Dermoscopic spectrum of mycosis fungoides: a retrospective observational study by the International Dermoscopy Society.

BACKGROUND: The dermoscopic features of classic patch stage mycosis fungoides (MF) have been described, but data on plaque and tumoral stage as well as rarer MF subtypes is limited. OBJECTIVE: To evaluate dermoscopic morphology and dermoscopic-pathological correlations of classic MF stages and investigate dermoscopic features of MF variants. METHODS: Patients with histopathologically confirmed lesions of classic MF (patch, plaque and tumoral stage) or folliculotropic, erythrodermic and poikilodermatous MF were included. Standardized evaluation of dermoscopic pictures of the included MF variants and comparative analysis and dermoscopic-pathological correlation assessment of different stages of classic MF were performed. RESULTS: A total of 118 instances were included (75 classic MF, 26 folliculotropic MF, 9 erythrodermic MF and 8 poikilodermatous MF). Linear/linear-curved vessels and white scales in the skin furrows were significantly associated with patch-stage MF, while clustered dotted vessels were related to plaque-stage MF and peripheral linear vessels with branches, ulceration and red globules separated by white lines to tumour-stage MF. Moreover, patchy white scales were significantly more common in patches and plaques compared to tumours, whereas focal bright white structureless areas were related to plaque and tumoral stage. Vessels histopathologically corresponded to dilated vascular structures in the dermis, orange structureless areas to either dermal hemosiderin (patch/plaque stage) or dense cellular infiltration (tumours), bright white lines/structureless areas to dermal fibrosis and ulceration to loss of epidermis. The main dermoscopic findings of folliculotropic MF were lack of hairs, dilated follicles and follicular plugs, while erythrodermic MF was mainly characterized by linear/dotted vessels, patchy white scales and focal orange structureless areas and poikilodermatous MF by focal white and brown structureless areas, white patchy scales and brown reticular lines. CONCLUSION: Dermoscopy may allow a more precise characterization of classic MF and reveal clues suggestive of the main MF variants.

Laryngeal mucous membrane pemphigoid serves as a prognostic factor for poor response to treatment with rituximab.

Mucous membrane pemphigoid (MMP) is an autoimmune blistering, scarring and occasionally mutilating disease that may progress to blindness or airway obstruction. Over the past few years, rituximab (RTX) has emerged as a potential therapeutic solution for MMP; however, the literature regarding its efficacy in the treatment of severe MMP is sparse. We studied four patients with recalcitrant MMP who were treated with RTX. Three of these had recalcitrant laryngeal disease; two were unresponsive to RTX, while the third patient achieved complete remission (CR) but relapsed twice. The fourth patient, who had oral and ocular disease, also achieved CR. In addition, we reviewed 143 cases of MMP treated with RTX reported in the literature to date. Of these, 120 had late observation endpoints, of whom 81 (67.5%) achieved CR, 24 (20%) received partial remission and 15 (12.5%) had no remission. Based on this study, the presence of laryngeal MMP seems to predict refractoriness to RTX treatment. In conclusion, we found that RTX can ameliorate the MMP course and that laryngeal involvement, which is known to be a prognostic factor for severe MMP, may also predict poor response to RTX.

Grover disease and bullous pemphigoid: a clinicopathological study of six cases.

Grover disease (GD) is an idiopathic dermatosis that typically manifests as itchy papules over the trunk in middle-aged men. Bullous pemphigoid (BP) is an autoimmune bullous disease that affects older people. Not only are the two diseases easily distinguishable on clinical grounds, they are also characterized by differences in histopathology, pathogenesis and response to treatment Thus, the co-occurrence of these two conditions in the same patient is usually considered coincidental. In this report, we present a multicentre retrospective analysis of six patients who developed both GD and BP over a short period of time, and in all cases but one, GD preceded BP. We discuss the clinical and histopathological features of these patients, and the suggested mechanisms of the diseases. We conclude that GD might predispose to the development of BP.

Survival, disease progression and prognostic factors in elderly patients with mycosis fungoides and Sézary syndrome: a retrospective analysis of 174 patients.

BACKGROUND: Advanced age at diagnosis is considered a poor prognostic factor in mycosis fungoides (MF) and Sézary syndrome (SS). OBJECTIVE: To evaluate the outcomes and prognostic factors in patients diagnosed at an advanced age (≥65 years) with MF/SS. METHODS: Survival, progression rates and various clinical and histopathological variables were studied in a group of 174 elderly patients diagnosed with MF/SS between 1992 and 2015 at a single referral cancer center in the United States. Kaplan-Meier estimates were used to determine survival and progression and Cox proportional hazards regression univariate and multivariate models were used to identify prognostic factors. RESULTS: Of 174 elderly patients, 76.4% were diagnosed with early-stage (clinical stages IA-IIA) and 23.6% with late-stage MF/SS (IIB-IV). Advanced age was associated with poor overall survival, but not with disease-specific survival (DSS) or progression-free survival (PFS). Gender, increasing clinical stage, T and B classifications, elevated lactate dehydrogenase (LDH) levels and development of large cell transformation (LCT) were significant predictors of poor survival or disease progression. Patients with early-stage MF and <10% total skin involvement (T1 classification) or patch-only disease (T1a/T2a) showed better PFS with no observed disease-specific mortality. Folliculotropic MF was associated with poor DSS in patients with early-stage disease. CONCLUSIONS: Older age at diagnosis of MF/SS does not predict worse disease-specific outcomes. Elderly patients with early-stage disease, specifically involving less than 10% of the skin surface with patches but without plaques or folliculotropism, have an excellent prognosis. However, the development of LCT is a strong prognostic indicator of poor survival in elderly patients with MF/SS.

Acneiform follicular mucinosis: an indolent follicular mucinosis variant unrelated to mycosis fungoides?

Follicular mucinosis (FM) can present as an acneiform eruption, and is usually a benign variant of primary FM unrelated to cutaneous T-cell lymphoma (CTCL). We report two cases of women in their twenties who presented with an acneiform rash on the face, arms and back. In both cases, pathological evaluation of the facial papules revealed predominantly mucinous degeneration of the follicular epithelium, with insufficient lymphocytic infiltration or atypia to diagnose mycosis fungoides. These cases are similar to previous reports of acneiform FM. As none of the reported cases progressed to CTCL, we consider that overdiagnosis and overtreatment should be avoided in acneiform FM, but recommend long-term follow-up.

Dermoscopy and the diagnosis of primary cutaneous B-cell lymphoma.

BACKGROUND: Primary cutaneous B-cell lymphomas (PCBCLs) are frequently misdiagnosed, and a biopsy is needed to attain the correct diagnosis. OBJECTIVE: To characterize the dermoscopic features of PCBCL. METHODS: In this retrospective observational study, we analysed the pathology reports of 172 newly diagnosed PCBCL for the initial clinical differential diagnosis. The dermoscopic images of 58 PCBCL were evaluated for dermoscopic features. Two dermoscopy experts, who were blinded to the diagnosis and the study objective, evaluated images from 17 cases for a dermoscopic differential diagnosis. RESULTS: Of 172 biopsy-proven PCBCL lesions, cutaneous lymphoma was suspected by the clinician in 16.3%; the leading diagnosis was basal cell carcinoma in 17.4%, and other skin neoplasms in 21%. Studying 58 PCBCL dermoscopic images, we most frequently identified salmon-coloured background/area (79.3%) and prominent blood vessels (77.6%), mostly of serpentine (linear-irregular) morphology (67.2%). Dermoscopic features did not differ significantly by subtype or location. Blinded evaluation by dermoscopy experts raised a wide differential diagnosis including PCBCL, arthropod bite, basal cell carcinoma, amelanotic melanoma and scar/keloid. CONCLUSIONS: Two dermoscopic features, salmon-coloured area/background and serpentine vessels, are frequently seen in PCBCL lesions. These characteristic dermoscopic features, although not specific, can suggest a possible diagnosis of PCBCL.

Misoprostol for primary versus secondary prevention of postpartum haemorrhage: a cluster-randomised non-inferiority community trial.

OBJECTIVE: To assess whether secondary prevention, which preemptively treats women with above-average postpartum bleeding, is non-inferior to universal prophylaxis. DESIGN: A cluster-randomised non-inferiority community trial. SETTING: Health sub-centres and home deliveries in the Bijapur district of Karnataka, India. POPULATION: Women with low-risk pregnancies who were eligible for delivery with an Auxiliary Nurse Midwife at home or sub-centre and who consented to be part of the study. METHODS: Auxiliary Nurse Midwifes were randomised to secondary prevention using 800 mcg sublingual misoprostol administered to women with postpartum blood loss ≥350 ml or to universal prophylaxis using 600 mcg oral misoprostol administered to all women during the third stage of labour. MAIN OUTCOME MEASURES: Postpartum haemoglobin ≤7.8 g/dl, mean postpartum blood loss and postpartum haemoglobin, postpartum haemorrhage rate, transfer to higher-level facilities, acceptability and feasibility of the intervention. RESULTS: Misoprostol was administered to 99.7% of women as primary prevention. In secondary prevention, 92 (4.7%) women had postpartum bleeding ≥350 ml, of which 90 (97.8%) received misoprostol. The proportion of women with postpartum haemoglobin ≤7.8 g/dl was 5.9 and 8.8% in secondary and primary prevention clusters, respectively [difference -2.9%, one-sided 95% confidence interval (CI) <1.3%]. Postpartum transfer and haemorrhage rates were low (<1%) in both groups. Shivering was more common in primary prevention clusters (P = 0.013). CONCLUSION: Secondary prevention of postpartum haemorrhage with misoprostol is non-inferior to universal prophylaxis based on the primary outcome of postpartum haemoglobin. Secondary prevention could be a good alternative to universal prophylaxis as it medicates fewer women and is an acceptable and feasible strategy at the community level. TWEETABLE ABSTRACT: Secondary prevention of postpartum haemorrhage with misoprostol is non-inferior to universal prophylaxis.

A novel homozygous deletion in EXPH5 causes a skin fragility phenotype.

Epidermolysis bullosa simplex (EBS) is the most common form of EB. Eight different genes have been implicated in the pathogenesis of different types of EBS, but a substantial portion of the cases cannot be attributed to mutations in known genes. Recently, recessive mutations in the gene EXPH5 (encoding exophilin-5, also known as Slac2-b) were identified in patients affected with a mild form of EBS. We used immunohistochemistry, Sanger sequencing and PCR-restriction fragment length polymorphism analysis to identify the cause of mild congenital skin fragility in a 3-year-old girl. No mutations were detected in KRT5 or KRT14, but we identified a novel homozygous deletion in EXPH5, which was found to cosegregate with the disease phenotype in the family. Our results further expand the spectrum of mutations in EXPH5. Appraisal of the present case against previously reported patients indicate that EXPH5 mutations result in a distinctive skin fragility phenotype, with minimal blistering compared with other forms of basal EBS.

The expanding spectrum of IgA pemphigus: a case report and review of the literature.

IgA pemphigus (IGAP) is a rare, distinct variant of pemphigus, currently classified, depending upon the histological features, immunofluorescence staining pattern and autoantibody profile, into two types: subcorneal pustular dermatosis (SPD) and intraepidermal neutrophilic IgA dermatosis. In a patient with a widespread blistering disease of the skin resembling SPD-type IGAP, we demonstrate the coexistence of IgA reactivity to both epidermal (desmocollins 2 and 3) and basement membrane-associated (BP180) proteins, suggesting the coexistence of atypical IGAP and linear IgA bullous dermatosis, respectively. This case, together with 20 previous reports of atypical IGAP, underscores the limitations of current classification schemes. Therefore, we suggest reclassifying these cases under the general term 'IGAP spectrum'.

Community-based distribution of misoprostol to prevent postpartum haemorrhage at home births: results from operations research in rural Ghana.

OBJECTIVE: To report on a rigorous distribution and monitoring plan to track misoprostol for community-based distribution to reduce postpartum haemorrhage (PPH) in rural Ghana. DESIGN: Operations research. SETTING: Rural Ghana. SAMPLE: Women in third trimester of pregnancy presenting to primary health centres (PHCs) for antenatal care (ANC). METHODS: Ghana Health Service (GHS), Millennium Village Projects, and the University of Illinois at Chicago conducted an operations research study designed to assess the safety, feasibility, and acceptability of community-based distribution of misoprostol to prevent PPH at home deliveries in rural Ghana. One thousand doses (3000 tablets, 200 µg each) were obtained from the Family Health Division of GHS. Three 200-µg tablets of misoprostol (600 µg) in foil packets were packaged together in secured transparent plastic packets labelled with pictorial messages and distributed to midwives at seven PHCs for distribution to pregnant women. MAIN OUTCOME MEASURES: Correct use of misoprostol in home deliveries and retrieval of unused misoprostol doses, PPH rates and maternal mortality. RESULTS: Of the 999 doses distributed to midwives, 982 (98.3%) were successfully tracked, with a 1.7% lost to follow-up rate. Midwives distributed 654 doses to women at third-trimester ANC visits. Of women who had misoprostol to use at home, 81% had an institutional delivery and were able to return the misoprostol safely to the midwife. Of the women that used misoprostol, 99% used the misoprostol correctly. CONCLUSIONS: This study clearly demonstrates that misoprostol distributed antenatally to pregnant women can be used accurately and reliably by rural Ghanaian women, and should be considered for policy implementation across Ghana and other countries with high home birth rates and maternal mortality ratios.

Crystal Structure of the Solid Electrolyte, RbAg4I5.

The crystal structure of the solid electrolyte RbAg(4)I(5) has been determined from single-crystal x-ray diffraction counter data. There are four RbAg(4)I(5) in a cubic unit cell with a = 11.24 A. The structure refinement, by least squares, is based on space group P4(1)3 (O(7)). The arrangement of the iodide ions is similar to that of the manganese atoms in beta-manganese, and provides 56 iodide tetrahedra per unit cell, which share faces in such manner as to provide diffusion paths for the silver ions. The occurrence of the two low-temperature phases of RbAg(4)I(5) has been established by x-ray diffraction and optical examination.

Crystal Structure of the Solid Electrolyte(C5H5NH) Ag5I6 at -30{degrees}C.

The crystal structure of pyridinium hexaiodopentaargentate, (C(5)H(5)NH) Ag(5)l(6), is unique among those of the halide and chalcogenide solid electrolytes in that face-sharing iodide octahedra as well as face-sharing tetrahedra and face-sharing between octahedra and tetrahedra provide the paths for silver ion transport. There are two formula units in a hexagonal cell, space group P6/mcc (D6h(2)). At -30 degrees C, the lattice constants are a = 11.97 +/- 0.02, c = 7.41 +/- 0.01 A. The structure has three sets of sites for the silver ions. At -30 degrees C two of these sets are apparently filled with the ten silver ions per unit cell, while the third set of tetrahedrally coordinated general positions is empty. Therefore, the conductivity at this temperature is limited by the thermal excitation of the silver ions into the empty tetrahedra.

Pressure-induced phases of sulfur.

At least three phases of sulfur may be induced at pressures between 16 and 65 kilobars. A fibrous form obtained at pressures of 27 kilobars and higher appears to be closely related to the form obtained when plastic sulfur is chilled and stretched. Crystals of the fibrous form are orthorhombic with a = 13.8, b = 32.4, and c = 9.25 A. Thus far the results are in accord with deductions made by Prins and co-workers that the sulfur atoms are arranged in helices with ten atoms and three turns per 13.8-A period, and that these helices are essentially close-packed. The unit cell contains 16 ten-atom chain lengths. The probable space groups to which the crystal may belong are C c m m, C c 2 m, or C c m 2(1). These imply that the structure must contain both right- and left-handed helices and that at least half the helices have some disorder about their axes. The other two phases appear to have structures related to that of the fibrous form, but analyses of them has not progressed as far. One of these phases appears to be uomegasulfur.