Clinical and demographic features of hidradenitis suppurativa: a multicentre study of 1221 patients with an analysis of risk factors associated with disease severity.

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic, relapsing and debilitating inflammatory disease associated with profound morbidity. AIM: In this multicentre study, we investigated the demographic and clinical features of HS, and determined risk factors of disease severity. METHODS: In total, 1221 patients diagnosed with HS from 29 centres were enrolled, and the medical records of each patient were reviewed. RESULTS: The mean age of disease onset was 26.2 ± 10.4 years, and almost 70% (n = 849) of patients were current or former smokers. Mean disease duration was 8.9 ± 8.4 years with a delay in diagnosis of 5.8 ± 3.91 years. Just over a fifth (21%; n = 256) of patients had a family history of HS. The axillary, genital and neck regions were more frequently affected in men than in women, and the inframammary region was more frequently affected in women than in men (P < 0.05 for all). Acne (40.8%), pilonidal sinus (23.6%) and diabetes mellitus (12.6%) were the most prevalent associated diseases. Of the various therapies used, antibiotics (76.4%) were most common followed by retinoids (41.7%), surgical interventions (32.0%) and biologic agents (15.4%). Logistic regression analysis revealed that the most important determinants of disease severity were male sex (OR = 2.21) and involvement of the genitals (OR = 3.39) and inguinal region (OR = 2.25). More severe disease was associated with comorbidity, longer disease duration, longer diagnosis delay and a higher number of smoking pack-years. CONCLUSIONS: Our nationwide cohort study found demographic and clinical variation in HS, which may help broaden the understanding of HS and factors associated with disease severity.

Morphological assessment of the stylohyoid complex variations with cone beam computed tomography in a Turkish population.

BACKGROUND: The aim of this investigation was to evaluate the length, thickness, sagittal and transverse angulations and the morphological variations of the stylohyoid complex (SHC), to assess their probable associations with age and gender, and to investigate the prevalence of it in a wide range of a Turkish sub-population by using cone beam computed tomography (CBCT). MATERIALS AND METHODS: The CBCT images of the 1000 patients were evaluated retrospectively. The length, thickness, sagittal and transverse angulations, morphological variations and ossification degrees of SHC were evaluated on multiplanar reconstructions (MPR) adnd three-dimensional (3D) volume rendering (3DVR) images. The data were analysed statistically by using nonparametric tests, Pearson's correlation coefficient, Student's t test, c2 test and one-way ANOVA. Statistical significance was considered at p < 0.05. RESULTS: It was determined that 684 (34.2%) of all 2000 SHCs were elongated (> 35 mm). The mean sagittal angle value was measured to be 72.24° and the mean transverse angle value was 70.81°. Scalariform shape, elongated type and nodular calcification pattern have the highest mean age values between the morphological groups, respectively. Calcified outline was the most prevalent calcification pattern in males. There was no correlation between length and the calcification pattern groups while scalariform shape and pseudoarticular type were the longest variations. CONCLUSIONS: We observed that as the anterior sagittal angle gets wider, SHC tends to get longer. The most observed morphological variations were linear shape, elongated type and calcified outline pattern. Detailed studies on the classification will contribute to the literature. (Folia Morphol 2018; 77, 1: 79-89).

Primary double teeth and their effect on permanent successors.

AIM: Understanding the effects of primary double tooth (PDT) on permanent successors is important to ensure healthy permanent occlusion and aesthetics. The aim of this study is to determine the prevalence and type of PDT, their effect on permanent successors, and the accompanying dental anomalies/pathologies in a Turkish population. MATERIALS AND METHODS: Study design: The records of 63 PDTs in 54 healthy Caucasian children among 10,000 patients were investigated. PDTs were classified according to Aguilo's classification. RESULTS: The prevalence of PDT was 0.6%. Of the 63 PDTs, 14.3% were type I, 11.1% were type II, 31.7% were type III, and 41.3% were type IV; one (1.6%) was a triple tooth. Aplasia of the permanent lateral incisor was observed most frequently in association with type I (56%) PDT. All PDTs associated with a supernumerary permanent tooth were type IV. Dental anomalies/pathologies such as odontoma, talon cusp were observed. Caries involvement was observed most frequently in type IV (58.3%) PDT. STATISTICS: The chi-squared test was used to determine whether successor aplasia depended on PDT type, and contingency coefficients (%) were calculated to determine the degree of association between aplasia and PDT type. CONCLUSION: Clinicians should assess PDT clinically and radiographically to determine whether they are associated with aplasia of permanent lateral incisors (type I) or supernumerary permanent teeth (type IV). Type IV of PDT should be sealed with sealant or resin.

Toll-like receptors and skin.

Toll-like receptors are important pattern recognition receptors which have key roles in both innate and adaptive immune responses. They are strongly associated with the pathogenesis of inflammatory and autoimmune diseases. Furthermore, Toll-like receptors have also been implicated in the pathogenesis of several skin diseases such as skin infections, psoriasis, acne vulgaris, lichen planus, Behçet's disease, leprosy, syphilis, Lyme disease, atopic dermatitis and allergic contact dermatitis, mycosis fungoides, non-melanoma skin cancers and melanoma. In this manuscript, the structure and functions of Toll-like receptors in immune responses, their impact on skin diseases and recent advances on therapeutic usage have been reviewed.

Changes in fundus autofluorescence of choroidal melanomas following treatment.

PURPOSE: We have previously shown that fundus autofluorescence (FAF) associated with pigmented choroidal lesions can be attributed to mainly lipofuscin (orange pigment) but also to hyperpigmentation, drusen, or fibrous metaplasia. The purpose of this study is to describe the effects of treatment on FAF in choroidal melanomas after plaque radiotherapy alone or in combination with transpupillary thermotherapy (TTT). METHODS: Retrospective chart review of eight consecutive patients with choroidal melanoma treated with plaque radiotherapy alone or in combination with TTT who underwent FAF photography before and after treatment. The correlation between FAF patterns and foci of orange pigment, hyperpigmentation, drusen, or fibrous metaplasia was evaluated. RESULTS: The median follow-up time was 4 (range 2-9) months. Foci of orange pigment and hyperpigmentation became larger and more numerous after treatment. Fibrous metaplasia was also increased. A complete correlation between increased FAF and orange pigment was found in all eight tumours (100%) before and after treatment. No correlation between hyperpigmentation and increased FAF was found before treatment but a partial correlation was found in all eyes after treatment. Before treatment, correlation between fibrous metaplasia was present in three eyes and increased FAF was partial in two eyes with no correlation in one case. After treatment, this correlation was partial in all presenting eyes (7). CONCLUSIONS: Following treatment, choroidal melanomas may show increased FAF, mainly due to an increase in the amount of lipofuscin (orange pigment) and hyperpigmentation.

Review of fundus autofluorescence in choroidal melanocytic lesions.

Fundus autofluorescence (FAF) imaging takes advantage of the fluorescent properties of some molecules, especially lipofuscin. FAF derives mainly from retinal pigment epithelium (RPE) and Bruch's membrane. Using confocal scanning laser ophthalmoscope (cSLO) we have previously shown that FAF associated with pigmented choroidal lesions can be attributed to mainly lipofuscin (orange pigment) within the RPE. Other causes of FAF include hyperpigmentation, drusen, or fibrous metaplasia probably because they also cause lipofuscin accumulation in the overlying RPE. There is a total or partial correlation between FAF and the foci of lipofuscin and hyperpigmentation in about 90% of the cases. The FAF patterns of choroidal melanocytic lesions were classified as patchy or diffuse. The patchy pattern was defined as the presence of distinct areas of increased FAF between areas of normal autofluorescence. The diffuse pattern was characterized by the presence of increased FAF with indistinct borders over a larger part (>50%) of the tumour in the absence of such intervening areas. Choroidal melanomas presented with either a diffuse or patchy pattern, whereas choroidal naevi demonstrated only the patchy pattern. Diffuse FAF pattern was more often associated with larger choroidal melanomas as well as with early venous and late hyperfluorescence on fluorescein angiography. Limitations of these observations depend on the field of depth of cSLO; thus, FAF from other planes could not be detected. Increased retinal thickness, intraretinal oedema, or presence of subretinal fluid may also affect the FAF signal.

Well-circumscribed orbital venous-lymphatic malformations with atypical features in children.

AIM: To report well-circumscribed orbital lymphatic-venous malformations (VLMs) with atypical clinical, imaging and pathological features in four paediatric patients. METHODS: Retrospective non-comparative case series of four patients aged 5-18 years old having a well-circumscribed orbital mass diagnosed histopathologically as orbital VLM. All patients underwent orbitotomy and total excision of the VLM. Pre- and postoperative visual acuity, proptosis and globe displacement produced by the orbital VLM, MRI findings, histopathological features, treatment, follow-up and prognosis were evaluated. RESULTS: No proptosis, visual acuity change or globe displacement was induced by the orbital VLM. One lesion was located superiorly, one medially and two inferonasally. On MRI, the orbital VLMs were isointense on T1-weighted images and hyperintense on T2-weighted images, demonstrated moderate contrast enhancement and had a heterogenous internal structure. Signal void areas and fluid-fluid levels were not observed on MRI. At a mean follow-up of 50 months, all patients remained free of recurrence clinically and retained preoperative visual acuities. Several histopathological features of the excised lesions supported an initial diagnosis of cavernous haemangioma, but the lesions were subsequently rediagnosed as orbital VLM when aggregates of lymphocytes and randomly arranged smooth muscle were noted. CONCLUSIONS: Well-circumscribed orbital VLMs in children can display atypical clinical, imaging and pathological features. MRI features of this entity are not characteristic of typical orbital VLMs. It may be possible to totally excise well-circumscribed orbital VLMs as in this series of four patients. Careful histopathological evaluation indicates the correct diagnosis.

Intravitreal bevacizumab for macular oedema secondary to branch retinal vein occlusion.

PURPOSE: To report short-term results of intravitreal bevacizumab (IVB) injection for macular oedema (ME) associated with branch retinal vein occlusion (BRVO). METHODS: Retrospective interventional case series of 12 eyes of 12 consecutive patients. IVB injections (1.25 mg/0.05 ml) were given as needed based on the presence of ME on optical coherence tomography. Changes in Snellen visual acuity (VA) and central macular thickness (CMT) were analysed using the Student's paired t-test. RESULTS: At a mean follow-up of 9.8 months (range: 6-17 months), the mean number of IVB injections given was 4 (range: 1-6). No ocular or systemic side effects were noted. The differences in logMAR VA between baseline and 1 month, between baseline and 6 months, and between baseline and final follow-up were statistically significant (P<0.05). The differences in CMT between baseline and 1 month, baseline and 6 months, and between baseline and final follow-up were also statistically significant (P<0.05). CONCLUSION: Short-term results indicate that IVB is useful in decreasing ME and improving VA in eyes with BRVO.

Nonpreserved human amniotic membrane transplantation for conjunctival reconstruction after excision of extensive ocular surface neoplasia.

PURPOSE: To report our experience on the use of nonpreserved human amniotic membrane transplantation (AMT) in ocular surface reconstruction after excision of extensive ocular surface neoplasia (OSN). DESIGN: Prospective noncomparative interventional case series. PARTICIPANTS: In all, 10 eyes of 10 consecutive patients with extensive OSN involving various areas of limbus, conjunctiva, and cornea (conjunctival carcinoma in situ, four eyes; squamous cell carcinoma, three eyes; malignant melanoma, two eyes; conjunctival-orbital lymphangioma, one eye) were included in this prospective noncomparative interventional case series. After excision of the neoplasia with 3-4 mm tumour-free margins, double freeze-thaw cryotherapy was applied to the margins of the remaining conjunctiva, and nonpreserved human amniotic membrane graft was sutured to the adjacent conjunctiva using 8/0 vicryl sutures and cornea using 10/0 nylon sutures, with the epithelial side facing up to cover the bare sclera and cornea. Postoperatively, topical corticosteroids were used for 3 months. RESULTS: After tumour excision and AMT, a satisfactory result with a wet, stable conjunctiva, and rapid and complete healing was observed in all eyes. Over a mean follow-up of 10.0 months (range, 6-27 months), all but one eye remained free of tumour recurrence. In one eye with conjunctival melanoma, there was a small recurrence, which was treated with excision and cryotherapy. Treatment complications were partial stem cell deficiency in two eyes and symblepharon formation in one eye. Immune graft rejection was not encountered. CONCLUSION: Nonpreserved human AMT appears to be useful for reconstruction of ocular surface following excision of extensive OSN.

Targetoid haemosiderotic haemangioma: dermoscopic monitoring of three cases and review of the literature.

Targetoid haemosiderotic haemangioma represents a new, rarely reported, distinctive, benign vascular tumour, characterized histopathologically by a biphasic growth pattern of dilated vascular structures in the superficial dermis lined by prominent hobnail endothelial cells and collagen dissecting, rather narrow neoplastic vessels in deeper parts of the lesion. In the initial stage, the lesion is seen as a small purple or violaceous papule, 2--3 mm in diameter. Over time, the ecchymotic ring expands peripherally until it disappears spontaneously. In the later stages, however, the central papule remains as a slightly raised dermal lesion with a purple to brownish discolouration. We report three cases whose repetitive cyclic morphological changes of targetoid haemosiderotic haemangiomas were monitored dermoscopically at 3-month follow-ups. Histopathological examination of each lesion identified the features of targetoid haemosiderotic haemangioma. To the best of our knowledge, our three cases are the first reported in the literature of targetoid haemosiderotic haemangiomas that were regularly monitored by dermoscopic examinations, enabling development of the different stages of the same lesion to be followed.

Malignant eccrine poroma in an unusual location.

Malignant eccrine poroma is a rare skin appendage tumour, originating from the intraepidermal and upper dermal eccrine ducts. The tumour either arises spontaneously or develops in a long-standing eccrine poroma, generally in elderly people over 60. Clinically, it tends to be a localized lesion, which manifests itself as a nodule or ulcerated tumour, favouring extremities. We report an 83-year-old female with an enlarging and bleeding tumour on her lumbosacral region. This lesion first appeared as a small pigmented papule and progressed to an erythematous patch with central papular portion and some peripheral pigmentation. Histopathology revealed malignant eccrine poroma. Also, immunohistochemically, diffuse and intense p53 staining was observed. Regular pigment pattern, brown globules and black dots were seen in dermoscopic examination. After performing a wide excision, 20 months of follow-up revealed no recurrence or metastasis of the tumour. This case represents an unusually located malignant eccrine poroma with some pigmentation.

Relationship between ossification of the stylohyoid ligament and enthesopathy: a comparative study.

OBJECTIVE: Stylohyoid apparatus might be an anatomic region in the cervical spine involved by enthesopathy. The aim of this study was to assess the elongation and/or ossification at the stylohyoid apparatus in the degenerative or inflammatory diseases such as ankylosing spondylitis (AS), psoriatic arthropathy (PsA) and cervical spondyloarthrosis (CS) in which cervical spine involvement can be seen. METHODS: Twenty-eight patients with AS, 25 patients with PsA, 31 patients with CS and 50 controls who did not have any complaints or symptoms related with elongated styloid process (SP) were included in the study. On the lateral cervical radiographs, the anterior and posterior aspects of each vertebral body and intervertebral disk were carefully evaluated for the presence and severity of syndesmophytes, osteophytes or ossification of the adjacent longitudinal ligaments in the patient group. The entire osseous length of the SP was measured on the lateral and lateral oblique mandibular or cervical views or the anteroposterior views radiographs in the patients and controls. RESULTS: There were statistically significant differences between the patients with AS and control group and between the patients with PsA and control group with respect to the length of SP. The dimension of syndesmophytes or ligamentous ossification of the cervical spine involving C5-6 intervertebral disk level were significantly correlated with length of SP in the patients with AS. CONCLUSION: Elongated SP might be another manifestation of enthesopaty in cervical spine of the spondyloarthropathies.

Conjunctival melanoma: risk factors for recurrence, exenteration, metastasis, and death in 150 consecutive patients.

OBJECTIVE: To identify the risk factors of conjunctival malignant melanoma that predict local tumor recurrence, orbital exenteration, distant metastasis, and tumor-related mortality. DESIGN: The clinical parameters of the patient, tumor, and treatment were analyzed in a nonrandomized fashion for their relation to 4 main outcome measures using Cox proportional hazards regression models. PARTICIPANTS: One hundred fifty consecutive patients. MAIN OUTCOME MEASURES: Local tumor recurrence, orbital exenteration, distant metastasis, and death from conjunctival melanoma. RESULTS: The Kaplan-Meier estimates of local tumor recurrence was 26% at 5 years, 51% at 10 years, and 65% at 15 years. The mean number of recurrences per patient was 1 (median, 0 recurrences). There was no recurrence in 98 patients (65%), 1 recurrence in 28 patients (19%), 2 recurrences in 11 patients (7%), 3 recurrences in 5 patients (3%), and 4 or more recurrences in 8 patients (5%). Using multivariate analysis, the factors correlated with local tumor recurrence were melanoma location (not touching the limbus) (P =.01) and pathological tumor margins (lateral margin involved) (P =.02). Multivariate analysis for features correlated with ultimate exenteration included initial visual acuity (20/40 OU or worse) (P<. 001), melanoma color red (P =.01), and melanoma location (not touching the limbus) (P =.02). Tumor metastasis was present in 16% of patients at 5 years, 26% of patients at 10 years, and 32% of patients at 15 years. Metastasis was first located in the regional lymph nodes in 17 cases, the brain in 4 cases, the liver in 3 cases, the lung in 2 cases, and was disseminated in 1 case. The risks for metastases using multivariate analysis included pathological tumor margins (lateral margin involved) (P =.002) and melanoma location (not touching limbus) (P =.04). Tumor-related death occurred in 7% patients at 5 years' follow-up and 13% at 8 years' follow-up. The risk factors for death using multivariate analysis included initial symptoms (lump) (P =.004) and pathologic findings (de novo melanoma without primary acquired melanosis) (P =.05). The technique of initial surgery was shown to be an important factor in preventing eventual tumor recurrence (P =.07), metastasis (P =.03), and death (P =.006) in the univariate analysis, but did not reach significance in the multivariate analysis. CONCLUSIONS: Conjunctival malignant melanoma is a potentially deadly tumor. In the present study, metastasis was detected in 26% of patients, and death occurred in 13% of patients at 10 years. Extralimbal melanoma and tumor involvement of the surgical margins were especially poor prognostic factors. Meticulous surgical planning using wide microsurgical excisional biopsy working with the "no touch" technique and supplemental alcohol corneal epitheliectomy and conjunctival cryotherapy is advised. Arch Ophthalmol. 2000;118:1497-1507

Superior oblique muscle palsy in a patient with orbital dermoid cyst.

We describe the clinical and radiologic findings and surgical outcome of an orbital dermoid cyst causing a superior oblique muscle palsy in a child. Superior oblique muscle palsy in childhood is most often congenital. Less common causes are trauma, vascular lesions, neoplasms, and infections.(1,2) The most common orbital lesions in children are dermoid and epidermoid cysts.(3-5) A dermoid cyst at the region of trochlea is suspected as the cause of superior oblique muscle palsy in our case. This unusual presentation of a dermoid cyst has not been reported previously.

Plaque radiotherapy for management of ciliary body and choroidal melanoma with extraocular extension.

PURPOSE: To report the results of plaque radiotherapy of ciliary body and choroidal melanoma with extraocular extension. METHODS: This retrospective study of 17 eyes of 17 patients with ciliary body and choroidal melanoma demonstrated extraocular extension that was clinically evident but less than 3 mm in thickness. In 14 eyes, the extraocular extension was located anteriorly and in three posteriorly. The outcome of plaque radiotherapy in terms of intraocular and extraocular tumor responses and the development of metastasis were analyzed. RESULTS: At a median follow-up of 63 months (range, 23 to 164 months) after plaque radiotherapy, all patients showed control of both the intraocular and extraocular components of ciliary body and choroidal melanoma. In no case did the intraocular or extraocular portion of the tumor relapse. The initial median thickness of the intraocular component was 4.9 mm, and the final median thickness was 2.4 mm. The initial median thickness of the extraocular component was 2.0 mm, and the final median thickness was 1.0 mm. The initial median basal diameter of the extraocular component was 3.5 mm, and the final median basal diameter was 2.0 mm. Of 17 patients, 11 (10 with anterior and one with posterior extraocular extension) showed partial regression in base and thickness of the extraocular extension, and in six there was little or no change. In those with anterior extrascleral extension, the sclera remained intact without clinically observable necrosis, and the scleral fibers were gradually more visible as the tumor regressed. Three patients (18%) died of metastatic melanoma at a median interval of 38 months after plaque radiotherapy. CONCLUSIONS: Plaque radiotherapy appears to be a reasonable treatment option for selected cases of ciliary body and choroidal melanoma with clinically visible extraocular extension less than 3 mm in thickness.

Plaque radiotherapy for uveal melanoma: long-term visual outcome in 1106 consecutive patients.

OBJECTIVE: To identify clinical predictive factors for visual outcome in a large series of patients who underwent plaque radiotherapy for uveal melanoma. DESIGN: Clinical factors, including patient data, tumor features, and radiation variables, were analyzed for their impact on visual acuity using Cox proportional hazards regression models. PARTICIPANTS: Patients with uveal melanoma and initial visual acuity of 20/100 or better in the affected eye who were treated with plaque radiotherapy between July 1976 and June 1992. MAIN OUTCOME MEASURES: Two end points were used to evaluate posttreatment visual acuity: (1) final visual acuity (good [20/20-20/100] vs poor [20/200 to no light perception]) and (2) loss of visual acuity (minimal [<5 lines Snellen visual acuity] vs moderate [> or = 5 lines Snellen visual acuity]). RESULTS: Of 1300 consecutive patients with uveal melanoma treated by plaque radiotherapy, 1106 had a visual acuity of 20/100 or better at the time of treatment. In this group, poor visual acuity was found in 34% at 5 years and 68% at 10 years of follow-up. From multivariable analysis, clinical factors that best predicted poor visual acuity were increasing tumor thickness, proximity to foveola of less than 5 mm, notched plaque shape, tumor recurrence, patient age 60 years or older, subretinal fluid, cobalt isotope, anterior tumor margin posterior to equator, and worse initial visual acuity. Moderate loss of visual acuity of 5 Snellen lines or more was found in 33% at 5 years and 69% at 10 years of follow-up. From multivariable analysis, clinical factors that best predicted moderate visual acuity loss included increasing tumor thickness, worse initial visual acuity, notched plaque shape, tumor recurrence, proximity to foveola of less than 5 mm, patient age of 60 years or older, subretinal fluid, and diabetes mellitus or hypertension. When analyzing visual outcome with regard to tumor thickness, ultimate poor visual acuity of 20/200 or worse at 5 years was found in 24% with a small melanoma (< or = 3.0 mm), 30% with a medium melanoma (3.1-8.0 mm), and 64% with a large melanoma (>8.0 mm). When analyzing visual outcome with regard to tumor proximity to visually important structures, tumors less than 5 mm from the optic disc or foveola demonstrated poor visual acuity in 35% at 5 years, whereas those 5 mm or more from the optic disc and foveola showed poor visual acuity in 25% at 5 years. CONCLUSIONS: Ultimate visual acuity after plaque radiotherapy for uveal melanoma depends on many factors, including patient age and general health, initial visual acuity, tumor location and size, subretinal fluid, radioactive isotope, and final tumor control. At 10 years' follow-up, 68% of patients demonstrate poor visual acuity. Visual acuity is most effectively preserved in eyes with small tumors outside a radius of 5 mm from the optic disc and foveola. Arch Ophthalmol. 2000;118:1219-1228