A Case with Hypotrichosis-Lymphedema-Telangiectasia Syndrome with Hair Shaft Fragility.

Introduction: Hypotrichosis-lymphedema-telangiectasia syndrome (HLTS) is a disease characterized by the failure of angiogenesis, vascularization, and hair formation caused by a mutation in the SOX18 gene. Case Presentation: We report a 15-year-old female patient presented with sparse hairs on her scalp and eyebrows and the absence of eyelashes and body hair since birth. We detected premature weathering due to abnormality of the hair shaft. Discussion/Conclusion: Detection of trichophytosis and split hair in light microscopy in a patient with sparse hair, telangiectasia, and lymphedema may help diagnose HLTS.

Isolated hair loss on the eyebrow: five cases with trichoscopic features.

Alopecia areta (AA) and trichotillomania (TTM) are common causes for hair loss on the eyebrows. Yellow dots, vellus hairs, anisotrichosis, empty follicular openings, and black dots were observed in the present study's patients with AA. Split hairs, question mark hairs, broken hairs, flame hairs, black dots, hairs with different lengths, and hemorrhagic areas were found in the patients with TTM. Trichoscopy is a very useful and helpful technic in distinguishing AA and TTM on the eyebrows.

Evaluation of the Demographic and Clinical Features of Patients With Digital Myxoid Pseudocysts and Their Response to Treatment.

BACKGROUND: Digital myxoid pseudocysts (DMPs) are the most common benign degenerative lesions of the nail unit. There is currently no consensus regarding first-line treatments for DMPs. OBJECTIVE: To evaluate demographic and clinical features and treatment methods of DMP and examine recurrence rates and factors that may affect recurrence. METHODS AND MATERIALS: All patients in the authors' clinic who were diagnosed with DMP between 2013 and 2020 were included. Treatment methods were categorized as surgical excision (SE), drainage and compression (DC), simple drainage (SD), and no treatment. Responses and recurrence rates after different treatment methods were investigated. RESULTS: Fifty-one lesions from 48 patients were included. Thirty-two lesions were treated with SE, 11 with DC, and 1 with SD, while 7 lesions were left untreated. The initial complete response rate after SE was significantly higher than that after DC. No statistically significant difference was found between the recurrence rates of the 2 treatment methods. Using intra-articular methylene blue during SE did not seem to affect recurrence rates. CONCLUSION: Drainage and compression seems to be a safe and effective first-line treatment option for DMPs located on the proximal nail fold and distal interphalangeal joint. SE is more favorable as a second-line treatment.

The role of dermoscopy in the diagnosis of subungual glomus tumors.

BACKGROUND AND OBJECTIVES: The dermoscopic features of glomus tumors have only been described in a few case reports. The aim of this research was to define the clinical and dermoscopic features of subungual glomus tumors. METHODS: Thirty-two patients with subungual glomus tumors were evaluated retrospectively. RESULTS: On the photographs, longitudinal erythronychia, longitudinal leukonychia, punctate leukonychia, splinter hemorrhage, isolated capillaries, distal notching, distal subungual hyperkeratosis, onycholysis, and onychoschizia were found. There was no statistical difference between the rates of detection of these findings by evaluation from clinical photographs alone and from both clinical and dermoscopic photographs. While ramified vessels with bluish spots could be detected in only five of 26 cases with bluish spots in their clinical photographs, these ramified vessels were seen in 14 cases in bluish spots in dermoscopic photographs (P = 0.004). Compared to clinical examination, dermoscopy was able to detect blue spots in three more cases. Lesion duration was higher in the cases with ramified vessels (P = 0.018). CONCLUSIONS: Dermoscopy seems to contribute to the clinical examination in displaying only ramified vessels located in bluish spots and in determining the localization of the subungual tumors. The presence of ramified vessel in the bluish spots is strongly related to lesion duration.

Is There a Pathogenetic Relationship Between Alopecia Areata and Familial Mediterranean Fever?

Alopecia areata (AA) is type of autoimmune, T-cell-mediated disease with abnormal expression of MHC Class I, a common reason for non-scarring hair loss. Familial Mediterranean fever (FMF) is a hereditary autoinflammatory disease characterized by periodic fever and serositis. Various diseases and conditions that may be related to FMF have been reported. It has been known that patients with FMF are vulnerable to MHC Class I-related diseases. The co-existence of the two MHC Class I group-associated entities, FMF and AA, has not been reported in the literature. Here, we present three cases with AA and FMF and discuss the possible common pathway in their pathogenesis.

Integrated Cadaveric Nail Surgery Course Improves Self-Assessed Knowledge and Competency of Dermatologists.

INTRODUCTION: Dermatologists see many patients with nail disorders requiring surgical interventions. However, nail surgery is often found to be difficult by dermatologists. The aim of this study was to evaluate the effectiveness of an integrated nail surgery course. METHODS: A nail surgery course model, combining anatomy, pathology, and dermatology with theoretical lectures and hands-on training on fresh-frozen cadaver, was designed for dermatologists. Before and after the course, the participants were asked to complete a questionnaire in which they assessed their knowledge and competence levels. RESULTS: Twenty-eight dermatologists completed the questionnaires. The majority of the dermatologists (79.6%) had limited nail surgery experience, previously. The most commonly cited reasons for lack of knowledge or low or moderate theoretical and/or practical level of knowledge of nail surgery were not having received theoretical and practical education during dermatology residency. The median scores of self-assessed knowledge and competency improved significantly after the course (p < 0.001). The majority (82.1%) of the participants were "quite" or "very" well satisfied. CONCLUSION: An integrative nail surgery course model seems beneficial and encouraging for dermatologists. Organizing this type of nail surgery course, especially in medical schools using fresh-frozen cadavers, may significantly improve dermatologists' knowledge and skills.

Subungual exostosis and subungual osteochondromas: a description of 25 cases.

BACKGROUND: Subungual exostosis (SE) and subungual osteochondroma (SO) are an uncommon, benign tumor of the distal phalanx. The purpose of this retrospective study was to evaluate clinical, demographical, and radiological features; treatment modalities; and follow-up results in SE and SO cases. METHODS: Twenty-five cases were confirmed histopathologically as SE or SO. At the time of admission, clinical data were obtained on the age and sex of the patient, duration of symptoms, presence of pain, previous diagnoses and treatments, concomitant systemic diseases, family history, lesional localization, clinical and radiological features of the lesion, surgical treatment methods, and duration of follow-up. RESULTS: There were 14 patients in the SE group and 11 patients in the SO group. There was no statistically significant difference in gender, age, duration of symptoms, or pain between the two groups. Regarding the locations of the lesions, two (14.28%) were on the hands, 12 (85.72%) lesions were on the toes, 11 (91.67%) of which were on the great toe, and one (8.33%) was on the small toe of the left foot in the SE group. All the osteochondroma lesions were located on the toes. In five (45%) cases, the lesion was on the great toe. Two patients had residual lesions in the SE group. Recurrence occurred in one case in the SO group. CONCLUSIONS: Clinically and histopathologically, SE and SO appear to be two different entities. When diagnosed correctly and treated appropriately, the lesions have good functional and cosmetic results, as well as a very low recurrence rate.

A Proposal for a New Severity Index for the Evaluation of Chronic Paronychia.

INTRODUCTION: Chronic paronychia (CP) is an inflammatory disease of the nail folds. Staging of CP is important for clinicians. We developed an objective scale that evaluates each finding of CP separately in addition to evaluating the treatment process and follow-up. METHODS: A new "chronic paronychia severity index scale" was developed to enable dermatologists to examine all features of CP. A previous categorical severity scale and this new scale were used for the evaluation of CP by 6 different dermatologists. The dermatologists evaluated the nails with both scales again 20 days later using randomly ordered photographs. RESULTS: Using the previous scale, the intra-observer intraclass correlation coefficient (ICC) values between the first and second evaluations were 0.767, 0.860, 0.734, 0.609, 0.900, and 0.840 for the 6 dermatologists. Using the new proposed scale, the intra-observer ICC values between the first and second evaluations were 0.930, 0.931, 0.942, 0.934, 0.938, and 0.920 for the 6 dermatologists. All intra-observer ICC values were higher for the results of the proposed scale than for those of the previous scale. The inter-observer ICC values were also higher for the proposed scale than for the previous scale for the 6 dermatologists. CONCLUSION: The new scale is a standardized, more suitable, objective, and valuable method to use in clinical practice and studies on CP.

Pregabalin treatment of three cases with brachioradial pruritus.

Brachioradial pruritus (BRP) is a rare type of chronic pruritus that usually localized at the dorsolateral part of the forearms. Itching, burning, or pain are common symptoms at the involved areas. The etiological factors are still unknown but sun exposure and/or cervical spine lesions seem to be trigerring or precipiting factors. Neuropathogenic mechanism plays role in etiopathogenesis of BRP, therefore, antiepileptic drugs such as gabapentin, oxcarbazepine. and pregabalin are suggested medications for BRP. Herein, we report three cases with BRP successfully treated with pregabaline.

The Epidemiology of Non-Melanocytic Benign and Malignant Skin Tumors in Pediatric Patients Attending to the Dermatology Department.

BACKGROUND: Non-melanocytic skin tumors are rarely seen in pediatric patients; although they are mostly benign, they remain to be elucidated by histopathological examination. The objective of the study was to describe the epidemiology of non-melanocytic skin tumors in children attending to our dermatology department. METHOD: The histopathologic studies of all skin punch and excisional biopsies of children up to 16 years old referred to our dermatology department between January 2007 and January 2012 were reviewed retrospectively. Melanocytic tumors and cystic and infectious lesions were excluded. Age, sex, location, and histopathologic diagnosis were recorded. The skin tumors were categorized. RESULTS: A total of 4,126 skin tumors were analyzed histopathologically, and 203 of the lesions were from children up to 16 years of age. Ninety-seven of the lesions from 91 patients were non-melanocytic skin tumors. Forty-seven (51.64%) were male, 44 (48.36%) were female, and mean age was 10.55 ± 4.31 years. Malignant tumor was 1.03% (one tumor) and benign tumors were 98.97% (96 tumors) of all. The most frequent non-melanocytic skin tumor was pilomatricoma with 22 lesions (22.68%), followed by pyogenic granuloma with 18 lesions (18.54%), and nevus sebaceous with 10 (10.3%) lesions. Cutaneous leukemic infiltrate was found to be the only malignant skin tumor in the study group. The most frequently affected age group was children aged > 13 to ≤ 16 years, which included 38 patients (41.7%). The majority of lesions were on head and scalp (32 tumors, 32.96%), followed by trunk (28 tumors, 28.84%) and upper limbs (22 tumors, 22.75%). CONCLUSION: The ratio of malignant to benign skin tumors in pediatric patients is found to be small. Pilomatricoma, pyogenic granuloma and nevus sebaceous are found to be the most frequent non-melanocytic skin tumors of children. The ratio of malignant tumors is very rare.

Hepatitis C and hepatitis B virus infections in the etiopathogenesis of pemphigus.

BACKGROUND: Previous studies have shown that some viral infections may be triggers for autoimmune diseases. The role of viral infections in the etiopathogenesis of pemphigus has also been investigated. OBJECTIVES: To investigate the relationship between pemphigus and the hepatitis B and C virus infections. METHODS: This retrospective study included 62 patients with pemphigus and 50 healthy controls of matching ages and genders. The control group included relatives who accompanied the orthopedic patients to the hospital but had no history of systemic and/or autoimmune diseases. RESULTS: The group of patients with pemphigus was composed of 43 (69.3%) females and 19 (29.7%) males, and the mean age was 48.08 ± 15.38. The hepatitis C virus antibody was negative in all of the patients. Hepatitis B surface antigen (HBsAg), hepatitis B surface antibody (HBsAb), or both, were studied in 44 of the 62 patients. Two (4.3%) of these patients were positive for HBsAg and found to have HBV infection. Fourteen of them were HBsAb positive. Two (4%) of the 50 control subjects showed hepatitis C virus antibody positivity, while only 1 (2%) patient with pemphigus displayed HBsAg positivity. There was no statistically significant difference between the two groups for hepatitis B and C virus infections. CONCLUSIONS: This study does not support an association between pemphigus and hepatitis B and/or C virus infections.

Hepatitis C and hepatitis B virus infections in the etiopathogenesis of pemphigus

BACKGROUND: Previous studies have shown that some viral infections may be triggers for autoimmune diseases. The role of viral infections in the etiopathogenesis of pemphigus has also been investigated. OBJECTIVES: To investigate the relationship between pemphigus and the hepatitis B and C virus infections. METHODS: This retrospective study included 62 patients with pemphigus and 50 healthy controls of matching ages and genders. The control group included relatives who accompanied the orthopedic patients to the hospital but had no history of systemic and/or autoimmune diseases. RESULTS: The group of patients with pemphigus was composed of 43 (69.3%) females and 19 (29.7%) males, and the mean age was 48.08 ± 15.38. The hepatitis C virus antibody was negative in all of the patients. Hepatitis B surface antigen (HBsAg), hepatitis B surface antibody (HBsAb), or both, were studied in 44 of the 62 patients. Two (4.3%) of these patients were positive for HBsAg and found to have HBV infection. Fourteen of them were HBsAb positive. Two (4%) of the 50 control subjects showed hepatitis C virus antibody positivity, while only 1 (2%) patient with pemphigus displayed HBsAg positivity. There was no statistically significant difference between the two groups for hepatitis B and C virus infections. CONCLUSIONS: This study does not support an association between pemphigus and hepatitis B and/or C virus infections. .

Impact of functional pruritus compared with mild psoriasis on quality of life: a cross-sectional questionnaire study in Turkey.

BACKGROUND: Functional pruritus (FP) is a type of chronic pruritus that is a somatoform disease in nature, triggered by psychogenic factors. Psoriasis is a chronic, inflammatory, and pruritic skin disease that causes significant cosmetic problems and psychological distress. OBJECTIVE: In this study, we aimed to investigate the effects of functional pruritus on quality of life and to compare these with the effects of mild psoriasis. METHODS: A total of 73 patients (40 with psoriasis and 33 with FP) were enrolled into the study. All the participants were asked to complete the Dermatology Life Quality Index (DLQI) and World Health Organization (WHO) Quality of Life, short form, Turkish version (WHOQOL-BREF-TR). Pruritus severity was evaluated subjectively with a visual analog scale (VAS) in both groups, and psoriasis severity was evaluated with the Psoriasis Area and Severity Index. Patients' age, gender, educational status, duration of disease, and medications were recorded. RESULTS: There was no significant difference in quality of life between the two groups. VAS values were significantly higher in the FP group. A negative correlation was found between age and the physical domain on the WHOQOL-BREF-TR, and also between the psychological domain on the WHOQOL-BREF-TR and the duration of the disease in FP patients. In psoriasis patients, a negative correlation was found between age and DLQI scores. CONCLUSION: FP has a negative impact on quality of life that is comparable to that of mild psoriasis. It seems that the visibility of psoriatic lesions and the cosmetic concerns in psoriasis patients do not result in a more severely impaired quality of life than in patients with FP.

Allergic contact dermatitis mimicking angioedema due to paraphenylendiamine hypersensitivity: a case report.

Active sensitization to paraphenylendiamine (PPD) and related compounds from temporary black henna tattoos has become an epidemic in the recent years. Hair dyes also include PPD like black henna tatoos which cause allergic contact dermatitis. Skin lesions of allergic contact dermatitis from PPD are mostly seen as an exudative erythema, an erythema multiforme-like eruption or a bullous contact dermatitis. We, herein, report a 27 year-old woman with an angioedema-like reaction occurring after the first exposure to hair dye who was unaware of being previously sensitized to PPD from black henna tattoo.

Waardenburg Syndrome type 1: A case report.

Waardenburg Syndrome (WS) is a rare hereditary disorder that is characterized by the clinical manifestations of oculocutaneous anomalies of pigmentation, congenital deafness, dystopia canthorum, and broad nasal root. It demonstrates both genetically and clinically heterogenous characteristics. In this article, we report an 11-month-old boy with WS1, one of four clinicat types of WS. He exhibited white forelock, hypopigmented macules and patches, heterochromia irides, and dystopia canthorum.

A giant benign clear cell hidradenoma on the anterior trunk.

Clear cell hidradenoma (CCH) is an uncommon variant of benign cutaneous adnexal tumors. These tumors are clinically asymptomatic, solitary dermal nodules. They occur most frequently on the scalp, face abdomen and extremities. Growth is slow and malignant change is rare. 45-year-old woman presented with a nodule which had begun 4 years ago as a small nodular asymptomatic lesion and had a central ulceration and a minimal hemorrhagic discharge on her anterior abdomen wall. On dermatologic examination there was a 6.5×5×4 cm non-tender, soft reddish purple nodule, with lobular appearance and ulceration. In the laboratory investigations, all hematologic and biochemical tests were normal. A computed tomography (CT) scan demonstrated a cystic tumor with lobulated contour with contrast enhancement. The lesion was excised totally. In histopathological examination, the tumor was composed of biphasic smaller dark polygonal cells and larger clear cells and coarse nuclear chromatine. There were duct like structures. Immunohistochemical investigation was done for the suspicion of malignancy. Cytoplasm of clear cells and of duct like structures showed PAS-positive and d-PAS resistant staining. There was a positive reaction to epithelial membrane antigen and carcinoembryonic antigen. The mitotic index in Ki 67 examination was low. All these findings confirmed the diagnosis of benign CCH.