RARE DISEASE
Rare cutaneous lupus erythematosus
Rare cutaneous lupus erythematosus
Rare cutaneous lupus erythematosus
Drug discovery
1
drug
With orphan designation
Overview
Rare cutaneous lupus erythematosus (CLE) encompasses uncommon autoimmune skin manifestations, including lupus panniculitis, chilblain lupus, and bullous lupus. These subtypes present with distinct features such as subcutaneous nodules, cold-induced lesions, or blistering eruptions, often alongside chronic photosensitivity and potential systemic involvement (SLE). Diagnosis relies on clinical-histopathological correlation, with management balancing cutaneous control and systemic disease monitoring [1][6][11][20].
Key Clinical Insights
Burden
Significant psychosocial impact: Depression, anxiety, and social isolation linked to scarring, dyspigmentation, and stigma [4][9].
Financial strain due to chronicity, with 38% of patients reporting income loss [4][9].
Scarring and alopecia (e.g., DLE) cause irreversible damage, worsening quality of life [2][6][9].
Therapies
First-line: Topical corticosteroids, calcineurin inhibitors (e.g., tacrolimus) [3][8][17].
Systemic: Antimalarials (hydroxychloroquine), methotrexate, or retinoids (e.g., acitretin) for refractory cases [3][8][17].
Refractory disease: Biologics (rituximab), IV immunoglobulins, or JAK inhibitors under investigation [3][8][17].
Categories: rare skin diseases
Research Papers
97 drug discovery papers about Rare cutaneous lupus erythematosus, with 1 next-in-class emerging drug candidate forecasted to outperform the average preclinical success rate. Recent publications:
97 drug discovery papers about Rare cutaneous lupus erythematosus, with 1 next-in-class emerging drug candidate forecasted to outperform the average preclinical success rate. Recent publications:
categories:
Small molecules
small molecules
2026-09-24 | 07 | CHILBLAIN LUPUS: A RARE FORM OF CUTANEOUS LUPUS ERYTHEMATOSUS - A CASE REPORT
Chilblain lupus erythematosus is a rare, cold-induced form of chronic cutaneous lupus that can occur in both genetic and sporadic forms. It is characterized by acral skin lesions and is commonly associated with autoimmunity and type I interferon pathway activation. Diagnosis is based on clinical features and histological findings. While topical and systemic therapies can be effective, the disease is often chronic and relapsing. We describe a case report of a 48-year-old woman with a history of chronic spontaneous urticaria and Raynaud’s phenomenon presented with a history of recurrent nodular lesions on the toes since 2015. The lesions, characterized by a burning sensation and worsening during winter, appeared as erythematous to violaceous plaques with light scaling. Laboratory investigations showed positive antinuclear antibodies (1:160) and anti-Smith antibodies, while cryoglobulins and cold agglutinins were absent. The diagnosis of CHLE was confirmed by skin biopsy, which showed perivascular lymphocytic infiltrate, interface dermatitis, and increased dermal mucin, and by nailfold capillaroscopy, which revealed severe microangiopathy and giant capillary loops. Initial management with hydroxychloroquine (200 mg twice daily), topical tacrolimus 0.1%, and warmth protection resulted in only partial improvement. However, after adding methotrexate (12.5 mg/week) to the regimen, the patient achieved almost complete clinical remission within six months. This stable remission has been maintained for six months to date, highlighting methotrexate as an effective add-on therapy for CHLE cases refractory to antimalarials.
2026-09-18 | Facial discoid lupus erythematosus complicated by a Candida tropicalis fungal granuloma in a patient with rhupus syndrome: a case report of successful treatment with oral itraconazole.
Discoid lupus erythematosus (DLE) is a chronic cutaneous subtype of lupus erythematosus characterized by inflammatory lesions that are highly susceptible to secondary infection, particularly in immunocompromised individuals. Cutaneous fungal granulomas caused by Candida tropicalis and superimposed on pre-existing DLE lesions are exceedingly rare and pose substantial diagnostic challenges. We report the case of a 48-year-old female patient with a 2-year history of rheumatoid arthritis (RA), who presented with facial lupus lesions in January 2024. During her disease course, she met the classification criteria for systemic lupus erythematosus (SLE), leading to a diagnosis of rhupus syndrome (RA-SLE overlap). After eight months of treatment with hydroxychloroquine and prednisone, her cutaneous lesions showed no improvement and instead progressed to nodules and pustules. Histopathological examination of a skin biopsy revealed classic features of DLE accompanied by granulomatous inflammatory infiltrates. Periodic acid-Schiff (PAS) staining and subsequent fungal culture confirmed C. tropicalis as the causative pathogen. The patient was ultimately diagnosed with DLE complicated by secondary C. tropicalis fungal granuloma in the setting of Rhupus syndrome. She received a 3-month course of combination therapy consisting of oral itraconazole (200 mg once daily) plus topical naftifine-ketoconazole cream, which resulted in complete clearance of all facial lesions. No recurrence was observed throughout a 12-month post-treatment follow-up period.
2026-08-14 | Atypical Lupus Erythematosus Profundus of the Flank Evaluated by Serial Computed Tomography and Managed with Hydroxychloroquine: A Case Report.
Lupus erythematosus profundus (LEP) is a rare form of chronic cutaneous lupus erythematosus that predominantly involves the subcutaneous adipose tissue. When it presents at an atypical site, LEP may mimic infection or neoplasia, delaying diagnosis. A 37-year-old woman presented with a progressively enlarging ulcerated indurated lesion on the left flank. An initial biopsy performed at another hospital raised concern for a lymphoproliferative disorder. Computed tomography (CT) demonstrated panniculitic changes in the left flank and additional clinically occult lesions in the right buttock and left thigh. An incisional biopsy from the thigh, selected on the basis of the CT findings, showed interface dermatitis, lymphoplasmacytic panniculitis, dermal mucin deposition, and granular IgM deposition along the dermoepidermal junction, supporting a diagnosis of LEP and arguing against cutaneous lymphoma. Treatment with prednisolone followed by adjunctive hydroxychloroquine (HCQ) led to ulcer healing and radiologic improvement, allowing gradual corticosteroid tapering without relapse to date. This case underscores the importance of considering LEP in atypical deep truncal lesions. In this patient, CT was useful for defining disease extent, identifying clinically occult lesions, selecting a biopsy site, and documenting treatment response. HCQ may also be a useful steroid-sparing adjunct in LEP.
2026-08-13 | Transforming the treatment of autoimmune skin diseases: a journey through biologic therapies.
Autoimmune blistering diseases and autoimmune connective tissue diseases with prominent skin involvement are increasingly managed with biologic and targeted therapies. We review the evidence supporting these approaches and their practical positioning. We performed a PubMed/MEDLINE literature review (inception to February 2026) on biologic therapies for pemphigus, pemphigoid spectrum disorders, LABD, EBA, cutaneous lupus erythematosus, and dermatomyositis, including selected non-biologic small molecules, focusing on validated cutaneous outcomes. Evidence is strongest for B-cell depletion in pemphigus (including dose-optimization strategies) and for IL-4/IL-13 pathway blockade in BP, with additional data for anti-IgE therapy in selected BP phenotypes. In CLE, the most consistent cutaneous datasets involve pDC/type I interferon-axis modulation and BAFF inhibition, largely derived from systemic lupus cohorts; TYK2 inhibition has shown controlled benefit in CLE. In dermatomyositis, CDASI-based evidence is strongest for IVIg, with anti-IFNβ therapy and the TYK2/JAK1 inhibitor brepocitinib (positive in a phase 3 trial) showing cutaneous benefit; other biologics show limited trial signals. Biologic/targeted therapies should be positioned by disease phenotype and evidence strength, prioritizing steroid-sparing strategies in pemphigus and BP and using CLASI/CDASI-anchored outcomes to interpret lupus and dermatomyositis data. Evidence for rare AIBDs remains mostly case-level and should be framed accordingly.
2026-07-28 | Two cases of therapy-refractory chilblain lupus erythematosus successfully treated with deucravacitinib.
Chilblain lupus erythematosus is a rare skin manifestation of lupus erythematosus (LE), which has proven particularly difficult to treat and therefore often presents a great challenge for patients and physicians. We treated two patients with cases of long-lasting, refractory chilblain LE with the selective, allosteric TYK2-inhibitor deucravacitinib. Both patients showed rapid improvement of their respective skin lesions and symptoms. The treatment was generally well tolerated with no severe adverse events. One of the patients experienced an intermittent occurrence of acne lesions. We therefore suggest deucravacitinib as a promising and safe off-label treatment option for chilblain LE.
antibodies
2026-09-01 | Anifrolumab for rituximab-induced SCLE and cryoglobulinemic vasculitis in primary Sjögren's disease: a case report.
Rituximab, a chimeric anti-CD20 monoclonal antibody, is widely used to treat autoimmune diseases and hematologic malignancies through B-cell depletion, yet paradoxical autoimmune phenomena, including drug-induced lupus erythematosus, have been increasingly recognized. While paradoxical autoimmune phenomena following rituximab therapy have been reported, drug-induced subacute cutaneous lupus erythematosus (DI-SCLE) represents an exceedingly rare complication. This case reports rituximab-induced SCLE in a patient with Sjögren's disease and demonstrates the dual therapeutic efficacy of anifrolumab in managing both drug-induced cutaneous lupus and interferon-driven systemic manifestations. A 69-year-old woman with established primary Sjögren's disease and cryoglobulinemic vasculitis developed biopsy-proven subacute cutaneous lupus erythematosus with clinical onset approximately 4 months following rituximab administration, with histopathological confirmation at Month 9. The patient presented with characteristic erythematous lesions on the trunk and back, which subsequently recurred with extension to the face and décolleté; histopathological examination revealed interface dermatitis and a positive lupus band test on direct immunofluorescence. Treatment with anifrolumab (300 mg intravenously every 4 weeks) resulted in complete resolution of rituximab-induced SCLE. Subsequent addition of low-dose mycophenolate mofetil (1,000 mg daily) achieved complete remission of cryoglobulinemic vasculitis, with sustained disease control maintained on this anifrolumab-based regimen. Clinicians should maintain vigilance for paradoxical cutaneous reactions following B-cell-depleting therapies, particularly in patients with underlying Sjögren's disease and anti-Ro/SSA positivity.
2026-08-01 | Anifrolumab use in Jaccoud Arthropathy, Chilblains and Refractory SLE - A Case Report and Literature Review
Background Anifrolumab, an anti-interferon I receptor monoclonal antibody, is the second biologic molecule to be recognized for treatment of active systemic lupus erythematosus (SLE). Jaccoud’s arthropathy (JA) a reducible, non-erosive subtype of musculoskeletal involvement in SLE patients, and Chilblain Lupus Erythematosus (CHLE), a subtype of cutaneous involvement presenting as a spectrum of acral cold-induced lesions that can progress to painful ulcerations, are 2 rare manifestations of SLE with treatment guidelines mostly relying on clinical cases and experts’ opinion. Case Report We report a case of a patient diagnosed with coexisting SLE, CHLE requiring amputation and mutilating JA. Clinical response on all aspects remained suboptimal after multiple lines of treatment including Hydroxychloroquine, high dose Prednisone, Mycophenolate mofetil, Ustekinumab, Azathioprine, Belimumab. She showed positive clinical response to Anifrolumab, with significant reduction of arthralgia and complete resolution of chilblains lesions. This was maintained at 1 year post introduction of Anifrolumab with only complication attributable to this treatment being a cutaneous abscess treated with topical and systemic antibiotics. Following this improvement, we decided to proceed to a narrative review of literature regarding management of both CHLE and JA. Guidelines did not recommend Anifrolumab directly for any of those 2 subtypes of SLE. The line of treatment after Belimumab in the most up to date Cases of both presentations were compiled, with specific interest in patients reported to have received Anifrolumab as part of their treatment regimen. We collected 10 cases of CHLE treated with Anifrolumab. We also found 1 case of JA that demonstrated improvement of arthralgia on Anifrolumab. Most of the patients had also failed to respond to multiple lines of treatment including trial of Belimumab and/or Rituximab. All cases responded completely to Anifrolumab within 8 to 20 weeks. We did not find reports of cases followed for longer intervals. Conclusion With our case, we are participating in the growing weight of evidence that Anifrolumab is a safe and potent treatment option that should be considered in refractory cutaneous lupus, including CHLE, and potentially in refractory arthralgia, even in JA subtypes.
2026-07-14 | Pregnancy and lactation during anifrolumab treatment in systemic lupus erythematosus.
Dear Editor, Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune disease that predominantly affects women of childbearing age. Cutaneous involvement represents one of the most common clinical manifestations of SLE and may occur in acute, subacute, or chronic forms. SLE disease activity can be assessed with the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and the severity of cutaneous disease activity using the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI). Management of mucocutaneous SLE during pregnancy and breastfeeding remains challenging. In recent years, novel targeted therapies such as anifrolumab, a monoclonal antibody directed against the type I interferon receptor, have shown efficacy in patients with active SLE, particularly in those with prominent mucocutaneous manifestations. [...].
2026-04-27 | Xanthomatous Reactions in Cutaneous Lupus Erythematosus-More Than an Incidental Finding?
Cutaneous lupus erythematosus (CLE) is a well-known autoimmune disorder within dermatology. However, findings of lipid-laden macrophages and xanthomas in a subset of these cases are less understood and usually labeled as a rare, incidental finding. For this reason, a very limited amount of literature has been published regarding the phenomenon. We reviewed 84 cases diagnosed as CLE from 2022 to 2024 from our dermatopathology laboratory. Of the 84 cases of CLE, 9 (11%) demonstrated xanthomatous changes, with 67% sub-classified as discoid lupus and 33% as lupus panniculitis. CLE with xanthomatous reactions may be more common than once thought, as our sampling revealed a prevalence of 11%. Further studies are needed to investigate prevalence as well as possible clinical correlations, as these could influence diagnosis, prognosis, and treatment for this subset of cases.
2026-03-01 | 5PSQ-142 Efficacy, safety and adherence of systemic lupus erythematosus treatment with belimumab supported by a mobile app developed by hospital pharmacy department
Background and Importance
Systemic lupus erythematosus (SLE) is a rare disease with highly variable manifestations. New therapies such as belimumab appear to reduce cumulative organ damage and disease flares, improving patient’s quality of life.
Aim and Objectives
This study aimed to evaluate the effectiveness, safety and adherence of belimumab, supported by a mobile application for pharmacological follow-up.
Material and Methods
A descriptive, observational, retrospective, single-centre study was conducted including all SLE patients treated with belimumab between 2013 and 2024, excluding clinical trials. Demographic, clinical, and biochemical data were collected, while safety and adherence information were obtained through a mobile app developed by the Pharmacy Department. Evaluations were performed at weeks 24, 52, 104, 208, and at study completion. Statistical analyses were conducted using STATA v18.0. Qualitative variables were expressed as percentages (frequency) and quantitative variables as median (interquartile range).
Results
46 patients without renal involvement were included, predominantly female (94.9%), with a mean age of 44.6(12,13) years. The Rheumatology Department prescribed most treatments (64.8%). Median treatment duration was 28.9 (40,21) months. Joint involvement (65.9%) and cutaneous symptoms (45.5%) were most frequent. Anti-dsDNA antibodies were positive in 91.1%. Before belimumab, 71.7% received hydroxychloroquine, 39.1% methotrexate, 26.1% azathioprine, 26.1% mycophenolate, 30.0% rituximab, and 17.4% cyclophosphamide. Baseline SLEDAI was 8.4 (4,15). At week 24, 78.6% achieved SLEDAI <4, increasing to 89.3% at week 52. Corticosteroid reduction was notable: 32.6% used ≤5 mg/day prednisone at baseline versus 78.3% at week 24 and 95.6% by week 208. Complement levels improved (C3 +9.3%; C4 +13.8%) and anti-dsDNA (-28.6%) and IgM (-19.1%) decreased at week 24, with sustained improvement through week 208. Patients provided informed consent to participate via a mobile app that issued medication reminders, recorded adverse reactions in real time, collected quality of life (EQ-5D-5L, 52.2%) and adherence (Morisky–Green, 60.9%) questionnaires, and allowed direct communication with pharmacists 24 hours. 100% patients adhered to the prescribed dosing schedule. The most frequent adverse events, all registered through the app, were recurrent infections (47.9%), headache (26.8%), and allergic reactions (14.1%).
Conclusion and Relevance
Belimumab effectively reduced SLE activity, corticosteroid dependence, and biochemical markers of organ damage. The mobile app improved adherence, quality of life assessment, and the detection and management of adverse reactions.
Conflict of Interest
No conflict of interest
combination
2026-09-08 | Lupus Panniculitis of the Buttock: A Refractory Case With Diagnostic Challenges
Lupus erythematosus panniculitis (LEP) is a rare form of chronic cutaneous lupus involving the subcutaneous fat.Ulcerative lesions are uncommon and may mimic infectious, inflammatory, or neoplastic disorders, leading to delayed diagnosis.We report the case of a 40-year-old woman with longstanding photosensitivity who presented with a painful left gluteal ulcer evolving over eight months.The lesion began as a deep inflammatory nodule and progressively ulcerated.Antibiotic therapy was ineffective.Laboratory tests showed positive antinuclear antibodies at 1:160 and positive anti-Sm/RNP antibodies, with negative anti-double-stranded DNA antibodies.Histopathology revealed lymphocytic lobular panniculitis consistent with lupus panniculitis.Treatment with hydroxychloroquine and systemic corticosteroids resulted in partial improvement, but relapses occurred during steroid tapering.Dapsone failed to control the disease.Methotrexate was subsequently introduced, leading to marked clinical improvement within three months.This case highlights an unusual ulcerative gluteal presentation of lupus panniculitis.Early biopsy and clinicopathological correlation are essential to exclude mimickers and guide appropriate immunomodulatory treatment.
2025-09-18 | Rowell’s syndrome: Literature review on therapeutical options and successful treatment of a case with methotrexate and belimumab combination
Rowells Syndrome (RS) is a rare condition characterized by Erythema Multiforme (EM)–like lesions observed in patients with Systemic Lupus Erythematosus (SLE) or Cutaneous Lupus Erythematosus (CLE), with characteristic histological features on skin biopsy and Direct Immunofluorescence.
small molecules
2026-09-24 | 07 | CHILBLAIN LUPUS: A RARE FORM OF CUTANEOUS LUPUS ERYTHEMATOSUS - A CASE REPORT
Chilblain lupus erythematosus is a rare, cold-induced form of chronic cutaneous lupus that can occur in both genetic and sporadic forms. It is characterized by acral skin lesions and is commonly associated with autoimmunity and type I interferon pathway activation. Diagnosis is based on clinical features and histological findings. While topical and systemic therapies can be effective, the disease is often chronic and relapsing. We describe a case report of a 48-year-old woman with a history of chronic spontaneous urticaria and Raynaud’s phenomenon presented with a history of recurrent nodular lesions on the toes since 2015. The lesions, characterized by a burning sensation and worsening during winter, appeared as erythematous to violaceous plaques with light scaling. Laboratory investigations showed positive antinuclear antibodies (1:160) and anti-Smith antibodies, while cryoglobulins and cold agglutinins were absent. The diagnosis of CHLE was confirmed by skin biopsy, which showed perivascular lymphocytic infiltrate, interface dermatitis, and increased dermal mucin, and by nailfold capillaroscopy, which revealed severe microangiopathy and giant capillary loops. Initial management with hydroxychloroquine (200 mg twice daily), topical tacrolimus 0.1%, and warmth protection resulted in only partial improvement. However, after adding methotrexate (12.5 mg/week) to the regimen, the patient achieved almost complete clinical remission within six months. This stable remission has been maintained for six months to date, highlighting methotrexate as an effective add-on therapy for CHLE cases refractory to antimalarials.
2026-09-18 | Facial discoid lupus erythematosus complicated by a Candida tropicalis fungal granuloma in a patient with rhupus syndrome: a case report of successful treatment with oral itraconazole.
Discoid lupus erythematosus (DLE) is a chronic cutaneous subtype of lupus erythematosus characterized by inflammatory lesions that are highly susceptible to secondary infection, particularly in immunocompromised individuals. Cutaneous fungal granulomas caused by Candida tropicalis and superimposed on pre-existing DLE lesions are exceedingly rare and pose substantial diagnostic challenges. We report the case of a 48-year-old female patient with a 2-year history of rheumatoid arthritis (RA), who presented with facial lupus lesions in January 2024. During her disease course, she met the classification criteria for systemic lupus erythematosus (SLE), leading to a diagnosis of rhupus syndrome (RA-SLE overlap). After eight months of treatment with hydroxychloroquine and prednisone, her cutaneous lesions showed no improvement and instead progressed to nodules and pustules. Histopathological examination of a skin biopsy revealed classic features of DLE accompanied by granulomatous inflammatory infiltrates. Periodic acid-Schiff (PAS) staining and subsequent fungal culture confirmed C. tropicalis as the causative pathogen. The patient was ultimately diagnosed with DLE complicated by secondary C. tropicalis fungal granuloma in the setting of Rhupus syndrome. She received a 3-month course of combination therapy consisting of oral itraconazole (200 mg once daily) plus topical naftifine-ketoconazole cream, which resulted in complete clearance of all facial lesions. No recurrence was observed throughout a 12-month post-treatment follow-up period.
2026-08-14 | Atypical Lupus Erythematosus Profundus of the Flank Evaluated by Serial Computed Tomography and Managed with Hydroxychloroquine: A Case Report.
Lupus erythematosus profundus (LEP) is a rare form of chronic cutaneous lupus erythematosus that predominantly involves the subcutaneous adipose tissue. When it presents at an atypical site, LEP may mimic infection or neoplasia, delaying diagnosis. A 37-year-old woman presented with a progressively enlarging ulcerated indurated lesion on the left flank. An initial biopsy performed at another hospital raised concern for a lymphoproliferative disorder. Computed tomography (CT) demonstrated panniculitic changes in the left flank and additional clinically occult lesions in the right buttock and left thigh. An incisional biopsy from the thigh, selected on the basis of the CT findings, showed interface dermatitis, lymphoplasmacytic panniculitis, dermal mucin deposition, and granular IgM deposition along the dermoepidermal junction, supporting a diagnosis of LEP and arguing against cutaneous lymphoma. Treatment with prednisolone followed by adjunctive hydroxychloroquine (HCQ) led to ulcer healing and radiologic improvement, allowing gradual corticosteroid tapering without relapse to date. This case underscores the importance of considering LEP in atypical deep truncal lesions. In this patient, CT was useful for defining disease extent, identifying clinically occult lesions, selecting a biopsy site, and documenting treatment response. HCQ may also be a useful steroid-sparing adjunct in LEP.
2026-08-13 | Transforming the treatment of autoimmune skin diseases: a journey through biologic therapies.
Autoimmune blistering diseases and autoimmune connective tissue diseases with prominent skin involvement are increasingly managed with biologic and targeted therapies. We review the evidence supporting these approaches and their practical positioning. We performed a PubMed/MEDLINE literature review (inception to February 2026) on biologic therapies for pemphigus, pemphigoid spectrum disorders, LABD, EBA, cutaneous lupus erythematosus, and dermatomyositis, including selected non-biologic small molecules, focusing on validated cutaneous outcomes. Evidence is strongest for B-cell depletion in pemphigus (including dose-optimization strategies) and for IL-4/IL-13 pathway blockade in BP, with additional data for anti-IgE therapy in selected BP phenotypes. In CLE, the most consistent cutaneous datasets involve pDC/type I interferon-axis modulation and BAFF inhibition, largely derived from systemic lupus cohorts; TYK2 inhibition has shown controlled benefit in CLE. In dermatomyositis, CDASI-based evidence is strongest for IVIg, with anti-IFNβ therapy and the TYK2/JAK1 inhibitor brepocitinib (positive in a phase 3 trial) showing cutaneous benefit; other biologics show limited trial signals. Biologic/targeted therapies should be positioned by disease phenotype and evidence strength, prioritizing steroid-sparing strategies in pemphigus and BP and using CLASI/CDASI-anchored outcomes to interpret lupus and dermatomyositis data. Evidence for rare AIBDs remains mostly case-level and should be framed accordingly.
2026-07-28 | Two cases of therapy-refractory chilblain lupus erythematosus successfully treated with deucravacitinib.
Chilblain lupus erythematosus is a rare skin manifestation of lupus erythematosus (LE), which has proven particularly difficult to treat and therefore often presents a great challenge for patients and physicians. We treated two patients with cases of long-lasting, refractory chilblain LE with the selective, allosteric TYK2-inhibitor deucravacitinib. Both patients showed rapid improvement of their respective skin lesions and symptoms. The treatment was generally well tolerated with no severe adverse events. One of the patients experienced an intermittent occurrence of acne lesions. We therefore suggest deucravacitinib as a promising and safe off-label treatment option for chilblain LE.
antibodies
2026-09-01 | Anifrolumab for rituximab-induced SCLE and cryoglobulinemic vasculitis in primary Sjögren's disease: a case report.
Rituximab, a chimeric anti-CD20 monoclonal antibody, is widely used to treat autoimmune diseases and hematologic malignancies through B-cell depletion, yet paradoxical autoimmune phenomena, including drug-induced lupus erythematosus, have been increasingly recognized. While paradoxical autoimmune phenomena following rituximab therapy have been reported, drug-induced subacute cutaneous lupus erythematosus (DI-SCLE) represents an exceedingly rare complication. This case reports rituximab-induced SCLE in a patient with Sjögren's disease and demonstrates the dual therapeutic efficacy of anifrolumab in managing both drug-induced cutaneous lupus and interferon-driven systemic manifestations. A 69-year-old woman with established primary Sjögren's disease and cryoglobulinemic vasculitis developed biopsy-proven subacute cutaneous lupus erythematosus with clinical onset approximately 4 months following rituximab administration, with histopathological confirmation at Month 9. The patient presented with characteristic erythematous lesions on the trunk and back, which subsequently recurred with extension to the face and décolleté; histopathological examination revealed interface dermatitis and a positive lupus band test on direct immunofluorescence. Treatment with anifrolumab (300 mg intravenously every 4 weeks) resulted in complete resolution of rituximab-induced SCLE. Subsequent addition of low-dose mycophenolate mofetil (1,000 mg daily) achieved complete remission of cryoglobulinemic vasculitis, with sustained disease control maintained on this anifrolumab-based regimen. Clinicians should maintain vigilance for paradoxical cutaneous reactions following B-cell-depleting therapies, particularly in patients with underlying Sjögren's disease and anti-Ro/SSA positivity.
2026-08-01 | Anifrolumab use in Jaccoud Arthropathy, Chilblains and Refractory SLE - A Case Report and Literature Review
Background Anifrolumab, an anti-interferon I receptor monoclonal antibody, is the second biologic molecule to be recognized for treatment of active systemic lupus erythematosus (SLE). Jaccoud’s arthropathy (JA) a reducible, non-erosive subtype of musculoskeletal involvement in SLE patients, and Chilblain Lupus Erythematosus (CHLE), a subtype of cutaneous involvement presenting as a spectrum of acral cold-induced lesions that can progress to painful ulcerations, are 2 rare manifestations of SLE with treatment guidelines mostly relying on clinical cases and experts’ opinion. Case Report We report a case of a patient diagnosed with coexisting SLE, CHLE requiring amputation and mutilating JA. Clinical response on all aspects remained suboptimal after multiple lines of treatment including Hydroxychloroquine, high dose Prednisone, Mycophenolate mofetil, Ustekinumab, Azathioprine, Belimumab. She showed positive clinical response to Anifrolumab, with significant reduction of arthralgia and complete resolution of chilblains lesions. This was maintained at 1 year post introduction of Anifrolumab with only complication attributable to this treatment being a cutaneous abscess treated with topical and systemic antibiotics. Following this improvement, we decided to proceed to a narrative review of literature regarding management of both CHLE and JA. Guidelines did not recommend Anifrolumab directly for any of those 2 subtypes of SLE. The line of treatment after Belimumab in the most up to date Cases of both presentations were compiled, with specific interest in patients reported to have received Anifrolumab as part of their treatment regimen. We collected 10 cases of CHLE treated with Anifrolumab. We also found 1 case of JA that demonstrated improvement of arthralgia on Anifrolumab. Most of the patients had also failed to respond to multiple lines of treatment including trial of Belimumab and/or Rituximab. All cases responded completely to Anifrolumab within 8 to 20 weeks. We did not find reports of cases followed for longer intervals. Conclusion With our case, we are participating in the growing weight of evidence that Anifrolumab is a safe and potent treatment option that should be considered in refractory cutaneous lupus, including CHLE, and potentially in refractory arthralgia, even in JA subtypes.
2026-07-14 | Pregnancy and lactation during anifrolumab treatment in systemic lupus erythematosus.
Dear Editor, Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune disease that predominantly affects women of childbearing age. Cutaneous involvement represents one of the most common clinical manifestations of SLE and may occur in acute, subacute, or chronic forms. SLE disease activity can be assessed with the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and the severity of cutaneous disease activity using the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI). Management of mucocutaneous SLE during pregnancy and breastfeeding remains challenging. In recent years, novel targeted therapies such as anifrolumab, a monoclonal antibody directed against the type I interferon receptor, have shown efficacy in patients with active SLE, particularly in those with prominent mucocutaneous manifestations. [...].
2026-04-27 | Xanthomatous Reactions in Cutaneous Lupus Erythematosus-More Than an Incidental Finding?
Cutaneous lupus erythematosus (CLE) is a well-known autoimmune disorder within dermatology. However, findings of lipid-laden macrophages and xanthomas in a subset of these cases are less understood and usually labeled as a rare, incidental finding. For this reason, a very limited amount of literature has been published regarding the phenomenon. We reviewed 84 cases diagnosed as CLE from 2022 to 2024 from our dermatopathology laboratory. Of the 84 cases of CLE, 9 (11%) demonstrated xanthomatous changes, with 67% sub-classified as discoid lupus and 33% as lupus panniculitis. CLE with xanthomatous reactions may be more common than once thought, as our sampling revealed a prevalence of 11%. Further studies are needed to investigate prevalence as well as possible clinical correlations, as these could influence diagnosis, prognosis, and treatment for this subset of cases.
2026-03-01 | 5PSQ-142 Efficacy, safety and adherence of systemic lupus erythematosus treatment with belimumab supported by a mobile app developed by hospital pharmacy department
Background and Importance
Systemic lupus erythematosus (SLE) is a rare disease with highly variable manifestations. New therapies such as belimumab appear to reduce cumulative organ damage and disease flares, improving patient’s quality of life.
Aim and Objectives
This study aimed to evaluate the effectiveness, safety and adherence of belimumab, supported by a mobile application for pharmacological follow-up.
Material and Methods
A descriptive, observational, retrospective, single-centre study was conducted including all SLE patients treated with belimumab between 2013 and 2024, excluding clinical trials. Demographic, clinical, and biochemical data were collected, while safety and adherence information were obtained through a mobile app developed by the Pharmacy Department. Evaluations were performed at weeks 24, 52, 104, 208, and at study completion. Statistical analyses were conducted using STATA v18.0. Qualitative variables were expressed as percentages (frequency) and quantitative variables as median (interquartile range).
Results
46 patients without renal involvement were included, predominantly female (94.9%), with a mean age of 44.6(12,13) years. The Rheumatology Department prescribed most treatments (64.8%). Median treatment duration was 28.9 (40,21) months. Joint involvement (65.9%) and cutaneous symptoms (45.5%) were most frequent. Anti-dsDNA antibodies were positive in 91.1%. Before belimumab, 71.7% received hydroxychloroquine, 39.1% methotrexate, 26.1% azathioprine, 26.1% mycophenolate, 30.0% rituximab, and 17.4% cyclophosphamide. Baseline SLEDAI was 8.4 (4,15). At week 24, 78.6% achieved SLEDAI <4, increasing to 89.3% at week 52. Corticosteroid reduction was notable: 32.6% used ≤5 mg/day prednisone at baseline versus 78.3% at week 24 and 95.6% by week 208. Complement levels improved (C3 +9.3%; C4 +13.8%) and anti-dsDNA (-28.6%) and IgM (-19.1%) decreased at week 24, with sustained improvement through week 208. Patients provided informed consent to participate via a mobile app that issued medication reminders, recorded adverse reactions in real time, collected quality of life (EQ-5D-5L, 52.2%) and adherence (Morisky–Green, 60.9%) questionnaires, and allowed direct communication with pharmacists 24 hours. 100% patients adhered to the prescribed dosing schedule. The most frequent adverse events, all registered through the app, were recurrent infections (47.9%), headache (26.8%), and allergic reactions (14.1%).
Conclusion and Relevance
Belimumab effectively reduced SLE activity, corticosteroid dependence, and biochemical markers of organ damage. The mobile app improved adherence, quality of life assessment, and the detection and management of adverse reactions.
Conflict of Interest
No conflict of interest
combination
2026-09-08 | Lupus Panniculitis of the Buttock: A Refractory Case With Diagnostic Challenges
Lupus erythematosus panniculitis (LEP) is a rare form of chronic cutaneous lupus involving the subcutaneous fat.Ulcerative lesions are uncommon and may mimic infectious, inflammatory, or neoplastic disorders, leading to delayed diagnosis.We report the case of a 40-year-old woman with longstanding photosensitivity who presented with a painful left gluteal ulcer evolving over eight months.The lesion began as a deep inflammatory nodule and progressively ulcerated.Antibiotic therapy was ineffective.Laboratory tests showed positive antinuclear antibodies at 1:160 and positive anti-Sm/RNP antibodies, with negative anti-double-stranded DNA antibodies.Histopathology revealed lymphocytic lobular panniculitis consistent with lupus panniculitis.Treatment with hydroxychloroquine and systemic corticosteroids resulted in partial improvement, but relapses occurred during steroid tapering.Dapsone failed to control the disease.Methotrexate was subsequently introduced, leading to marked clinical improvement within three months.This case highlights an unusual ulcerative gluteal presentation of lupus panniculitis.Early biopsy and clinicopathological correlation are essential to exclude mimickers and guide appropriate immunomodulatory treatment.
2025-09-18 | Rowell’s syndrome: Literature review on therapeutical options and successful treatment of a case with methotrexate and belimumab combination
Rowells Syndrome (RS) is a rare condition characterized by Erythema Multiforme (EM)–like lesions observed in patients with Systemic Lupus Erythematosus (SLE) or Cutaneous Lupus Erythematosus (CLE), with characteristic histological features on skin biopsy and Direct Immunofluorescence.
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Drug Discovery Landscape
1 orphan drug designation for Rare cutaneous lupus erythematosus.
1 orphan drug designation for Rare cutaneous lupus erythematosus.
Drug | Therapy type | Orphan designation | Clinical trial status | Sponsor |
|---|---|---|---|---|
R-salbutamol sulphate | small molecules | EMA | Astion Pharma A/S |
“Terminated” – programs with discontinued clinical trials, or with no updates reported for 5 years after completion or 12 years after the last trial started.
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