Cutaneous metastasis as a first sign of adenocarcinoma of the cervix.

Cervical cancer remains one of the most common malignancies diagnosed in women as well as a leading cause of cancer related deaths in women worldwide. Cutaneous metastasis associated with cervical malignancy is a remarkably rare phenomenon. We present a patient whose cutaneous signs led to the diagnosis of metastatic adenocarcinoma of the cervix.

Neonatal pemphigoid gestationis: An atypical presentation of a rare disease.

Bullous pemphigoid (BP) is an autoimmune blistering disease characterized by urticarial plaques and/or vesicles and tense bullae. A unique presentation of BP can occur during pregnancy, the postpartum period after delivery, or with the initiation of contraception, in which case it is referred to as pemphigoid gestationis (PG). In rare instances, newborns born to mothers with PG may also present with blisters due to transplacental passage of maternal anti-bullous pemphigoid 180 (BP180) or 230 (BP230) immunoglobulin G (IgG). In this report, we present an unusual case of neonatal PG in an infant born to an asymptomatic mother without a previous diagnosis of PG.

Individualizing patient education for greater patient satisfaction.

The benefits of educational intervention on health outcomes has been widely discussed, but the most educational methods have not been addressed. We sought to assess preferred modes of education during an outpatient dermatology visit (ie, verbal instruction [VI], written instruction [WI], demonstration [DM], Internet resources [IR]). We secondarily looked at patient satisfaction with the educational methods used. The results indicate the most preferred method of education among 157 patients who completed a 12-question survey and areas where physicians may need to improve patient education.

A Case of Oral Lichen Planus Preceding the Diagnosis of Good Syndrome.

Good syndrome (GS) is a rare condition characterized by thymoma and immune deficiency with a poorly understood mechanism in which patients have reduced immunoglobulin levels and circulating B-cells along with impaired T-cell function. GS is often accompanied by autoimmune and inflammatory conditions, and in this report, we present a case of refractory oral lichen planus (OLP) preceding the diagnosis of GS. In this case, a patient with a history of OLP was diagnosed with GS and common variable immunodeficiency (CVID) following thymectomy and was treated with intravenous immunoglobin (IVIG). Additionally, he was found to have pure red cell aplasia managed with cyclosporine. His oral symptoms worsened, and he presented to dermatology. Treatment was initiated with topical clobetasol and tacrolimus for his OLP, and fluconazole was started for concomitant oral candidiasis. His OLP has remained under satisfactory control with this regimen; however, he requires close surveillance for malignancy given his increased risk of oral squamous cell carcinoma (OSCC) with immunosuppression and active OLP. Although rare, clinicians should be aware of GS and its association with erosive OLP along with the heightened risk of infection in these patients.

Skin-Related Quality of Life During Autoimmune Bullous Disease Course.

Importance: Autoimmune bullous diseases (AIBDs) are chronic relapsing-remitting conditions with significant morbidity. Skin-related quality of life (SRQL) may vary by AIBD subtype and disease type. Disease severity and flare severity can be difficult to define; SRQL can offer a key insight. Objectives: To investigate the Skindex-16 score as an SRQL measure in AIBD subtypes during flare and nonflare states and to evaluate Skindex-16 construct validity. Design, Setting, and Participants: This retrospective cross-sectional study was conducted from September 1, 2016, to February 1, 2020, among 192 patients at the University of Utah Health autoimmune dermatology clinic with pemphigoid, pemphigus, dermatitis herpetiformis, and linear immunoglobulin A disease. Patients had an encounter-associated diagnosis, Skindex-16 scores, and self-reported flare status. Statistical analysis was performed from March 2022 to June 2023. Exposure: Autoimmune bullous disease subtype and patient-reported flare status. Main Outcomes and Measures: Skindex-16 domain scores (emotions, symptoms, and functioning; range, 0-100, where 0 indicates no effect on SRQL and 100 maximum effect) and individual item scores were described by disease and flare status. Flare scores were expected to be higher by at least the standard error of measurement (SEm). Convergent validity was assessed using Spearman correlation among Skindex-16 scores, serologic titers, and other patient-reported outcome measures. Floor or ceiling domain scores (<20% of sample scoring either lowest or highest possible domain scores, respectively) were assessed for Skindex-16. Structural validity was assessed using confirmatory factor analysis (CFA). Results: The study included 192 patients with 212 visits (median age, 68 years [IQR, 58-76 years]; 123 of 212 women [58.0%]) with Skindex-16 scores (64 in flare state and 148 in nonflare state). Median Skindex-16 domain scores were higher for all disease categories among patients in the flare state compared with those in the nonflare state (pemphigoid [emotions: flare, 52.4 (IQR, 38.1-69.0); nonflare, 7 (IQR, 0-17); symptoms: flare, 37.5 (IQR, 29.2-58.0); nonflare, 13 (IQR, 0-25); functioning: flare, 26.7 (IQR, 10.0-56.7); nonflare, 0 (IQR, 0-3)]; pemphigus [emotions: flare, 54.8 (IQR, 31.0-81.0; nonflare, 0 (IQR, 0-19); symptoms: flare, 58.3 (IQR, 41.7-70.8); nonflare, 4 (IQR, 0-12.5); functioning: flare, 26.7 (IQR, 13.3-83.3); nonflare, 0 (IQR, 0-3.33)]; dermatitis herpetiformis [emotions: flare, 72.6 (IQR, 34.7-90.5); nonflare, 14.3 (IQR, 2.4-26.2); symptoms: flare, 69 (IQR, 31.3-85.4); nonflare, 12.5 (IQR, 0-29.2); functioning: flare, 38.3 (IQR, 5.0-63.2); nonflare, 0 (IQR, 0-13.3)]. This difference exceeded SEm cut points. Cronbach α was greater than 0.80 for all domains and AIBDs. Moderate or low correlations were seen with desmoglein 1 and bullous pemphigoid 180 titers. Moderate correlation existed between Skindex-16 and Patient-Reported Outcomes Measurement Information System Depression scores (emotions: ρ = 0.40; symptoms: ρ = 0.41; functioning: ρ = 0.48), and strong correlation existed between Skindex-16 and patient-reported disease severity (emotions: ρ = 0.71; symptoms: ρ = 0.73; functioning: ρ = 0.66). Floor domain scores greater than 20% were seen among patients in the nonflare state, but ceiling domain scores were rare (<10% for all domains); CFA model fit was poor. Conclusions and Relevance: In this cross-sectional study, SRQL was highly associated with flare of AIBDs. Skin-related quality of life was worse during periods without flare among patients with pemphigoid and dermatitis herpetiformis compared with pemphigus, highlighting residual SRQL morbidity. Skindex-16 showed good construct validity, but the poor CFA model fit needs further research. Clinical measurement of SRQL in AIBDs can add critical disease-severity information.

Dermal dendritic cells in psoriasis, nummular dermatitis, and normal-appearing skin.

BACKGROUND: The reason psoriasis (PSO) favors extensor skin is unknown. We hypothesized that PSO may involve extensor skin preferentially because of differences in the number or type of dermal dendritic cells (dDCs) between flexural and extensor skin. OBJECTIVE: We sought to compare dDC type and distribution in normal-appearing flexural and extensor skin, PSO, and nummular dermatitis (ND). METHODS: Using immunohistochemical markers, the number, distribution, and type of Langerhans cells, myeloid dendritic cells (DCs), and plasmacytoid DCs was compared in normal-appearing skin, PSO, and ND. RESULTS: Significant differences in dDC density were not identified between flexural and extensor skin, although extensor skin contained fewer CD11a(+) and CD11c(+) cells. Compared with normal-appearing skin, cells expressing CD11a, CD11c, CD123, CD303, and CD207 were increased in PSO. ND lesions showed similar increases. No significant difference between PSO and ND was evident with the exception of decreased S100A6(+) cells in PSO. LIMITATIONS: We did not study seasonal variation in DC density or assess nonlesional skin from patients with PSO. CONCLUSIONS: The data did not support the hypothesis that PSO favors extensor skin because of differences in DC localization. However, dDCs were significantly increased in PSO by comparison with normal-appearing skin, supporting existing evidence that they are involved in the overall pathogenesis of PSO.

Rheumatic manifestations of skin disease.

PURPOSE OF REVIEW: There is an increasing interest in improving the understanding of pathophysiology, outcome measures, and therapies of rheumatic skin disease. Increasingly, studies are using the skin as a primary endpoint for evaluating therapies. This will review the current state of the art for the most common rheumatic skin diseases. RECENT FINDINGS: A number of medications, including biologics such as tumor necrosis factor alpha and interferon, have been associated with onset of cutaneous lupus. The cutaneous lupus erythematosus area and severity index has been further validated and utilized in a number of studies. Smoking continues to be associated both with presence and refractoriness of cutaneous lupus erythematosus to therapy. There are several tools now available for evaluating the skin disease of dermatomyositis, but there is a need for new effective therapies. Measurement of skin disease in scleroderma continues to be a challenge, and there is a need for more effective therapies. Several studies show efficacy of intravenous iloprost for severe Raynaud's and skin ulcers, and of bosentan for digital ulcers. SUMMARY: The present review covers new outcome measures, treatments, and unusual manifestations of cutaneous lupus, dermatomyositis, scleroderma, and rheumatoid arthritis. There have been a number of new studies related to validation of disease activity measures, as well as their use in evaluation of new therapies for these conditions. Validated outcome measures are required to perform meaningful studies, and will facilitate organized epidemiologic, quality of life, and therapeutic studies.

Cdc7 expression in melanomas, Spitz tumors and melanocytic nevi.

BACKGROUND: Cdc7 is a serine-threonine kinase required for initiation of DNA replication that may play a role in the development and progression of melanoma. MATERIALS AND METHODS: Tissue microarrays containing 40 melanomas, 40 Spitz tumors and 30 nevi were constructed. Staining for Cdc7 was scored semiquantitatively according to intensity and extent, and the values were converted into composite scores. RESULTS: Nodular melanomas, atypical Spitz tumors and superficial spreading melanomas had the highest scores (nodular melanomas, 3.67; atypical Spitz tumors, 2.78 and superficial spreading melanomas, 2.44). Typical Spitz nevi, dysplastic nevi and ordinary nevi had the lowest scores. Cdc7 expression in melanomas differed significantly from non-Spitz nevi (p < 0.001). The difference was also significant when invasive melanomas were compared with dysplastic nevi (p < 0.005) and when invasive melanomas were compared with non-atypical Spitz nevi (p < 0.001). However, there was no significant difference between invasive melanomas and atypical Spitz tumors (p = 0.69) or between dysplastic nevi and ordinary nevi (p = 0.73). CONCLUSION: Cdc7 expression differs significantly among cutaneous melanocytic neoplasms and can be evaluated by routine immunohistochemical methods. The results suggest that differences in Cdc7 expression may account for some of the differences between malignant melanomas and benign melanocytic nevi.

Acquired kinking of the hair caused by acitretin.

Alopecia, hypertrichosis, and hirsutism may be caused by a variety of medications. Drug-induced alterations in the texture or structure of the hair shaft, however, are much less common. We report a female patient who presented with acquired generalized kinking of the hair 6 months after the initiation of acitretin therapy for psoriasis. The hair change has persisted despite reductions in the dose of acitretin. To our knowledge, this is the first report of hair kinking induced by acitretin. It has been proposed that retinoid therapy may affect keratinization of the inner root sheath to cause this structural hair shaft change.

Plaque-like myofibroblastic tumor of infancy.

Fibrohistiocytic neoplasms are relatively uncommon in infancy and childhood. We report an unusual spindle cell tumor occurring in two infants within the first 3 months of life. These tumors histologically resembled dermatofibromas, but the young age of onset, large size, and plaque-like morphology were distinctly different from those of dermatofibromas. The features of these neoplasms are discussed and the differential of other spindle cell tumors of infancy is reviewed.

Incidence of Dermatomyositis in a Nationwide Cohort Study of US Veterans.

This cohort study evaluates the incidence of dermatomyositis and its trend over time in the US Department of Veterans Affairs health care system.