancer prevention methods and educating high-risk and at-risk individuals, we may assume that a National Screening Day has a crucial impact on the public health system.

Skin Manifestations Associated with Autoimmune Liver Diseases: a Systematic Review.

Autoimmune liver diseases, which include mainly autoimmune hepatitis, primary biliary cholangitis, primary sclerosing cholangitis, and the variant syndromes, are often associated with extrahepatic autoimmune diseases. However, the association with cutaneous diseases is less well described. In the present article, we provide a systematic literature review on skin manifestations linked to each of these four autoimmune liver diseases, excluding skin manifestations of systemic diseases. The association of autoimmune hepatitis with vitiligo is well known, with a particular striking association with type 2 autoimmune hepatitis, a condition occurring almost entirely in children and adolescents, much rarer and more aggressive than type 1 autoimmune hepatitis; probable associations are also identified with alopecia areata, psoriasis, and pyoderma gangrenosum. Primary biliary cholangitis is not linked to lichen planus as previously assumed, but to vitiligo, psoriasis and the very rare amicrobial pustulosis of the folds. The proposed diagnostic criteria for this latter condition include the presence of anti-mitochondrial autoantibodies, the serological hallmark of primary biliary cholangitis. The very strong association of primary sclerosing cholangitis with inflammatory bowel diseases hampers the search for an association with skin diseases, since inflammatory bowel diseases have a strong association with various dermatological condition, including neutrophilic dermatoses and erythema nodosum. Nevertheless, a probable association of primary sclerosing cholangitis with psoriasis is identified in this review. Variant syndromes, also called overlap syndromes, are likely associated with vitiligo as well, which is not surprising, since autoimmune hepatitis is a feature of these conditions and they may share regions of the MHC.

Cutaneous and Mucosal Manifestations of Sjögren's Syndrome.

Sjögren's syndrome is currently considered an "autoimmune epithelitis," as exocrine glands, especially salivary and lacrimal, are progressively destructed by an immune-mediated process associated with specific serum autoantibodies and local lymphocyte infiltrate. Xerostomia remains a key complain in patients with Sjögren's syndrome but should be evaluated also for other causes such as xerogenic medications, followed by radiation and chemotherapy for head and neck cancers, hormone disorders, infections, or other connective tissue diseases. Further, xerophtalmia (also known as dry eye) frequently associated with keratoconjunctivitis sicca cumulatively affects approximately 10-30% of the general population with increasing incidence with age and is more frequently secondary to non-autoimmune diseases. On the other hand, numerous patients with Sjögren's syndrome manifest signs of systemic dryness involving the nose, the trachea, the vagina, and the skin, suggesting that other glands are also affected beyond the exocrine epithelia. Skin involvement in Sjögren's syndrome is relatively common, and various manifestations may be present, in particular xeroderma, eyelid dermatitis, annular erythema, and cutaneous vasculitis. Additional skin non-vasculitic manifestations include livedo reticularis which may occur in the absence of vasculitis, and localized nodular cutaneous amyloidosis possibly representing lymphoproliferative diseases related to Sjögren's syndrome. The treatment of skin and mucosal manifestations in Sjögren's syndrome is similar regardless of the cause, starting from patient education to avoid alcohol and tobacco smoking and to pursue dental hygiene. In conclusion, a strict collaboration between the dermatologist and the rheumatologist is essential in the adequate management of Sjögren's syndrome skin and mucosal manifestations.

Swiss Practice Recommendations for the Management of Hidradenitis Suppurativa/Acne Inversa.

Hidradenitis suppurativa (HS) is a painful, inflammatory, debilitating skin disease with a chronic intermittent course. The central pathogenetic event seems to be the occlusion of the hair follicle. HS has a 1-year prevalence of about 1%. It typically presents after puberty with painful, deep-seated, inflamed lesions in the apocrine gland-bearing areas of the body: most commonly the axillae, inguinal, and anogenital regions. HS has a high negative impact on patients' quality of life even in patients with only limited disease burden, and the diagnosis of HS is often made with a long diagnostic delay. In this practical short version we present diagnostic and therapeutic recommendations which are based on a systematic literature search as well as an informal expert consensus of Swiss dermatologists and dermatosurgeons.

Primary Biliary Cholangitis Associated with Skin Disorders: A Case Report and Review of the Literature.

Primary biliary cholangitis (PBC) is a rare autoimmune cholestatic liver disease. It is often associated with extrahepatic autoimmune diseases. Skin disorders are sporadically reported in association with PBC. We report an unusual case of PBC associated with acquired reactive perforating dermatosis (ARPD) and present a review of the literature on skin disorders associated with PBC. Our patient presented to the dermatology department with generalized pruritus associated with nodular perforating skin lesions on the trunk, and cholestatic liver disease of unknown origin. After having established both diagnosis of ARPD and PBC, she was managed in an interdisciplinary manner, and both her skin and liver conditions improved gradually. Only one similar case is reported in the literature, in that case, the liver disease was not treated. By reviewing the literature, we found that lichen planus, vitiligo, and psoriasis are the most frequent skin disorders associated with PBC. However, there is only limited data about specific skin disorders associated with PBC. This case report of a patient with PBC associated with ARPD underlines the importance of interdisciplinary management of patients with rare liver diseases combined with rare skin disorders. The present review of the literature shows that probably, immune-mediated skin conditions are not more frequent in PBC patients than in the general population. However, the available data are scant; there is a need for high-quality data on skin conditions associated with PBC.

Successful Treatment of Relapsing Bowen's Disease with Ingenol Mebutate: The Use of Dermoscopy to Monitor the Therapeutic Response.

Ingenol mebutate (IM) has recently been approved for the topical treatment of actinic keratoses. It appears to have a dual mechanism of action: rapid necrosis after gel application and a subsequent immune-mediated response, which targets any residual dysplastic epidermal cells. We report the successful treatment of a woman, who had been relapsing into Bowen's disease (BD) on her right forefinger for 8 years. During her clinical history, she had received an allogeneic, HLA-identical stem cell transplant for myeloproliferative syndrome with a JAK2V617F mutation and lobectomy of the pulmonary right lower lobe for adenocarcinoma. We used dermoscopy to monitor the therapeutic response of BD. We discuss IM gel as a possible therapeutic option for BD.

Swiss S1 Guidelines on the Systemic Treatment of Psoriasis Vulgaris.

Psoriasis vulgaris is a common, chronic inflammatory skin disease with a prevalence of 1.5-2% in Western industrialized countries. A relevant percentage of patients suffer from moderate-to-severe psoriasis and experience a significant reduction in quality of life. The choice of an adequate therapy could help to prevent disease and exacerbation of comorbidity, which could increase quality of life, avoid hospitalization and avoid reduction of working days. The present guidelines are focused on the initiation and management of systemic therapies in cases of moderate-to-severe plaque-type psoriasis in adults to optimize treatment response, adherence and quality of life. This first version of the Swiss S1 guidelines presents therapeutic recommendations which are based on a systematic literature search as well as an informal expert consensus of dermatologists in Switzerland.