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Pigmented mammary Paget's disease is a very rare variant of mammary Paget's disease linked to an underlying carcinoma in almost all cases. We present the case of a 62-year-old female patient who came to our attention for the evaluation of a monolateral asymptomatic pigmented lesion of the right nipple, which turned out to be a pigmented mammary Paget's disease unassociated to an underlying malignancy – an extremely rare entity only anecdotally reported in literature. The two main peculiarities of our patient's lesion, the importance of immunohistochemistry in the differential diagnosis and the theories on its pathogenesis are discussed. Further studies are necessary to establish the best treatment options. Click here for the corresponding questions to this CME article.

Bullous pemphigoid (BP) is the most common autoimmune blistering skin disease, characterized by the development of autoantibodies against hemidesmosomal components BP180 and BP230. The mainstay of therapy is topical and systemic corticosteroids (CS) and immunosuppressors. As this pathology mainly involves the elderly, subjects often have numerous comorbidities that influence the clinical management. Omalizumab is a recombinant humanized monoclonal anti-IgE antibody which has recently emerged as a promising treatment for BP in patients for whom CS are contraindicated or conventional treatments have failed to control the disease. For this study, we selected five patients who presented with corticosteroid-dependent BP with a contraindication to the use of other immunosuppressive treatments. The objectives of our study were to evaluate the effectiveness of omalizumab in controlling BP and allowing to decrease the dosage of systemic CS, assessing the effects of omalizumab on the clinical manifestations and the titers of circulating anti-BP180 and BP230 antibodies, IgE and eosinophils. A reduction in the dose of systemic CS was possible in 100% of the patients and complete resolution of the clinical picture was seen in 100% for skin lesions and in 40% for pruritus. A reduction of circulating IgE was found in 40%, anti-BP180 and BP230 IgGs were decreased in 60% and eosinophils in 80%.

Lombardy, Italy was one of the most heavily impacted areas in the world during the height of the Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) pandemic, quickly becoming the epicenter within Italy. There have been 845,898 cases reported to date July 19, 2021 within Lombardy, accounting for approximately 20% of the cases overall in Italy.1 During the height of the pandemic, concern was raised over the use of biologics for psoriasis as they necessitate some degree of immunomodulation.2.

Since the onset of the coronavirus disease 2019 (COVID-19) pandemic, there has been an open debate on the impact of biological drugs used in the treatment of psoriasis. To define whether patients under treatment with biologics suffer from increased morbidity and mortality from COVID-19, compared to psoriatic patients treated only with topical drugs, we designed an observational monocentric prevalence study recording the personal and clinical data of psoriatic patients, with focus on the presentation of signs and symptoms related to COVID-19 in the period of time ranging from 1 January 2020 to 31 May 2020. A total of 180 patients were enrolled into two groups: 100 patients in the topical therapy group and 80 patients in the biological therapy group. No statistically significant difference was found between the groups regarding the prevalence of COVID-19 infection and symptoms at a bivariable analysis with adjustment for confounders. In conclusion, psoriatic patients under treatment with biologics do not seem to be more susceptible to COVID-19 compared to other psoriatic patients and we suggest not interrupting treatment with biological drugs, even in areas suffering from active outbreaks of the disease.

During the lockdown period most planned visits have been postponed and the number of accesses to Emergency Department (ED) has dramatically reduced. The aim of our study is to analyze the impact of the lockdown on the number, type and severity of Dermatological ED diagnosis. We performed a retrospective review of all dermatological consultations in the ED of IRCSS San Matteo during the lockdown period in Italy (February 22nd ‐ May third 2020) and compared them with those from the same period in 2019. We noticed a sharply reduction in the number of dermatological consultations requested in the ED: from 164 patients in 2019 to 33 in 2020. Some diagnostic categories showed a significant difference with a higher incidence of vasculopathic lesions (0.6% vs 12.1%, P

Linear IgA bullous dermatosis (LABD) is characterized by presence of multiple IgA autoantibodies, and a comparatively lesser number of IgG antibodies, directed against different hemidesmosomal antigens. The main autoantigens are LAD-1, LABD-97, BP180 and BP230, type VII collagen and laminin 332. We retrospectively studied the serology of 54 Italian patients with LABD using enzyme-linked immunosorbent assay (ELISA), immunoblotting assay, and indirect immunofluorescence on monkey oesophagus and salt-split skin. Among these, indirect immunofluorescence of salt-split skin elicits the greatest sensitivity. Sixty-three percent of the sera were observed to be positive, with a lamina lucida pattern observed in 48%, a sub-lamina densa pattern in 2% and a mixed pattern in 13% of the cases. IgA reactivity to LAD-1 on immunoblotting was found in 52% of sera, to BP180-NC16A by ELISA in 32% and to BP230 in 26%. Only 17% of patients possessed circulating IgG autoantibodies. LAD-1 was determined to be a major autoantigen of the lamina lucida subtype. Combined serological assays demonstrated a high sensitivity (82%), suggesting that this approach could support diagnosis when a biopsy is not feasible or direct immunofluorescence results are negative.

Objective. Our objective was to characterize the demographic information, clinical features, and laboratory data of patients with dermatitis herpetiformis (DH). Methods. In this multicentre cross-sectional study, consecutive patients with a new diagnosis of DH that referred to nine different Italian centers between 2011 and 2016 were characterized assessing demographic, clinical and laboratory findings, and evaluating gender and age differences across selected variables. Results. A total of 151 patients were included. Among them, 81 (53.6%) were males and 70 (46.4%) were females, with a male to female ratio of 1.2 : 1. The median age at the time of diagnosis was 41 years (range 0–85). Males had a significant longer diagnostic delay if compared to females (9 vs. 3 months, respectively; p=0.01). Direct immunofluorescence was positive in 94.7% of the patients, while duodenal biopsy showed partial to total villous atrophy in 70.1% of patients. All the females resulted positive to at least one of the antibodies tested, while a total of 12 male patients (10.5%) tested negative to celiac-specific antibodies. Female patients had a high rate (14.1%) of autoimmune thyroiditis. Conclusions. Our study confirmed some of the most relevant data regarding DH that have been previously reported in the literature. In addition, we found a reduced diagnostic delay in females with respect to males, possibly related to the higher sensitivity of serologic testing in females with DH compared to males. Finally, we demonstrated that intestinal involvement could be severe in patients with DH and that females should be tested for thyroiditis.

Anderson-Fabry disease (AFD) is a rare X-linked lysosomal storage disease, caused by defects of the alpha-galactosidase A (GLA) gene. AFD can affect the heart, brain, kidney, eye, skin, peripheral nerves, and gastrointestinal tract. Cardiology (hypertrophic cardiomyopathy), neurology (cryptogenic stroke), and nephrology (end-stage renal failure) screening studies suggest the prevalence of GLA variants is 0.62%, with diagnosis confirmation in 0.12%. OBJECTIVES: This study sought to expand screening from these settings to include ophthalmology, dermatology, gastroenterology, internal medicine, pediatrics, and medical genetics to increase diagnostic yield and comprehensively evaluate organ involvement in AFD patients. METHODS: In a 10-year prospective multidisciplinary, multicenter study, we expanded clinical, genetic, and biochemical screening to consecutive patients enrolled from all aforementioned clinical settings. We tested the GLA gene and α-galactosidase A activity in plasma and leukocytes. Inclusion criteria comprised phenotypical traits and absence of male-to-male transmission. Screening was extended to relatives of probands harboring GLA mutations. RESULTS: Of 2,034 probands fulfilling inclusion criteria, 37 (1.8%) were carriers of GLA mutations. Cascade family screening identified 60 affected relatives; clinical data were available for 4 affected obligate carriers. Activity of α-galactosidase A in plasma and leukocytes was diagnostic in male subjects, but not in female subjects. Of the 101 family members harboring mutations, 86 were affected, 10 were young healthy carriers, and 5 refused clinical evaluation. In the 86 patients, involved organs or organ systems included the heart (69%), peripheral nerves (46%), kidney (45%), eye (37%), brain (34%), skin (32%), gastrointestinal tract (31%), and auditory system (19%). Globotriaosylceramide accumulated in organ-specific and non-organ-specific cells in atypical and classic variants, respectively. CONCLUSIONS: Screening probands with clinically suspected AFD significantly increased diagnostic yield. The heart was the organ most commonly involved, independent of the clinical setting in which the patient was first evaluated. Copyright © 2016 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

Patients undergoing allogeneic stem cell transplantation are at high risk for infection with a variety of pathogens during different phases of the procedure. Human infections due to Bartonella spp. are viewed as emerging diseases typical in, although not exclusive to, immunosuppressed patients, in particular those with AIDS, organ transplants and haematological malignancies. We describe four patients, three children and one adult, who developed vegetating papillomatous lesions exclusively on the oral mucosae. They shared a history of haematological malignancy and allogeneic bone marrow/stem cell transplantation, and later developed chronic graft-versus-host disease, also involving the oral mucosae. Histopathologically, the vegetating lesions were characterized by a diffuse neoangiogenesis, granulation-like tissue, and a mixed cell infiltrate predominantly composed of neutrophils. Gram-negative bacteria were found in the endothelial cells of the vessels in the deeper portion of the corium by electron microscopy. In three cases, DNA of B. henselae was detected by polymerase chain reaction (PCR), and confirmed by sequencing of the PCR products. All the lesions healed after systemic antibiotic therapy, although some recurred after months, and regressed again after systemic antibiotic treatment associated with conservative surgical excision.

Primary cutaneous T-cell lymphomas are exceedingly rare in children and adolescents. However, mycosis fungoides (MF) is the most frequent primary cutaneous lymphoma diagnosed in childhood. Two cases of MF in siblings (a 14-year-old boy and his 10-year-old sister) are reported. On the basis of clinical features (histopathological and immunophenotypical findings) a diagnosis of MF patch lesions was made in both siblings. Since recent data in the literature have underlined a high frequency of the HLA-DQB1*03 allele in patients with familial MF (including child patients), the HLA profile of the patients was analysed, indicating the presence of a haplotype (HLA-DQB1*03,*03 in the girl, HLA-DQB1*02,*03 in the boy) corresponding with that described in recent literature. Two rare and exceptional cases of MF in siblings are reported, highlighting the presence of a peculiar haplotype.

Sir,Patients affected by chronic B-cell lymphatic leukaemia(CBLL) and, more rarely, other oncohaematologicaldiseases may present with papules, plaques, nodules andvesico-bullous lesions on exposed areas (1–3). Theselesions are usually considered an exaggerated reaction toinsect bites, although the patients not always had ahistory (except for the seasonal presentation of cuta-neous findings), the clinical picture, and response totreatment suggestive of insect bite (3, 4). This phenom-enon has been described in about 40 patients affected bylymphoproliferative disorders, 95% of whom had CBLL(1–7). Weed (1) first gave the definition of ‘exaggerateddelayed hypersensitivity to mosquito bites’ and reportedthis condition only in patients affected with CBLL.Later, Houston & Keene (2) described a case ofexaggerated insect bite-like reaction also in a patientwith lymphocytic lymphoma. In 1986, Rosen et al. (3)studied 10 patients and suggested that the cutaneouslesions could be linked, in some way, to the onco-haematological conditions, without explaining the exactpathway.Five patients affected by different B lymphoprolifera-tive disorders, who presented with pruritic papules,nodules and vesico-bullous lesions on exposedareas during spring and summer time, are reported.We discuss an immuno-allergic mechanism, involvingboth allergic reaction to insect bite and the impair-ment of the immune response in oncohaematologicalpatients.CASE REPORTSFrom 1995 to 2001, three patients affected by CBLL andtwo by non-Hodgkin B-cell lymphomas attended ourdepartment with polymorphous, erythematous cuta-neous papules and plaques, some of them evolving intobullous lesions. During spring-summer all the patientsdeveloped very itchy lesions, plaques (Fig. 1) andsometimes bullae, mainly localized on upper and/orlower limbs and on the face. Three patients referred tohave been bitten by mosquitoes, the other two deniedthis occurrence. At the time of the clinical examination,all the patients were living in or close to the area ofPavia, Italy, where seasonal infestations of mosquitoes(Aedes) are particularly widespread. All the patientsunderwent a 4-mm punch biopsy, necessary for ahistopathologic evaluation; a direct immunofluores-cence test was carried out for four patients, to excludeautoimmune bullous diseases.At the time of the eruption, one patient was ontreatment with VACOP-B protocol (adriblastina, cyclo-phosphamide, etoposide, vincristine, bleomycin, predni-sone), two patients with chlorambucil and one withcyclophosphamide. An 87-year-old patient was notunder treatment. Blood analysis revealed peripheraleosinophilia in three patients out of five. Stool analysissearching for parasites was carried out in those threepatients and proved negative. IgE was in the normalrange in all the patients. Serum protein electrophoresisrevealed that total immunoglobulins were in the normalrange or little lower in all the patients, while all of thempresented a different degree of decrease of IgG, IgMand/or IgA in sera. The other clinical and serologicalfindings were unremarkable or consistent with theirhaematological condition.The histopathology was characterized by a variety offindings, all of which were consistent with an arthropodbite reaction. In particular, a wedge-shaped, superficialor superficial-deep perivascular and often also inter-stitial infiltrate was present. It was mainly composed ofeosinophils in association with lymphocytes and rarelyneutrophils. The density or the depth of the infiltratevaried from case to case, also according to the age of thelesion. An oedema of the subpapillary dermis wasalways evident. One patient presented a subepidermalvesicle. In another patient spongiosis could be seen and

Sir, Human giardiasis, caused by Giardia intestinalis, a  agellate protozoan parasite that colonizes the small bowel, is a worldwide infection (1). Giardia infection is usually asymptomatic but intestinal illness may occur (2–5). Several reports describe the association of allergy with increased levels of total serum IgE antibodies and of speciŽ c IgE antibodies against food allergens in patients aVected by giardiasis, and Giardia infection may determine altered absorption of food antigens causing allergic sensitization (6). Cutaneous signs may be virtually indistinguishable from those of atopic dermatitis (7, 8). Acute reactions such as urticaria or asthma have also been described (9–11). We here report a patient aVected by giardiasis, with lichen-planus-like lesions as the sole clinical feature.

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