Autoimmune Skin Conditions: Cutaneous Lupus Erythematosus.

Cutaneous lupus erythematosus (CLE) is a spectrum of autoimmune skin conditions associated with systemic lupus erythematosus (SLE). CLE and SLE may exist concurrently or independently. Accurate recognition of CLE is crucial because it may herald systemic disease onset. Lupus-specific skin conditions include acute cutaneous lupus erythematosus (ACLE) which manifests as a malar or butterfly rash; subacute cutaneous lupus erythematosus (SCLE); and chronic cutaneous lupus erythematosus, which includes discoid lupus erythematosus (DLE). All three types of CLE present as pink-violet macules or plaques with unique morphology, in areas of sun-exposed skin. Association with SLE differs: ACLE is most closely associated, with SCLE in the middle, and DLE the least so. All types of CLE are pruritic, sting, and burn, and DLE can result in disfiguring scarring. All CLE is exacerbated by UV light exposure and smoking. Diagnosis combines clinical evaluation with skin biopsy. Management focuses on mitigating modifiable risk factors and using pharmacotherapy. UV protection includes use of sun protective factor (SPF) 60 or higher sunscreens containing zinc oxide or titanium dioxide, avoidance of sun exposure, and use of physical barrier clothing. Topical therapies and antimalarial drugs are first-line, followed by systemic therapies (eg, disease-modifying antirheumatic drugs, biologic therapies [eg, anifrolumab, belimumab], or other advanced systemic drugs).

Platelet Rich Plasma for the Treatment of Scarring Alopecia Due to Discoid Lupus Erythematosus.

Platelet-rich plasma (PRP) is an autologous concentration of plasma from a patient’s blood containing platelets up to 7 times higher than normal plasma. Originally indicated to improve connective tissue regeneration in orthopedic surgery, PRP has also proven to be advantageous in the treatment of androgenic alopecia (AGA), alopecia areata (AA), and other forms of non-scarring alopecia.

Patterns of Skin Disease in the Context of a High Prevalence HIV Population in Botswana.

The authors reviewed outpatients in a tertiary dermatology clinic in Botswana to expand knowledge on patterns of skin disease in this population with a high prevalence of human immunodeficiency virus (HIV). Approximately one-third of new and follow-up patients were HIV positive. Common dermatologic conditions included eczematous eruptions, viral and fungal infections, malignant neoplasms, vascular disorders, disorders of pigmentation, and mechanical/physical injury-related disorders. HIV has impacted patterns of dermatologic disease in Botswana, with Kaposi sarcoma being the most frequently biopsied condition. Given the shortage of dermatology specialists, resources should be allocated toward education and management of these most prevalent skin conditions.

Cosmetic treatment in patients with autoimmune connective tissue diseases: Best practices for patients with lupus erythematosus.

The cutaneous manifestations of lupus, especially chronic cutaneous lupus erythematosus, are a source of significant morbidity and can negatively impact patient quality of life. While the active inflammatory component of the disease may be adequately treated, patients are frequently left with residual skin damage and disfiguring aesthetic deficits. Dermatologists lack guidelines regarding the use and safety of various reconstructive and cosmetic interventions in this patient population. Laser treatments are largely avoided in the lupus population because of the possible photodamaging effects of ultraviolet and visible light. Similarly, given the autoimmune nature of this disease, some physicians avoid injectable treatment and grafts because of the concern for disease reactivation via antigenic stimulation. In the second article in this continuing medical education series we compile available data on this topic with the goal of providing evidence-based guidance on the cosmetic treatment of patients with lupus erythematosus with a focus on chronic cutaneous lupus erythematosus.

Pigmented linear discoid lupus erythematosus following the lines of Blaschko: A retrospective study of a Chinese series.

BACKGROUND: Linear cutaneous lupus erythematosus is a rare subtype of lupus erythematosus (LE) that develops linear lesions following the lines of Blaschko. Linear cutaneous lupus erythematosus may present as various subtypes of LE, including linear discoid lupus erythematosus. There are few reports about pigmentedlinear discoid lupus erythematosus in the literature. AIMS: We aimed to summarize the clinical and pathological features of patients with pigmented linear discoid lupus erythematosus following the lines of Blaschko. METHODS: Eighteen patients with pigmented linear discoid lupus erythematosus attending the outpatient department of the Dermatology, Peking Union Medical College Hospital, China, were enrolled in the study. We recorded clinical data including sex, age at onset, disease duration, location and distribution of the lesions, symptoms, trigger factors, antinuclear antibody (ANA) testing, therapy, and therapeutic responses. Histopathological features were also summarized. RESULTS: All 18 patients presented with well-defined brownish pigmented linear or segmental macules or plaques, following the lines of Blaschko. All the lesions were located on the head or neck. Unilaterally distributed lesions were found in 94.4% of patients. Two patients showed low titers of ANA in a speckled pattern. No systemic involvement or progression to systemic LE was noted. The patients were clinically diagnosed as pigmented lichen planus (55.6%), pigmented linear discoid lupus erythematosus (33.3%), and linear morphea (11.1%) before histopathological examination. LIMITATIONS: The study was retrospective and direct immunofluorescence was not performed. Not all patients' information was available and 4 patients were lost to follow-up because their contact information was changed. CONCLUSION: Pigmented linear discoid lupus erythematosus mostly occurs on the head and neck. It manifests as brownish macules along the lines of Blaschko. Differentiation between pigmented linear discoid lupus erythematosus and other dermatoses that have a linear distribution can be difficult both clinically and pathologically, but histological details can help distinguish them.

Practice-based differences in paediatric discoid lupus erythematosus.

BACKGROUND: Children with discoid lupus erythematosus (DLE) are at risk for disfigurement and progression to systemic lupus erythematosus (SLE). Consensus is lacking regarding optimal care for children with DLE. OBJECTIVES: The aim of this study was to compare practice patterns among paediatric dermatologists/rheumatologists treating paediatric DLE. METHODS: An online survey was sent to 292 paediatric rheumatologists in the Childhood Arthritis and Rheumatology Research Alliance and 200 paediatric dermatologists in the Pediatric Dermatology Research Alliance. Consensus was defined as ≥ 70% agreement. RESULTS: Survey response rates were 38% (76 of 200) for dermatology and 21% (60 of 292) for rheumatology. Both specialties agreed that screening labs should include complete blood counts with differential, urinalysis, complement levels, erythrocyte sedimentation rate, antinuclear antibody and other autoantibodies, hepatic function and renal function/electrolytes. Both specialties agreed that arthritis or nephritis should prompt intensified evaluation for SLE. No other patient features achieved consensus as disease-modifying risk factors. Hydroxychloroquine was agreed upon as first-line systemic therapy, but consensus was lacking for second- or third-line treatment. CONCLUSIONS: We found few areas of consensus and significant practice differences between paediatric dermatologists and rheumatologists treating DLE. Knowledge gaps include risk factors for SLE, optimal screening and treatment of refractory skin disease.

Racial Disparities in the Incidence of Primary Chronic Cutaneous Lupus Erythematosus in the Southeastern US: The Georgia Lupus Registry.

OBJECTIVE: Relative to studies of systemic lupus erythematosus (SLE), epidemiologic studies of chronic cutaneous lupus erythematosus (CCLE) are rare and are limited to populations with no racial diversity. We sought to provide minimum estimates of the incidence of primary CCLE (CCLE in the absence of SLE) in a population comprised predominantly of white individuals and black individuals in the southeastern region of the US. METHODS: The Georgia Lupus Registry allowed for the use of multiple sources for case-finding, including dermatology and rheumatology practices, multispecialty health care facilities, and dermatopathology reports. Cases with a clinical or clinical/histologic diagnosis of CCLE were classified as definite. Cases ascertained exclusively from dermatopathology reports were categorized as probable. Age-standardized incidence rates stratified by sex and race were calculated for discoid lupus erythematosus (DLE) in particular and for CCLE in general. RESULTS: The overall age-adjusted estimates for combined (definite and probable) CCLE were 3.9 per 100,000 person-years (95% confidence interval [95% CI] 3.4-4.5). The overall age-adjusted incidences of definite and combined DLE were 2.9 (95% CI 2.4-3.4) and 3.7 (95% CI 3.2-4.3) per 100,000 person-years, respectively. When capture-recapture methods were used, the age-adjusted incidence of definite DLE increased to 4.0 (95% CI 3.2-4.3). The black:white and female:male incidence ratios for definite DLE were 5.4 and 3.1, respectively. CONCLUSION: Our findings underscore the striking racial disparities in susceptibility to primary CCLE, with black individuals having a 3-fold to 5-fold increased incidence of CCLE in general, and DLE in particular, compared with white individuals. The observed sex differences were consistent with those reported previously, with a 3 times higher risk of DLE in women compared with men.

A comprehensive review of immune-mediated dermatopathology in systemic lupus erythematosus.

Lupus erythematosus (LE) is an autoimmune disease with a broad clinical spectrum ranging from cutaneous lesions to severe systemic manifestations. The pathogenesis of the disease and the immunological mechanisms for the heterogeneities in lupus remain unclear. The LE-specific cutaneous manifestations are generally divided into three categories: acute cutaneous LE (ACLE), subacute cutaneous LE (SCLE) and chronic cutaneous lupus erythematosus (CCLE). Clinically, lupus patients with skin lesions can be divided into two subsets based on the organs involved: cutaneous LE, such as DLE and SCLE, which appears only as a skin manifestation, and systemic lupus erythematosus (SLE), e.g., ACLE, which involves other organs, such as kidneys, joints, and the hematopoietic system. However, lupus is an aggressive disease, and cutaneous lupus and systemic lupus partially overlap. Fewer than 5% of DLE patients and approximately 50% of SCLE patients might develop major organ damage and then develop SLE in subsequent years. Furthermore, there are no predictive biomarkers in clinical use. To the best of our knowledge, increasing evidence from clinical trials has revealed that early intervention can either reduce or delay the onset of severe manifestations. Therefore, identification of certain biomarkers in skin lesions or circulation from patients with skin lesions to predict future flares and advise treatment is an unmet need. In this review, we comprehensively describe the subtypes of LE with pathological criteria and clinical manifestations; summarize the up-to-date evidence on certain cell distributions, such as keratinocytes, neutrophils, dendritic cells, T cells and B cells, in skin and peripheral blood; and discuss their pathogenic roles and their potential roles in predictive diagnosis and as therapeutic targets.

Lupus erythematosus and localized scleroderma coexistent at the same sites: a rare presentation of overlap syndrome of connective-tissue diseases.

Overlap syndromes are known to occur with connective-tissue diseases (CTDs). Rarely, the overlap occurs at the same tissue site. We report the case of a patient with clinical and histopathologic findings consistent with the presence of discoid lupus erythematosus (DLE) and localized scleroderma within the same lesions. Based on our case and other reported cases in the literature, the following features are common in patients with an overlap of lupus erythematosus (LE) and localized scleroderma: predilection for young women, photodistributed lesions, DLE, linear morphology clinically, and positivity along the dermoepidermal junction on direct immunofluorescence. Most patients showed good response to antimalarials, topical steroids, or systemic steroids.

Review of treatment for discoid lupus erythematosus.

Discoid lupus erythematosus (DLE) is a chronic cutaneous disease characterized by inflammatory plaques that, in the absence of prompt diagnosis and treatment, may lead to disfiguring scarring and skin atrophy. However, there is limited evidence for which treatments are most effective. Currently, no medications have been approved specifically for the treatment of DLE. Many of the drugs described in the literature were developed for use in other immune disorders. This review will summarize current therapeutic options for DLE and their supporting evidence with discussion of prevention, topical measures, physical modalities, and systemic therapies, including newer potential therapies.

Neglect leads to extremes: maggots and malignancy in a case of discoid lupus erythematosus.

Discoid lupus erythematosus (DLE) is a chronic form of cutaneous lupus erythematosus that runs an indolent course. The rare complications of DLE include scarring, mutilation, non-healing ulceration, cicatricial alopecia and malignancy. DLE progresses to systemic lupus erythematosus (SLE) in around 5% of localized cases and 22% of generalized cases. We report a case of DLE, presenting with a six-month history of ulcerated fungating plaques and small crusted nodules superimposed on DLE plaques over both the forearms. Two weeks prior to the presentation, maggots were also noticed on these plaques. Skin biopsies from these lesions were suggestive of squamous cell carcinoma (SCC) and keratoacanthoma. A wide surgical excision of the tumor followed by partial split-thickness skin grafting was performed with complete healing of the lesions. No recurrence has been noted 18 months from follow-up.

Primary scarring alopecias.

Scarring alopecia or cicatricial alopecia results from follicular damage that is sufficient to cause the destruction and replacement of pilosebaceous structures by scar tissue. Primary scarring alopecias represent a group of disorders that primarily affect the hair follicles, as opposed to secondary scarring alopecias, which affect the dermis and secondarily cause follicular destruction. Inflammation may predominantly involve lymphocytes or neutrophils. Cicatricial alopecias that mainly involve lymphocytic inflammation include discoid lupus erythematosus, lichen planopilaris, frontal fibrosing alopecia, central centrifugal alopecia, and pseudopelade (Brocq). Cicatricial alopecias that are due to predominantly neutrophilic inflammation include folliculitis decalvans, tufted folliculitis, and dissecting cellulitis of the scalp. Folliculitis keloidalis is a cicatricial alopecia with a mixed inflammatory infiltrate.

Unilateral eyelid involvement as single presentation of discoid lupus erythematosus: a clinical conundrum.

We report a case of a 30-year-old woman with discoid lupus erythematosus (DLE) involving only a single lower eyelid. The diagnostic delay is explained by the unspecific clinical and histopathology picture and lack of specific changes in the first biopsy specimen taken. The diagnosis was based on later histological and immunological studies. Palpebral involvement has rarely been reported as the first and sole manifestation of the disease.

Discoid lupus erythematosus at a site of previous injury.

A 71-year-old man with three patches of discoid lupus erythematosus (DLE) confined to the right preauricular region drew our attention because of the unusual linear arrangement of the lesions. Twenty-five years previously, the patient had suffered a trauma in the same area from falling off his motorcycle. We believe that, despite the great lapse in time, this injury may have facilitated the onset of DLE in the very same area, through long-term destabilization of the local neuroimmune network. The case fits the recently coined concept of the immunocompromised district, a cutaneous region with altered immune control, more susceptible to harbouring opportunistic infections, tumours, and immune disorders.

Ethical issues in inpatient consultative dermatology.

Ethical issues commonly arise in inpatient dermatology consultations. We describe the case of an inpatient suffering from a severe, undiagnosable skin condition that raises key principles of medical ethics. The four principles include autonomy, nonmaleficence, beneficence, and justice. Ethical situations faced by inpatient dermatologists are similar to those experienced by medicine hospitalists, as compared with outpatient dermatologists, given the unique clinical setting in which inpatient dermatology is practiced. Other inpatient services, colleagues, and ethics committees are useful resources for inpatient dermatologists faced with difficult ethical situations.

[Schizophrenic psychosis with polycystic ovarian syndrome and lupus erythematosus]. / Schizophrene Psychose bei Koinzidenz von Polycystischem Ovarsyndrom und Lupus erythematodes.

We present the case of a patient with cycloid paranoid psychosis, polycystic ovarian syndrome (PCOS) and lupus erythematosus discoides, whose peculiar course of illness illustrates the interdependence of endocrinological, immunological and psychosocial factors.

Morphea with discoid lupus erythematosus.

The presence of lupus erythematosus with morphea in the same patient has rarely been reported. In this case, we describe a woman with the overlap of discoid lupus erythematosus with superficial morphea, diagnoses that are supported by histopathologic features and laboratory studies.