RARE DISEASE
Acrodermatitis continua of Hallopeau
Acrodermatitis continua of Hallopeau
Acrodermatitis continua of Hallopeau
Drug discovery
104
papers
On emerging therapies
Overview
Acrodermatitis continua of Hallopeau (ACH) is a rare, chronic pustular psoriasis variant characterized by sterile pustules on digit tips, progressive nail dystrophy (onychodystrophy/anonychia), and potential osteolysis. It typically follows trauma or infection and is resistant to conventional therapies. Classified as localized pustular psoriasis, it causes significant pain and disability due to tissue destruction [1][4][6].
Burden
Chronic disability: 70–80% develop irreversible nail loss; 30–40% progress to osteolysis [1][9][20]
Functional impact: Severe pain (DLQI >20), digit deformities, and impaired fine motor skills [6][9][18]
Psychosocial: High treatment dissatisfaction due to recurrent flares and limited therapeutic options [2][18]
Therapies
First-line: Topical steroids/calcineurin inhibitors ± systemic retinoids (acitretin) or methotrexate [3][14][18]
Biologics: Anti-TNF-α (adalimumab), IL-17/23 inhibitors (secukinumab, ustekinumab), and IL-36R antagonists (spesolimab) show superior response (44–75% efficacy) vs traditional agents [2][5][11][13]
Challenges: Low long-term efficacy (26.3% excellent response); frequent recalcitrance requires therapy escalation [2][3][8]
Categories: rare genetic diseases, rare skin diseases
Research Papers
104 drug discovery papers about Acrodermatitis continua of Hallopeau. Recent publications:
104 drug discovery papers about Acrodermatitis continua of Hallopeau. Recent publications:
categories:
Small molecules
small molecules
2026-08-08 | Marked improvement of isolated acrodermatitis continua of hallopeau with upadacitinib: a case report and literature review.
Acrodermatitis continua of Hallopeau (ACH) is a rare and chronic variant of pustular psoriasis characterized by sterile pustules, periungual inflammation, and progressive nail destruction. Due to its rarity and overlapping clinical features with other nail disorders, ACH is frequently underrecognized and remains difficult to treat, with no standardized therapeutic guidelines. Here, we report a case of long-standing, isolated ACH showing marked improvement after treatment with the selective Janus kinase 1 (JAK1) inhibitor upadacitinib. A 54-year-old man presented with a 13-year history of recurrent periungual erythema, sterile pustules, and progressive nail dystrophy affecting multiple digits. The disease was refractory to topical corticosteroids, systemic antibiotics, acitretin, and 1 year of secukinumab therapy. Following initiation of upadacitinib 15 mg daily, rapid clinical improvement was observed within 4 weeks, with reduction of subungual pustules and periungual inflammation. Nail involvement subsequently improved gradually, accompanied by nail regrowth and a decrease in the modified Nail Psoriasis Severity Index score from 66 at baseline to 16 at week 24 and 13 at month 9. No adverse or unanticipated events were observed during follow-up. This case highlights the potential role of JAK1 inhibition as an effective therapeutic strategy for refractory ACH and supports further investigation into JAK-targeted therapies in pustular psoriasis spectrum disorders.
2026-06-08 | Apremilast for Acrodermatitis Continua of Hallopeau: Report of Two Cases and Review of the Literature.
Acrodermatitis continua of Hallopeau (ACH) is a rare, localized variant of pustular psoriasis characterized by painful paronychia. It primarily affects the distal phalanges and is frequently refractory to standard topical treatments. Establishing an optimal systemic therapy is clinically challenging, particularly for women of childbearing age, given the teratogenicity of retinoids and methotrexate, as well as the adverse effect profiles associated with cyclosporine. Herein, we present two cases of Japanese women in their 30s with recalcitrant ACH who were treated with apremilast. In Case 1, erythema and pain resolved within two months. In Case 2, while skin lesions responded favorably, persistent joint symptoms and headaches necessitated a switch to risankizumab. To evaluate broader utility, we analyzed 10 ACH cases (our two and eight from literature). Clinical efficacy for skin lesions was observed in 90% (9/10), and the response rate for joint pain reached 80% (4/5), with a median response time of 2 months. Notably, apremilast also demonstrated a favorable safety profile even with comorbidities like viral hepatitis. Although 30% of patients experienced mild adverse events (e.g., nausea, headache), the medication was generally well-tolerated. While this review is limited by potential publication bias inherent to case reports, and treatment responses vary, with one study showing limited efficacy, apremilast may be considered among systemic treatment options. Given its non-teratogenic nature and cost-effectiveness compared to biologics, we recommend apremilast as one of the first-line systemic treatment options for ACH patients resistant to topical regimens, especially those of childbearing potential.
2025-12-17 | Clinical phenotype of 504 pustular psoriasis patients from Europe and Egypt: a multi-center observational study.
Pustular psoriasis (PP) comprises a group of rare autoinflammatory skin diseases characterized by sterile pustules. Previous studies have focused on Western or East-Asian cohorts, leaving North African populations underrepresented. This study aimed to describe the demographic and clinical features of a large cohort of European and Egyptian PP patients. We collected data on 504 PP patients from 12 centers across five European countries and Egypt, analyzing their demographic profiles, clinical presentations, and treatment histories. Of 504 PP patients (424 Europeans and 80 Egyptians), 78 (15.5%) had generalized pustular psoriasis (GPP), 374 (74.2%) had palmoplantar pustulosis (PPP), 12 (2.4%) had acrodermatitis continua of Hallopeau (ACH), and 40 (7.9%) had other or overlapping PP subtypes. PPP was more prevalent in Europeans (83.0% vs. 27.5%), whereas GPP was more prevalent in Egyptians (60.0% vs. 7.0%). Europeans included more women (80.0% vs. 39.6% in GPP and 79.4% vs. 22.7% in PPP; both p < 0.001) and were significantly older (59.9 ± 16.8 vs. 36.1 ± 18.6 years in GPP and 62.2 ± 16.4 vs. 40.2 ± 13.8 in PPP; both p < 0.001). European GPP patients had higher rates of psoriasis vulgaris (PV) than Egyptian GPP patients (28.6% vs. 10.4%; p = 0.043). Treatment patterns varied, with cyclosporine being frequently used in Europe, while Egyptians more commonly received methotrexate and steroids. This study highlights significant variations between European and Egyptian PP patients. Europeans presented more commonly with PPP, whereas Egyptian patients had more GPP and fewer coexisting PV diagnoses.
2025-09-30 | A Methodical Approach to a 15-Year Diagnostic Enigma: Unmasking Acrodermatitis Continua of Hallopeau Through Dermoscopy, Histopathology, and Structured Therapeutic Sequencing
Background: Acrodermatitis continua of Hallopeau (ACH) is a rare, debilitating variant of pustular psoriasis. Its profound clinical mimicry of common infections, particularly onychomycosis, often leads to extensive diagnostic delays and ineffective treatments, causing significant patient morbidity. This report details a case with a 15-year history of misdiagnosis, illustrating a structured methodological approach to diagnosis and management. Case presentation: A 40-year-old Indonesian woman presented with a 15-year history of painful pustular lesions and severe onychodystrophy affecting seven digits, refractory to numerous antimicrobial therapies. The diagnostic process was systematically re-evaluated; dermoscopy revealed features inconsistent with onychomycosis (dotted vessels, hemorrhagic spots), prompting a definitive skin and nail matrix biopsy. Histopathology confirmed pustular psoriasis with pathognomonic Kogoj's spongiform pustules and Munro's microabscesses. Treatment was initiated with cyclosporine (3.3 mg/kg/day), leading to rapid remission. However, the development of gingival hyperplasia and hypertension necessitated a transition to weekly methotrexate (15 mg). The patient achieved and maintained clinical remission on this regimen. Conclusion: This case underscores the necessity of a high index of suspicion for ACH in chronic, treatment-resistant nail dystrophy. It demonstrates that a methodical application of dermoscopy and histopathology is indispensable for overcoming clinical mimicry. The main lesson is that structured diagnostic evaluation and sequenced therapy, responsive to adverse events, are crucial for achieving long-term remission and restoring quality of life.
2025-07-09 | Successful management of Acrodermatitis Continua of Hallopeau with Methotrexate: A case report
Acrodermatitis Continua of Hallopeau is a rare, chronic, and localised variant of pustular psoriasis.The recalcitrant nature of the disease accompanied with significant nail and bone destruction poses a therapeutic challenge.Various treatment options of ACH are available however standard guidelines of treatment is not yet available.Methotrexate can be a valuable and cost-effective systemic agent of choice in Acrodermatitis Continua of Hallopeau, particularly in cases refractory to topical therapy, resource-constrained settings where access to bioloic therapies are limited, as evidenced in our patient.
antibodies
2026-07-23 | Genotype-Phenotype Correlations and Clinical Outcomes of Spesolimab in Acrodermatitis Continua of Hallopeau: A Multicenter Retrospective Cohort Study.
To explore genotype-phenotype associations of IL36RN variants and clinical outcomes of spesolimab treatment in acrodermatitis continua of Hallopeau (ACH). This multicenter retrospective study included 14 ACH patients with IL36RN genotyping from 2019 to 2025. Clinical features were compared across all genotypes. Medical records of individuals who received spesolimab were reviewed to describe treatment responses, with a follow-up observation period up to 56 weeks. Compared with monoallelic or wild-type genotypes, biallelic IL36RN variants were associated with earlier disease onset age (23 vs 48 and 47 years, P <0.05) and higher baseline disease severity (median GPPGA score, 4 vs 3 and 2, P <0.001). Among 6 patients with biallelic, refractory ACH who received spesolimab, including 3 isolated cases that were administered at a 450-mg dose, overall percentage improvement across disease activity and patient-reported measures occurred rapidly, reaching 54% at week 1 and 74% at week 4. Relapse occurred in 4 patients between weeks 31 and 44, with disease activity remaining lower than baseline and improvement observed after re-treatment. Spesolimab was associated with clinical improvement in refractory ACH with biallelic IL36RN mutations, including isolated ACH treated with a 450-mg dose, supporting further evaluation of dose individualization below the approved 900-mg regimen for GPP flares.
2026-06-25 | Case Report: Local injection of an IL-17 inhibitor successfully treats Acrodermatitis continua of Hallopeau and avoids immune shift.
Acrodermatitis continua of Hallopeau (ACH) is a refractory, chronic, sterile pustular psoriasis predominantly involving the acral regions. Although biologic agents targeting the interleukin-17 (IL-17) pathway have demonstrated clinical promise, systemic administration may induce eczematous eruptions, known as "immune drift," due to Th17/Th2 immune imbalance. This report presents a novel and successful case, being the first to describe a patient with refractory ACH who was successfully treated with local injections of an IL-17 inhibitor, thereby avoiding immune drift. A 13-year-old female patient with ACH showed poor responses to systemic acitretin, upadacitinib, and topical calcipotriol/betamethasone. After switching to systemic Xeligekimab, her hand and foot pustules improved rapidly, but eczematous eruptions appeared. Subsequently, the treatment was switched to diluted Xeligekimab administered via divided local injections around the lesions on the hands and feet. By week 4, periungual lesions markedly improved; by week 16, erythema and pustules had largely resolved, and scaling significantly improved. The Palmoplantar Pustulosis Area and Severity Index (PPPASI), Dermatology Life Quality Index (DLQI), and Nail Psoriasis Severity Index (NAPSI) all decreased substantially, and no immune drift recurred. This case suggests that local injection of an IL-17 inhibitor may effectively control skin lesions while potentially avoiding systemic immune imbalance, offering a preliminary novel treatment approach for refractory ACH that requires further validation.
2026-06-09 | Image 1_Case Report: Local injection of an IL-17 inhibitor successfully treats Acrodermatitis continua of Hallopeau and avoids immune shift.jpeg
Acrodermatitis continua of Hallopeau (ACH) is a refractory, chronic, sterile pustular psoriasis predominantly involving the acral regions. Although biologic agents targeting the interleukin-17 (IL-17) pathway have demonstrated clinical promise, systemic administration may induce eczematous eruptions, known as “immune drift,” due to Th17/Th2 immune imbalance. This report presents a novel and successful case, being the first to describe a patient with refractory ACH who was successfully treated with local injections of an IL-17 inhibitor, thereby avoiding immune drift. A 13-year-old female patient with ACH showed poor responses to systemic acitretin, upadacitinib, and topical calcipotriol/betamethasone. After switching to systemic Xeligekimab, her hand and foot pustules improved rapidly, but eczematous eruptions appeared. Subsequently, the treatment was switched to diluted Xeligekimab administered via divided local injections around the lesions on the hands and feet. By week 4, periungual lesions markedly improved; by week 16, erythema and pustules had largely resolved, and scaling significantly improved. The Palmoplantar Pustulosis Area and Severity Index (PPPASI), Dermatology Life Quality Index (DLQI), and Nail Psoriasis Severity Index (NAPSI) all decreased substantially, and no immune drift recurred. This case suggests that local injection of an IL-17 inhibitor may effectively control skin lesions while potentially avoiding systemic immune imbalance, offering a preliminary novel treatment approach for refractory ACH that requires further validation.
2026-03-19 | Case Report: Periungual Xeligekimab injection for refractory Acrodermatitis continua of Hallopeau.
This case report presents the novel and successful use of periungual Xeligekimab injections for managing refractory Acrodermatitis Continua of Hallopeau (ACH), representing the first documented application of this localized administration route for the IL-17A inhibitor. It contributes to the scientific literature by demonstrating a promising alternative therapeutic strategy for treatment-resistant ACH, highlighting the potential for enhanced local efficacy and minimized systemic exposure. The patient was a 36-year-old woman with a two-decade history of ACH affecting her digits, characterized by persistent periungual and subungual erythema, sterile pustules, significant nail plate dystrophy with thickening and fragmentation, and associated digital swelling, tenderness, and restricted motion. Prior therapies, including topical corticosteroids, phototherapy, and systemic tofacitinib, had proven ineffective. Diagnosis was confirmed clinically and supported by MRI, which revealed active bone marrow edema in the distal phalanges. The therapeutic intervention involved initial periungual injections of a diluted Xeligekimab formulation, which led to partial improvement. This was followed by a series of injections using undiluted Xeligekimab (100 mg/mL). This escalation resulted in marked clinical resolution: complete clearance of pustules and inflammation, healthy nail regrowth, and resolution of digital swelling and pain. Concurrently, the Dermatology Life Quality Index and pain scores dramatically improved, and follow-up MRI showed substantial resolution of the underlying bone marrow edema, confirming the treatment's efficacy on both cutaneous and deep osseous inflammation.
2026-01-23 | Genotype–Phenotype Correlations and Clinical Outcomes of Spesolimab in Acrodermatitis Continua of Hallopeau: A Multicenter Retrospective Cohort Study
This is the tables and clinical figures supplemented in this article：Genotype–Phenotype Correlations and Clinical Outcomes of Spesolimab in Acrodermatitis Continua of Hallopeau: A Multicenter Retrospective Cohort Study
small molecules
2026-08-08 | Marked improvement of isolated acrodermatitis continua of hallopeau with upadacitinib: a case report and literature review.
Acrodermatitis continua of Hallopeau (ACH) is a rare and chronic variant of pustular psoriasis characterized by sterile pustules, periungual inflammation, and progressive nail destruction. Due to its rarity and overlapping clinical features with other nail disorders, ACH is frequently underrecognized and remains difficult to treat, with no standardized therapeutic guidelines. Here, we report a case of long-standing, isolated ACH showing marked improvement after treatment with the selective Janus kinase 1 (JAK1) inhibitor upadacitinib. A 54-year-old man presented with a 13-year history of recurrent periungual erythema, sterile pustules, and progressive nail dystrophy affecting multiple digits. The disease was refractory to topical corticosteroids, systemic antibiotics, acitretin, and 1 year of secukinumab therapy. Following initiation of upadacitinib 15 mg daily, rapid clinical improvement was observed within 4 weeks, with reduction of subungual pustules and periungual inflammation. Nail involvement subsequently improved gradually, accompanied by nail regrowth and a decrease in the modified Nail Psoriasis Severity Index score from 66 at baseline to 16 at week 24 and 13 at month 9. No adverse or unanticipated events were observed during follow-up. This case highlights the potential role of JAK1 inhibition as an effective therapeutic strategy for refractory ACH and supports further investigation into JAK-targeted therapies in pustular psoriasis spectrum disorders.
2026-06-08 | Apremilast for Acrodermatitis Continua of Hallopeau: Report of Two Cases and Review of the Literature.
Acrodermatitis continua of Hallopeau (ACH) is a rare, localized variant of pustular psoriasis characterized by painful paronychia. It primarily affects the distal phalanges and is frequently refractory to standard topical treatments. Establishing an optimal systemic therapy is clinically challenging, particularly for women of childbearing age, given the teratogenicity of retinoids and methotrexate, as well as the adverse effect profiles associated with cyclosporine. Herein, we present two cases of Japanese women in their 30s with recalcitrant ACH who were treated with apremilast. In Case 1, erythema and pain resolved within two months. In Case 2, while skin lesions responded favorably, persistent joint symptoms and headaches necessitated a switch to risankizumab. To evaluate broader utility, we analyzed 10 ACH cases (our two and eight from literature). Clinical efficacy for skin lesions was observed in 90% (9/10), and the response rate for joint pain reached 80% (4/5), with a median response time of 2 months. Notably, apremilast also demonstrated a favorable safety profile even with comorbidities like viral hepatitis. Although 30% of patients experienced mild adverse events (e.g., nausea, headache), the medication was generally well-tolerated. While this review is limited by potential publication bias inherent to case reports, and treatment responses vary, with one study showing limited efficacy, apremilast may be considered among systemic treatment options. Given its non-teratogenic nature and cost-effectiveness compared to biologics, we recommend apremilast as one of the first-line systemic treatment options for ACH patients resistant to topical regimens, especially those of childbearing potential.
2025-12-17 | Clinical phenotype of 504 pustular psoriasis patients from Europe and Egypt: a multi-center observational study.
Pustular psoriasis (PP) comprises a group of rare autoinflammatory skin diseases characterized by sterile pustules. Previous studies have focused on Western or East-Asian cohorts, leaving North African populations underrepresented. This study aimed to describe the demographic and clinical features of a large cohort of European and Egyptian PP patients. We collected data on 504 PP patients from 12 centers across five European countries and Egypt, analyzing their demographic profiles, clinical presentations, and treatment histories. Of 504 PP patients (424 Europeans and 80 Egyptians), 78 (15.5%) had generalized pustular psoriasis (GPP), 374 (74.2%) had palmoplantar pustulosis (PPP), 12 (2.4%) had acrodermatitis continua of Hallopeau (ACH), and 40 (7.9%) had other or overlapping PP subtypes. PPP was more prevalent in Europeans (83.0% vs. 27.5%), whereas GPP was more prevalent in Egyptians (60.0% vs. 7.0%). Europeans included more women (80.0% vs. 39.6% in GPP and 79.4% vs. 22.7% in PPP; both p < 0.001) and were significantly older (59.9 ± 16.8 vs. 36.1 ± 18.6 years in GPP and 62.2 ± 16.4 vs. 40.2 ± 13.8 in PPP; both p < 0.001). European GPP patients had higher rates of psoriasis vulgaris (PV) than Egyptian GPP patients (28.6% vs. 10.4%; p = 0.043). Treatment patterns varied, with cyclosporine being frequently used in Europe, while Egyptians more commonly received methotrexate and steroids. This study highlights significant variations between European and Egyptian PP patients. Europeans presented more commonly with PPP, whereas Egyptian patients had more GPP and fewer coexisting PV diagnoses.
2025-09-30 | A Methodical Approach to a 15-Year Diagnostic Enigma: Unmasking Acrodermatitis Continua of Hallopeau Through Dermoscopy, Histopathology, and Structured Therapeutic Sequencing
Background: Acrodermatitis continua of Hallopeau (ACH) is a rare, debilitating variant of pustular psoriasis. Its profound clinical mimicry of common infections, particularly onychomycosis, often leads to extensive diagnostic delays and ineffective treatments, causing significant patient morbidity. This report details a case with a 15-year history of misdiagnosis, illustrating a structured methodological approach to diagnosis and management. Case presentation: A 40-year-old Indonesian woman presented with a 15-year history of painful pustular lesions and severe onychodystrophy affecting seven digits, refractory to numerous antimicrobial therapies. The diagnostic process was systematically re-evaluated; dermoscopy revealed features inconsistent with onychomycosis (dotted vessels, hemorrhagic spots), prompting a definitive skin and nail matrix biopsy. Histopathology confirmed pustular psoriasis with pathognomonic Kogoj's spongiform pustules and Munro's microabscesses. Treatment was initiated with cyclosporine (3.3 mg/kg/day), leading to rapid remission. However, the development of gingival hyperplasia and hypertension necessitated a transition to weekly methotrexate (15 mg). The patient achieved and maintained clinical remission on this regimen. Conclusion: This case underscores the necessity of a high index of suspicion for ACH in chronic, treatment-resistant nail dystrophy. It demonstrates that a methodical application of dermoscopy and histopathology is indispensable for overcoming clinical mimicry. The main lesson is that structured diagnostic evaluation and sequenced therapy, responsive to adverse events, are crucial for achieving long-term remission and restoring quality of life.
2025-07-09 | Successful management of Acrodermatitis Continua of Hallopeau with Methotrexate: A case report
Acrodermatitis Continua of Hallopeau is a rare, chronic, and localised variant of pustular psoriasis.The recalcitrant nature of the disease accompanied with significant nail and bone destruction poses a therapeutic challenge.Various treatment options of ACH are available however standard guidelines of treatment is not yet available.Methotrexate can be a valuable and cost-effective systemic agent of choice in Acrodermatitis Continua of Hallopeau, particularly in cases refractory to topical therapy, resource-constrained settings where access to bioloic therapies are limited, as evidenced in our patient.
antibodies
2026-07-23 | Genotype-Phenotype Correlations and Clinical Outcomes of Spesolimab in Acrodermatitis Continua of Hallopeau: A Multicenter Retrospective Cohort Study.
To explore genotype-phenotype associations of IL36RN variants and clinical outcomes of spesolimab treatment in acrodermatitis continua of Hallopeau (ACH). This multicenter retrospective study included 14 ACH patients with IL36RN genotyping from 2019 to 2025. Clinical features were compared across all genotypes. Medical records of individuals who received spesolimab were reviewed to describe treatment responses, with a follow-up observation period up to 56 weeks. Compared with monoallelic or wild-type genotypes, biallelic IL36RN variants were associated with earlier disease onset age (23 vs 48 and 47 years, P <0.05) and higher baseline disease severity (median GPPGA score, 4 vs 3 and 2, P <0.001). Among 6 patients with biallelic, refractory ACH who received spesolimab, including 3 isolated cases that were administered at a 450-mg dose, overall percentage improvement across disease activity and patient-reported measures occurred rapidly, reaching 54% at week 1 and 74% at week 4. Relapse occurred in 4 patients between weeks 31 and 44, with disease activity remaining lower than baseline and improvement observed after re-treatment. Spesolimab was associated with clinical improvement in refractory ACH with biallelic IL36RN mutations, including isolated ACH treated with a 450-mg dose, supporting further evaluation of dose individualization below the approved 900-mg regimen for GPP flares.
2026-06-25 | Case Report: Local injection of an IL-17 inhibitor successfully treats Acrodermatitis continua of Hallopeau and avoids immune shift.
Acrodermatitis continua of Hallopeau (ACH) is a refractory, chronic, sterile pustular psoriasis predominantly involving the acral regions. Although biologic agents targeting the interleukin-17 (IL-17) pathway have demonstrated clinical promise, systemic administration may induce eczematous eruptions, known as "immune drift," due to Th17/Th2 immune imbalance. This report presents a novel and successful case, being the first to describe a patient with refractory ACH who was successfully treated with local injections of an IL-17 inhibitor, thereby avoiding immune drift. A 13-year-old female patient with ACH showed poor responses to systemic acitretin, upadacitinib, and topical calcipotriol/betamethasone. After switching to systemic Xeligekimab, her hand and foot pustules improved rapidly, but eczematous eruptions appeared. Subsequently, the treatment was switched to diluted Xeligekimab administered via divided local injections around the lesions on the hands and feet. By week 4, periungual lesions markedly improved; by week 16, erythema and pustules had largely resolved, and scaling significantly improved. The Palmoplantar Pustulosis Area and Severity Index (PPPASI), Dermatology Life Quality Index (DLQI), and Nail Psoriasis Severity Index (NAPSI) all decreased substantially, and no immune drift recurred. This case suggests that local injection of an IL-17 inhibitor may effectively control skin lesions while potentially avoiding systemic immune imbalance, offering a preliminary novel treatment approach for refractory ACH that requires further validation.
2026-06-09 | Image 1_Case Report: Local injection of an IL-17 inhibitor successfully treats Acrodermatitis continua of Hallopeau and avoids immune shift.jpeg
Acrodermatitis continua of Hallopeau (ACH) is a refractory, chronic, sterile pustular psoriasis predominantly involving the acral regions. Although biologic agents targeting the interleukin-17 (IL-17) pathway have demonstrated clinical promise, systemic administration may induce eczematous eruptions, known as “immune drift,” due to Th17/Th2 immune imbalance. This report presents a novel and successful case, being the first to describe a patient with refractory ACH who was successfully treated with local injections of an IL-17 inhibitor, thereby avoiding immune drift. A 13-year-old female patient with ACH showed poor responses to systemic acitretin, upadacitinib, and topical calcipotriol/betamethasone. After switching to systemic Xeligekimab, her hand and foot pustules improved rapidly, but eczematous eruptions appeared. Subsequently, the treatment was switched to diluted Xeligekimab administered via divided local injections around the lesions on the hands and feet. By week 4, periungual lesions markedly improved; by week 16, erythema and pustules had largely resolved, and scaling significantly improved. The Palmoplantar Pustulosis Area and Severity Index (PPPASI), Dermatology Life Quality Index (DLQI), and Nail Psoriasis Severity Index (NAPSI) all decreased substantially, and no immune drift recurred. This case suggests that local injection of an IL-17 inhibitor may effectively control skin lesions while potentially avoiding systemic immune imbalance, offering a preliminary novel treatment approach for refractory ACH that requires further validation.
2026-03-19 | Case Report: Periungual Xeligekimab injection for refractory Acrodermatitis continua of Hallopeau.
This case report presents the novel and successful use of periungual Xeligekimab injections for managing refractory Acrodermatitis Continua of Hallopeau (ACH), representing the first documented application of this localized administration route for the IL-17A inhibitor. It contributes to the scientific literature by demonstrating a promising alternative therapeutic strategy for treatment-resistant ACH, highlighting the potential for enhanced local efficacy and minimized systemic exposure. The patient was a 36-year-old woman with a two-decade history of ACH affecting her digits, characterized by persistent periungual and subungual erythema, sterile pustules, significant nail plate dystrophy with thickening and fragmentation, and associated digital swelling, tenderness, and restricted motion. Prior therapies, including topical corticosteroids, phototherapy, and systemic tofacitinib, had proven ineffective. Diagnosis was confirmed clinically and supported by MRI, which revealed active bone marrow edema in the distal phalanges. The therapeutic intervention involved initial periungual injections of a diluted Xeligekimab formulation, which led to partial improvement. This was followed by a series of injections using undiluted Xeligekimab (100 mg/mL). This escalation resulted in marked clinical resolution: complete clearance of pustules and inflammation, healthy nail regrowth, and resolution of digital swelling and pain. Concurrently, the Dermatology Life Quality Index and pain scores dramatically improved, and follow-up MRI showed substantial resolution of the underlying bone marrow edema, confirming the treatment's efficacy on both cutaneous and deep osseous inflammation.
2026-01-23 | Genotype–Phenotype Correlations and Clinical Outcomes of Spesolimab in Acrodermatitis Continua of Hallopeau: A Multicenter Retrospective Cohort Study
This is the tables and clinical figures supplemented in this article：Genotype–Phenotype Correlations and Clinical Outcomes of Spesolimab in Acrodermatitis Continua of Hallopeau: A Multicenter Retrospective Cohort Study
Access all drug discovery papers and probability of success in trials forecasts:
Access all drug discovery papers and probability of success in trials forecasts:
Drug Discovery Landscape
0 orphan drug designations.
0 orphan drug designations.
Let's accelerate rare disease drug discovery
Let's accelerate drug discovery
Get access to Explority AI’s forecasts to outperform average preclinical success rates. Whether you’re expanding your R&D pipeline, evaluating a partnership, or simply have a question — we’d love to hear from you.