JEADV clinical practice最新文献

{"title":"Bullous Pemphigoid Presenting as Immunoglobulin M Pemphigoid","authors":"D. Didona,&nbsp;R. Maglie,&nbsp;A. M. Sequeira Santos,&nbsp;V. Schwarz,&nbsp;M. Hertl,&nbsp;F. Solimani","doi":"10.1002/jvc2.70006","DOIUrl":"https://doi.org/10.1002/jvc2.70006","url":null,"abstract":"<p>Bullous pemphigoid (BP) is the most frequent autoimmune blistering disease (AIBD) of the skin [<span>1</span>]. Usually, BP affects patients in their seventh or eighth decade of life and is elicited by circulating immunoglobulin (Ig) G autoantibodies targeting BP180 (Collagen XVII) and/or BP230, two components of the dermo-epidermal junction (DEJ) [<span>1</span>]. In recent years, some unclear evidence of rare sub-variants of BP triggered by pathogenic IgM directed against BP180, known as IgM pemphigoid, have been reported. However, the relevance of IgM in the pathogenesis of AIBD is still under debate [<span>2</span>]. Reported cases of IgM pemphigoid tend to have a more favourable and manageable disease course compared to classical BP. Yet, the question raised by the analysis of the more recent literature is whether IgM pemphigoid is rather triggered by barely detectable IgG (or IgA) antibodies.</p><p>Here, we describe an otherwise healthy 51-year-old female patient who was admitted to our clinic showing tense blisters on erythematous skin on both elbows for 3 years (Figure 1A,B). The oral mucosa was not affected, and the patient complained of itch limited to the affected areas. A histological examination showed a dermo-epidermal cleft and sparse neutrophilic and eosinophilic infiltration (Figure 1C). Serological examination with a commercially available enzyme-linked immunosorbent assay (ELISA) kit (EUROIMMUN, Lübeck, Germany) did not detect circulating antibodies against components of the DEJ (BP180, BP230, collagen VII, laminin 332). Direct immunofluorescence analysis of perilesional skin showed the absence of IgG and IgA deposition, yet a strong linear tissue-bound IgM, together with a mild and discontinuous linear C3 deposition (Figure 1D−G). By indirect immunofluorescence on salt-split skin, we confirmed the absence of IgA and IgG and the presence of IgM binding to the epidermal side of the split (Figure 1H). A pattern typical for BP [<span>1</span>]. We repeated ELISA after 2 and 4 weeks, which confirmed the absence of IgG against BP180 and BP230. Furthermore, there was no evidence for a monoclonal IgM gammopathy, immunodeficiency, cryoglobulinemia or hyper IgM syndrome. According to the pathological and immunological findings, a diagnosis of IgM pemphigoid was done. We started a topical treatment with clobetasol propionate 0.05% cream twice a day, which rapidly led to improvement of skin lesions without scarring. To investigate the presence of specific IgM against BP180 better, we performed a western blot analysis (WB) with recombinant BP180 protein. WB results confirmed the presence of anti-BP180 IgM, but we also found a weaker presence of IgG and IgA (Figure 2). Exclusive detection of IgM has been reported in BP, epidermolysis bullosa acquisita and mucous membrane pemphigoid [<span>2, 3</span>]. The concomitant presence of tissue-bound IgM and C3 is plausible, given the described complement-fixing ability of IgM [<span>4</span>]. ","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"4 4","pages":"887-889"},"PeriodicalIF":0.5,"publicationDate":"2025-02-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.70006","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144923699","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Scarring Alopecia in a 66-Year-Old Woman","authors":"Sergio Castillo Pinto,&nbsp;Lina M. Isaza,&nbsp;Isabel Cristina Cuellar","doi":"10.1002/jvc2.621","DOIUrl":"https://doi.org/10.1002/jvc2.621","url":null,"abstract":"&lt;p&gt;A 66-year-old female with no significant medical history, referred from a rural area, presented with a 1-week history of ocular pain associated with ipsilateral erosions and tense bullae involving the left scalp and periocular region. On examination, extensive areas of alopecia were observed on the majority of the scalp, with no visible follicular openings, along with erosions and tense bullae on the left side of the scalp and periocular area. No involvement of other body areas or mucous membranes was noted (Figures 1 and 2). Initially, the differential diagnosis included herpes zoster or lichen planus pemphigoides with Wolf's isotopic response. A comprehensive ocular examination by an ophthalmologist ruled out herpetic infection or other structural damage to the eye. Notably, the patient reported similar episodes in her twenties, which had resulted in cicatricial alopecia over large areas of the scalp. Considering the clinical findings and the diagnostic alternatives, an incisional biopsy was performed for histopathology, direct immunofluorescence (DIF), and the salt-split test (Figures 3 and 4).&lt;/p&gt;&lt;p&gt;Cicatricial alopecia refers to a group of disorders that cause permanent hair loss due to the destruction of hair follicles. It can be classified as primary, involving lymphocytic, neutrophilic, mixed, or nonspecific etiologies, or secondary, resulting from physical factors such as trauma, thermal or surgical damage. Among these causes, BPP is rarely found as a possible aetiology.&lt;/p&gt;&lt;p&gt;BPP is a rare autoimmune blistering disease considered a variant of mucous membrane pemphigoid (MMP). To date, 65 cases have been reported in the literature with histopathological confirmation and positive DIF [&lt;span&gt;1-3&lt;/span&gt;]. BPP is characterised by chronic and recurrent subepidermal bullae that most commonly appear on the scalp and face, leading to secondary scarring and cicatricial alopecia. Mucous membrane involvement is reported in only about 22.2% of cases and tends to be mild [&lt;span&gt;3-6&lt;/span&gt;].&lt;/p&gt;&lt;p&gt;Diagnosis is based on clinical and histopathological correlation, with findings of subepidermal blisters and superficial dermal infiltrate containing eosinophils, along with DIF showing IgG and C3 deposits along the BMZ [&lt;span&gt;3, 4&lt;/span&gt;]. When available, serologic studies such as indirect immunofluorescence and ELISA can help to confirm the diagnosis by identifying autoantibodies with specific target antigens [&lt;span&gt;7&lt;/span&gt;]. If serologic tests are not available, the salt-split test can be a useful tool in DIF [&lt;span&gt;7&lt;/span&gt;]. This test helps determine whether antibodies are binding to the epidermal or dermal side of the BMZ, providing information about the possible targeted antigens, which is not possible with DIF alone [&lt;span&gt;7&lt;/span&gt;]. The pattern of antibody deposits in the BMZ, along with the salt-split test, helps identify the specific antigens involved; distinguishing between n-serrated and u-serrated patterns, and determining whether binding o","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"4 2","pages":"624-626"},"PeriodicalIF":0.0,"publicationDate":"2025-02-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.621","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144191176","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Bullous Eruption with Cholestasis in Pregnancy","authors":"Sara Al Janahi,&nbsp;Yusra S. Al Ali","doi":"10.1002/jvc2.623","DOIUrl":"https://doi.org/10.1002/jvc2.623","url":null,"abstract":"&lt;p&gt;A 26-year-old female, 27 weeks pregnant (G2P1) presented to our clinic with a 2-week history of a progressively worsening, urticarial and bullous eruption that started on the abdomen and subsequently spread to the trunk and extremities. She reported significant nocturnal pruritus, disrupting her sleep andpredominately affecting her palms and soles. Her previous pregnancy had been uneventful. She was also not known to have atopy or any preceding dermatologic conditions. Clinical examination revealed pink to erythematous urticarial papules and plaques, with bullae at the periphery, distributed over the extremities and trunk, involving the umbilicus and sparing the peri-umbilical striae (Figure 1). Laboratory investigations were significant for elevated bile salts 16.5 μmol/L (normal &lt; 10 μmol/L in pregnant women). All other investigations were unremarkable. Two 4-mm punch biopsies were taken from the abdomen for histopathology with hematoxylin and eosin (H&amp;E) staining and immunofluorescence studies (Figure 2).&lt;/p&gt;&lt;p&gt;The clinicopathologic correlation was suggestive of pemphigoid gestationis (PG) with co-existing intrahepatic cholestasis of pregnancy. The patient's obstetrician started treatment with ursodeoxycholic acid (UCDA) 500 IU three times per day. Dermatologic management consisted of oral prednisolone 0.5 mg/kg, antihistamines, topical steroids, and emollients. The patient demonstrated improvement in pruritus and skin lesions, noted at a 2-week follow-up visit where most of the lesions resulted in post-inflammatory hyperpigmentation. The improvement continued throughout her pregnancy. Attempts to taper the dose of oral prednisolone resulted in a flare of disease, marked by the appearance of new bullae. Thus, she remained on the same dose until delivery. The patient delivered a healthy baby girl. There was no noted flare with delivery or in the postpartum period.&lt;/p&gt;&lt;p&gt;PG is an autoimmune bullous disorder with clinical and histologic overlap with bullous pemphigoid (BP). PG may occur at any stage of pregnancy but tends to favour the third trimester. The aetiology is due to anti-basement membrane zone auto-antibodies to BPAG2 (BP 180, collagen XVII). There is a strong association with HLA-DRs DRB1*0301 (HLA-DR3) and DRB1*0401/040X (HLA-DR4) [&lt;span&gt;1&lt;/span&gt;]. Clinically, patients may experience intense pruritus with erythematous urticarial papules and plaques classically on the abdomen that often spread to the rest of the body [&lt;span&gt;2&lt;/span&gt;]. Mucosal surfaces are often spared [&lt;span&gt;1&lt;/span&gt;]. Histopathology, as in this case, demonstrated eosinophilic spongiosis of the epidermis with oedema of the upper and mid dermis. A perivascular lymphohistiocytic inflammation with abundant eosinophils was noted (Figure 2a). Direct Immunofluorescence (DIF) was significant for linear deposition of C3 (2 + ) and IgG (1 + ) along the basement membrane (Figure 2b).&lt;/p&gt;&lt;p&gt;Immunofluorescence studies are considered the gold standard for diagnosis of PG,","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"4 2","pages":"627-629"},"PeriodicalIF":0.0,"publicationDate":"2025-02-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.623","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144191177","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Efficacy of Calcipotriol/Betamethasone Ointment in Facial Discoid Dermatosis","authors":"Alberto Murtas,&nbsp;Cristina Mugheddu,&nbsp;Luca Pilloni,&nbsp;Franco Rongioletti,&nbsp;Laura Atzori","doi":"10.1002/jvc2.625","DOIUrl":"https://doi.org/10.1002/jvc2.625","url":null,"abstract":"<p>Facial Discoid Dermatosis (FDD) is a rare chronic skin condition characterised by persistent, annular erythematous and desquamative papules on the face. Its aetiology is unclear, making differential diagnosis challenging. Key clinical features include pink-orange, minimally scaling lesions limited to the face, sudden onset with long-term stability, and resistance to treatment. We report a new case of FDD in a patient in his late 30's, unresponsive to various treatments until improvement with topical calcipotriol/betamethasone ointment. A review of 22 cases reveals a female predominance and broad age range. Histopathology consistently shows hyperkeratosis, parakeratosis, acanthosis, psoriasiform hyperplasia, follicular plugging, and perivascular lymphocytic infiltrate. Many cases, including ours, also reported the presence of abundant Demodex folliculorum.</p>","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"4 2","pages":"544-551"},"PeriodicalIF":0.0,"publicationDate":"2025-02-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.625","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144190691","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cutaneous Tumours With Perineural Involvement in a 42-Year-Old Woman","authors":"Ting Fong Yeo,&nbsp;Caitlin Borowsky,&nbsp;Dabean Faraj,&nbsp;Mohammad Elnaggar,&nbsp;Daryl Teo,&nbsp;Wael Hamarneh,&nbsp;Simon Dixon,&nbsp;Rami Salha,&nbsp;Pick-Ngor Woo","doi":"10.1002/jvc2.627","DOIUrl":"https://doi.org/10.1002/jvc2.627","url":null,"abstract":"&lt;p&gt;A 42-year-old Caucasian woman presented to us with a 1-year history of lesion on right cheek and a 1-month history of lesion on her nose. Her right cheek lesion initially appeared red and raised before becoming crusty. A similar lesion emerged on her nasal dorsum without any associated trauma. Examination revealed a 10 mm indurated plaque with a central pitted scar on her right cheek, and a 20 mm erythematous plaque with yellow crust on the dorsum of her nose (Figure 1a). Her medical history includes right vestibular schwannoma (which was removed with ventriculoperitoneal shunt in situ), postneurosurgical trigeminal neuralgia, a congenital blind right eye and psoriasis. She was initially diagnosed with a keratoacanthomas (KAs) and referred to maxillofacial team for a surgical excision. While awaiting surgery, her right cheek lesion regressed, and her nasal lesion quadrupled in size within weeks (Figure 1b). Surgical excision was abandoned in favour of multiple nasal biopsies and an MRI of her neck. Histology confirmed a well-differentiated squamous cell carcinomas (SCCs) (Figure 2a,b) and her MRI showed no lymphadenopathy. The patient family history revealed that her sister and two aunts had similar recurrent cutaneous tumours that self-regressed, leaving pitted scars.&lt;/p&gt;&lt;p&gt;Further questioning revealed a family history of FSD in her sister and two aunts. Genetic testing subsequently confirmed FSD with a pathogenic variant of the TGFBR1 gene designated c.1059_1062delinsCAATAAp.(Leu354AsnfsTer4), whereas no variants in the &lt;i&gt;NF1&lt;/i&gt; gene were found. Following a multidisciplinary team (MDT) discussion, her condition was stabilised with a treatment regime with cryotherapy, imiquimod cream and oral acitretin.&lt;/p&gt;&lt;p&gt;Her acitretin dose was increased to 40 mg/day, and prednisolone was initiated when she developed right infra-orbital swelling and reduced sensation on the right side of her face (Figure 3a). A MRI of her neck (Figure 3b) and biopsy demonstrated soft tissue inflammation. Despite treatment, she continued developing similar lesions on her lips, left upper eyelid and right cheek, which were treated with cryotherapy.&lt;/p&gt;&lt;p&gt;A month later, she developed severe neuropathic pain on the left side of her face and swelling in her cheek (Figure 3c), requiring referral to the pain clinic. Prednisolone was continued, however her Acitretin dose was reduced to 30 mg due to dry lips. An MRI of her neck showed perineural enhancement along her left infraorbital nerve (Figure 3d). The persistent pain and evolving nasal tumour caused significant psychosocial distress. Multiple MDT discussions led to a decision for nonsurgical management with Imiquimod cream which was discontinued shortly after due to intolerance and cryotherapy for active lesions. Radiotherapy was not recommended for treating FSD. Serial MRI scans showed stable progress of the infra-orbital nerve distribution and eventual resolution over a 2-year period. To date, she continues treatment wit","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"4 2","pages":"633-636"},"PeriodicalIF":0.0,"publicationDate":"2025-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.627","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144191171","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Case of Anti-TNFα Therapies Induced Lupus Panniculitis Successfully Improved With a JAK Inhibitor (Baricitinib)","authors":"Cristina Grechin,&nbsp;Jane Doheny,&nbsp;Keith Pilson,&nbsp;Donough Howard,&nbsp;Muireann Roche","doi":"10.1002/jvc2.626","DOIUrl":"https://doi.org/10.1002/jvc2.626","url":null,"abstract":"<p>Lupus erythematosus panniculitis (LEP) is a rare variant of cutaneous lupus erythematosus (1%–3% of CLE). There are two case reports to date in the literature of LEP potentially triggered by anti-TNF-α therapies. Clinically, it is characterised by tender, erythematous subcutaneous indurated nodules or plaques on fatty body areas. It can occur frequently alone or in combination with systemic erythematous lupus or discoid lupus. The pathogenic mechanism of anti-TNF-α therapies induced lupus panniculitis is not well understood. Our case report and literature review highlight that anti-TNF-α therapies can induce lupus panniculitis, especially in the rheumatoid arthritis cohort of patients.</p>","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"4 2","pages":"540-543"},"PeriodicalIF":0.0,"publicationDate":"2025-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.626","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144191173","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Successful Psoriasis Management With Deucravacitinib After Guselkumab-Associated Eosinophilic Pneumonia","authors":"Katie K. Lovell,&nbsp;Steven R. Feldman","doi":"10.1002/jvc2.614","DOIUrl":"https://doi.org/10.1002/jvc2.614","url":null,"abstract":"<p>Eosinophilic pneumonia (EP) involves the accumulation of eosinophilic infiltrates in the lung parenchyma, potentially triggered by smoking, infections, or medications, including biologics for psoriasis. This case report describes a 49-year-old female with a history of psoriasis and chronic pancreatitis, who developed EP after initiating guselkumab, an IL-23 inhibitor. Following a 4-month hiatus from ustekinumab due to insurance issues, she received guselkumab for a psoriasis flare (60% BSA). Shortly before the second dose, she presented with pleuritic chest pain, cough, and shortness of breath. CT revealed extensive ground-glass infiltrates, and bronchoscopy with bronchoalveolar lavage (BAL) showed 52% eosinophils, indicating EP. Two months postdischarge, follow-up CT showed near-complete resolution of opacities, and 4 months later, after tapering off of systemic corticosteroids, her psoriasis flared again (60% BSA). Due to EP and preference for a nonbiologic treatment, deucravacitinib, a TYK2 inhibitor, was initiated, resulting in well-controlled psoriasis (BSA = 0%) after 3 months, with only mild, resolved acne. This case suggests deucravacitinib as a viable option for patients experiencing noninfectious pneumonia from prior biologic therapy and highlights the importance of monitoring for pulmonary symptoms in patients on biologics.</p>","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"4 2","pages":"529-531"},"PeriodicalIF":0.0,"publicationDate":"2025-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.614","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144191174","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Erythematoviolaceous Papules and Plaques on Sun-Exposed Areas","authors":"Ana C. Martín-Zamora,&nbsp;Alejandra Gamboa-Flores,&nbsp;Jaime Pozuelo-Díaz,&nbsp;Marcela Campos-Hidalgo","doi":"10.1002/jvc2.618","DOIUrl":"https://doi.org/10.1002/jvc2.618","url":null,"abstract":"<p>A 71-year-old Hispanic female patient presented to the dermatology department with a 6-year history of cutaneous lesions. Personal medical history was notable for hypertension, diabetes mellitus, and previous breast cancer. Her chronic treatment consisted of metformin, insulin, irbesartan and omeprazole. She reported lesions appearing first on her scalp and face with significant pruritus and then extending to the back of her upper extremities, with worsening after sun-exposure. Previous treatment with topical and oral steroids had a poor response.</p><p>On physical examination, erythematoviolaceous papules and plaques, some with annular configuration and diffuse white scaling were seen (Figure 1). Dermoscopy revealed shiny white structures in some areas. In other parts, a pinkish–red background with whitish scales, orange–yellow structureless areas, and a mixed vascular pattern (dotted, linear and branched vessels) was evidenced (Figure 2).</p><p>Laboratory testing revealed anaemia (haemoglobin: 11.1 mg/dL) with normal leucocyte and platelet count. Kidney and liver function tests were normal. Positive antinuclear antibodies (ANA) and extractable nuclear antigen antibodies (ENA) were found, with detection of Anti-Ro and Anti-La antibodies. Anti-ds-DNA antibodies were negative. No other lab anomalies were detected. Skin biopsies from her back and left arm were done (Figure 3).</p><p>CLE/LP overlap syndrome is very rare, with few cases published so far. Diagnostic criteria define classic CLE/LP overlap syndrome as patients with mixed clinical characteristics of both CLE and LP, histopathological characteristics consistent with LP (with or without CLE features), and positive serologic markers of CLE (positive ANA with 1:80 or higher titers, ENA antibodies, double-stranded DNA antibodies, or antiphospholipid antibodies) [<span>1, 2</span>]. Our patient fulfilled all the criteria. Direct immunofluorescence (DIF) can be helpful, but is not necessary for diagnosis, and can present features of either CLE or LP [<span>2</span>].</p><p>According to literature, cutaneous lesions in this entity most often affect the extremities, face, and trunk. They have been described as centrally atrophic, hypopigmented, with scaling, sometimes painful or pruritic [<span>3, 4</span>]. However, a wide variety of different morphologies have been reported, with oral compromise also being present in many cases [<span>2</span>]. Our patient presented with lesions in photo-exposed areas, some of them morphologically consistent with lichen planus and some more suggestive of psoriasiform subacute cutaneous lupus. She also presented autoantibodies more commonly found in the subacute form of CLE. The coexistence of these findings has been reported before [<span>5, 6</span>].</p><p>In addition to presenting clinical overlapping features of both CLE and LP, dermoscopic findings in our patient were also consistent with both conditions. Classic dermoscopic elements described in subac","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"4 2","pages":"621-623"},"PeriodicalIF":0.0,"publicationDate":"2025-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.618","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144191170","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Management of Cutaneous Lesions in Brunsting–Perry Pemphigoid (Bpp): A Single-Center Retrospective Cohort Study","authors":"Shirley P. Parraga,&nbsp;Matthew L. Hrin,&nbsp;Sarah N. Rimmer,&nbsp;Joseph L. Jorizzo","doi":"10.1002/jvc2.70000","DOIUrl":"https://doi.org/10.1002/jvc2.70000","url":null,"abstract":"&lt;p&gt;Brunsting–Perry pemphigoid (BPP) is a rare, autoimmune skin disorder within the spectrum of mucous membrane (cicatricial) pemphigoid characterized by subepidermal blistering of the head, neck, and shoulders [&lt;span&gt;1, 2&lt;/span&gt;]. The development of autoantibodies against hemidesmosomal proteins including BP180 and BP230 disrupts the integrity of the epidermal basement membrane [&lt;span&gt;2-4&lt;/span&gt;]. BPP's rarity has precluded extensive study [&lt;span&gt;4, 5&lt;/span&gt;]. We assessed the outcomes of 10 BPP patients treated with methotrexate (MTX) or mycophenolate mofetil (MMF).&lt;/p&gt;&lt;p&gt;Institutional review board was obtained to review the medical records of patients evaluated at Wake Forest Baptist Health's dermatology clinic who received MTX or MMF for BPP skin lesions between 2010 and 2020. Diagnoses were established based on (1) clinical features of lesions, (2) positive direct immunofluorescence (DIF) assays (&lt;span&gt;&lt;/span&gt;&lt;math&gt;\u0000 &lt;semantics&gt;\u0000 &lt;mrow&gt;\u0000 &lt;mrow&gt;\u0000 &lt;mo&gt;±&lt;/mo&gt;\u0000 &lt;mspace&gt;&lt;/mspace&gt;\u0000 &lt;/mrow&gt;\u0000 &lt;/mrow&gt;\u0000 &lt;/semantics&gt;&lt;/math&gt;C3 deposits along the basement membrane zone), (3) histopathologic findings, and (4) ELISA/immunoblot positivity. Patients who did not meet these criteria were excluded. Outcomes were categorized as complete response (CR) (asymptomatic, no new lesion development), partial response (PR) (improving lesions, reduced erythema and inflammation), and no response (NR).&lt;/p&gt;&lt;p&gt;Our patients were primarily White (100%) males (60%) with a mean age of 62 years (range: 48–82) (Table 1). Lesions were presented on the trunk (40%), face (20%), mouth (70%), scalp (40%), and genitals (10%) (Figure 1). Patients had face (25%), scalp (50%), and buccal mucosal biopsies (25%) (Figure 2). Seven patients (70%) had positive ELISA results for IgG antibodies against full-length BP 180 recombinant proteins; four patients (40%) had positive anti-BP 230 IgG antibodies. All patients (100%) had negative antibody production against collagen VII. Patients had a mean disease duration of 27 and 42 months before MTX and MMF initiation, respectively. Medications failed before MTX or MMF included topical corticosteroids (30%), prednisone (30%), tacrolimus “swish and spit” (20%), magic mouthwash (hydrocortisone, lidocaine, diphenhydramine, nystatin) (30%), doxycycline (20%), minocycline (10%), dapsone (10%), and azathioprine (10%). Seven patients (70%) were systemic therapy naïve. Three patients (30%) had pretreatment systemic therapy failure on a mean prednisone dose of 21.7 mg.&lt;/p&gt;&lt;p&gt;Two treatment groups were analyzed: MTX and MMF. Patients received either MTX or MMF as initial treatment. Six patients received treatment with MTX; four with MMF (Table 2). One patient was discontinued from MTX and started on MMF. Mean time to initial response was 2.9 months (standard deviation [SD], 1.9 months) and 2.7 months (SD, 2.4 months) for ","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"4 2","pages":"580-583"},"PeriodicalIF":0.0,"publicationDate":"2025-01-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.70000","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144191193","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"An Evaluation of Generic and Disease-Specific Patient-Reported Outcome Measures to Assess the Impact of Percentage of Scalp Hair Loss on Health-Related Quality of Life in a European Population","authors":"K. A. Hanson,&nbsp;S. Marwaha,&nbsp;S. K. Kurosky,&nbsp;M. Harries,&nbsp;P. Anderson,&nbsp;J. Piercy,&nbsp;V. Basey,&nbsp;J. Austin,&nbsp;E. H. Law","doi":"10.1002/jvc2.591","DOIUrl":"https://doi.org/10.1002/jvc2.591","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Hair loss due to alopecia areata (AA) can negatively impact patients’ health-related quality of life (HRQoL). Patient-reported outcome measures (PROMs) like the EQ-5D-5L and the Alopecia Areata Patient Priority Outcomes (AAPPO) represent treatment outcomes and can guide decision-making. However, the EQ-5D-5L potentially underestimates AA-specific impacts, while the AAPPO may provide a more disease-specific assessment.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Objectives</h3>\u0000 \u0000 <p>This study uses the EQ-5D-5L and the AAPPO emotional symptom (ES) and activity limitations (AL) subscales to characterise HRQoL among patients with AA.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This analysis uses secondary data from the Adelphi Alopecia Areata Disease Specific Programme (AA-DSP), a survey of dermatologists and patients with AA in five European countries. Included patients completed a survey containing the EQ-5D-5L and AAPPO. Descriptive summary statistics were reported for AAPPO ES/AL subscales and EQ-5D-5L scores, overall and stratified by physician-reported percentage of scalp hair loss (%SHL). Cramer's V effect sizes were calculated across each of the AAPPO ES/AL and EQ-5D anxiety/depression and usual activities items to examine the relationship between varying degrees of SHL and the PROMs.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Four hundred thirty-five patients with AA completed the AAPPO and EQ-5D-5L. The mean (SD) overall EQ-5D-5L index and EQ-VAS score was 0.85 (0.15) and 74.07 (17.86), respectively. The mean (SD) overall AAPPO ES and AL subscale scores were 1.70 (1.03) and 0.88 (0.93), respectively. For the AAPPO ES and AL items, effect sizes were largest between the extreme values of SHL (0%–10% vs. 100%), while the effect sizes between intermediate SHL groups (e.g., 11%–20% and 21%–49%) tended to be smaller. The two EQ-5D-5L items demonstrated lower effect size values.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Our findings suggest the AAPPO suitably discriminates between clinically relevant groups of patients with AA. The EQ-5D-5L was not as effective in measuring the specific psychological or social dimensions by SHL, potentially underestimating disease burden among patients with AA.</p>\u0000 </section>\u0000 </div>","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"4 2","pages":"451-457"},"PeriodicalIF":0.0,"publicationDate":"2025-01-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.591","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144191246","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}