Extensive Skin Involvement Research Articles

Diffuse Geometric Erosions on the Skin of a Premature Infant, Fraternal Twin Unaffected

Diffuse Geometric Erosions on the Skin of a Premature Infant, Fraternal Twin Unaffected

Repeat limited fasciectomy is a safe and effective treatment for recurrence of Dupuytren's disease.

The results of surgery for Dupuytren's disease can be compromised by the potential for disease recurrence and loss of function. Selecting which patients will benefit from repeat surgery, when to operate, and what procedure to undertake requires judgement and an understanding of patient expectations and functional needs. We undertook this study to investigate patient outcomes and satisfaction following repeat limited fasciectomy for recurrent Dupuytren's disease. We prospectively identified all patients presenting with recurrence of Dupuytren's disease who were selected for surgical treatment with repeat limited fasciectomy surgery between January 2013 and February 2015. Patients were assessed preoperatively, and again at a minimum of five years postoperatively. We identified 43 patients who were carefully selected for repeat fasciectomy involving 54 fingers. Patients with severe or aggressive disease with extensive skin involvement were not included; in our practice, these patients are instead counselled and preferentially treated with dermofasciectomy. The primary outcome measured was change in the Michigan Hand Outcomes Questionnaire (MHQ) score. Secondary outcomes were change in finger range of motion, flexion contracture, Semmes-Weinstein monofilament (SWM) values, and overall satisfaction. There was a significant improvement in MHQ scores, across all domains, with a mean overall score increase of 24 points (p < 0.001). The summed flexion contracture across the metacarpophalangeal joint (MCPJ) and the proximal interphalangeal joint (PIPJ) reduced from means of 72.0° (SD 15.9°) to 5.6° (SD 6.8°) (p < 0.001). A significant increase in maximal flexion was seen at the MCPJ (p < 0.001) but not the PIPJ (p = 0.550). The mean overall satisfaction score from the visual analogue scale was 8.9 (7.9 to 10.0). Complications were uncommon although five fingers showed reduced sensibility at final follow-up. Our study shows that repeat limited fasciectomy for selected patients presenting with recurrence of Dupuytren's disease can be an effective and safe treatment resulting in excellent patient-reported outcomes and levels of satisfaction. Cite this article: Bone Joint J2021;103-B(5):946-950.

Toxic Epidermal Necrolysis (TEN)/Stevens-Johnson Syndrome (SJS) Epidemiology and Mortality Rate at King Fahad Specialist Hospital (KFSH) in Qassim Region of Saudi Arabia: A Retrospective Study.

Background Toxic epidermal necrolysis (TEN) and Stevens-Johnson syndrome (SJS) are life-threatening conditions caused by drug reactions. There are multiple causative drugs and different risk factors associated with SJS/TEN. Objectives To study the epidemiology of SJS/TEN and associated mortality rate in Qassim region, Saudi Arabia. Methodology. A retrospective chart review of all patients with the diagnosis of SJS/TEN who were admitted to King Fahad Specialist Hospital (KFSH) in Qassim region, Saudi Arabia, for the period between Jan 2014 to Jan 2019. The Careware information health system is used at KFSH, and patients were identified searching the diagnosis SJS/TEN. Results Total of 10 patients with diagnosis of SJS/TEN were admitted to KFSH for the period from Jan 2014 to Jan 2019. Antibiotics were the culprit in 5 out of 10 patients. 9 out of 10 patients survived with good outcome. One patient with the diagnosis of TEN died, given extensive skin involvement complicated by sepsis. Conclusion Despite the limitation of this study given small sample size, this is the first study of its kind that discusses the epidemiology of SJS/TEN in Saudi Arabia. We found the estimated incidence rate of SJS/TEN in Qassim region to be 7.6 cases per million person-years. Antibiotics and antiepileptics were the culprits in 8 out of 10 patients.

Microneedling Treatment of Striae Distensae in Light and Dark Skin With Long-Term Follow-Up

Striae distensae have notoriously been difficult to treat due to their extensive involvement of nonfacial skin. Microneedling with its lack of thermal injury during microneedling treatment renders it a viable treatment option in darker skin tones and nonfacial regions due to the reduced risk of postinflammatory hyperpigmentation. To describe the clinical results and side effects of microneedling in a series of 25 individuals with striae distensae. Twenty-five consecutive adults (SPT I-V) with striae distensae involving the trunk and extremities were treated using a microneedling device. No additional treatments (topical or intralesional) were applied. Two assessors blinded to treatment protocol rated clinical improvement of striae on a 5-point scale. Side effects were monitored and tabulated. Patients received 1 to 3 consecutive monthly treatments. All striae improved at least 50% after an average of 1.8 treatments, and 28% of patients demonstrated more than 75% clinical improvement. Striae in thicker skin regions (e.g., buttocks/thighs) showed comparable clinical improvement than those in thinner skin areas (e.g., breasts) and did not require additional treatment sessions. Side effects were limited to transient erythema in all skin phototypes. No infections or dyspigmentation were observed. The clinical results obtained in this study support the safe and effective treatment of striae distensae with microneedling in light and dark skin tones in various body locations. Standardization of treatment protocols are anticipated with further (ongoing) studies.

Incontinentia pigmenti in boys: Causes and consequences

Incontinentia pigmenti (IP) is an X-linked genodermatosis caused by mutation of the NEMO/IKBKG gene. While lethal in male foetuses, heterozygous females survive because of X-inactivation mosaicism. Herein we discuss 9 male patients with IP. This is an observational, descriptive, retrospective, multicentre, French study carried out with the help of the SFDP research group. Statistical analysis was performed both on our own patients and on those reported in the literature. Nine boys with no family history of IP but with typical neonatal skin reactions were included. Genetic analysis of blood (n=8) and skin biopsy (n=3) confirmed the diagnosis of IP by identification of common deletion of the IKBKG/NEMO gene (exons 4 to 10) in the state of somatic mosaic in 6 and 2 cases respectively. Where analysed, the karyotype was normal (n=6). Over a median follow-up period of 48 months (3 months to 10 years), 3 patients had neurological abnormalities, 2 had severe ophthalmologic abnormalities, and 1 had dental abnormalities. Extensive skin involvement is a systemic risk factor, unlike cutaneous scarring. IP in boys is often due to a mosaic mutation that should be sought in blood and skin. Long-term neurological and ophthalmological monitoring is essential, especially in cases of extensive skin involvement.

Skin wrinkling of the upper arms: a case of mid-dermal elastolysis

Mid-dermal elastolysis is a rare acquired elastic tissue disorder with about 100 cases reported in the literature. It is characterized by localized patches of finely wrinkled skin on the shoulder and upper extremities and a band-like loss of elastic tissue in the mid-dermal layer on biopsy. Some patients may have symptoms of discomfort, erythema, and/or pruritis. Mid-dermal elastolysis is predominantly seen in young to middle-aged Caucasian females and extensive skin involvement may lead to cosmetic concerns. Furthermore, it is important to rule out other disorders of elastic fiber that are associated with systemic involvement. We present a case of MDE, discuss the differential diagnosis, and describe characteristic clinical features and histology findings of each condition.

Severe Pericardial Effusion Heralding Scleroderma Renal Crisis: A Case Report and the Literature Review

Introduction: Both scleroderma renal crisis and severe pericardial effusion are rare and life-threatening complications of systemic sclerosis. This article describes a case of scleroderma renal crisis heralded by severe pericardial effusion. Case Presentation: The patient was a 39-year-old woman with a history of 8 years diffuse cutaneous scleroderma and extensive skin involvement who referred to Imam Khomeini Hospital Complex, Tehran, Iran, with worsening dyspnea and pleuritic chest pain. Echocardiography revealed severe pericardial effusion, which was drained by the pericardial window. Thereafter, the patient became gradually anuric and her blood pressure was uncontrolled despite maximum doses of antihypertensive agents, including captopril. The serum creatinine level was increased progressively; thus, hemodialysis started. Concomitantly, microangiopathic hemolytic anemia complicated the course of the disease, which responded to plasma exchange. Kidney biopsy revealed pieces of evidence indicating thrombotic microangiopathy. Conclusions: This case report denotes to a probable association between scleroderma the renal crisis and severe pericardial effusion. Therefore, physicians should be vigilant about the renal crisis in patients with systemic sclerosis and pericardial effusion.

Management of pemphigus vulgaris with rituximab: A case report and brief review on emerging treatment options

Pemphigus vulgaris is the most common autoimmune vesiculobullous disorders. It presents with flaccid bullae on the skin and erosions on the mucosa. Severe cases can be life-threatening and hence prompt treatment can prevent untoward consequences. Earlier, steroids were the mainstay of treatment but it causes further medical problems. Nevertheless, new studies have emerged about the pathogenesis of the diseases and treatment protocols have been updated with newer drugs that had comparatively less adverse effects. We report a case of pemphigus vulgaris with extensive involvement of skin and oral mucosa treated with rituximab and adjuvant immunosuppressants and a brief update on various treatment protocols.

Breast Cancer Tissue Markers, Genomic Profiling, and Other Prognostic Factors: A Primer for Radiologists.

An understanding of prognostic factors in breast cancer is imperative for guiding patient care. Increased tumor size and more advanced nodal status are established independent prognostic factors of poor outcomes and are incorporated into the American Joint Committee on Cancer (AJCC) TNM (primary tumor, regional lymph node, distant metastasis) staging system. However, other factors including imaging findings, histologic evaluation results, and molecular findings can have a direct effect on a patient's prognosis, including risk of recurrence and relative survival. Several microarray panels for gene profiling of tumors are approved by the U.S. Food and Drug Administration and endorsed by the American Society of Clinical Oncology. This article highlights prognostic factors currently in use for individualizing and guiding breast cancer therapy and is divided into four sections. The first section addresses patient considerations, in which modifiable and nonmodifiable prognostic factors including age, race and ethnicity, and lifestyle factors are discussed. The second part is focused on imaging considerations such as multicentric and/or multifocal disease, an extensive intraductal component, and skin or chest wall involvement and their effect on treatment and prognosis. The third section is about histopathologic findings such as the grade and presence of lymphovascular invasion. Last, tumor biomarkers and tumor biology are discussed, namely hormone receptors, proliferative markers, and categorization of tumors into four recognized molecular subtypes including luminal A, luminal B, human epidermal growth factor receptor 2-enriched, and triple-negative tumors. By understanding the clinical effect of these prognostic factors, radiologists, along with a multidisciplinary team, can use these tools to achieve individualized patient care and to improve patient outcomes. ©RSNA, 2018.

Quality of life in patients with mycosis fungoides and Sezary syndrome is significantly worse in female patients, Sézary syndrome and those with more extensive skin involvement

Background: Patients with Mycosis fungoides/Sézary Syndrome (MF/SS) have a poor quality of life (QoL) and a combination of skin, pruritus and cancer QOL questionnaires are usually used in combination in clinical trials. The Skindex-29 is a self-administered questionnaire to comprehensively measure the effects of skin disease on patient's quality of life (QoL). We sought to evaluate QOL in patients with MF/SS enrolled in the PROCLIPI (Prospective Cutaneous Lymphoma International Prognostic Index) study using Skindex-29.

Femoral cartilage thickness in patients with systemic sclerosis: It’s relation to vitamin D

Systemic sclerosis (SSc) is a chronic autoimmune progressive connective tissue disease characterized by widespread vascular, immune and fi brotic changes of the skin and internal organs. Articular cartilage thickness previously investigated in few. Lower levels of vitamin D have been demonstrated in SSc patients and may be related to more sever disease of longer duration and extensive skin involvement. Aim of this study was to compare the levels of vitamin D and femoral cartilage thickness (FCT) in SSc patients with that of controls and to analyze the associations between the (FCT), vitamin D levels and SSc-disease parameters.

Oligosecretory Myeloma With Amyloidosis and Alopecia.

Amyloidosis is a systemic illness characterized by the extracellular deposition of abnormal proteins in body tissues and organs. In addition to renal involvement, amyloidosis can also present with a variety of skin manifestations, though rarely with alopecia. Sixteen cases of alopecia secondary to systemic amyloidosis are reported. There is one reported case that presented with alopecia universalis. We report a case of a 68-year-old woman presenting with alopecia universalis, rapid decline in kidney function, and nephrotic syndrome who was found to have multiple myeloma-associated AL amyloidosis (immunoglobulin light chain). Her serological workup including serum electrophoresis was negative and she underwent renal biopsy. Pathology revealed eosinophilic material within the mesangium that was Congo-red positive, had apple-green birefringence under polarized light, and ultramicroscopically appeared as fibrillary material. Subsequent bone marrow examination showed a diffuse increase in plasma cells with atypia indicating plasma cell neoplasm. This case underlines several interesting aspects of multiple myeloma and the way it may present with amyloidosis. The lack of monoclonal spike on electrophoresis yet positive light chain analysis deserves special attention by clinicians to avoid a missed diagnosis. The extensive skin involvement also raises several questions regarding the pathologic mechanisms of alopecia in a patient with amyloidosis.

Atypical Hand, Foot, and Mouth Disease Caused by Coxsackievirus A6 in Denmark: A Diagnostic Mimicker.

Since 2008, outbreaks of atypical hand, foot, and mouth disease (HFMD) in children and adults have been reported worldwide. The majority of these outbreaks are caused by a new lineage of Coxsackie virus A6 (CV-A6) presenting a more severe clinical phenotype than the classical childhood HFMD caused by CV-A16. Between June 2014 and January 2016, 23 cases of atypical HFMD disease presented at a Dermatology Department at a regional University Hospital in Denmark. Patients were referred by general practitioners and dermatologists with a variety of clinical diagnoses, including eczema herpeticum, vasculitis, syphilis, dermatophytid, erythema multiforme and Stevens-Johnson syndrome. Three adults and 3 children required hospitalization due to extensive skin involvement and fever. All reported patients had laboratory-confirmed enterovirus infection. This study demonstrated an upsurge in atypical HFMD caused by CV-A6 in the Region of Southern Denmark and that atypical HFMD can be difficult to diagnose clinically as it may mimic other severe skin diseases.

SEZARY SYNDROME MIMICKING GENERALIZED PSORIASIS VULGARIS

Background: Sezary syndrome is the one type of cutaneous T cell. This disease is characterized by reddish patches or plaques on the skin which extends to whole body into erythroderma, lymphadenopathy and presence of atypical lymphocytes called Sezary cells.Purpose: To know clinical manifestation, examination and management of Sezary syndrome which clinically resembles generalized psoriasis.Case: A man 60 years old came with scaly plaques reddish brown on almost of his body accompanied by lymphadenopathy on the supraclavicular lymph node right and left and accompanied by intense itchy also. Another clinical features were alopecia, palmoplantar hyperkeratosis, onychodysthropy, facies leonine without anesthesia on the lesion and without enlargement of peripheral nerve. From laboratory test, there is an increasing in the number of leukocytes, from the peripheral blood smear examination found Sezary cells and histopathology showed focal athrophy and acanthosis of the epidermis and dense infiltration of lymphocytes in the dermo-epidermal junction and superficial dermis. Case management: Patient received methotrexate (MTX) 3 x 5 mg (1 cylcle) with mometasone furoate 0,1% cream and CTM 3x1 tablet for adjunctive therapy. Methotrexte was discontinued because there are increasing of liver function and deterioration of patient’s condition. After 25 days of treatment, the patient got sepsis and then the patient died. Conclusion: Early onset of Sezary syndrome in this case is difficult to know because the clinical manifestation is similar with psoriasis vulgaris. Supporting examination such as laboratory test, blood smears and histopathology examination could help diagnosis. The presence of lymphadenopathy, atypical lymphocytes in the peripheral blood and extensive skin involvement reflecting the poor prognosis. The most common cause of death was sepsis.

A Simple Dressing Technique Following Dermofasciectomy and Full Thickness Skin Grafting of the Fingers in the Treatment of Severe Dupuytren's Contracture.

Dupuytren's disease with severe finger contractures and recurrent contractures following previous surgery often have extensive skin involvement. In these severe cases, excision of the diseased chord along with the involved skin is a good option to reduce the risk of recurrance. The resulting skin defect can be covered with a full thickness skin graft (FTSG) or a cross finger flap. Cross finger flaps have donor finger morbidity and hence a full thickness graft is usually preferred. The FTSG extending to the midlateral margins on both sides of the finger reduces the risk of joint contracture due to graft shrinkage. Once the FTSG is sutured in place, the standard practice is to compress and secure the graft to its recipient bed with a tie-over dressing and this can be time consuming. We present a simple dressing technique to secure the FTSG without the need for a tie-over dressing.

Age at disease onset may help to further characterize the disease phenotype in psoriatic arthritis

ObjectiveTo evaluate whether the age of disease presentation helps to better characterize the disease phenotype in PsA. MethodsRetrospective cohort study that included 205 consecutive patients fulfilling the CASPAR criteria for PsA. Study outcomes were assessed using univariate and multivariate analyses according to the age of onset of both skin and joint disease (cut off at 40years). ResultsEarly onset psoriasis (EOP) showed more extensive skin involvement (OR 2.3, P=0.011), axial pattern as disease onset (OR 4.6, P=0.009) and mixed pattern during evolution (OR 2.4, P=0.019), family history of both psoriasis (OR 3.1, P=0.003) and PsA (OR 4.0, P=0.021), higher prevalence of HLA-C*06 (OR 2.03, P=0.03) and HLA-B*27 (OR 2.7, P=0.02). Early onset arthritis (EOA) had more family history of PsA (OR 2.9, P=0.007), and HLA-B*27 positivity (OR 5.9, P<0.0001). Patients with late onset arthritis (LOA) were more likely to have DM (OR 4.0, P=0.009), hypertension (OR 2.5, P=0.004), dyslipidemia (OR 2.3, P=0.011), and obesity (OR 1.7, P=0.012). Late onset psoriasis (LOP) tended to have more obesity (OR 1.9, P=0.035), DM (OR 9.4, P<0.0001), hypertension (OR 4.1, P<0.0001), and ischemic heart disease during follow-up (OR 5.9, P=0.021). In multivariate analysis, LOP predicted DM development (OR 12.1, P=0.006). LOA was shown to be an independent risk factor for hypertension (OR 5.2, P=0.039). ConclusionAge at disease onset exerts a strong influence on several domains of disease phenotype in PsA. Therefore, this descriptor should be considered a good stratification option for epidemiological and genetic studies in PsA.

Donor Type, Chronic Gvhd Subtype , and the Severity of Chronic Gvhd at the Time of Initiation of Chronic Gvhd Treatment Affect Failure-Free Survival in the Patients with Chronic Gvhd

Introduction: Chronic graft versus host disease (cGVHD) is one of the major complications after allogeneic hematopoietic cell transplantation (allo-HCT). Several prognostic factors have been proposed to predict the outcomes of cGVHD including progressive type onset, extensive skin involvement, thrombocytopenia and NIH global score (NIH GS). Most studies have been focusing on the factors at the diagnosis of cGVHD without consideration of baseline characteristics prior to allo-HCT. We attempted to evaluate the prognostic factors for the outcomes of cGVHD treatment including the characteristics at the start of cGVHD treatment as well as prior to HCT.Method: We retrospectively reviewed 668 consecutive patients who underwent allo-HCT between 2004 and 2012 at the Princess Margaret Cancer Centre, Toronto, Canada, among whom 277 patients diagnosed as cGVHD and received systemic corticosteroids as a frontline cGVHD therapy. Chronic GVHD was classified and graded using the NIH consensus criteria. We evaluated non-relapse mortality (NRM), relapse and failure-free survival (FFS). FFS was defined as time to a switch in systemic therapy, NRM or relapse. The Kaplan-Meier method was used for FFS. The cumulative incidences of NRM, relapse and the treatment switch (TS) were calculated considering competing risks. Multivariate analysis was performed using the Cox proportional hazard regression model for FFS.Results: With a median follow-up duration of 26 months, the median time from HCT to cGVHD treatment was 183 days (range, 61-828). 102 patients (36.8%) were classified as classical cGVHD and 175 (63.2%) as overlap syndrome. At the start of cGVHD treatment 25 patients (9.0%) had mild cGVHD by the NIH GS, 189 (68.2%) moderate and 63 (22.7%) severe.Median age at allo-HCT was 51 year-old (range, 19-70). 162 patients (58.5%) were males and 65 (23.5%) patients were gender match of female donor to male recipient. 257 patients (92.8%) received peripheral blood stem cells (PBSC).175 grafts (63.2%) were from matched sibling donors (MSD). 180 patients (65%) received myeloablative conditioning. GVHD prophylaxis was calcineurin inhibitor (CNI) and methotrexate (n=82, 29.6%), CNI and mycophenolate mofetil (n=141, 50.9%), CNI and T-cell depletion (n=37, 13.5%) or others (n=17, 6.1%).The median FFS was 255 days (95% CI, 218-321). The severity of cGVHD, NIH GS correlated with FFS: median FFS was 164 days in severe vs 238 days in moderate vs 304 days in mild (p=0.001). The overlap syndrome was associated with a shorter FFS than classical cGVHD (223 vs 329 days, p=0.015). Patients receiving MSD graft showed longer FFS (329 days) than unrelated donor (196 days; p=0.004).The cumulative incidence of TS was 47.7% at 1 year. The NRM was 7.1% and relapse rate was 6.8% at 1 year. The MSD was associated with a lower 1-year NRM than the unrelated donors (4.2% vs 12.3%, p=0.003) while no difference between 2 groups for TS (p=0.731) or relapse at 1 year (p=0.565).Patients with overlap syndrome had higher NRM at 1 year than with classical cGVHD (10.0% vs 2.2%, p=0.009), but no differences in TS or relapse at 1 year (p=0.167 and p=0.138). Chronic GVHD severity by NIH GS showed a significant correlation with TS (28% in mild, 51.9% in moderate, and 43.8% in severe grade at 1 year, p=0.02) and NRM (4% in mild, 3.6% in moderate, and 19.1% in severe grade at 1 year, p<0.001), but with relapse (p=0.784).Multivariate analysis for FFS confirmed that the use of unrelated donor showed a worse FFS (hazard ratio (HR) 1.660, p=0.001). FFS was also associated with the severity of cGVHD, NCC GS (mild vs moderate vs severe; HR 1 vs 2.1 vs 2.9, p=0.002) and the cGVHD subtype (classical vs overlap, HR 1 vs 1.39, p=0.028).We then assigned score 0 for NIH GS mild, 1 for moderate, and 2 for severe; for NIH subtype, score 0 for classical and 1 for overlap; for donor types, score 0 for MSD and 1 for unrelated donors. After summation of the scores, we regrouped them into low (score 0, n=11, 3.9%), intermediate (score 1-2, n=168, 60.6%), and high risk groups (score 3-4, n=98, 35.3%). The risk stratification model correlated nicely with FFS (FFS duration, 1977 days in low vs 341 days in intermediate, and 150 days in high risk group, p<0.001).Conclusion: the use of unrelated donor, overlap subtype of chronic GVHD and severe grade of chronic GVHD at the time of initiation of chronic GVHD treatment affect adversely on failure-free survival. DisclosuresKim:Novartis Pharmaceuticals: Consultancy, Research Funding; Bristol-Myers Squibb: Consultancy, Research Funding.

Distinctive cutaneous and systemic features associated with antitranscriptional intermediary factor-1γ antibodies in adults with dermatomyositis

Antibodies against transcriptional intermediary factor (TIF)-1γ are associated with malignancy in dermatomyositis (DM). Identification of clinical findings associated with anti-TIF-1γ antibodies in DM is a high priority for both patient diagnosis and risk assessment. We sought to define the clinical phenotype of patients with anti-TIF-1γ DM. Using a novel, sensitive, and specific assay for anti-TIF-1γ antibodies, we retrospectively tested plasma from 134 adult patients with DM and examined associations between anti-TIF-1γ antibodies and particular clinical and laboratory features. In all, 55 (41%) patients had autoantibodies to TIF-1γ. Anti-TIF-1γ positive patients were less likely to have systemic features including interstitial lung disease, Raynaud phenomenon, and arthritis/arthralgia. Patients with TIF-1γ autoantibodies had more extensive skin involvement, and some patients manifested characteristic findings including palmar hyperkeratotic papules, psoriasis-like lesions and a novel finding of hypopigmented and telangiectatic ("red on white") patches. This was a retrospective study from a single tertiary referral center. TIF-1γ is the most commonly targeted DM-specific autoantigen in adults in a large US cohort. Although these patients tend to have less systemic involvement, their skin disease is often extensive and characteristic. Recognition of cutaneous findings in anti-TIF-1γ positive patients may allow more accurate and timely diagnosis and effective treatment of patients with DM.

Combination Trimodality Therapy Using Vismodegib for Basal Cell Carcinoma of the Face.

Background. For large basal cell carcinomas (BCCs) of the head and neck, definitive surgery often requires extensive resection and reconstruction that may result in prolonged recovery and limited cosmesis. Vismodegib, a small-molecule inhibitor of the hedgehog pathway, is approved for advanced and metastatic BCCs. We present a case of advanced BCC treated with combination of vismodegib, radiotherapy, and local excision resulting in excellent response and cosmesis. Case Presentation. A 64-year-old gentleman presented with a 5-year history of a 7 cm enlarging right cheek mass, with extensive vascularization, central ulceration, and skin, soft tissue, and buccal mucosa involvement. Biopsy revealed BCC, nodular type. Up-front surgical option involved a large resection and reconstruction. After multidisciplinary discussion, we recommended and he opted for combined modality of vismodegib, radiotherapy, and local excision. The patient tolerated vismodegib well and his right cheek lesion decreased significantly in size. He was then treated with radiotherapy followed by local excision that revealed only focal residual BCC. Currently, he is without evidence of disease and has excellent cosmesis. Conclusions. We report a case of locally advanced BCC treated with trimodality therapy with vismodegib, radiotherapy, and local excision, resulting in excellent outcome and facial cosmesis, without requiring extensive resection or reconstructive surgery.

Severe sepsis with Stevens Johnson syndrome caused by Mycoplasma pneumoniae – case presentation

Background Mycoplasma pneumoniae is widely known as the etiological agent of “atypical pneumonia”, the most common clinical aspects of the infection being bronchiolitis and acute tracheobronchitis. It can also determine extrapulmonary manifestation such as ear, nose and throat infections, neurological, cardiac or dermatological manifestations. Dermatological involvement is second most common, after respiratory infections, and it can vary from urticaria-like rashes to Stevens-Johnson syndrome. Stevens-Johnson syndrome is severe form of immunecomplex–mediated hypersensitivity complex characterized by a hallmark of skin lesions spanning from mild forms to extensive involvement of skin and mucosa. It can be caused by a viral or bacterial infection or it can be drug induced.