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Epidermolytic ichthyosis (EI) is a rare inherited ichthyosis related to heterozygous mutations in the Keratin 1 or Keratin 10 genes. Because of the broad phenotypic spectrum, it is sometimes difficult to differentiate it from other keratinopathic ichthyoses (KI) in clinical practice. We report an intriguing case of KI presenting as generalized ichthyosis in a reticulate pattern surrounding islands of normal skin, epidermolytic hyperkeratosis and binucleate cells on histopathology, and heterozygous mutation in KRT10. Through this case, we would like to demonstrate the importance of genetic studies and genotype-phenotype correlation in diagnosing such challenging cases.

Byline: Gauri. Mahabal, Leni. George, Dincy. Peter, Meera. Thomas, Biju. George, Vikram. Mathews, Aby. Abraham, Alok. Srivastava, Susanne. Pulimood Hyperacute GVHD (HaGVHD) is a rare complication of hematopoietic stem cell [...]

Febrile ulceronecrotic Mucha‐Habermann disease (FUMHD), a lymphocyte‐mediated inflammatory skin disorder, is considered a severe variant of pityriasis lichenoides et varioliformis acuta that can lead to a fatal outcome if not managed in a timely fashion. Children with FUMHD can have systemic complications involving various organs. The scarcity of reported cases and the absence of well‐designed studies or randomized clinical trials to evaluate different therapeutic modalities pose a major challenge in treating this potentially life‐threatening disorder. We report a five‐year‐old child with FUMHD and seizures treated unsuccessfully with a combination of systemic steroids, methotrexate, dapsone, and oral erythromycin, who improved rapidly and achieved disease control with just a single infusion of low‐dose intravenous immunoglobulin. [ABSTRACT FROM AUTHOR]

Objective: Early surgical debridement is vital for favorable outcomes in acute invasive fungal sinusitis (AIFS). Our study aimed to propose guidelines with tailored, conservative surgical procedures based on areas of involvement and evaluate their usefulness in avoiding repeated debridement. Methods: This retrospective observational study was conducted on 150 AIFS patients operated on with the proposed surgical guidelines from May to June 2021 at a tertiary care hospital. Data including demography, comorbidities, surgical procedures, revision surgery, and outcome were collected and analyzed. Results: All 150 patients underwent bilateral endoscopic sinonasal debridement. Among them, 108 patients (72%) had current or recent coronavirus disease (COVID) infection. Ninetytwo patients (61.3%) required additional procedures based on disease extent. Twenty patients (15.4%) required revision debridement because of progressive or recurrent disease. Mean age of this group was 46.15 (standard deviation ±11.2) years with a strong male predominance (9:1). Seventeen had diabetes mellitus, 12 suffered from active COVID-19 infection and six had received corticosteroids. None of the 31 patients who had recovered from COVID-19 or had no comorbidities required revision surgery. Age, gender, and comorbidities were not significant predictors for revision surgery. Fourteen patients (70%) underwent second surgery within one month of primary surgery. Predominant disease locations were alveolus and palate (55% each), and in 80% the site was uninvolved at primary surgery. The most common revision procedure was inferior partial maxillectomy (60%). At follow-up, all were asymptomatic with no evidence of disease. Conclusion: The proposed surgical guidelines for AIFS allow for adequate surgical debridement with preservation of optimum functional status. Low revision surgery rates and good outcomes with minimal morbidity validate its usefulness. [ABSTRACT FROM AUTHOR]

Although Worst pattern of invasion (WPOI) is one of the histopathological (HP) markers that has been utilized in risk stratification of oral squamous cell carcinoma (OSCC) patients, its potential as an independent predictive factor for lymph node metastasis (LNM) and prognosis is least analyzed. Aim of the study is to analyze the relationship of various HP parameters to WPOI, their propensity for lymph node metastasis and prognostic value. This retrospective study included 140 patients diagnosed with resectable OSCC who underwent definitive surgery. Multiparametric HP risk assessment was done on the postoperative specimen and patients were categorized as low-risk WPOI (Type 1–3), and high-risk group (type 4 and 5). After categorization, 36.1% patients had low-risk WPOI and 63.9% had high-risk WPOI. Significant association was noted between WPOI and patient's age (p = 0.001), nodal stage (p = 0.001), lymphovascular invasion (LVI) (p = 0.006) and neural invasion (p = 0.001). 87% patients with nodal metastasis had high risk WPOI. LVI (p = 0.014) and WPOI (p < 0.001) had significant predictive role in LNM. High-risk WPOI and bone involvement were found to be predictive factors for overall survival, and only high risk WPOI had strong correlation with disease free survival having significant poor prognosis. Analyzing WPOI is essential in reporting HP specimens in OSCC. High-risk WPOI can act as an independent predictor for LNM, early recurrence and poor prognosis. Incorporation of WPOI into TNM staging is recommended to improve clinician's ability to prognosticate and individualize treatment strategies. [ABSTRACT FROM AUTHOR]

Objective: We aimed to study the literature on chronic granulomatous invasive fungal sinusitis to elucidate the changing trends in the management of the disease. Data Sources: Using specific keywords, we searched the PubMed, PubMed Central, and Scopus databases over the past 50 years, which yielded 938 articles in the English language. Review Methods: Scrutiny of 147 relevant articles revealed 15 homogenous case series (255 cases of histologically proven chronic granulomatous fungal sinusitis alone) and 8 heterogeneous case series (patients with other types of fungal sinusitis included), which were analyzed in detail (all with >5 cases each). Conclusions: The disease typically affected middle‐aged adults with immunocompetence. Most reports were from Sudan, India, and Saudi Arabia. A slowly progressive orbital, cheek, or palatal mass with proptosis (88.2%) or sinonasal symptoms (39.2%) was typical. Ethmoid (57.2%) and maxillary (51.4%) sinuses were chiefly affected with intracranial extension in 35.1%. Aspergillus flavus (64%) was the most frequent isolate reported. Endoscopic excision (78.8%) followed by azole therapy was the preferred treatment in recent reports. Orbital exenteration and craniotomy were infrequently performed. Complete resolution or improvement was reported in 91.3% of patients. Mortality ranged from 5.9% to 22.2%. There is a trend in the literature toward less radical and disfiguring surgery and preferential use of azoles, with good outcomes even in advanced cases. Implications for Practice: Chronic granulomatous fungal sinusitis should be diagnosed on the basis of well‐defined histopathologic features. A combination of endoscopic sinus surgery and azole therapy usually yields good outcomes. [ABSTRACT FROM AUTHOR]

We report salt-induced swelling transitions of a lamellar complex of the anionic polyelectrolyte, poly(acrylic acid sodium salt) (PAANa), and the cationic amphiphile, didodecyldimethylammonium chloride (DDAC). Increasing the concentration of NaCl in the solution is found to lead to a collapsed → swollen → collapsed transition of the complex. The swelling transition is driven by an abrupt increase in PAANa adsorption on DDAC bilayers above a threshold salt concentration. The lamellar periodicity of the swollen phase is not determined by the thickness of the adsorption layer, and additional mechanisms have to be invoked to understand the extent of its swelling. The swelling transition is not observed for the highest molecular weight of PAANa used, but a gradual transformation between the two collapsed structures is seen on increasing the salt concentration. The polyelectrolyte chains desorb from the bilayers at a very high salt concentration, in a process similar to the well-known destabilization of complexes of oppositely charged polyelectrolytes. However, unlike the PAANa chains, the polymer-free bilayers do not disperse uniformly in the solution. Instead, they form a collapsed lamellar stack containing very little water due to the van der Waals attraction between them. The occurrence of an abrupt swelling transition at intermediate salt concentrations in this system contrasts sharply with the gradual swelling reported in other complexes with increasing salt concentration. Furthermore, this behavior does not seem to have been anticipated by theories of complexation of oppositely charged macroions. More experiments are required for a clear understanding of the interactions stabilizing the different phases observed in this system. [ABSTRACT FROM AUTHOR]

Background: Salivary duct carcinoma (SDC) is an extremely rare and aggressive subtype of salivary gland cancer with high morbidity and mortality and poor response to treatment. The current options of treatment include radical surgery followed by radiotherapy (RT) with or without chemotherapy. The aim of this study was to analyse the patterns of recurrences, possible predictors of outcome and role of RT in a cohort of patients with non-metastatic SDC. Methods: A retrospective review of patients treated between 2010 and 2019 with histologically proven non-metastatic SDC was conducted. Results: Sixteen patients were included in the series. Median follow-up was 25 months. Progression-free survival (PFS) and overall survival (OS) at 12 months were 61% and 80%, respectively. Seven out of the 16 patients had disease progression, distant metastases being most frequent. Four patients died due to disease progression. PFS was significantly worse for patients with pathological neck node positivity (p = 0·036) and peri-parotid nodes (p = 0·007). Local control was significantly associated with RT (p = 0·011). Addition of any chemotherapy, regardless of either concurrent or adjuvant, had no impact on the PFS or OS. Pathological neck node positivity with nodal stage of N2 or higher correlated significantly with worse OS (p = 0·031). Conclusion: Salivary ductal carcinoma is an aggressive malignancy with high metastatic potential. Inferior prognosis was observed among patients who had metastatic deposits in either cervical nodes or peri-parotid nodes on histopathology. As systemic failures are more predominant among these patients, larger prospective trials are needed to formulate an optimum strategy for choice and sequencing of first-line systemic therapy. [ABSTRACT FROM AUTHOR]

Cationic surfactants form complexes with DNA in aqueous solutions. In the case of single-chain surfactants that self-assemble into micelles, these complexes have a two-dimensional columnar structure. We have studied the structural polymorphism of such complexes by varying the chain length of the surfactant. A variety of columnar structures are observed, such as square, hexagonal and superhexagonal. Formation of these different structures can be qualitatively understood in terms of the interplay between entropic and electrostatic contributions to their free energy. [ABSTRACT FROM AUTHOR]