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Background: Anti‐nuclear antibodies (ANA), anti‐extractable nuclear antigens (ENA) and anti‐dsDNA antibodies are often associated with cutaneous lupus erythematosus (CLE), with variable frequency depending on skin subtype. However, specific data based on large case‐series on the pathogenetic, diagnostic and prognostic meaning of such autoantibodies are still lacking. Objective: To characterize the correlations between CLE subtypes as well as LE‐non‐specific skin lesions and their autoantibody pattern. Methods: Epidemiological, clinical and immunopathological data of 619 Italian patients with CLE and LE‐non‐specific skin lesions were analysed. Differences in age, sex, clinical features and autoantibody profile were evaluated in each LE subgroup. Results: Anti‐nuclear antibodies (P < 0.0001), anti‐dsDNA (P < 0.0001), ENA (P = 0.001), anti‐Sm (P = 0.001), anti‐RNP (P = 0.004) and anti‐histone (P = 0.005) antibodies were associated with SLE. A strong association between ANA (P < 0.0001) and anti‐dsDNA (P < 0.0001) and female gender was also found: positive ANA and positive anti‐dsDNA had a higher prevalence among females. Chronic CLE resulted to be negatively associated with ENA (OR = 0.51, P < 0.0001), anti‐Ro/SSA (OR = 0.49, P < 0.0001) and anti‐dsDNA (OR = 0.37, P < 0.0001). Intermittent CLE resulted to be negatively associated with ENA (OR = 0.50, P = 0.007) and ANA (OR = 0.61, P = 0.025). Subacute CLE resulted to be associated with ENA (OR = 5.19, P < 0.0001), anti‐Ro/SSA (OR = 3.83, P < 0.0001), anti‐Smith (OR = 2.95, P = 0.004) and anti‐RNP (OR = 3.18, P = 0.007). Acute CLE resulted to be strongly associated with anti‐dsDNA (OR = 6.0, P < 0.0001) and ANA (OR = 18.1, P < 0.0001). LE‐non‐specific skin lesions resulted to be significantly associated with systemic involvement. Livedo reticularis was significantly associated with ENA (P = 0.007) and anti‐Ro/SSA (P = 0.036). Palpable purpura and periungual telangiectasia were significantly associated with ANA. Conclusion: According to our findings, some well‐known associations between CLE subtypes and autoantibody profile were confirmed; moreover, specific association between autoantibodies and LE‐non‐specific skin lesions was highlighted. A strict association between anti‐ENA and anti‐Ro/SSA antibodies and livedo reticularis, ANA and palpable purpura, and ANA and periungual telangiectasia was evidenced. [ABSTRACT FROM AUTHOR]

While the lesions on the body were resolved, those on the face persisted 15 days after the first treatment; hence, a cycle of benzoate benzyl benzoate 25% cream was started. Dear Editor, Scabies is commonly noted to be localized on the wrists, skin folds, hands, feet and genitalia. However, during the pandemic period, the prolonged use of the COVID mask may have favoured the development of lesions on the face for prolonged skin contact with the mite-contaminated mask. [Extracted from the article]

Although neurosyphilis ( NS) keeps plaguing worldwide, often with oligosymptomatic and atypical manifestations, the most recent reports fail to provide useful information, like details of the clinical history and even of the previous early therapy. We conducted a survey of the literature of the last 5 years on the clinical presentation of NS, recording the aforementioned inaccuracies. One hundred and thirty-seven articles were collected, reporting on 286 patients. General paresis was the commonest form (49%), often manifesting with cognitive impairment and psychiatric symptoms. Syphilitic meningitis was found in 63 patients (22%), mainly with ocular or auditory involvement. Meningovascular and tabetic form were both found in 12% of cases. Gummatous and epileptic manifestations were rare. Perusal of the literature confirms that NS prevalence is increasing, often with manifestations that are atypical for timing and type of lesions. Unfortunately, many articles are lacking of critical information, like an accurate clinical history and timing of the therapy making difficult to assess the effectiveness of penicillin in preventing NS. [ABSTRACT FROM AUTHOR]

Background Dermatological emergencies exist and should not be underestimated. On the other hand, many accesses to the emergency department ( ED) of patients with dermatological conditions are unjustified. Objective Our main objective is to describe dermatological conditions seen in an ED observation unit ( EDOU). Secondly, our aim is to identify alarm symptoms and signs of 'true emergencies'. Methods We conducted a prospective study, including dermatological patients admitted to EDOU of the University Hospital of San Martino, Genoa, Italy, in 3 years. Results Overall 372 patients were studied. The most common condition seen was infection (41.67%) (mainly bacterial), followed by atypical exanthem (13.98%) and vasculitis (11.29%). The highest rate of cases peaked in May (13%); infectious diseases showed two peaks (spring and autumn). Conclusion Given constraints involved in health care today, it is crucial to understand which dermatological presentations are 'true emergencies'. In such a scenario knowing the epidemiology of dermatological emergencies and the alarming skin signs and symptoms might be useful. [ABSTRACT FROM AUTHOR]

Background Epidermolysis bullosa acquisita ( EBA) is an autoimmune blistering disease characterized by tissue-bound and circulating autoantibodies to the dermal-epidermal junction. The autoantibody target is type VII collagen (Col VII) which is involved in dermal-epidermal adhesion. Diagnosis is made by clinical and histopathological findings, linear deposition of autoantibodies at the dermal-epidermal junction detected by direct immunofluorescence, and binding to the dermal side of salt-split skin by indirect immunofluorescence ( IIF). However, the detection of specific anti-Col VII reactivity has an important confirmatory value. Methods The humoral immune response in EBA sera was analysed by (i) IIF on human skin, (ii) a commercial Col VII ELISA, and (iii) immunoblotting on Col VII produced by an epithelial cell line. Objective The aim of this study was to compare the sensitivity of different approaches for the serological diagnosis of EBA. Results The vast majority of EBA sera (79.2%) bound to the Col VII non-collagenous domains by a commercial ELISA, while a small proportion of patients (12.5%) exclusively reacted to the collagenous domain by immunoblotting. Of note, the autoantibodies reactivity to Col VII was more frequently detected by IB (91.7%) than by IIF (83.3%) and ELISA (79.2%). Interestingly, 2 out of 24 sera recognized Col VII epitopes undetectable in the native secreted protein but present in the context of extracellular matrix proteins, as assessed by immunomapping on Col VII-deficient skin. Conclusion Our findings show that the use of multiple assays allows to improve diagnostic performance. An algorithm for efficient serological diagnosis of EBA is proposed. [ABSTRACT FROM AUTHOR]

I Editor i Syphilis is known to be "the great imitator": since the patient's history is often deceiving and the spectrum of clinical signs and symptoms is extremely broad, diagnosis may be particularly challenging.1,2 Tertiary syphilis is rarely seen nowadays but it should be taken into account when dealing with granulomatous disorders of unclear nature.1-4 Indeed, up to 25% of the patients with untreated primary syphilis develop symptoms of tertiary syphilis, usually occurring 3-5 years after primary infection and involving several organs, mainly skin, heart and central nervous system.1 We recently observed an unsuspected case of syphilitic I gummas i in a patient whose history was definitively not evocative for sexually transmitted diseases (STDs). This case was interesting not only for the deceiving patient's history but especially for the clinical aspect of the cutaneous lesions, given the rarity, nowadays, of benign tertiary syphilis ( I gummas) i in clinical practice. CSF testing is crucial for the correct diagnosis and therapeutic approach, also in case of asymptomatic neurosyphilis.6 Even if the patient's history is not evocative for STDs, as in our case, syphilis should be considered when approaching granulomatous lesions of unclear nature. [Extracted from the article]

Oral Diseases (2012) 18, 442-458 Pemphigus vulgaris (PV) is the most common type of pemphigus. PV pathogenesis is still debated, and treatment remains challenging. We investigated five controversial topics: (1) What are the target antigens in PV? (2) Do desmogleins adequately address PV pathophysiology? (3) How does acantholysis occur in PV? (4) Is PV still a lethal disease? (5) What is the role of rituximab (RTX) in PV treatment? Results from extensive literature searches suggested the following: (1) Target antigens of PV include a variety of molecules and receptors that are not physically compartmentalized within the epidermis. (2) PV is caused by a variety of autoantibodies to keratinocyte self-antigens, which concur to cause blistering by acting synergistically. (3) The concept of apoptolysis distinguishes the unique mechanism of autoantibody-induced keratinocyte damage in PV from other known forms of cell death. (4) PV remains potentially life-threatening largely because of treatment side effects, but it is uncertain which therapies carry the highest likelihood of lethal risk. (5) RTX is a very promising treatment option in patients with widespread recalcitrant or life-threatening PV. RTX's cost is an issue, its long-term side effects are still unknown, and randomized controlled trials are needed to establish the optimal dosing regimen. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to the article "Necrobiotic granuloma annulare of the penis: a case responding to anti-tuberculosis therapy with a review of the literature," in the previous issue.

A 54-year-old woman presented with a 5-month history of tender nodules in both nasolabial folds that had developed 4 months after the injection of hyaluronic acid (HA) (Restylane®) for wrinkles. The patient was treated with 1.5 mg/day betamethasone for 6 days and her lesions disappeared within 1 week. About 8 days after stopping therapy, however, new nodules developed at the same site, on previously healthy buttocks, and on old scars.On examination, nodules of about 0.5–1 cm in size were palpable at the nasolabial folds, and red nodules were present on the buttocks () and on two old scars. Laboratory tests disclosed an increased protein C reaction (7.9 mg/L; normal value,< 5 mg/L) and acetyl-converting enzyme test (14.5 U/L; normal value,< 9 U/L). A chest X-ray was normal. Lung function tests showed a decreased lung CO diffusion, and chest axial tomography disclosed fibrosis, increased parenchyma density, and calcifications, findings suggestive of a diagnosis of lung sarcoidosis. An X-ray of the hands showed some bone cysts. Interestingly, two granulomatous lesions were observed at the sites of venipuncture.Histology of a gluteal lesion biopsy showed a deep granuloma with epithelioid and Langhans cells in the absence of necrobiosis. Sarcoidosis was diagnosed and the patient was given 50 mg/day prednisone with clear clinical improvement of cutaneous lesions in about 6 months. Decreased parenchyma density was also observed by chest axial tomography. [ABSTRACT FROM AUTHOR]

Patients with pemphigus may produce antibodies against molecules other than the classical transmembranal ones. Recently, for example antibodies to 230 kDa antigens have been found in association with antibodies to intercellular substance. To better understand their prevalence, clinical correlates and prognostic significance of bands other than 130 and 160 kDa, we studied 67 pemphigus sera. About one-fourth of patients revealed multiple heterogeneous bands and 13% the 230 kDa band. When challenged with the recombinant protein rBPP55, the carbossiterminal portion of hullous pemphigoid major antigen, all 230 kDa-positive-sera proved negative. Caution is to be recommended in interpreting pemphigus sera with a hand migrating at the 230 kDa level. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to the article about a 67-year-old man with pemphigus herpetiformis (PH) associated with prostate cancer.

AbstractBackgroundThe epidemiology of bullous pemphigoid (BP) is not clear because of the heterogeneity of the disease, and its possible association with internal malignancies has been under debate for many years. We report the findings of a 2-year study on incident BP cases in the Liguria region of Italy. Subjects and methodsThirty-two patients with BP were collected over the 2-year period. Diagnosis was made based on clinical findings and confirmed by histology, direct immunofluorescence (DIF) and indirect immunofluorescence (IIF) with salt-split skin and monkey oesophagus, and immunoblotting (IB). All patients were thoroughly investigated for possible malignancies and all were followed up for 6 months to monitor the response to treatment. ResultsDIF showed linear deposits at the dermoepidermal junction in all but one patient. IIF gave positive findings for 15 sera tested with monkey oesophagus and 20 tested with salt-split skin. IB gave positive findings in 19 cases. There was a malignancy in six cases, but no clinical or immunological features that could be considered to predict this occurrence. ConclusionsThe findings of this study are in accordance with most of the data found in the literature, including the fact that IgG serum levels did not predict the course of the disease. Contrary to previous indications, IgE levels were not indicative of disease course either. Mucosal lesions, erythema multiform-like lesions, negative IIF findings and antibodies to AgPB2 were not a prediction for the development of malignancy. [ABSTRACT FROM AUTHOR]

The article focuses on a study conducted on prevalence of herpes zoster (HZ) and pityriasis rosea in patients with psoriasis. Topics discussed include HZ occurring in a million people in the U.S. annually as a result of declining cellular immunity and transitory immunosuppression, Psoriasis being triggered by pityriasis rosea (PR) and prevalence of HZ in psoriatic patients being higher than expected.

A letter to the editor is presented which discusses the case of a 37-year-old man with mycocis fungoides (MF) who developed pyoderma gangrenosum-like CD30+ cutaneous T-cell lymphoma after one year of treatment.

Presents a letter to the editor on the disease of allergic contact dermatitis published in the November 1999 issue of the "Journal of the European Academy of Dermatology and Venereology."

Psoriatics proved to he less frequently sensitized than patients attending a patch test clinic, but not less than those with other dermatological diseases. Previous claims of impairment of immunoreactivity in psoriasis, possibly resulting in a reduced prevalence of diseases with immunological pathogenesis, were not confirmed. The contrast may be explained by the clinical heterogeneity of psoriasis. Current classifications do not include in a separate set, lesions which, by clinical features and symptoms, would be diagnosed as allergic contact dermatitis if the patient did not, in fact, already have psoriasis.

Examines the case of a 26-year-old woman with a design painted on the right shoulder on henna dye. Development of itchy erythematooedematous reaction which produced the same original design; Treatment of topical corticosteroid on the inflamed area; Investigation shows the use of powder hair dye which ingredients are not specified.

Drug-induced bullous pemphigoid (DBP) associated to biologics administered for psoriasis is rare. DBP has been described especially in association with anti-TNF-α drugs and anti-IL12 and 23, but never in relation to guselkumab (anti-IL23). We report the case of a 76-year-old male patient with severe psoriasis (PASI 20), presenting with generalized tense bullae and erosions after being recently switched to guselkumab therapy. Histology and direct immunofluorescence confirmed the suspect of bullous pemphigoid (BP). Guselkumab administration was interrupted, low-dose oral corticosteroid therapy was introduced and after only 1-month remission was obtained with no new lesions appearing. As outlined in the presented case, DBP's onset typically follows the introduction of a new drug in patients taking polypharmacy. In addition, DBP may spontaneously regress after discontinuation of the triggering drug and it responds very rapidly to steroid therapy. Up to date, DBP has been described after biological therapy for psoriasis in 11 patients, following administration of ustekinumab, efalizumab, etanercept, secukinumab, and adalimumab. Conversely, DBP after guselkumab therapy for psoriasis has never been reported in published studies. We highlight the need to face and document increasing, though rare, side effects of biologic therapies, as new biologic molecules are being constantly developed and administered to psoriatic patients, to promptly interrupt treatment when needed., (© 2021 The Authors. Dermatologic Therapy published by Wiley Periodicals LLC.)

We present the case of a 92-year-old woman, with bullous pemphigoid (BP) and a concomitant nevus comedonicus (NC) presenting as an asymptomatic, linear lesion of the entire lower left limb, formed by multiple comedones. Dermoscopy of the NC and histopathology confirmed the clinical and dermoscopic suspect of giant congenital nevus comedonicus. The two entities presented no overlap. In this article, we speculate that a mutation of the NEK9 gene, associated with NC, probably altering the normal follicular differentiation in NC lesions, may hypothetically also influence the expression of BPAG2 in NC. This might possibly influence a protective role of NC lesions towards BP. Undoubtedly, genetic studies would be needed to confirm or reject the proposed hypothesis., (© 2021 Japanese Dermatological Association.)

Lack of safety data on pregnant women determines difficulty in choosing the correct biologic agent to treat psoriasis in women of childbearing potential. Studies have postulated a role of IL-23 in unexplained recurrent spontaneous abortions. This gives rise to consideration about use of anti-IL-23 drugs in treatment of psoriasis in women of childbearing potential., (© 2021 The Authors. Dermatologic Therapy published by Wiley Periodicals LLC.)

Annular elastolytic giant cell granuloma (AEGCG) is a rare granulomatous skin disorder, characterized by erythematous plaques with elevated borders and hypopigmented center, occurring mainly on sun exposed-skin. Histologically it presents with elastophagocytosis and elastolysis. There is no established first line treatment for AEGCG, especially for the generalized form. In a small number of cases, antimalarial drugs and tranilast, associated to topical or oral steroids, have been proposed to treat generalized AEGCG with partial benefits. We herein present the case of a patient with AEGCG aged 74 years, who was unresponsive to classical therapies, and then successfully treated with methotrexate., (© 2020 Wiley Periodicals LLC.)

During the COVID-19 pandemic, various cutaneous manifestations have been described as associated with SARS-CoV2 infection. It is debated if skin lesions could represent a diagnostic or prognostic indicator. Specifically, it is unclear whether skin lesions may be used to perform an early diagnosis and/or to predict worse outcomes. In this review, we described the cutaneous signs so far reported as COVID-19-related and discussed their incidence, clinico-pathological features, and diagnostic and prognostic value., (© 2020 Wiley Periodicals LLC.)

Genital psoriasis (GenPs) is a frequent manifestation of psoriasis, causing distress, especially in women. We prospectively studied a population of 74 psoriatic women with severe and generalized psoriasis eligible to biologic therapy, to examine which biologic therapy is more effective on GenPs and to study possible associations between PASI severity and GenPs. Overall, 25/74 (34%) had GenPs: 6 received Ixekizumab, 7 Ustekinumab, 8 Adalimumab, 2 Secukinumab, 1 Etanercept, 1 Certolizumab. Therapies were administered based on PASI severity, independently from the presence of GenPs. Side effects, PASI score, sPGA-G scale for GenPs were recorded at time 0 and after 6 month of therapy. The mean sPGA-G scale value was 2.8 before treatment. After biologic therapy, all patients except one, improved of at least one point. Mostly, patients treated with anti-IL17 (Secukinumab, Ixekizumab) and anti-IL12/23 (Ustekinumab) improved. Mean PASI ranged from 10 to 16.3 before treatment. After 6 months of therapy, 4 anti-TNFα patients, 6 anti-IL17 and 1 anti-IL12/23, reached PASI 90. At time 0, no correlation between PASI and sPGA-G was visible (Pearson r = 0.10, p = .620). From our data, GenPs apparently responds favorably to IL17A inhibitors, but further studies, based on larger numbers of patients, are needed., (© 2019 Wiley Periodicals, Inc.)

Bullous pemphigoid, mucous membrane pemphigoid, and pemphigus vulgaris are different cutaneous autoimmune blistering diseases, with complex pathogenic mechanisms. In all of them, a type-2 response is thought to have a central role. Interleukin 4 and Interleukin 13 are crucial cytokines in type-2 response. Treatment of these conditions is often challenging. Dupilumab, a recombinant fully human IgG4 monoclonal antibody with binding specificity to human interleukin-4 receptor IL-4Rα, has the potential to inhibit both IL-4 and IL-13. We propose IL-4Rα as a theoretical drug target for cutaneous autoimmune bullous diseases., (© 2019 The Authors. Dermatologic Therapy published by Wiley Periodicals, Inc.)

Ivermectin is a drug approved for the treatment of papulopustular rosacea (PPR). Although clinical guidelines recommend the use of ivermectin as the first-line treatment in patients with almost clear and mild rosacea, studies concerning its use on them are lacking. This study investigated the effectiveness and the tolerability of ivermectin in almost clear to severe rosacea and assessed the antiparasitic effect on Demodex mites. This is a retrospective study based on 50 patients affected by PPR and treated with topical ivermectin 1% once daily over 16 weeks. The disease severity, the patient-examined improvement, and the safety assessment of patients were evaluated. Demodex mites were studied with the standardized skin surface biopsy. PPR to all severity achieved a therapeutic success. The number of inflammatory lesions was significantly decreased in almost clear (p < .0001), mild, moderate, and severe (p < .001) forms. A complete remission of inflammatory lesions was achieved by almost clear (p < .001) and mild (p = .005) with 82% with none-to-mild cutaneous adverse events. Thirty-two percent were positive for Demodex mites, and all of them turned negative after 16 weeks. Ivermectin is an effective treatment not only in moderate to severe PPR but also in almost clear/mild rosacea., (© 2019 Wiley Periodicals, Inc.)

In recent years, dermatologic manifestations in oncohematologic patients have become more common. The aim of our study was to determine the incidence and heterogeneity of skin manifestations in patients followed at our Hematology Department. This observational monocentrical study was conducted on 60 patients. We divided the observed conditions in exanthematous, purpuric, vesicular-bullous, papulonodular, urticarial, and eczematous manifestations. Moreover, all lesions were classified according to pathogenesis, in (a) specific skin manifestations, caused by neoplastic skin infiltration; (b) immune-mediated manifestations, based on immunological mechanisms; and (c) skin lesions due to immunodeficiency. Altogether, 73 clinical manifestations were reported. Specific manifestations (a) were detected in 15.1% of the cases, mainly with papulonodular appearance. Immune-mediated manifestations (b) were found in 37 cases (50.7%), particularly with eczematous or exanthematous appearance, and leukemia was the malignancy most frequently reported in these patients. Eventually, lesions due to immunosuppression (c) were reported in 34.2% of the cases. They were represented by infections and cutaneous malignancies, and usually manifested with papulonodular lesions. Skin lesions in oncohematologic patients are a common event. A multidisciplinary approach based on the collaboration between the hematologist and the dermatologist is crucial to achieve a proper diagnosis, and correctly manage these manifestations., (© 2018 John Wiley & Sons, Ltd.)