Discoid Lupus Research Articles

Uncommon manifestation of discoid lupus erythematosus in a human immunodeficiency virus patient.

Uncommon manifestation of discoid lupus erythematosus in a human immunodeficiency virus patient.

Discoid lupus erythematosus in an 8-year-old girl: A rare case

Discoid lupus erythematosus in an 8-year-old girl: A rare case

Evaluation of the Involvement of Heme Oxygenase-1 Expression in Discoid Lupus Erythematosus Lesions.

Discoid lupus erythematosus (DLE) is a chronic autoimmune disease that primarily affects the skin, causing red, scaly patches that may be disfiguring and can cause permanent scarring. This study aimed to investigate the potential clinical and therapeutic applications of heme oxygenase-1 (HMOX1) in the context of DLE. Immunohistochemical staining and bioinformatics analysis were performed on skin biopsy samples from DLE patients to examine the levels of HMOX1 and to correlate with markers of inflammation. Our study revealed a negative correlation between HMOX1 levels and the inflammatory status of DLE lesions, as well as an inverse correlation between HMOX1 levels and the infiltration of M1 macrophages and activated mastocytes. These findings suggest that HMOX1 plays a crucial role in the regulation of inflammation in DLE and could be a potential therapeutic target and biomarker for DLE.

BI16 (P41) An emerging role of interferon-targeted therapy for the treatment of cutaneous systemic lupus erythematosus: a prospective real-world evaluation of anifrolumab for refractory cutaneous lupus

Abstract Cutaneous lupus erythematosus (CLE), particularly discoid lupus erythematosus (DLE), is frequently unresponsive to standard therapies, including antimalarial and immunosuppressive therapies. Recent evidence has identified the importance of type-1 interferon (IFN-1) in the pathogenesis of disease. Anifrolumab is a monoclonal antibody that targets IFN-1 and has been licensed for the treatment of systemic lupus erythematosus (SLE). It is unclear which clinical subtypes of CLE are associated with an IFN-1 signature and may be responsive to anifrolumab therapy. We sought to evaluate the efficacy of anifrolumab on (i) DLE and (ii) rituximab-refractory CLE; and (iii) to compare IFN-specific biomarkers and (iv) early and late immunophenotypic and clinical responses. Anifrolumab was available through an early access to medicines scheme. Patients with SLE for whom the phenotype was predominantly cutaneous were treated with anifrolumab 300 mg intravenously every 4 weeks. The Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) and Dermatology Life Quality Index (DLQI) were reported. Seven patients were enrolled (five with DLE, one with chilblain and one with subacute CLE). All patients were female (median age 46 years; median disease duration 17 years). The median number of failed therapies was six: rituximab in six, belimumab in two and thalidomide in four patients. Four patients required long-term oral prednisolone > 10 mg daily. Median baseline CLASI activity score was 17 and DLQI was 16/30. Median CLASI score reduced after 4 weeks to 6 (range 0–13; P = 0.016) with a median decrease from baseline of 60% (range 19–100%), which was maintained at 6 months. Clinical responses were associated with significant improvements in DLQI (P < 0.001) and EQ-5D visual analogue scale (P = 0.002) by 3 months. All patients had a marked suppression of IFN-stimulated genes at 4 weeks (mean difference 2.92; P < 0.01). Treatment was generally well tolerated with the exception of one patient who had shingles complicated by sensorineural hearing loss while on anifrolumab and prednisolone > 15 mg daily. This required prolonged aciclovir for effective management. This case series highlights the potential role for anifrolumab in managing treatment-resistant CLE with particular responses observed in DLE and chilblain lupus. The need for enhanced awareness of viral infections, particularly varicella-zoster virus, needs to be considered in higher-risk patients.

P41 An emerging role of interferon targeted therapy for the treatment of cutaneous systemic lupus erythematosus: a prospective real-world evaluation of anifrolumab for refractory cutaneous lupus

Abstract Cutaneous lupus erythematosus (CLE), particularly discoid lupus erythematosus (DLE), is frequently unresponsive to standard therapies, including antimalarial and immunosuppressive therapies. Recent evidence has identified the importance of type-1 interferon (IFN-1) in the pathogenesis of disease. Anifrolumab is a monoclonal antibody that targets IFN-1 and has been licensed for the treatment of systemic lupus erythematosus (SLE). It is unclear which clinical subtypes of CLE are associated with an IFN-1 signature and may be responsive to anifrolumab therapy. We sought to evaluate the efficacy of anifrolumab on (i) DLE and (ii) rituximab-refractory CLE and (iii) to compare IFN-specific biomarkers and (iv) early and late immunophenotypic and clinical responses. Anifrolumab was available through an early access to medicines scheme. Patients with SLE for whom the phenotype was predominantly cutaneous were treated with anifrolumab 300 mg intravenously every 4 weeks. The Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) and Dermatology Life Quality Index (DLQI) were reported. Seven patients were identified (five with DLE, one with chilblain and one with subacute CLE were enrolled). All patients were female (median age 46 years; median disease duration 17 years). The median number of failed therapies was six: rituximab in six, belimumab in two and thalidomide in four patients. Four patients required long-term oral prednisolone > 10 mg daily. The median baseline CLASI activity score was 17 and DLQI was 16/30. The median CLASI score reduced after 4 weeks to 6 (range 0–13; P = 0.016) with a median decrease from baseline of 60% (range 19–100%), which was maintained at 6 months. Clinical responses were associated with significant improvements in DLQI (P < 0.001) and the EQ-5D visual analogue scale (P = 0.002) by 3 months. All patients had a marked suppression of IFN-stimulated genes at 4 weeks (mean difference 2.92; P < 0.01). Treatment was generally well tolerated with the exception of one patient who had shingles complicated by sensorineural hearing loss while on anifrolumab and prednisolone > 15 mg daily. This required prolonged aciclovir for effective management. This case series highlights the potential role for anifrolumab in managing treatment-resistant CLE with particular responses observed in DLE and chilblain lupus. The need for enhanced awareness of viral infections, particularly varicella-zoster virus, needs to be considered in higher-risk patients.

CPC07 Lupus comedonicus: a not so common entity

Abstract Lupus comedonicus (LC) is an exceedingly rare variant of chronic cutaneous lupus erythematosus (CCLE), with only 13 reported cases in the literature to date. While its aetiology remains unknown, hallmarks include the presence of comedones on erythematous plaques, particularly in seborrhoeic areas. We report a case of LC involving the scalp, concha bowl and upper back. A 53-year-old female smoker with a 20-pack-year history and previous unprovoked pulmonary embolism presented to dermatology with a 2-year history of alopecia and rash affecting her face and upper back. She was initially reviewed by oral medicine for ulcers, where erythematous buccal mucosal plaques with white striations were seen. The initial biopsy showed a patchy lichenoid inflammatory infiltrate, raising suspicions for lichen planus; however, repeat biopsy highlighted perivascular inflammation and keratin plugging suggestive of lupus. Full skin examination revealed patchy scarring alopecia with perifollicular scale and erythema. Erythematous infiltrated plaques were seen on the face with similar more indurated lesions on the upper back associated with open comedones. The eponymous Shuster sign was observed in the left conchal bowl, with associated follicular occlusion. Laboratory studies all returned within normal limits, including antinuclear antibody titres and complement levels. Differential diagnosis included lupus erythematosus tumidus, discoid lupus erythematosus and LC. Lesional biopsies taken from her back confirmed LC, highlighting lichenoid inflammation involving follicles and interfollicular skin, with superficial and deep perivascular infiltrate. We commenced oral hydroxychloroquine at 200 mg once daily for management, with topical mometasone furoate 0.1% to be used while awaiting its full effect. Smoking cessation has also been advised given the potentially antagonistic effect of smoking with lupus and hydroxychloroquine. Hypotheses of the pathogenesis of LC include the effect of sun exposure. Ultraviolet radiation is known to not only enhance the activity of CCLE but may also contribute to the formation of comedones. Actinic damage alters collagen of normal skin, promoting sebum retention and follicular plugging. Given its infrequency, LC could be mistaken for benign conditions, including acne vulgaris, naevus comedonicus and Favre–Racouchot disease. It is plausible that the small case numbers are due to current underdiagnoses. It is important for dermatologists to be aware of and accurately diagnose LC. Apart from its potentially destructive, scarring complications, especially in untreated and misdiagnosed cases, it is associated with systemic lupus in 30% of cases. Delay in diagnosis and therapeutic management can be associated with significant potential morbidity and even mortality.

H22 Barking up the right tree: history of quinine

Abstract Quinine is considered to be one of the most important medical discoveries of the 17th century through its use in treating malaria. Since then, its applications have expanded, and quinine continues to be one of the most widely used drugs in dermatology. Quinine is found in the bark of the Cinchona tree found in the jungles of the Andean region of South America. The exact nature of its discovery remains unclear; however, there are tales of the Spanish Countess of Chinchon being cured of a recurring fever by using liquid containing Cinchona bark. This prompted her to return to Spain in 1638 with the seeds and precious bark; in 1742, botanist Carl Linnaeus named the tree Cinchona in her honour. During the early 17th century, the bark was ground into a powder and mixed with wine. In 1820, French scientists Pierre Pelletier and Joseph Caventou developed the processes to extract quinine from Cinchona bark, improving the potency of the medicine. In the 19th century, as the role of quinine in treating malaria spread, Europeans in the British colonies started planting Cinchona trees to avoid importing large amounts of the drug. Owing to its bitter taste, quinine was mixed with sugar and soda, creating the first tonic water. The tonic water was mixed with gin to make the bitterness of quinine more palatable. Winston Churchill claimed that this drink ‘saved more Englishmen’s lives, and minds, than all the doctors in the Empire’ (Simonetti O, Contini C, Martini M. The history of Gin and Tonic; the infectious disease specialist long drink. When gin and tonic was not ordered but prescribed. Infez Med 2022; 30:619–26). The first documented use of quinine to treat dermatological conditions was produced by Payne in 1894, who reported the successful treatment of skin lesions in discoid lupus erythematosus (Payne J. A postgraduate lecture on lupus erythematosus Clin J 1894; 4:223–9). In 1951, a case series in the Lancet of 20 patients with lupus erythematosus and rheumatoid arthritis who were successfully treated with quinacrine, led to the extensive use of antimalarials to treat skin and joint conditions (Page F. Treatment of lupus erythematosus with mepacrine. Lancet 1951; 258:755–8). This was later succeeded by chloroquine: a synthetic analogue was first synthesized in the 1930s by Hans Andersag. However, it was noted to have significant toxicities, which led to the production of hydroxychloroquine, a similar drug with a much-improved side-effect profile. After 400 years, quinine and its synthetic derivatives continue to play a major role in managing dermatological conditions.

BH13 Is this dissecting cellulitis of the scalp in a White child?

Abstract Dissecting cellulitis of the scalp (DCS) is characterized by recurrent pustules and sinus tract formation leading to scarring and alopecia. Follicular obstruction is a key pathogenic event and is associated with hidradenitis suppurativa and acne conglobate. Commonly, DCS is found in adult men of Afro-Caribbean descent (Arneja J, Vashi C, Gursel E, Lelli J. Management of fulminant dissecting cellulitis of the scalp in the pediatric population: case report and literature review. Can J Plast Surg 2007; 15:211–14). We describe a rare case of a White child who presented with features suggestive of DCS. An 11-year-old boy was referred with a 2-year history of patches of alopecia affecting the occipital scalp which was initially treated as tinea capitis with antifungal creams, but this was ineffective. On examination, there was diffuse, boggy subcutaneous swelling with scarring, overlying erythema and patches of alopecia on the occipital scalp. A course of erythromycin and oral terbinafine showed a partial response of the affected areas, although subsequent new patches of alopecia were noted with a similar manifestation of bogginess and overlying erythema. Systemic examination, blood tests and skin scrapings were unremarkable. While continuing erythromycin, he was counselled for isotretinoin and a referral to a tertiary centre has been made to guide further management. Paediatric scarring alopecia encompasses congenital and acquired causes (Imhof R, Cantwell H, Proffer S et al. The spectrum of pediatric scarring alopecia: a retrospective review of 27 patients seen at Mayo Clinic. Pediatr Dermatol 2021; 38:580–4). The differential diagnosis for our patient included tinea capitis causing irreversible alopecia, and being aware of this phenomenon with late presentations and delayed treatment is vital. Other differentials are discoid lupus and cutaneous morphoea. However, the clinical features in this case are more in keeping with DCS, which is scarcely reported in the paediatric literature. Histological confirmation is not always practical with children, and follow-up to assess course of the disease is prudent. Importantly, the discussion of permanent hair loss with a child and parent remains challenging, and clinicians should be aware of the associated psychosocial impact.

Effects of Hydroxychloroquine and Tacrolimus on Discoid Facial Lupus Erythematosus in a Dog

Discoid lupus erythematosus is lupus in dogs an autoimmune disease that affects the skin. A 6-year-old, 38 kg, male German Shepherd dog was taken to the Small Animal Hospital at the University in Kyiv, Ukraine with a history of progressive skin lesions. The indications of discoid lupus erythematosus in dogs manifested as red, scaly macules or papules on the skin's surface. These gradually develop into follicular plugging, disc-shaped plaques with adherent scales, and peripheral hyperpigmentation. The oral hydroxychloroquine was used for medication and the prescribed dosage was 5.0 mg per kilogram of the dog's weight, administered once daily. The hydroxychloroquine was gradually reduced and discontinued within a month. Concomitant with the hydroxychloroquine treatment, the application of tacrolimus cream (Protopic® 0.03%) was initiated. The veterinary physician also advised the dog owner to limit sun exposure to avoid any adverse effects. After a four-week period, there was a decrease in pruritus and erythema, and plaques had flattened although the skin still had some patchy hyperpigmentation. Approximately 3-4 weeks later, the veterinarian determined that the dog had achieved clinical remission as all the skin lesions had become completely flattened. The use of deproteinized calf blood extract gel (Solcoseril® Gel for external 4.15 mg/1 g, Legacy led to the complete disappearance of the initial redness and prevented the appearance of new skin lesions. These results can be considered as a safe and effective alternative to conventional treatment methods.

An Analysis of Literature on Topical Steroid Withdrawal in Dermatology

Topical steroids have been used to treat a variety of dermatological conditions and are one of the most commonly prescribed drugs in dermatology. These are Food and Drug Administration (FDA)-approved medications for treating diseases such as eczema, vitiligo, atopic dermatitis, discoid lupus erythematosus, and more. The mechanism of action of topical corticosteroids consists of anti-inflammatory, anti-mitotic, and immunosuppressive effects. Patients on topical corticosteroid treatments must be monitored carefully as there is a potential for adverse effects, especially in treatments lasting longer than several weeks. While when properly prescribed and monitored topical corticosteroid usage is considered safe, there have been growing concerns surrounding topical corticosteroid abuse, phobia, and withdrawal. Current research has focused on new therapeutic strategies and management protocols to improve long-term patient outcomes and mitigate the adverse effects of these drugs. In this narrative review, we describe the recent findings of clinical manifestations, patient perspectives in the literature, current knowledge, and misinformation and abuse pertaining to topical steroids.

Clinical and histopathological features of rostrolateral nasal alar arteriopathy of German shepherd dogs.

Dermal arteritis of the nasal philtrum (DANP) has been described in large-breed dogs. To characterise clinically distinct, discrete fissures of the dorsolateral nasal alae associated with severe bleeding in German shepherd dogs (GSDs). Fourteen privately owned GSDs with linear rostrolateral nasal alar fissures and a histopathological diagnosis of nasal vasculopathy. Retrospective analysis of medical records and histological slides. Mean age of onset was 6 years. Before biopsy, episodic arteriolar bleeding was noted in 11 of the 14 (79%) dogs. Slide analysis revealed enlarged nasal arterioles with expanded vascular tunics and luminal stenosis beneath ulcers. Histopathological lesions consistent with mucocutaneous pyoderma and/or facial discoid lupus erythematosus were present in 5 of the 14 (36%) dogs. Enlarged arterioles stained blue with Alcian blue and Masson's trichrome stains, consistent with deposition of mucin and collagen, respectively. Immunohistochemical stains (neutrophil myeloperoxidase, IBA1, CD3) were performed. CD3 was negative for all dogs, whilst neutrophil myeloperoxidase and IBA1 occasionally demonstrated intramural neutrophils (3 of the 14 dogs, 21%) or histiocytes (1 of the 14 dogs, 7%) in altered vessels, respectively. All dogs underwent medical management and/or surgical excision. Treatments included tacrolimus, prednisone, ciclosporin-modified, pentoxifylline, antimicrobials and doxycycline/niacinamide. No dogs were treated with antimicrobials alone. For seven dogs with long-term follow-up, treatment response was complete in five (71%) and partial in two (29%), with six of the seven (86%) receiving immunomodulatory treatments to maintain remission. Nasal alar arteriopathy of GSDs shares histopathological changes with DANP. It has characteristic clinical and histopathological features and appears amenable to immunomodulation.

Clinical characteristics of palmoplantar discoid lupus erythematosus: A multicenter retrospective review of 43 patients

AbstractBackgroundPalmoplantar discoid lupus erythematosus (ppDLE) is a rare manifestation of chronic cutaneous lupus erythematosus that can result in painful, hyperkeratotic, and/or erosive lesions on the palms and soles. Despite this penchant to cause significant morbidity, the natural history of ppDLE and its response to conventional treatment approaches remain poorly understood.ObjectivesTo characterize the initial presentation, treatment response, and association with systemic lupus erythematosus (SLE) of ppDLE.MethodsWe performed an International Classification of Diseases (ICD‐9 and ICD‐10) code and natural‐language query for medical records from Brigham and Women's Hospital and Massachusetts General Hospital to identify all cases of ppDLE based on biopsy and/or expert opinion from January 2000 through October 2022. Demographics, clinical features, and treatment data were analysed.ResultsForty‐three patients (median age, 29.5 years [range, 8−70 years]; 36 female [83.7%]) with ppDLE were identified. Mean time from initial clinical presentation of ppDLE symptoms to diagnosis was 14.5 months (n = 34). Of the 43 patients, 74.4% developed coexistent SLE. A mean of 5.9 (standard deviation, 2.7) lines of therapy were required to achieve control of ppDLE, with the majority of patients requiring the initiation of at least two concomitant systemic medications.ConclusionsHerein, we present the largest cohort of patients with ppDLE in the literature to date. Despite a high incidence of coexistent SLE, patients experienced lengthy diagnostic delays. Even with appropriate diagnosis, an overwhelming trend towards treatment‐refractory disease was observed.

Epidemiology and demographics of cutaneous lupus erythematosus in Colombia between 2015 and 2019.

Cutaneous lupus erythematosus (CLE) is classified into three groups - acute, subacute, and chronic - based on clinical and histopathological characteristics. The risk of systemic manifestations varies among these groups. There are few studies on CLE epidemiology. For this reason, this paper aims to describe CLE prevalence and demographics in Colombia between 2015 and 2019. This descriptive, cross-sectional study used the international classification of diseases, tenth revision, for CLE subtypes, utilizing official data from the Colombian Ministry of Health. In people older than 19 years, 26,356 CLE cases were registered, yielding a prevalence of 76 cases per 100,000 population. CLE was more frequent in females, at a 5:1 ratio compared to males. The most common clinical presentation was discoid lupus erythematosus, in 45% of cases. The majority of cases occurred in people between 55 and 59 years old. This is the first study that describes CLE demographics in adults in Colombia. Findings regarding clinical subtypes and female predominance are consistent with those in the medical literature.

Lupus nephritis with clinical and laboratory features of humoral immune deficiency in a 29-year-old female with a novel NCF1 variant consistent with autosomal recessive chronic granulomatous disease

Chronic Granulomatous Disease (CGD) is a rare, inherited disorder of the innate immune system characterized by defects in the phagocyte NADPH oxidase complex. This results in poor phagocyte function causing frequent infections (bacterial and fungal) and chronic inflammation. Here we discuss a rare presentation of lupus nephritis and antibody deficiency in an adult female patient with a novel homozygous nonsense mutation in the NCF-1 gene causing autosomal recessive CGD.Our patient is a 29-year-old female with history of ITP, biopsy-confirmed lupus nephritis, systemic lupus erythematosus (SLE), idiopathic avascular necrosis of the hip, and previous diagnosis of common variable immune deficiency (CVID) treated with Immunoglobulin replacement therapy (IVIG). She presented to our clinic to establish care in the setting of persistent skin abscesses/cellulitis, poor wound healing, necrotic lymphadenitis, and recurrent urinary tract infections and pneumonia despite years of IVIG therapy. She had near-normal total IgG, absent IgA and IgE, and protective vaccine antibody titers (pneumococcus, diphtheria, and tetanus) prior to the start of IVIG. Immunophenotyping demonstrated quantitatively normal number of total B cells, absent CD27+ memory B cells, and normal T cell subsets. She had reassuring lymphocyte proliferation with mitogen stimulation and normal TLR function. Dihydrorhodamine (DHR) flow cytometric assay demonstrated abnormally low neutrophil oxidative burst activity. Gene sequencing revealed homozygous nonsense mutation in the NCF-1 gene (c.75-76del; pY26Hfs*26) and a pathogenic heterozygous variant in FANCM (c.3732-3735dup; p. Leu1246Asnfs*8). She was started on daily prophylactic trimethoprim-sulfamethoxazole and itraconazole. She has marked decrease in infections/hospitalizations and her lupus nephritis is well controlled with hydroxy-chloroquine. She is being referred to the NIH for HSCT evaluation. The majority of CGD cases in the United States are due to X-linked CGD (70%) and AR variants in NCF1 (20%). While rare, discoid lupus erythematosus and SLE have been documented with X-linked forms of CGD. Similarly, there are few case reports of antibody deficiency in patients with CGD. This is the first documented case of AR CGD with SLE/lupus nephritis and humoral immune defects. Additionally, we report a disease-causing, novel variant in the NCF1 gene. This case report adds to the existing literature on CGD and associated diseases.

Abstract #1394329: An Association Between Stiff Person Syndrome and Type 1 Diabetes

Stiff-person syndrome (SPS) is a rare neuromuscular disorder characterized by progressive muscular spasms and rigidity. Anti-glutamic acid decarboxylase (anti-GAD) antibodies are found in type 1 diabetes mellitus (T1DM). Thirty percent of patients diagnosed with SPS have T1DM. This relationship infers that SPS and T1DM share pathogenetic features. We describe a case of a 66-year-old female with a history of SPS who developed T1DM soon after the diagnosis was made. A 66-year-old female with a history of SPS, T1DM, and discoid lupus was referred to our university endocrinology clinic for diabetes management. She had ataxia, loss of balance, and muscle stiffness since 2012. The patient was diagnosed with spinal cerebellar ataxia. She was not treated with any specific agents and continued to have left-sided muscle stiffness with preserved strength. She denied weakness, pain, paresthesia, or muscle atrophy but had difficulty ambulating due to rigidity. The diagnosis of SPS was made based on positive anti-GAD antibodies (normal < 0.02) and positive N-channel antibodies in April 2016. She required hospital admission one month later and received solumedrol and IVIG. Her HbA1c was 6.5% (normal < 5.7% of total Hgb), and glucose levels were between 200-300 mg/dL, which were treated with basal and bolus insulin. She was discharged home in May 2016 with no diabetic medications. The patient developed polyuria and polydipsia with 11 Ibs unintentional weight loss 20 days after hospital discharge. She was referred to the endocrine clinic in June 2016 for further workup. Her HbA1c was 11% with C-peptide less than 0.01 (normal 0.78-5.20 ng/mL). Basal and bolus insulin were initiated, and she remains on an insulin regimen. SPS affects an estimated one person per million per year. Decreased level of gamma amino butyric acid (GABA) in the central nervous system (CNS) in SPS has been implicated, which causes a loss of neural inhibition. Glutamic acid decarboxylase (GAD) is the main enzyme required for the GABA inhibitory pathway. Anti-GAD antibodies decrease inhibitory function within the CNS and cause increased muscle activities and subsequent muscle spasms. While most patients with T1DM do not develop SPS, most with anti-GAD-positive SPS will have or develop T1DM. Either SPS or T1DM may be diagnosed first. Awareness of these associations is essential for early diagnosis and treatment of these disorders.

Verrucous squamous cell carcinoma occurring as a complication of generalized discoid lupus erythematosus in a nonsmoker

Verrucous squamous cell carcinoma occurring as a complication of generalized discoid lupus erythematosus in a nonsmoker

Verrucous disseminated discoid lupus erythematosus with oral and palmoplantar lesions treated with hydroxychloroquine

Lupus erythematosus (LE) is an autoimmune inflammatory disease with a wide spectrum of cutaneous and systemic manifestations. A unifying concept of this disease is difficult to develop as clinical features of patients with LE show a great variation. Our objective was to present a case of verrucous disseminated discoid lupus erythematosus (DLE) with atypical morphology, extensive involvement, and having oral and palmoplantar lesions. For confirmation of diagnosis, histopathology was done, which showed characteristic findings of DLE. Combination therapy of hydroxychloroquine with oral and topical corticosteroids resulted in resolution of skin lesions. Sometimes, DLE lesions may pose a diagnostic and therapeutic challenge, especially in a case of unusual presentation, as in the given case of hypertrophic LE. These cases require adequate therapy with clinical and histopathological follow-up considering that squamous cell carcinoma may arise in long-standing lesions.

Systemic Lupus Erythematosus in Men: Brief Description of Clinical and Laboratory Findings

Background: Systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterized by the presence of nuclear autoantibodies, which can cause autoimmune complex formation and inflammation of multiple organs. It is known to affect women more frequently than men and is typically diagnosed during reproductive age, where the female-male ratio has been reported as high as 12:1. The etiology of SLE is not fully understood, but both genetic predisposition and environmental triggers are believed to be involved. Because of the multitude of presentations, manifestations, and serological abnormalities in patients with SLE, diagnosis can be challenging. Chronic discoid lupus and photosensitivity have been found in men with SLE as far as skin findings are concerned, there is less joint involvement in these patients, however, severe systemic findings predominate, such as serositis, pleurisy, central nervous system involvement with the presence of seizures. , thrombotic events and lupus nephropathy of the acute proliferative glomeruonephritis variety, serologically there is no consensus yet, nor in the specific clinical characteristics but the presence of anti-dsDNA anti-Sm antibodies and anticardiolipin antibodies is also frequently found. Therapeutic approaches predominantly involve immunomodulation and immunosuppression and are targeted to be the specific organ manifestation, with the aim of achieving low disease activity. Conclusion: We considered that although male patients with lupus are not commonly seen, the manifestations are life threatening, and timely diagnosis and treatment of the disease will lead to a better outcome for these patients.

Tacrolimus in Solution as an Option to Inflammatory Conditions of the Scalp.

Several dermatological diseases lead to inflammatory conditions of the scalp. Most of these afflictions are recalcitrant and require long term maintenance treatment. We present a case series where topical tacrolimus was used in a solution vehicle for these conditions. A total of 22 patients (aged 24-90 years) with confirmed diagnosis of lichen planus pilaris (LPP), discoid lupus (DL), frontal fibrosing alopecia (FFA), erosive pustulosis of the scalp (EPS) or folliculitis decalvans (FD) were evaluated and treated with tacrolimus solution (0.1%) applied twice daily for 1 month, then once daily for another month and alternate days for 4 months. Efficacy was evaluated by an investigator global assessment, clinical and dermoscopic evaluation at weeks 4, 8 and 24. The safety assessment included monitoring of all adverse events. The study included 13 patients with LPP, 2 with DL, 2 with FD, 2 with EPS and 3 with AFF. After 1 month, 14 patients (63.6%) had a good response and 7 (31.8%) had excellent response. After 2 months, 16 patients (72.7%) had excellent response, and this response was persistent after 6 months of treatment. Tacrolimus in solution, even if not yet commercially available, was an effective and well tolerated alternative for the maintenance treatment of inflammatory conditions of the scalp.

A linked physiologically based pharmacokinetic model for hydroxychloroquine and metabolite desethylhydroxychloroquine in SARS-CoV-2(-)/(+) populations.

Hydroxychloroquine (HCQ) is Food and Drug Administration (FDA)-approved for malaria, systemic and chronic discoid lupus erythematosus, and rheumatoid arthritis. Because HCQ has a proposed multimodal mechanism of action and a well-established safety profile, it is often investigated as a repurposed therapeutic for a range of indications. There is a large degree of uncertainty in HCQ pharmacokinetic (PK) parameters which complicates dose selection when investigating its use in new disease states. Complications with HCQ dose selection emerged as multiple clinical trials investigated HCQ as a potential therapeutic in the early stages of the COVID-19 pandemic. In addition to uncertainty in baseline HCQ PK parameters, it was not clear if disease-related consequences of SARS-CoV-2 infection/COVID-19 would be expected to impact the PK of HCQ and its primary metabolite desethylhydroxychloroquine (DHCQ). To address the question whether SARS-CoV-2 infection/COVID-19 impacted HCQ and DHCQ PK, dried blood spot samples were collected from SARS-CoV-2(-)/(+) participants administered HCQ. When a previously published physiologically based pharmacokinetic (PBPK) model was used to fit the data, the variability in exposure of HCQ and DHCQ was not adequately captured and DHCQ concentrations were overestimated. Improvements to the previous PBPK model were made by incorporating the known range of blood to plasma concentration ratios (B/P) for each compound, adjusting HCQ and DHCQ distribution settings, and optimizing DHCQ clearance. The final PBPK model adequately captured the HCQ and DHCQ concentrations observed in SARS-CoV-2(-)/(+)participants, and incorporating COVID-19-associated changes in cytochrome P450 activity did not further improve model performance for the SARS-CoV-2(+) population.