
Introduction: Bullous pemphigoid (BP) is the most common autoimmune blistering disorder affecting the elderly population. While the etiology is predominantly idiopathic, physical trauma has been identified as a potential trigger. Most trauma-induced cases reported in the literature are associated with surgical incisions, burns, or open wounds. The development of generalized BP following a closed fracture, where the skin integrity remains initially intact, is an exceptionally rare phenomenon. Case Report: We report the case of an 85-year-old woman who presented with a generalized blistering eruption three months after sustaining a closed fracture of the left radius. Physical examination revealed tense, fluid-filled bullae on an erythematous base involving the trunk and extremities. Histopathological analysis demonstrated a subepidermal blister with eosinophilic infiltration. The diagnosis was confirmed by direct immunofluorescence showing linear deposition of IgG and C3 at the dermo-epidermal junction, and enzyme-linked immunosorbent assay (ELISA) detecting high titers of anti-BP180 autoantibodies. The patient was treated with a tapering course of systemic prednisone, initiated at 90 mg/day. This regimen led to the cessation of new blister formation within two weeks and complete clinical remission with normalization of inflammatory markers within three weeks. Conclusion: This case highlights that closed skeletal trauma, even without surgical intervention, can serve as a precipitating factor for generalized BP. The mechanism likely involves the release of sequestered antigens and epitope spreading due to internal tissue damage. Clinicians should maintain a high index of suspicion for BP in elderly patients presenting with cutaneous eruptions following orthopedic injuries to ensure prompt diagnosis and effective management.
Pagetoid Reticulosis (PR) is a rare variant of mycosis fungoides, characterized by localized patches or plaques mainly on the distal extremities with intraepidermal proliferation of neoplastic T cells. Typically, PR exhibits a CD4-positive, CD8-negative immunophenotype, whereas a CD4–/CD8– double-negative profile is distinctly uncommon, yet in localized pagetoid reticulosis it may be associated with an indolent clinical course and favorable therapeutic response. In this report, we present a case of a healthy 58-year-old male with a three-year history of a persistent erythematous plaque on the left forearm. Histopathological analysis demonstrated atypical lymphocytes with prominent epidermotropism. Immunohistochemical staining showed double-negative CD4/CD8 phenotype with positive CD3, CD5, and CD7. The lesion responded completely to topical therapy followed by localized radiotherapy, with clinical remission at follow-up.
Introduction Alopecia areata (AA) is an autoimmune hair loss disorder often triggered by genetic and environmental factors. Acute, massive shedding is unusual and may indicate a potent immune trigger. Case Presentation We report a 34-year-old woman with Hashimoto thyroiditis who developed rapid, diffuse AA ten days after bee stings. Clinical examination showed marked hair loss (SALT score 30→70) affecting scalp, eyebrows, and eyelashes. Laboratory workup revealed elevated IgE and venom-specific hypersensitivity. Treatment with corticosteroid pulses followed by oral methylprednisolone and methotrexate led to progressive hair regrowth. Conclusion This case highlights Hymenoptera envenomation as a rare trigger of acute AA, emphasizing the importance of environmental history and immune evaluation in sudden-onset autoimmune hair loss.
Introduction: Generalized extragenital lichen sclerosus with morphea overlap is a rare inflammatory and fibrosing dermatosis. Severe bullous-ulcerative disease remains therapeutically challenging and can cause major morbidity. Case Presentation: We report a 54-year-old woman with decade-long progressive generalized extragenital lichen sclerosus-morphea overlap, causing disabling hand sclerosis and widespread painful bullous-ulcerative leg lesions. Conventional first-line and extended immunomodulatory or immunosuppressive treatments, including potent topical corticosteroids, UVA-1 phototherapy, extracorporeal photopheresis, methotrexate, hydroxychloroquine, multiple biologics, and JAK inhibitors, showed little sustained benefit and were sometimes associated with deterioration or relevant immunosuppression. Intravenous immunoglobulin, initiated for secondary antibody deficiency, was associated with disease stabilization and complete ulcer healing; rituximab was subsequently continued as part of B-cell-directed background therapy. Persistent cutaneous inflammation under IVIG and rituximab was accompanied by elevated interferon-associated mediators, including interleukin-18, CXCL9, and CXCL10. Addition of anifrolumab, a type I interferon receptor antagonist, led to marked suppression of inflammatory activity, softening of sclerotic skin, normalization or reduction of several cytokine and chemokine abnormalities, and improved quality of life. Conclusion: In severe therapy-refractory lichen sclerosus-morphea overlap, combined IVIG, rituximab, and anifrolumab may help control skin inflammation, sclerosis and skin ulceration. This case supports cytokine-guided, mechanism-based salvage therapy in selected therapy-refractory patients.
Introduction: Cutaneous angiosarcoma (cAS) is a rare and aggressive malignancy of endothelial origin, most commonly affecting the scalp and face in elderly patients. Diagnosis is often challenging due to histological overlap with other vascular or epithelioid tumors. Secondary cAS typically arises following radiotherapy or chronic lymphedema. Case Presentation: We report a rare case of secondary cAS in a 33-year-old woman developing from a congenital giant abdominal hemangioma, without prior radiation or lymphedema. The lesion progressed from erythema to a large non-healing ulcer. Histopathology revealed poorly differentiated vascular tumor cells, confirmed by immunohistochemistry showing positivity for CD31, ERG, and FLI-1. Due to the extensive lesion, surgical excision was not feasible, and only supportive care was provided. Conclusion: This case emphasizes the importance of early recognition and biopsy in atypical vascular lesions. Secondary cAS may arise from pre-existing vascular anomalies even in the absence of classic risk factors, highlighting the need for individualized management.
Introduction:Acne vulgaris may lead to persistent sequelae, including post-inflammatory erythema, hyperpigmentation, and atrophic scarring. Multimodal regenerative approaches combining autologous biological therapies with light-based devices are increasingly used in clinical practice, although published clinical evidence remains limited. Case Presentations:We report 3 female patients with distinct acne-related skin sequelae: (1) post-inflammatory erythema with active inflammatory lesions, (2) post-inflammatory hyperpigmentation with residual acne activity, and (3) atrophic acne scarring. Each patient was treated with a tailored multimodal protocol combining photothermal biomodulated autologous platelet-rich plasma (PRP), laser therapy (non-ablative or ablative erbium-doped yttrium aluminum garnet), and light-emitting diode (LED) phototherapy. Clinical outcomes were retrospectively assessed using serial clinical photographs obtained during routine follow-up. Where image quality allowed, approximate inflammatory lesion counts and semi-quantitative ordinal estimates of erythema, hyperpigmentation, scar visibility, and texture change were added post hoc. Follow-up ranged from 1 month to 12 months. All 3 patients showed photographic improvement in inflammatory activity, pigmentation, skin texture, and/or scar appearance at available follow-up time points. Treatments were well tolerated, with only transient local reactions such as redness and swelling. Conclusions:These case reports illustrate the feasibility and tolerability of combining photothermal biomodulated autologous PRP with laser and LED therapies for different acne sequelae. Because no validated objective scoring system was applied prospectively, the findings should be interpreted as descriptive and hypothesis generating. These observations support further controlled studies to evaluate standardized protocols and clinical outcomes.
Introduction:SPINK5-syndromic epidermal differentiation disorder (also known as Netherton syndrome [NS]) is a severe chronic genetic disorder characterized by skin inflammation, severe atopy, and trichorrhexis invaginata (TI). Causative SPINK5 gene mutations result in defective serine protease inhibitor LEKTI, leading to unopposed serine protease activity and resulting in impaired skin barrier and type 2 inflammation. TI is the result of an intermittent keratinization defect of the hair cortex, a problem without specific treatment available to date. Case Presentation:We diagnosed NS in a 67-year-old woman who had severe skin manifestations and a hair-growth defect since childhood. Scalp hair was sparse in the nuchal and temporoparietal regions. Trichoscopy of these areas showed numerous "bamboo" hairs that broke off during contact with the dermatoscope. We initiated treatment with dupilumab and optimized the topical therapy. At a 3-month follow-up, improvement of rash, pruritus, and quality of life was reported. Furthermore, the previously brittle short hair was replaced with longer hair and normal hair shafts in the previously affected areas. Conclusions:TI, as part of NS, has a variable presentation, biologics currently treating cutaneous inflammation, but little is known about their effects on hair biology. In this report, we show that dupilumab treatment not only proved efficient for control of skin inflammation and pruritus but was also associated with near-complete resolution of "bamboo" hair and promoted hair growth. Especially how alterations in the inflammatory response in NS can be linked to a structural defect have not been addressed in the current literature so far.
Introduction:Sclerodactyly is commonly associated with systemic sclerosis (SSc), occurring alongside Raynaud's phenomenon and disease-specific autoantibodies. The combination of occupational silica exposure and SSc is known as Erasmus syndrome. However, isolated digital fibrosis in silica-exposed individuals without systemic features or SSc-specific autoantibody positivity has not yet been described. Case Presentation:A 40-year-old stonemason presented with a 6-month history of progressive fingertip sclerosis, fissuring, and pain, in combination with hand dermatitis, which was initially diagnosed and treated as hand dermatitis alone. Examination revealed distal digital tapering, loss of skin markings, telangiectasia, and abnormal nailfold capillaries, together with dermatitis, without Raynaud's phenomenon or systemic involvement. High-resolution computed tomography demonstrated upper-lobe-predominant pulmonary nodules consistent with chronic silicosis. Autoimmune serology showed a weakly positive antinuclear antibody of 1:80 with negative SSc-specific autoantibodies. Under the 2013 ACR-EULAR criteria, the patient did not meet the diagnostic threshold for SSc. Dermatitis improved with exposure cessation and topical tacrolimus therapy, while sclerodactyly persisted. Conclusion:This case highlights isolated sclerodactyly as a potential manifestation of silica exposure in the absence of SSc or disease-specific autoantibodies. In this case, it permitted earlier detection and management of silicosis. Given the potential for progression to SSc, early identification and multidisciplinary surveillance may help prevent progression and associated morbidity.
Introduction: En coup de sabre (ECDS) and Parry-Romberg syndrome (PRS) are rare, localised scleroderma subtypes, often considered to be overlapping variants of morphea. Hemi masticatory spasm (HMS), a distinct rare neuromuscular disorder characterised by unilateral paroxysmal contraction of the jaw muscles, has rarely been reported in association with these conditions. Case Presentation: A 20-year-old man presented with a linear, cicatricial alopecic patch over the left parietal scalp, along with indurated and bound-down skin over the left cheek. This was followed by progressive depression and asymmetry of the left midface, accompanied by paroxysmal spasms during mastication and swallowing. Examination revealed cicatricial alopecia over the left parietal region and an atrophic plaque over the ipsilateral cheek. The left lower lip and tongue were atrophic, while the ipsilateral masseter muscle was visibly hypertrophied. Histopathological findings of the cheek lesion were consistent with morphea. A clinical diagnosis of co-existing ECDS, PRS, and HMS was made. Botulinum toxin type A was used to treat HMS, with marked reduction in symptoms and improvement in quality of life. Conclusion: This case highlights the rare but meaningful association between ECDS, PRS, and HMS, underscoring the importance of early recognition and multidisciplinary intervention. Botulinum toxin remains a valuable therapeutic option for managing symptomatic HMS in such contexts.
Introduction: Juvenile dermatomyositis (JDM) is a rare autoimmune inflammatory myopathy of childhood. Initial manifestations may be atypical and lead to delays in diagnosis. Case report: An 11-year-old boy with a history of trauma of the left knee was initially treated with systemic antibiotics without clinical improvement. On physical examination, ulcerated plaques composed of multiple fistulous tracts with abundant purulent discharge were observed. A skin biopsy revealed findings consistent with calcinosis cutis. The autoantibody panel was positive for anti-Mi-2 antibodies, along with elevated muscle enzymes (CK = 2892 U/L). A diagnosis of JDM was established, and systemic treatment with prednisone at 1 mg/kg/day was initiated, resulting in an adequate clinical response of the lesions. Conclusion: JDM is a rare condition with a variable clinical presentation in the pediatric population. This case highlights the importance of considering JDM in the differential diagnosis of patients presenting with calcinosis, even in the absence of classic cutaneous manifestations and with only subtle or initially unrecognized muscle involvement.
Introduction: Extramammary Paget’s disease (EMPD) is a rare cutaneous adenocarcinoma that typically arises in apocrine gland-rich areas of the skin (genital, perianal, axillary regions) of older adults. Clinically, it often presents as a chronic eczematous or pruritic plaque that can be mistaken for benign dermatoses. Case Report: We report a 65-year-old postmenopausal woman with a 3-year history of a slowly expanding, itchy perineal plaque. The lesion was initially misdiagnosed as dermatitis and treated unsuccessfully with topical steroids, antifungals, and antibiotics. Examination revealed an indurated, verrucous plaque on the perineum with areas of flesh-colored, hypopigmented, and violaceous discoloration. There were no lymphadenopathy or other lesions. A punch biopsy showed epidermal hyperkeratosis and acanthosis with scattered pagetoid cells (large pale pleomorphic cells with atypical nuclei and mitoses) throughout the epidermis. Immunohistochemistry demonstrated tumor cell positivity for cytokeratin 7 (CK7), carcinoembryonic antigen (CEA), gross cystic disease fluid protein-15 (GCDFP-15), and HER2, consistent with primary EMPD. Metastatic workup, including pelvic MRI and colonoscopy, revealed no underlying internal malignancy. The patient underwent wide local excision with clear margins. Conclusion: This case highlights the importance of considering EMPD in chronic refractory anogenital dermatoses of the elderly. The CARE checklist was utilized in the writing of this case. Early skin biopsy and histopathologic examination are crucial for diagnosis. Definitive treatment with surgical excision offers the best chance for cure, and thorough evaluation for underlying carcinoma is essential in guiding management and follow-up.
Introduction:Punctate porokeratosis (PK) is a rare variant of PK characterized by small, keratotic papules on the palms and soles. It may occur in adolescence or early adulthood, and it is diagnosed mainly based on the histopathological findings of a cornoid lamella. In this report, we described a case with atypically larger papules and distinct dermoscopic features. Case Presentation:A 36-year-old woman presented with a 1-year history of asymptomatic hyperkeratotic papules on both hands. Physical examination showed multiple, discrete, translucent, hyperkeratotic papules measuring 2-8 mm in diameter (larger than the classic papules of 1-2 mm in diameter) on the palms and fingers. Dermoscopic examination revealed a light red background with yellowish-white scales and irregular vascular structures, whereas the typical "double-track" sign was absent. Conclusion:Histopathological examination is essential for diagnosing punctate PK, particularly in cases where lesions are unusually large or dermoscopic presentations are atypical.
Introduction: Martorell hypertensive ischemic leg ulcers (HYTILUs) is a rare, painful, and rapidly progressive lower extremity ulcer associated with long-standing, poorly controlled systemic hypertension. It most commonly affects obese women between the fifth and seventh decades of life and is characterized by microvascular arteriolosclerosis with preserved distal pulses. Diagnostic delay is frequent due to clinical overlap with venous ulceration, calciphylaxis, vasculitis, and pyoderma gangrenosum. Case Presentation: A 27-year-old obese African American man with uncontrolled hypertension presented with a 2-year history of bilateral, painful, non-healing lower extremity ulcers. The lesions began as erythematous indurated plaques that progressively ulcerated despite compression therapy, debridement, and enzymatic wound care. On examination, he had deep, punched-out necrotic ulcers on the dorsal feet and toes, marked non-pitting edema, intact pedal pulses, and severe hypertension (210/104 mm Hg). Extensive laboratory, microbiologic, and vascular investigations were unremarkable, and Doppler studies excluded significant arterial or venous occlusive disease. Histopathology was nondiagnostic but did not support vasculitis or infection. Further evaluation revealed decompensated systolic heart failure with a left ventricular ejection fraction of 27% due to idiopathic cardiomyopathy. Following aggressive cardiovascular optimization and diuresis resulting in substantial fluid loss, the patient’s edema and ulcerations improved markedly with conservative wound care alone. Notably, he developed spontaneous auto-amputation of the distal phalanx of the right second toe, a complication not previously described in association with HYTILU. Conclusion: This case highlights an unusual demographic presentation of HYTILU in a young male with severe systolic heart failure and documents digital auto-amputation as a novel complication. The patient’s clinical course underscores the central role of uncontrolled hypertension and microvascular compromise in disease pathogenesis, as well as the importance of addressing systemic cardiovascular pathology to achieve wound healing. Early recognition of HYTILU and differentiation from mimickers such as calciphylaxis and pyoderma gangrenosum are essential as management strategies differ substantially. Maintaining a high index of suspicion in atypical populations may prevent misdiagnosis, inappropriate interventions, and irreversible tissue loss.
Introduction: Xeroderma pigmentosum (XP) is a group of genodermatoses with autosomal recessive inheritance, comprising 9 subtypes designated A to J plus a variant (V) type, clinically characterized by increased photosensitivity, with specific subtypes more prone to ocular disease and progressive neurodegeneration. Case presentation: Three patients with the XP-V variant, a pair of sisters and an unrelated individual, presented with a novel homozygous c.1245-1G>A intronic splice site variant in the POLH gene classified as likely pathogenic. They exhibited numerous freckles and hypo- and hyperpigmented lesions that evolved into basal cell and squamous cell carcinomas, as well as thin malignant melanocytic lesions, photophobia, conjunctival telangiectasis, ectropion, and pterygia, without neurological symptoms. Conclusion: The possibility of dealing with a new founder effect for this specific variant in the State of Minas Gerais, in the Southeast region of Brazil, is suggested.
Introduction: 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. Case Presentation: 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. Conclusion: 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.
Introduction: Cutaneous angiosarcoma is a rare and highly aggressive vascular malignancy that commonly arises on the scalp and face of elderly individuals. It frequently metastasizes to visceral organs, whereas ocular involvement is exceedingly uncommon. Case Presentation: We report a case of clinically suspected eyelid conjunctival metastasis from cutaneous angiosarcoma in a man in his 60s. The primary tumor involved the left scalp and cheek and was treated with intensity-modulated radiotherapy combined with weekly paclitaxel for 7 months. Although an initial response was observed, the disease progressed with distant metastases to the lungs, liver, and brain. During the terminal stage, a rapidly enlarging hemorrhagic lesion developed on the conjunctiva of the right eyelid, outside the previously irradiated field. Because of the patient’s poor general condition, histopathological confirmation was not feasible. Based on the clinical course and characteristic appearance, the lesion was clinically diagnosed as conjunctival metastasis. Short-course palliative radiotherapy achieved effective hemostasis and symptomatic relief. Conclusion: This case highlights an unusual metastatic presentation of cutaneous angiosarcoma and supports the role of palliative radiotherapy in managing symptomatic ocular lesions.
Introduction: Eosinophilic annular erythema (EAE) is an inflammatory dermatosis characterized clinically by pruritic annular erythematous lesions and histopathologically by eosinophilic dermal infiltration, usually without flame figures. To date, neither standardized therapeutic guidelines nor established biomarkers reflecting disease activity have been identified. Case Presentation: A 50-year-old woman was referred to us with a 1-month history of annular erythema that was refractory to topical corticosteroids and antihistamines. The patient had no medical history of any diseases including bronchial asthma and allergic rhinitis, beyond rectal cancer. The patient did not take any medicines including dietary supplements. Physical examination showed annular erythematous lesions on the lower extremities and trunk. Blood examination showed eosinophil count of 500/µL and thymus and activation-regulated chemokine (TARC), 1,959 pg/mL, with no autoantibodies. Histopathological examination of annular erythema revealed dense perivascular infiltration of eosinophils in the superficial dermis. After exclusion of major differential diagnoses, a diagnosis of EAE was established. Low-dose systemic corticosteroid therapy was initiated but the disease was exacerbated with increasing serum TARC level of 17,310 pg/mL. However, the addition of dapsone at 50 mg/day to prednisolone at 0.5 mg/kg/day resulted in rapid resolution of newly developed lesions along with decreased serum TARC level, allowing for subsequent tapering and discontinuation of corticosteroid. Conclusion: This single case suggests that serum TARC levels may reflect disease activity in EAE. However, further studies are needed to determine whether TARC can serve as a reliable disease activity biomarker in this disease.
Introduction:Cutaneous squamous cell carcinoma (cSCC) is a common malignant skin cancer arising from epidermal squamous cells, typically developing on chronically sun-exposed skin. The clinical diagnosis of cSCC is usually rather straightforward, although one should be aware of rare and atypical subtypes. Case Presentation:An 83-year-old woman, hospitalized for the workup of auricular fibrillation, presented a 10 × 5.5 cm, indolent, indurated, slightly purulent, tumoral mass on her left hand, that progressively increased in volume since around 2 years. There was no functional failure and no locoregional adenopathies. The first punch biopsy favored a keratoacanthoma. The culture of the purulent discharge revealed no microorganisms. MRI did not reveal any deep soft tissue or bone invasion. Excisional surgery was performed under local anesthesia. Histology revealed a well-differentiated cSCC-like exophytic tumor with an endophytic architecture composed of tortuous, keratin-filled, interconnected crypts, highly suggestive of cSCC of the cuniculatum type. Conclusion:Carcinoma cuniculatum is an exceptional subtype of cSCC, with an indolent clinical evolution and a well-differentiated histological presentation. Dermatologists and plastic surgeons should be aware of this rare type of cSCC that is to be differentiated from verrucous carcinoma, keratoacanthoma, cSCC and pseudo-epitheliomatous hyperplasia. Aggressive therapy must be avoided as deep invasion is exceptional, the growth is highly indolent and locoregional or systemic metastatic lesions are nearly never observed. Surgical excision is the cornerstone of treatment.
Introduction: Primary subcutaneous candidiasis is a rare, severe opportunistic infection. While Candida albicans is typically a commensal, it can become invasive in immunocompromised hosts, often mimicking other necrotic skin conditions. Case Presentation: We describe an 83-year-old male with high-risk myelodysplastic syndrome (MDS) and acute myeloid leukemia who developed a nonhealing eschar-covered ulcer on his left shin following a minor gardening injury. Despite multiple courses of broad-spectrum antibiotics, the lesion persisted. Initial superficial cultures were dismissed as colonization. However, multiple deep tissue biopsies and high-throughput internal transcribed spacer 2 sequencing confirmed invasive C. albicans. The patient was treated with intravenous Caspofungin for 3 weeks, leading to significant wound healing. Conclusion: This case highlights the diagnostic challenge of “atypical” fungal ulcers. It underscores the importance of deep tissue biopsies and molecular sequencing in neutropenic patients to differentiate invasive pathogens from surface colonization and prevent systemic dissemination.
Introduction: This case report describes the clinical course and treatment of generalized pustular psoriasis (GPP) flare with intravenous (i.v.) spesolimab, an anti-IL-36R antibody, and subsequent long-term disease management with subcutaneous (s.c.) spesolimab. Case Presentation: A 72-year-old female patient had an ongoing GPP flare for 1 month before presenting to the hospital for flare and related multiorgan failure. She was successfully treated with 2 infusions of 900 mg i.v. spesolimab 1 week apart. She then initiated therapy with 300 mg s.c. spesolimab every 4 weeks for the long-term treatment of GPP. Rapid and significant improvement of skin and systemic symptoms was observed within 1 week of receiving i.v. spesolimab. However, the severity of the patient’s prolonged flare deteriorated her functional status, and she required rehabilitation post-discharge. At follow-ups, s.c. spesolimab demonstrated favorable efficacy and tolerability, with no flare recurrence. Mild superficial desquamation reappeared in the days before consecutive spesolimab doses, with complete skin recovery achieved post-dose. Conclusion: This case underscores the serious complications arising from untreated flare and the need for long-term management of chronic GPP. The patient’s ongoing post-flare symptoms highlight the chronicity of GPP, and continuous targeted treatment with spesolimab improved both her symptoms and quality of life.