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This article presents a case study of a 1-day-old male infant with congenital erosions on the index finger and foot. The diagnosis was neonatal pemphigus vulgaris, a transient autoimmune bullous disorder caused by the transfer of IgG autoantibodies from a mother with pemphigus vulgaris. The infant's mother had a history of pemphigus vulgaris, but had no active lesions during pregnancy. The erosions were managed with topical treatments and resolved completely after 3 weeks. Neonatal pemphigus vulgaris is self-limiting and typically resolves within a few weeks, with no reported progression to adult pemphigus vulgaris. [Extracted from the article]

Sweet syndrome is a neutrophilic dermatosis associated with multiple disorders. This retrospective case-series study of patients with Sweet syndrome in a tertiary hospital in Spain from 2001 to 2021, explores clinicopathological characteristics of Sweet syndrome and variables associated with malignancy, presence of autoinflammatory disorders and differences between histological subtypes. A total of 93 patients were identified: 30% idiopathic, 34% malignancy-associated, 29% reactive to infections or drug-associated, and 6% with an autoimmune/inflammatory condition. Acute myeloid leukaemia was the most common malignancy (16/93) followed by myelodysplastic syndrome (7/93). Patients with acute myeloid leukaemia presented isolated flares, marked cytopaenia and rapid response to treatment, whereas myelodysplastic syndrome followed a chronic-recurrent course. The most frequent associated medications and inflammatory disorders were filgrastim and hydroxyurea (n=2); and inflammatory bowel disease (n=4). In addition, 3 patients were diagnosed with VEXAS syndrome. Male sex (p=0.006), fever (p=0.034), increased erythrocyte sedimentation rate (p<0.001), anaemia (p<0.001), and thrombocytopaenia (p<0.001) were associated with malignancy. Histologically, patients were classified as classic (60%), histiocytoid (22.5%) or subcutaneous (15%), with pain (p=0.011) and nodules (p<0.001) being associated with subcutaneous-Sweet syndrome. Sweet syndrome in the context of cytopaenia should alert the presence of malignancy. An acquired autoinflammatory condition should be explored in relapsing Sweet syndrome with myelodysplastic syndrome. A minimum follow-up of 6 months is recommended. [ABSTRACT FROM AUTHOR]

Granuloma annulare (GA) is a granulomatous, idiopathic, inflammatory skin disorder characterized by the formation of papules and plaques with annular and acral distribution.1 GA is often limited and self-resolving, but in some cases, it can be generalized and refractory to treatments.1 New advances in the pathophysiology of GA have favoured Janus kinase (JAK) inhibitors as a promising therapeutic option.1,2 Here, we report three cases of resistant generalized GA successfully treated with tofacitinib. Further studies are necessary to assess the safety and the long-term remission of GA patients treated with tofacitinib, and to study the efficacy of other JAK inhibitors in these patients. In addition, other JAK inhibitors such as baricitinib and upadacitinib have recently been used in two generalized GA patients with good results.7,8 In conclusion, advances in GA pathophysiology have allowed the introduction of JAK inhibitors as a new treatment option. [Extracted from the article]

Background: Extracorporeal photopheresis (ECP) has been increasingly used as a second-line therapy for graft-versus-host disease (GVHD) but there is no consensus regarding the best therapeutic schedule.Study Design and Methods: Our offline ECP schedule for treating patients with GVHD was retrospectively reviewed. Patients with acute GVHD were treated on 2 days per week for the first 2 weeks, followed by 1 day per week for 2 more weeks. After the first month of treatment, patients received treatment 1 day every 2 weeks for a minimum of 16 ECP procedures. Patients with chronic GVHD were treated on 1 day per week for 4 weeks followed by 1 day every 2 weeks for a minimum of 14 ECP procedures.Results: Our series comprises 21 (45%) patients with acute GVHD and 26 (55%) patients with chronic GVHD who received 667 ECP procedures. A median (interquartile range [IQR]) of 1.0 (1.0-1.12) total blood volume was processed. Patients with acute and chronic GVHD received ECP procedures during a median of 49 (IQR, 14-103) and 180 (IQR, 111-274) days, respectively. Mild citrate-induced symptoms were present in 98 (46%) and 232 (51%) procedures in patients with acute and chronic GVHD, respectively. Overall response rate (ORR) and overall survival (OS) were 57 and 38% (95% confidence interval [CI], 17%-59%), respectively, for patients with acute GVHD. For patients with chronic GVHD, ORR and OS were 77 and 61% (95% CI, 18%-87%), respectively.Conclusion: Our new offline ECP schedule for treating patients with acute and chronic GVHD was efficacious and safe. [ABSTRACT FROM AUTHOR]

Autoinflammatory diseases include disorders with a monogenic cause and also complex conditions associated to polygenic or multifactorial factors. An increased number of both monogenic and polygenic autoinflammatory conditions have been identified during the last years. Although skin manifestations are often predominant in monogenic autoinflammatory diseases, clinical and histopathological information regarding their dermatological involvement is still scarce. Monogenic autoinflammatory diseases with cutaneous expression can be classified based on the predominant lesion: (1) maculopapular rashes or inflammatory plaques; (2) urticarial rashes; (3) pustular, pyogenic or neutrophilic dermatosis-like rashes; (4) panniculitis or subcutaneous nodules; (5) vasculitis or vasculopathy; (6) hyperkeratotic lesions; (7) hyperpigmented lesions; (8) bullous lesions; and (9) aphthous lesions. By using this classification, this review intends to provide clinical and histopathological knowledge about cutaneous involvement in monogenic autoinflammatory diseases. [ABSTRACT FROM AUTHOR]

Hypereosinophilic syndrome (HES) is often associated with cutaneous manifestations, mostly pruritic lesions, urticaria and angioedema. Mucosal lesions are rarely seen in HES but, when present, are usually the first manifestation of the disease. The clinical presentation may be heterogeneous, including erosions, aphthae or ulcers, and can be easily confused with other mucocutaneous disorders. Here, we present the case of a 64‐year‐old man with severe chronic erosive oral mucositis simulating pemphigus in which the finding of persistent eosinophilia and elevation of B12 vitamin serum levels raised the suspicion of HES. The FIP1L1–PDGFRA fusion gene (4q12) was detected by fluorescence in situ hybridization and the patient was treated with imatinib mesylate with complete response of the disease. [ABSTRACT FROM AUTHOR]

Dear Editor, Cutaneous chronic graft-versus-host disease (cGVHD) mimics different autoimmune dermatoses such as lichen planus (lichenoid cGVHD) or localized scleroderma (sclerodermatous cGVHD).1 We describe two patients who developed autoimmune bullous diseases (AIBD) after hematopoietic cell transplantation (HCT). A diagnosis of bullous pemphigoid (BP) was made as the patient fulfilled two out of three diagnostic criteria.2 Cutaneous biopsies were not performed as the patient did not present cutaneous lesions when he was evaluated in our clinic. In patient 2, we found autoantibodies against different cutaneous antigens detected by ELISA (in immunoblot, proteins are more thoroughly denatured and may not always be detected). [Extracted from the article]

Pancreatic panniculitis is a rare type that only occurs in 2–3% of all patients with pancreatic diseases. It is usually described in association with benign pancreatic disease and less commonly in association with pancreatic carcinoma. We describe a case of pancreatic panniculitis as the first manifestation of underlying ampullary adenocarcinoma and a new case of pancreatitis, panniculitis and polyarthritis (PPP‐Syndrome). Pancreatic panniculitis may be the cutaneous manifestation of pancreatic allograft rejection after simultaneous pancreas‐kidney transplantation. [ABSTRACT FROM AUTHOR]

It has been suggested that bullous pemphigoid is associated with an increased risk of malignancy, but the evidence is inconsistent. Therefore, a meta‐analysis was conducted to explore this association. PUBMED and Embase were searched for studies investigating the association between bullous pemphigoid and malignancy. This meta‐analysis included 16 studies with a total of 9398 cases of bullous pemphigoid. The rate of malignancy in patients with bullous pemphigoid was 11% (95% CI: 9–14, P < 0.001); 9% (95% CI: 6–13, P < 0.003) for women and 13% (95% CI: 9–18, P < 0.03) for men, with a statistically insignificant higher risk in men (OR = 1.30, 95% CI: 0.99–1.71, P = 0.06). The event rate was 9% (95% CI: 5–14, P < 0.001) in the Asian population and 13% (95% CI: 10–17, P < 0.001) in the European population, with a statistically significant lower risk in the Asians population (OR = 0.69, 95% CI: 0.57–0.84; P < 0.001). The event rate of malignancy was higher in patients with bullous pemphigoid than in matched controls (OR = 2.08, 95% CI: 1.22–3.55; P = 0.005). The overall event rate of malignancy was higher in the bullous pemphigoid group than in matched controls. Caution is required when interpreting these results, as potential confounding variables were not controlled for. [ABSTRACT FROM AUTHOR]

Pneumocystis jirovecii pneumonia (PCP) is a relatively rare complication in non-HIV patients receiving immunosuppressive treatment. Since the introduction of tumour necrosis factor-α inhibitors cases of this complication have increased. We report the case of a 54-year-old, HIV-negative patient, who presented to our department with a long history of pustular psoriasis with poor response to traditional treatments. During the last admission he developed a severe flare that was unresponsive to cyclosporine, therefore infliximab was initiated. After the third dose he developed PCP that required admission to the intensive care unit, with a positive response to i.v. administration of trimethoprim/sulfamethoxazole. During follow up a mutation in the IL36RN gene compatible with an IL-36RN deficiency was found and anakinra was started, with rapid improvement of his psoriasis. PCP is a severe complication in patients receiving immunosuppressive therapy and is probably underreported by dermatologists. There are no clinical guidelines for PCP prophylaxis in dermatological patients who will receive immunosuppressive or biological treatments. We believe that it is necessary to report the cases of PCP to assess the real impact of this complication and develop appropriate prophylaxis guidelines. [ABSTRACT FROM AUTHOR]

Lichen planus pemphigoides is an extremely rare autoimmune blistering disorder characterised by clinical and histopathological features of lichen planus, and a subepidermal blistering disease with antibodies to the basement membrane, very similar to bullous pemphigoid. Several years ago, we reported that the sera of patients with lichen planus pemphigoides reacted with amino acids 46-59 of domain BP180 NC16A, an epitope not usually recognised by bullous pemphigoid sera.[1] Autoantibodies in lichen planus pemphigoides react with a novel epitope within the C-terminal NC16A domain of BP180. [Extracted from the article]

The article presents a case study of a 74-year-old white man presented with multiple erythematous lesions, who was on oral hypoglycemic agents. The histological examination of the patient revealed epidermal atrophy with atypical keratinocytes and dyskeratosis. The patient was diagnosed for chemotherapy-induced inflammation of actinic keratoses. The article discusses 5-Fluorouracil as a cytotoxic drug that acts as an antimetabolite.

Pemphigoid gestationis (PG) is a subepidermal autoimmune blistering disease characterized by self-reactive T and B cells specific for the transmembrane hemidesmosomal protein type XVII collagen/BP180. Major T and B cell epitopes are located within the immunodominant 16 non-collagenous domain A (NC16A) of type XVII collagen. The aim of the present study was to map the pathogenically relevant epitopes targeted by blister-inducing patients' autoantibodies. For this purpose, we used an in vitro model of autoantibody-induced leukocyte-dependent dermal-epidermal separation. Pre-adsorption against a recombinant form of the NC16A region abolished the blister-inducing potential of autoantibodies from all PG patients. Using overlapping synthetic peptides, we demonstrated that PG autoantibodies bind to two defined epitopes within the NC16A region (aa 500-514 and aa 511-523). Importantly, pre-adsorption using an affinity matrix containing these epitopes completely abolished dermal-epidermal separation induced by PG autoantibodies. This study identifies the epitopes relevant for blister induction in PG and should facilitate the development of an antigen-specific immunoadsorption therapy for this disease. [ABSTRACT FROM AUTHOR]