Evaluation of the Quality of Life and the Demographic and Clinical Characteristics of Patients With Pemphigus With Oral Mucosal Involvement: A Multicenter Observational Study.

INTRODUCTION: Pemphigus vulgaris (PV) is an autoimmune disease primarily affecting the oral mucosa. OBJECTIVES: This study aimed to determine the demographic, clinical and treatment characteristics of PV patients with oral mucosal involvement and to assess the impact on their quality of life. METHODS: We conducted a prospective observational study among 106 patients diagnosed with PV and presenting oral mucosal involvement. Demographic data, clinical and treatment characteristics, and quality of life questionnaires were recorded. RESULTS: The study included 106 patients, 55 (51.89%) were male and there was a predominance of the mucocutaneous subtype in 83 individuals (78.38%). Oral mucosa was the initial site of manifestation in 44 patients (41.51%). Bilateral buccal mucosa was the most frequently affected site. The predominant symptom reported was a burning sensation, noted in 91 patients (85.85%). Oral mucosal examination revealed erosions in 85.85% of the patients. Systemic steroids were the most commonly administered treatment, and rituximab was used in 18 patients (16.98%). A positive and significant correlation was found between pemphigus severity and Oral Health Impact Profile-14, Dermatology Life Quality Index and Dermatological Quality of Life Scale scores (P < 0.05). The presence of superficial ulcers, flaccid bullae, lesion diameter ≥1 cm, and >10 lesions were factors that markedly diminished quality of life. Complete response to treatment was noted in all patients administered rituximab. CONCLUSIONS: The most common area of involvement was bilateral buccal mucosa, and the severity of PV closely correlated with a decline in quality of life measures. These results highlight the need for careful clinical oversight of PV, taking into account its effects on patients quality of life.

Multiple indurated nodular lesions on the lip, upper trunk and limbs of an 80-year-old man.

We report multiple indurated nodular lesions on the lip, upper trunk and extremities of an 80-year-old man.

Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: 11-year Demographic Clinical and Prognostic Characteristics.

Background: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are acute, life-threatening, severe drug reactions. Randomized studies on these diseases are difficult to perform. Aims and Objectives: The purpose of this study was to summarize the demographic and clinical characteristics of patients with SJS and TEN in a tertiary hospital in Turkey. Materials and Methods: We evaluated the records of 33 patients with SJS and TEN who were followed in our clinic or examined between January 2008 and June 2019, retrospectively. Age, sex, time of admission to hospital, causative drug, presence of concomitant disease, skin findings, mucosal involvement, the severity-of-illness score for TEN, the medication used, antibiotic use, transfer to intensive care, development of complications, and death or discharge status were noted. Results: Of the 33 patients, 11 (33.3%) had SJS, 3 (9.1%) had SJS/TEN overlap, and 19 (57.6%) had TEN. The majority (60.6%) of the patients were female. Nineteen (57.6%) patients had one, and 13 (39.4%) had more than one suspected drug exposure in their history. The most commonly suspected drugs were antibiotics. Twelve (36.4%) patients had intensive care unit hospitalization. Ten (30.3%) patients died. Conclusion: The demographic data of our study were consistent with the literature. Similar to the literature, antibiotics were the most common reaction-causing drugs. However, antiepileptic drugs, which were more frequently reported in other studies, were identified as suspicious in only one patient. We believe that our study will contribute to the determination of characteristics of this rare disease with real-life data.

Experience with Omalizumab for the treatment of chronic spontaneous urticaria in a tertiary center: real life experience.

PURPOSE: Chronic spontaneous urticaria (CSU) is defined as urticaria and/or angioedema that appears spontaneously due to known or unknown causes and lasts for at least 6 weeks. Omalizumab, an anti-IgE antibody that binds circulating free IgE, has recently emerged as a promising treatment for CSU, a condition which impairs patients' quality of life. We aimed to contribute real life data by reporting our experience with omalizumab in the treatment of intractable CSU. METHODS: Of 140 patients treated with omalizumab in our clinic between September 2013 and January 2018, 86 CSU patients with available current data were retrospectively evaluated in terms of sex, age, urticaria duration, urticaria activity score over 7 days (UAS7) before and after omalizumab, relapses and time to relapse, length of remission after omalizumab cessation, adverse events, and comorbidities. RESULTS: The mean age of the patients was 45.5 ± 14.3 years and 73.3% were women. Mean duration of urticaria before initiation of omalizumab therapy was 54.5 ± 67 months. All patients had used antihistamines before starting omalizumab treatment. The mean number of omalizumab doses was 11.9 ± 9.3. The mean duration of omalizumab treatment was 13.3 ± 10.4 months. Mean UAS7 score was 38.9 ± 4.1 before the start of omalizumab treatment, and 7.9 ± 10.5 after treatment. Treatment was discontinued in 10 patients (11.6%) due to nonresponse or loss of effect. Four patients (4.65%) experienced adverse events. Treatment was discontinued in 1 patient (1.16%) due to side effects. Of the 55 patients whose treatment was discontinued after their symptoms resolved, 31 (56.3%) relapsed after omalizumab cessation. Twenty-four patients (43.6%) did not relapse after omalizumab cessation. CONCLUSIONS: Our results show that omalizumab was an effective treatment for intractable CSU and did not cause any serious adverse effects other than asthenia, vertigo, and injection site reaction in four patients. These findings are relevant because they reflect real-life data.

Extranasal extranodal NK/T cell lymphoma: A post-transplantation lymphoproliferative disease.

Extranodal NK/T cell lymphoma (ENKTL) is a rare form of non-Hodgkin lymphoma. It mostly occurs in the upper respiratory tract. Cutaneous involvement can be seen among the extranasal ENKTLs. After solid organ and haematopoietic stem cell transplantation, post-transplantation lymphoproliferative disease because of immunosuppressive therapy is usually B cell-derived; T and NK/T cell-derived disease is rarely seen. A 43-year-old female patient who had renal transplantation 14 years ago presented with cutaneous ulceration and subcutaneous nodules located in the abdomen. The patient was diagnosed with ENKTL, nasal type. Although it is rare, ENKTL nasal type is a lymphoproliferative disease that should be considered in the differential diagnosis of ulcerated cutaneous tumoural lesions.

Long-term Follow-up of Positive Surgical Margins in Basal Cell Carcinoma of the Face.

BACKGROUND: Basal cell carcinoma (BCC) in central facial locations and tumors with positive margins are at a higher risk of recurrence. The most effective treatment is total excision, which includes an adequate pathological margin. OBJECTIVE: To evaluate the outcome of the patients who underwent surgery for BCCs of the head and neck and of those who had positive surgical margins where Mohs surgery is not available. METHODS: This study was conducted at Ege University Medical School between 2004 and 2014. One hundred thirty patients with 154 BCC who underwent surgical excision were included. In the histopathologic report, the existence of positive margin, BCC subtype, localization of the tumor, and distance of margins to the tumor were evaluated. RESULTS: Twenty-three lesions (14.9%) of 22 patients revealed positive surgical margins. Six patients (26.1%) had recurrences on the surgical site. The BCC subtypes of recurrent patients were reported to be multifocal superficial in 2 (33.3%), infiltrative (16.7%) in 1, and micronodular (50%) in 3. CONCLUSION: Patients with superficial multifocal or micronodular tumors should undergo reoperation because of high recurrence rates.

Cutaneous side effects of chemotherapy in pediatric oncology patients.

Pediatric oncology patients can present with various skin lesions related to both primary disease and immunosuppressive treatments. This study aimed to evaluate the cutaneous side effects of chemotherapy in pediatric oncology patients. Sixty-five pediatric oncology patients who were scheduled to undergo chemotherapy from May 2011 to May 2013 were included in the study. Three patients were excluded from the results, as 2 patients died during treatment and 1 patient withdrew from the study; therefore, a total of 62 patients were evaluated for mucocutaneous findings. Patients were grouped according to their oncological diagnoses and a statistical analysis was performed. There was no statistical significance in the incidence of cutaneous side effects of chemotherapy among the different diagnostic groups. Awareness among dermatologists of the possible cutaneous side effects of chemotherapy in pediatric patients and their causes can promote early diagnosis and treatment in this patient population.

Discrete papular dermal mucinosis with Hashimoto thyroiditis: a case report.

The cutaneous focal mucinoses are a group of connective tissue disorders characterized by deposition of mucin found either focally or diffusely in the dermis. A 47-year-old woman presented with asymptomatic flesh-colored papules on the neck, inguinal area, intergluteal area, vulvar area, and extremities of 5 months' duration. There was no history of preceding trauma or insect bites. The patient had undergone a subtotal thyroidectomy 21 years prior but had not used any thyroid medication before she was referred to our clinic. Thyroid ultrasonography was consistent with Hashimoto thyroiditis. During dermatologic examination, flesh-colored, well-defined, smooth papules that measured approximately 1.5 x 1 cm in size on the genital region, fingers, face, and scalp were seen. Histopathologic examination of a lesional biopsy revealed no abnormalities in the epidermis. Alcian blue staining showed that abundant deposits of dermal mucin had replaced collagen in the dermis.

White fibrous papulosis of the neck.

A 56-year-old white man with multiple, discrete nonfollicular papules on the neck is presented. Clinical and histopathologic features were compatible with the entity of white fibrous papulosis of the neck (WFPN). Pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE) and WFPN are further clinicopathologic patterns of intrinsic aging. Clinically, WFPN is characterized by isolated, whitish papules, whereas those of PXE-PDE are yellowish and often coalesce to form "cobblestone" plaques. Our case showed clearly marginated whitish papules. The major histopathologic feature of WFPN is superficial dermal fibrosis with scant elastolysis; in PXE-PDE, there is papillary dermal elastolysis but no sign of fibrosis. No recurrence was performed in the 3 years' follow-up in our case. Surgical treatment may be considered in such cases with well-circumscribed lesions.

Skin diseases in Turkish renal transplant recipients.

BACKGROUND: Organ transplant recipients are predisposed to a variety of cutaneous complications due to immunosuppressive therapy. We aimed to determine the prevalence and the clinical spectrum of skin diseases in renal transplant recipients (RTRs). METHODS: One hundred and eleven RTRs were examined at the Renal Transplantation Center in Ege University Hospital between October 1999 and October 2001. The effects of age, gender and duration time after transplantation on cutaneous manifestations were evaluated and the dermatologic manifestations in RTRs were compared with findings in a control group consisting of 100 patients. The t-test, chi2 test and Fisher's exact test were used for statistical analysis. RESULTS: Seventy-five patients (66.4%) had an infection of the skin, 66 patients (58.4%) had drug-related manifestations, and 11 patients (9.7%) had premalignant or malignant skin lesions. Human papilloma virus (HPV) infections were the most common skin lesions. There was no significant relation between age and gender and the incidence of skin diseases in RTRs. The incidence of HPV infections, tinea versicolor and premalignant and malignant lesions increased with the duration of immunosuppression. The incidence of infectious skin diseases, especially HPV infections and tinea versicolor, was higher in the study group than in the control group. CONCLUSIONS: In this study, we observed that cutaneous lesions, especially those caused by infectious diseases, had a higher frequency in RTRs. The findings emphasize the importance of regular dermatological screening in these patients, which can provide early diagnosis and a better quality of life for RTRs.

ITP as an initial manifestation of subacute cutaneous lupus erythematosus.

Subacute cutaneous lupus erythematosus (SCLE) is an entity characterized by widespread polycyclic lesions that heal without scarring. Skin lesions with marked ultraviolet sensitivity are distributed in an annular and/or psoriasiform configuration. Idiopathic thrombocytopenic purpura, which is an autoimmune disease (ITP), is mediated by a destructive immunoglobulin G antibody response to the platelets' membrane components. We report a case of subacute cutaneous lupus erythematosus initially manifested as thrombocytopenia, which was diagnosed as idiopathic thrombocytopenic purpura (ITP) and treated with splenectomy. Seven months later, development of cutaneous involvement followed the diagnosis of ITP. The clinical and histological features of the lesions were compatible with SCLE. Serological evaluations showed a negative anti-nuclear antibody test and an elevated anti-SSB/La antibody level. Symptoms for systemic involvement were negative. Although the clinical features such as photosensitivity, discoid rash, and thrombocytopenia were in favor of SLE, the patient did not fulfill the criteria of the American Rheumatism Association (ARA) for SLE.

Isotretinoin (13-cis-retinoic acid) associated atrial tachycardia.

We describe a 16-year-old boy who presented with palpitations for 1 week while being on isotretinoin treatment for nodulocystic facial acne for 3 months. Twenty four-hour Holter monitoring showed frequent premature atrial beats and episodes of nonsustained atrial tachycardia. He never had any episodes of palpitations previously. His complaints almost disappeared within a week after stopping the treatment. He remained asymptomatic since the discontinuation of the drug. The temporal relationship between isotretinoin treatment and patient's symptoms in the presence of documented arrhythmia suggests a drug-related cause. As a result, clinicians should be aware of the possible arrhythmogenic effect of isotretinoin.

Delayed diagnosis in a case of lupus vulgaris with unusual localization.

Lupus vulgaris is the most common form of cutaneous tuberculosis, and the usual sites of involvement are the head and neck. We present a forty-six-year-old woman with lupus vulgaris on the external surface of the left leg and foot, an unusual site. Based on histopathological and clinical features, this case was diagnosed as lupus vulgaris with unusual localization.

Non-melanoma skin cancers between the years of 1990 and 1999 in Izmir, Turkey: demographic and clinicopathological characteristics.

The incidence of nonmelanoma skin cancers (NMSCs) has been rapidly increasing in epidemic proportions all over the world. The purpose was to evaluate the demographic and clinico-pathological patterns of skin cancers in Izmir, a region with high ultraviolet light exposure. The study was retrospective and hospital-based. The data related to all NMSC patients registered from January 1, 1990, to December 31, 1999, in the Dermatology Department of Ege University Medical School in Izmir, were collected and analyzed according to the personal statistics, anatomic location, dimensions of the lesion and clinical and histopathological type of skin cancer. A total number of 2,879 patients, aged between 20 and 96 years, were included in the study; among them 1,633 were men and 1,246 were women. The mean age of the patient population was 62.81 years, with a standard deviation of 12.50 years. Among the tumour patients, there were 2,062 basal cell carcinomas (BCCs), 746 squamous cell carcinomas (SCCs) and 71 both BCC and SCC; the total number of lesions was 3,186 (2,339 BCCs, 847 SCCs). Of all the NMSC lesions studied, the highest percentage (46.6%) were located on the face. For the BCCs, the most frequent histopathological subtype was the solid pattern (50.74%). The mean male: female ratios for SCC and BCC lesions were 2.86 and 1.01, respectively. Nodular and ulcerative forms (69.8%) were the most commonly found clinical types. Of all NMSCs, 78.4% had diameters in the 11 to 20 mm. BCC is the most common type of skin cancer diagnosed in our region. Among the NMSC patients, farming was the most widespread and risky job. The majority of SCC tumors were observed mostly in men 60 years old or above. This is the first comprehensive description of NMSCs in our region.