Cutaneous Lesions In Patients Research Articles

Evaluation of interleukin-23 expression in classic kaposi’s sarcoma and AIDS-Associated

Kaposi’s sarcoma (KS) is a low-grade vascular tumor that presents most frequently with skin lesions. Multiple factors are related to KS pathogenesis. The interleukin-23 (IL-23) is an immunomodulatory cytokine with pro-carcinogenic role. The aim of this study was to evaluate the detection of IL-23 in cutaneous lesions of patients with KS classic and KS-AIDS. This was a cross-sectional study conducted at the public Hospital in Natal, Rio Grande do Norte, Brazil. This study included 13 biopsies of patients which presented KS with histopathological confirmation. For detection of IL-23, the immunohistochemical analysis was made with monoclonal antibody anti-IL-23. The immunostaining for IL-23 in biopsies was positive in 61.5% (8/13) of cases. Of seven lesions classified as patch-stage, three were positive for IL-23 (42.8%); two of three samples classified as plaque-stage were positive for IL-23 (66.7%); all the nodular-stage lesions were positive for IL-23. The positivity found in KS-AIDS lesions was 66.7% (6/9) and KS classic 50% (2/4). Our data supports the concept that IL-23 might be a pro-carcinogenic mediator, detected in KS lesions, probably favoring the progression of the tumor. KEY WORDS: Sarcoma; Kaposi; interleukin-23; tumor microenvironment.

Cutaneous Manifestations of COVID-19 in Critical Care.

SARS-CoV-2 infection can cause virus-mediated endothelial dysfunction, which in turn may lead to coagulopathy and ischemic microangiopathy. In the critical care population, cutaneous skin manifestations related to vascular compromise due to COVID-19 include livedo and purpura. These lesions can be difficult to differentiate from other dermatologic conditions seen in this population, including skin failure and deep-tissue pressure injuries. In addition, similarities in underlying pathophysiological mechanisms of these skin conditions can cause diagnostic overlap. Skin failure is known to occur in critical care patients owing to disease severity and shunting of blood to vital organs. COVID-19-related ischemic lesions can mimic the clinical course of deep-tissue pressure injury. The viral endothelial dysfunction present in patients with COVID-19 decreases tissue tolerance, which can result in an increased risk of hospital-acquired pressure injury. Extrinsic factors can also complicate diagnosis of cutaneous lesions in patients with COVID-19.

Exploring the Association Between History of Psoriasis (PSO) and Disease Activity in Patients with Psoriatic Arthritis (PsA).

IntroductionPsoriatic arthritis (PsA) is a common inflammatory disease affecting the peripheral and axial skeleton. History of psoriasis (PSO), either personal or family history, is an important factor in the diagnosis of PsA. We investigated the association between history of PSO and clinical characteristics of PsA.MethodsPsA patients were consecutive recruited from 2019 to 2020. These patients were subjected to clinical, biochemical, and radiographic examinations, and disease activity was evaluated. Continuous and categorical variables analyses were presented.ResultsAll registered patients (296 cases) met the classification criteria of PsA. They were divided into three groups based on the history of psoriasis (PSO), as: (1) 145 patients with PSO themselves (pPsA); (2) 96 patients with family history of PSO (fPsA); (3) 55 patients with family history and coexisting PSO themselves (fPsA/PSO). Compared to fPsA/PSO, the levels of CRP, ESR, uric acid, DAPSA, BASDAI, ASDAS, and BASFI were lower in fPsA, but similar to pPsA. The severity of sacroiliitis tended to be more severe in fPsA/PSO than fPsA (OR2 vs. 3 0.508; 95% CI 0.272 to 0.949, p < 0.05). No significant differences were found in HLA-B-27 and common inflammatory articular and extra-articular manifestations among the three groups. Furthermore, there were no differences in LEI, TJC, SJC, and DAS28CRP. Interestingly, a correlation was found between the ages of individuals with PSO and the onset of arthritis, and the earliest arthritis onset occurred in fPsA/PSO patients (p < 0.001).ConclusionsOur study demonstrates that currently existing cutaneous lesions in patients themselves are correlated with disease activity and severity of axial joint damage, whereas family history does not have an evident impact on the disease activity of PsA.

Different cell death mechanisms are involved in leprosy pathogenesis

Leprosy is a chronic granulomatous disease that remains a serious public health problem in developing countries. According to the Madrid classification, leprosy presents in four clinical forms: two immunologically unstable forms (indeterminate and borderline) and two stable polar forms (tuberculoid and lepromatous). In leprosy, the relationship of cell death to clinical disease outcome remains unclear. Therefore, we investigated the extent of autophagy and different cell death mechanisms—such as apoptosis, necroptosis, and pyroptosis—in cutaneous lesions of patients with leprosy, as well as the role of these mechanisms in clinical disease progression. This cross-sectional analytical study included 30 patients with a confirmed diagnosis of leprosy, with 10 patients in each of the following groups: lepromatous (LL), tuberculoid (TT), and indeterminate (II) leprosy groups. For histopathological analysis, skin samples were subjected to haematoxylin-eosin staining and immunostaining for apoptotic and necroptotic markers. The results indicated that FasL expression was much higher in the LL form than in the TT and II forms. Similar results (higher expression in the LL form than in the TT and II forms) were observed for caspase 8, RIP1, and RIP3 expressions. MLKL, BAX, and caspase 3 expression levels were highest in the LL form, especially in globular foamy macrophages. Beclin-1 expression was highest in the TT form but was low in LL and II forms. Caspase 1 expression was highest in the LL form, followed by that in the TT and II forms. In conclusion, our study elucidates the role of different cell death mechanisms in the pathophysiology of various forms of leprosy and suggests measures that may be used to control the host response to infection and disease progression.

28423 Treatment of cutaneous lesions in patient with blue rubber bleb nevus with diode laser

Blue rubber bleb nevus (BRBN) is a rare congenital skin disorder characterized by numerous, diffuse venous malformations of the cutaneous. The treatment of cutaneous lesions is not usually required except for cosmetic purposes while previous methods to remove cutaneous lesions including ND:YAG laser, photocoagulation, sclerotherapy, surgery often could lead to scars. We report a case of BRBN who had a good response to diode laser without adverse effects. A 16-month-old girl presented to our hospital with multiple, discrete, dark blue to violaceous soft compressible nodules on the skin as well as palm and sole. Especially, a large violaceous perianal lesion was about 4 cm in diameter that was at high risk for hemorrhage due to an anal fissure making the children occasionally bleeding while a bowel movement. Most of the lesions presented at birth remained stable in size and number. Abdominal ultrasound has not been discovered any abnormalities. Histopathology image of the cutaneous lesion shows variably sized, irregular and thin-walled vessels lined by a single layer of endothelial cells in the dermis. Immunohistochemical stain demonstrates endothelial cells positive for CD31. The girl has been treated using 810-nm diode laser at the setting 30-35 J/cm2, 30 ms for the cutaneous lesions on her back when the diagnosis of BRBN was established. After four times of treatment, a significant reduction in color and size was achieved on the back cutaneous lesions without adverse effects. In conclusion, this is the case of BRBN showing a good response to diode laser.

Skin Manifestations in COVID-19: Prevalence and Relationship with Disease Severity.

Background: Data on the clinical patterns and histopathology of SARS-CoV-2 related skin lesions, as well as on their relationship with the severity of COVID-19 are limited. Methods and Materials: Retrospective analysis of a prospectively collected cohort of patients with SARS-CoV-2 infection in a teaching hospital in Barcelona, Spain, from 1 April to 1 May 2020. Clinical, microbiological and therapeutic characteristics, clinicopathological patterns of skin lesions, and direct immunofluorescence and immunohistochemical findings in skin biopsies were analyzed. Results: Fifty-eight out of the 2761 patients (2.1%) either consulting to the emergency room or admitted to the hospital for COVID-19 suspicion during the study period presented COVID-19 related skin lesions. Cutaneous lesions could be categorized into six patterns represented by the acronym “GROUCH”: Generalized maculo-papular (20.7%), Grover’s disease and other papulo-vesicular eruptions (13.8%), livedo Reticularis (6.9%), Other eruptions (22.4%), Urticarial (6.9%), and CHilblain-like (29.3%). Skin biopsies were performed in 72.4%, including direct immunofluorescence in 71.4% and immunohistochemistry in 28.6%. Patients with chilblain-like lesions exhibited a characteristic histology and were significantly younger and presented lower rates of systemic symptoms, radiological lung infiltrates and analytical abnormalities, and hospital and ICU admission compared to the rest of patients. Conclusion: Cutaneous lesions in patients with COVID-19 appear to be relatively rare and varied. Patients with chilblain-like lesions have a characteristic clinicopathological pattern and a less severe presentation of COVID-19.

Clinical picture and treatment of cutaneous lesions in patients with neurofibromatosis type 1.

IntroductionThe most common type of neurofibromatosis is Recklinghausen disease (NF-1). A major feature of NF1 is the development of localized cutaneous neurofibromas. Such types of lesions are manifested in > 99% of adults with NF1 and are responsible for major negative effects on quality of life.AimTo present the clinical picture of patients with NF-1 and to report authors’ own observations related to their surgical treatment.Material and methodsA retrospective study aiming to present a group of patients with Recklinghausen disease was planned. The study was carried out in 39 patients with NF-1.ResultsAll patients had cutaneous neurofibromas localized in different parts of the body. The three most frequent localizations were the abdomen, back, and face. Surgical treatment was performed in 31 patients. All patients had discomfort due to the presence of cutaneous nodules especially because their visibility and multiplicity.ConclusionsDue to the polymorphism of the clinical picture and unpredictable course of the disease, there is no one standard of treatment of patients with Recklinghausen disease. Excision of tumours in the early stage of their development gives better functional and aesthetic results.

Study of cutaneous manifestations in chronic kidney disease patients on maintenance hemodialysis at a tertiary care centre

&lt;p class="abstract"&gt;&lt;strong&gt;Background:&lt;/strong&gt; Chronic kidney disease (CKD) is a progressive loss of kidney function over a period of months or years through five stages, presents with various cutaneous manifestations regardless of its aetiology. Patients of CKD disease on hemodialysis treatment which prolong the life expectancy and can also produce skin lesions. Skin changes described in patients with chronic renal disease are also found in patients with renal failure undergoing treatment with either peritoneal dialysis or hemodialysis. High percentage of patients receiving dialysis complains of pruritus that may be severe, in some instances the pruritus worsens with dialysis. The aims of the present study were to study the pattern of cutaneous lesions in patients with chronic kidney disease and to determine the incidence of skin manifestations specific to and associated with CKD.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Methods:&lt;/strong&gt; Hundred patients of CKD on MHD with cutaneous manifestations attending skin outpatient department, nephrology out-patient department, dialysis center and patients admitted in medical wards were included in the study.&lt;strong&gt;&lt;/strong&gt;&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Results:&lt;/strong&gt; The common cutaneous manifestations were xerosis 91%, pallor 78% and pruritus 69%. Nail manifestations include onycholysis 37% and half and half nails 33%. Hair manifestations in 24% patients.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Conclusions:&lt;/strong&gt; Most common cutaneous manifestation was xerosis, followed by pallor. Most common nail manifestation was onyholysis.&lt;/p&gt;

Fish tank granuloma: An emerging skin disease in Iran mimicking Cutaneous Leishmaniasis.

ObjectiveMycobacterium marinum causes a rare cutaneous disease known as fish tank granuloma (FTG). The disease manifestations resemble those associated with Cutaneous Leishmaniasis (CL). The aim of this study was to determine whether FTG was the cause of cutaneous lesions in patients who were referred to the Parasitology laboratory of Imam Reza Hospital in Mashhad to be investigated for CL.Materials/MethodsOne hundered patients, clinically diagnosed with CL between April 2014 and March 2015, were included in this study. Ziehl-Neelsen staining was performed to identify acid-fast Mycobacterium in addition to bacterial cultures using Löwenstein-Jensen medium. Skin lesion samples were also collected and kept on DNA banking cards for PCR testing.ResultsTwenty-nine of the 100 individuals with skin lesions, and therefore suspected of suffering from CL, tested positive for Mycobacterium marinum by PCR. Of these, 21 (72.4%) were male and 8(27.6%) were female. In 97% of these cases the lesions were located on hands and fingers. These patients had a history of manipulating fish and had been in contact with aquarium water. A sporotrichoid appearance was observed in 58.6% of the patients with mycobacterial lesions; 67% of patients had multiple head appearance.ConclusionPatients suspected to have CL and who test negative for CL could be affected by FTG. Therefore, after obtaining an accurate case history, molecular diagnosis is recommended for cases that give a negative result by conventional methods.

A study of cutaneous manifestations in diabetes mellitus patients in tertiary care centre

&lt;p class="abstract"&gt;&lt;strong&gt;Background:&lt;/strong&gt; Diabetes is the most common metabolic disorder with increased prevalence in developing countries like India. It affects almost all organs among which skin is affected by the acute metabolic derangments as well as by chronic degenerative complications. The aims were to study the pattern of cutaneous lesions in patients with diabetes mellitus and to determine the incidence of skin manifestations specific to and associated with diabetics.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Methods:&lt;/strong&gt; Hundred cases of diabetes mellitus with cutaneous manifestations attending skin outpatient department and other patients admitted in medical wards were included in the study.&lt;strong&gt;&lt;/strong&gt;&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Results:&lt;/strong&gt; The common skin disorders were cutaneous manifestations (67%), dermatosis more commonly associated with diabetes (36%), neuropathic and ischemic diabetic skin disease (11%).&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Conclusions:&lt;/strong&gt; Most common cutaneous manifestation was cutaneous infections followed by dermatosis more commonly associated with diabetes. Among cutaneous infections fungal infections were more common followed by bacterial infections. Cutaneous infections were more common in patients with poor glycaemic control and the association was found to be statistically significant.&lt;/p&gt;

Recurrent Erythema Nodosum in a Child with a &lt;i&gt;SHOC2&lt;/i&gt; Gene Mutation

We report the case of a 6-year-old male who developed recurrent erythema nodosum (EN) at the age of 3 years. The patient exhibited hypertelorism, low-set ears, micrognathia, moderate intellectual disability, thin long fingers, loose anagen hair, and prominent palmoplantar wrinkles. A heterozygous single nucleotide variant in the SHOC2 gene (c.4 A > G, p.S2G) was identified. Patients with a SHOC2 mutation exhibit a unique combination of ectodermal abnormalities including darkly pigmented skin and loose anagen hair. This report is the first to describe EN in a patient with SHOC2 mutation, and to examine the patient's hair using scanning electron microscopy. We hypothesize that the RAS/MAPK pathway is associated with the pathogenesis of cutaneous lesions in patients with SHOC2 mutations via autoinflammation and disturbance of epithelial stem cells.

Study of new onset cutaneous manifestations in Rheumatic Diseases

Background: Skin manifestations are an important clue to underlying rheumatological conditions and at times the first manifestation of the disease. Their identification helps in diagnosis, classification and follow up of these diseases. Hence we conducted this study to assess the new onset cutaneous lesions in patients with rheumatic diseases and correlate skin lesions with disease activity and study the response to therapy over a period of 3 months. Materials and Methods: This prospective observational study was done in KEM Hospital, Mumbai over 18 months recruiting 78 patients, presenting to Rheumatology OPD / wards with new onset skin manifestations. Disease activity was calculated as per standard indices for each rheumatological disease. Skin lesions appearing due to adverse effects of drugs or unrelated to the disease were excluded from the study. The outcome of the skin lesions was assessed at 3 months follow up. Results: Mean age of patients was 38 years with 91% being females. SLE was the most common diagnosis. The most common skin lesions were malar rash among SLE patients; rheumatoid nodules in patients of RA; Sclerodactyly in the Scleroderma patients and Heliotrope rash amongst the dermatomyositis patients. The mean SLEDAI score in the group with LE non -specific lesions was significantly higher compared to the group with LE-specific lesions (P<0.0001). At 3 months there was statistically significant reduction in SLEDAI score after treatment in SLE patients. In patients of RA, 74% patients showed reduction in DAS 28 ESR score with treatment at 3 months. Systemic sclerosis patients failed to show significant improvement in Modified Rodnan’s Skin Score after 3 months of treatment. Conclusions: Among all rheumatological conditions SLE presents most often with skin involvement. Patients with LE specific lesions have lower disease activity (SLEDAI score) as compared to LE nonspecific lesions. At 3 months follow up the response to treatment is good in SLE patients with reduction in SLEDAI scores and also in RA patients with reduction in DAS 28 ESR scores.

Mycobacterium decipiens sp. nov., a new species closely related to the Mycobacterium tuberculosis complex.

Two mycobacterial strains with close similarity to the Mycobacterium tuberculosis complex (MTBC) were isolated from cutaneous lesions of patients in the USA and Italy. At the phenotypic level, similarities to the MTBC included slow growth rate, rough morphotype of the unpigmented colonies and nearly identical high-performance liquid chromatography profiles of mycolic acids. In contrast to the MTBC, the strains were niacin- and nitrate-negative, and catalase-positive both at 68 °C and in semi-quantitative tests. The clinical isolates were more closely related to M. tuberculosis than to any other known mycobacterium and scored positive with commercial DNA probes (Hologic AccuProbe M. tuberculosis). Both average nucleotide identity and genome-to-genome distance suggested the strains are different from the MTBC. Therefore, given the distinguishing phenotypic and genomic-scale differences, we submit that the strains belong to a new species we have named Mycobacteriumdecipiens with type strain TBL 1200985T (=ATCC TSD-117T=DSM 105360T).

Approach to skin and soft tissue infections in non-HIV immunocompromised hosts.

Skin and soft tissue infections are frequent contributors to morbidity and mortality in the immunocompromised host. This article reviews the changing epidemiology and clinical manifestations of the most common cutaneous pathogens in non-HIV immunocompromised hosts, including patients with solid organ transplants, stem cell transplants, solid tumors, hematologic malignancies, and receiving chronic immunosuppressive therapy for inflammatory disorders. Defects in the innate or adaptive immune response can predispose the immunocompromised host to certain cutaneous infections in a predictive fashion. Cutaneous lesions in patients with neutrophil defects are commonly due to bacteria, Candida, or invasive molds. Skin lesions in patients with cellular or humoral immunodeficiencies can be due to encapsulated bacteria, Nocardia, mycobacteria, endemic fungal infections, herpesviruses, or parasites. Skin lesions may reflect primary inoculation or, more commonly, disseminated infection. Tissue samples for microscopy, culture, and histopathology are critical to making an accurate diagnosis given the nonspecific and heterogeneous appearance of these skin lesions due to a blunted immune response. As the population of non-HIV immunosuppressed hosts expands with advances in medical therapies, the frequency and variety of cutaneous diseases in these hosts will increase.

Enhanced mRNA expression of plasminogen activator inhibitor-1 in livedoid vasculopathy lesions.

Thrombosis and inflammation play an important role in pathophysiology of livedoid vasculopathy (LV). Plasminogen activator inhibitor-1 (PAI-1) is the main physiological inhibitor of fibrinolysis and is a pivotal modulator in a broad range of biological processes. The study specimens were retrospectively selected from archives of pathology department. We investigated PAI-1 mRNA expression in the paraffin blocks of patients with biopsy-proven LV and controls. We analyzed the presence of thrombus, fibrinoid necrosis, ulcer, and epidermal atrophy in study samples. The correlation between histologic findings and PAI-1 expression was investigated. Analyses were performed in 14 LV patients (mean age 31±20, 79% female) and 4 controls (mean age 64±19, 50% female). PAI-1 gene expression was significantly higher in LV compared to the control group (median 7.74 (Iqr:13.94) vs 1.0 (0.31)), P=.011. Histological analysis displayed that fibrinoid necrosis was present on all patients with LV, 61.5% displayed thrombus, 46.2% displayed ulcer, and 15.4% displayed epidermal atrophy. Overall, we did not observe any discerning difference in PAI-1 expression between the LV blocks with or without thrombus, fibrinoid necrosis, or epidermal atrophy, yet the LV specimens that displayed ulcer histologically had higher PAI-1 mRNA expression compared to those without ulcer (median 13.98 (Iqr:19.21) vs 2.86 (5.59)), (P=.046). PAI-1 mRNA expression is significantly increased in cutaneous lesions of patients with LV. Histological finding of ulcer is associated with increased PAI-1 expression in LV specimen. In the current era of PAI-1 inhibitors, enhanced local PAI-1 expression can form a novel and local therapeutic target in LV.

Progressive cutaneous Cryptococcosis complicated with meningitis in a myasthenia gravis patient on long-term immunosuppressive therapy \u2013 a case report

BackgroundCryptococcosis is an opportunistic infection caused by the encapsulated yeast Cryptococcus neoformans and most remarkably manifests in HIV-infected individuals, especially in the settings of very low CD4 count. Development of cryptococcosis in HIV-uninfected individuals is exceedingly rare and usually signifies a marked immunodeficiency. Cryptococcosis in association with myasthenia gravis or thymoma has been previously documented in only very few cases in the literature.Case presentationWe reported a complicated case of severe cutaneous cryptococcosis in a 39-year-old Vietnamese male patient with myasthenia gravis on long-term immunosuppressive therapy. The patient presented with a five month history of recurrent and progressive skin lesions that later on progressed into cryptococcal meningitis.ConclusionThrough this case, we aimed to emphasize the importance of including cutaneous cryptococcosis in the differential diagnosis of cutaneous lesions in patients on chronic immunosuppressive therapy. The cutaneous manifestations of cryptococcosis can be the first clue for a disseminated disease, which makes early recognition crucial and life-saving.

Comparison of cutaneous manifestations of diabetic with nondiabetic patients: A case-control study

Background: Diabetes mellitus is a metabolic disorder characterized by raised fasting and postprandial blood glucose levels and a variety of multisystem complications. The prevalence of skin manifestations seems to be similar between type 1 and type 2 diabetes mellitus patients. Cutaneous manifestations usually develop following the diagnosis of diabetes, but in some patients, they are the initial presenting signs, thereby helping in the early diagnosis of diabetes mellitus. Objectives: The objective of this study is to describe the cutaneous lesions in patients with type 2 diabetes mellitus and to compare the pattern of skin manifestations in diabetics and nondiabetics in coastal Karnataka and neighboring districts of Kerala. Methodology: This case-control study included 400 patients with type 2 diabetes mellitus and cutaneous manifestations attending the outpatient Departments of Dermatology and General Medicine and those admitted under these departments. A total of 400 age and sex-matched nondiabetic patients were included as controls. Results: Cutaneous infections were noted in 148 (37%) diabetics of which fungal infections were the most common seen in 106 (26.5%) patients, followed by xerosis in 121 (30.25%) and acrochordons in 71 (17.75%) patients. Other dermatoses associated with diabetes mellitus noted were acanthosis nigricans (5.5%), scleredema diabeticorum (0.25%), diabetic bullae (0.5%), and Kyrle's disease (1%). Cutaneous changes associated with neurovascular complications included diabetic foot in 3%, diabetic dermopathy in 2%, and pigmented purpuric dermatosis in 0.25% of the diabetics. Conclusion: A joint effort between dermatology and general medicine is necessary for the early recognition and treatment of the skin conditions and also to ensure adequate metabolic control.

Dermatoscopy in the evaluation of cutaneous lesions in patients with familial atypical multiple-mole melanoma syndrome

Skin cancer is currently the most common cancer type afflicting North Americans today and with an aging population the incidence is likely to increase. Certain skin cancers such as melanoma can result in significant morbidity and mortality especially if not identified and treated early. The traditional ABCDE rule (asymmetric shape, irregular borders, variation in colour, diameter &gt;6 mm and evolution/elevation of the lesion) used to identify concerning tumours will in fact miss many early melanomas. This problem is exacerbated in patients with hereditary forms of melanoma such as Familial Atypical Multiple- Mole Melanoma (FAMMM) syndrome where multiple atypical nevi satisfying the ABCDE criteria are present and would therefore warrant biopsy. Most of these biopsies would be unnecessary and would increase the patient’s morbidity. Dermatoscopy is a clinical tool that provides a bridge between clinical and histopathologic evaluation and has proven to be helpful in triaging suspicious lesions in the general population and to be a first-line screening tool to increase the detection of early melanomas in patients with FAMMM.

First Presentation of Hereditary Leiomyomatosis and Renal Cell Cancer Syndrome in Pregnancy.

Reed's syndrome, also known as hereditary leiomyomatosis and renal cell cancer (HLRCC) syndrome, is an autosomal dominant condition in which affected individuals may develop cutaneous leiomyomas, uterine fibroids, and renal cell carcinoma. This report describes a unique case of HLRCC because it presented in pregnancy with development of cutaneous pilar leiomyomas. Review of the literature for previous cases of Reed's syndrome during pregnancy including PubMed and Medline search. Genetic testing of this patient demonstrated a mutation in the fumarate hydratase (FH) gene. Review of the literature showed only 1 previous case series that described the onset of cutaneous lesions during pregnancy. This case serves as a reminder that there may exist a correlation between pregnancy and the first manifestation of cutaneous lesions in patients with HLRCC, and thus an increased clinical suspicion is warranted during this period.

The spectrum of histopathologic findings in cutaneous lesions in patients with Still disease.

Still disease is a rare disorder characterized by seronegative arthralgias/arthritis, spiking fever, and either an evanescent salmon-colored rash or persistent papules and plaques. We describe the clinical and biopsy findings in 10 patients with the evanescent rash of Still disease. Fourteen biopsy specimens were studied from seven women and three men with a mean age of 44.4 years. The skin lesions were typically erythematous macules, papules, or plaques with a median duration of 5 weeks. All patients had systemic symptoms, including fever and arthralgias. The infiltrate was predominantly lymphocytic in six biopsy specimens, approximately equal lymphocytic and neutrophilic in four biopsy specimens, and predominantly (although never exclusively) neutrophilic in four biopsy specimens. Other findings included focal vacuolar interface changes, neutrophilic eccrine hidradenitis, epidermal neutrophils, dermal mucin, and acanthosis associated with numerous upper epidermal dyskeratotic cells. It is important to be aware of the broad histologic spectrum that may be encountered in Still disease and to consider Still disease in the differential diagnosis of neutrophil-rich, lymphocyte-rich, and mixed inflammatory dermatoses. While the histologic findings seen in biopsy specimens of the evanescent rash are nonspecific, a distinctive variant also exists characterized by prominent epidermal apoptosis, especially involving the upper layers.