Peri-articular bone formation involving the temporomandibular joint: a narrative summary and Delphi consensus of a new classification system.

Heterotopic ossification (HO) is defined as 'bone where it does not belong'. Given the historical variety of nomenclature and eponyms, there is significant confusion about the etiology, pathogenesis, classification, and treatment of HO related to the temporomandibular joint (TMJ). The existing classifications for TMJ HO have shortcomings: they relate to specific populations, use differing imaging studies and demographic data, do not universally include alloplastic/autologous replacements, are based variously on radiological and/or clinical presentations that cannot always be combined, and were largely developed to assist oral and maxillofacial surgeons in surgical management. These deficiencies make it problematic to compare studies, draw valid conclusions, and pursue research. The aim of this study was to develop a new, more inclusive classification for TMJ HO. Currently available classifications were evaluated and a Delphi-type system used to build consensus from clinicians and researchers to develop a new system. Fourteen unique classifications for TMJ ankylosis/HO were identified. In light of the biological specifics related to heterotopic calcification of extracellular matrix versus heterotopic formation of actual bone, the group recommends a more unambiguous term - peri-articular bone formation - and proposes a new classification. This will help clinicians and researchers to study, describe, and manage various types of ectopic bone associated with the TMJ.

Which comorbid conditions and risk factors affect the outcome of and progression to total temporomandibular joint replacement?

Surgery is an effective modality to reduce pain and increase range of motion (ROM) in TMJ disorders. The aim of this study was to determine which comorbidities and risk factors affect outcomes and progression to total joint replacement (TJR). A retrospective cohort study of patients who underwent TJR between 2000- 2018 at MGH was conducted. Primary outcome was successful vs unsuccessful surgery. Success was defined as pain score ≤ 4 and ROM ≥ 30 mm; failure was defined as lack of either or both. Secondary outcome was differences between patients undergoing TJR only (group A) and those undergoing multiple surgeries progressing to TJR (group B). 99 patients (82 females, 17 males) were included. Mean follow-up was 4.1 years; mean age at first surgery was 34.2 (range 14-71) years. Unsuccessful outcomes were associated with high preoperative pain, low preoperative ROM, and higher number of surgeries. Male sex favored successful outcome. 75.0% group A and 47.6% group B had successful outcome. Group B had more females, higher postoperative pain, lower postoperative ROM, and used more opioids compared to group A. High preoperative pain, low preoperative ROM, and more surgeries were associated with poorer outcomes and frequent opioid use.

No evidence of resistance to itraconazole in a prospective real-world trial of dermatomycosis in India.

BACKGROUND: The prevalence of superficial fungal infections in India is believed to have increased substantially in the past decade. We evaluated the treatment outcomes and risk factors associated with clinical response to a treatment course of itraconazole for the management of dermatomycosis in India. METHODS: In this real-world, prospective pilot study (August 2019 to March 2020), adult participants (18-60 years), diagnosed with T. cruris or T. corporis, received itraconazole 200 mg/day (any formulation) orally for 7 days, and were followed for an additional 7 days. RESULTS: The study was terminated early due to the COVID-19 pandemic. Of 40 enrolled participants (mean [SD] age, 35.5 [12.73] years; {62.5%}] male; 37 received itraconazole and 20 (50%) completed the study. The median (range) Clinical Evaluation Tool Signs and Symptoms total score at baseline was 5.5 (2-10). Clinical response of "healed" or "markedly improved" based on the Investigator Global Evaluation Tool at day 7 (primary objective) was 42.9% (12/28; 95% CI: 24.53%, 61.19%). Itraconazole minimum inhibitory concentration for identified microorganisms, T. mentagrophytes species complex (91.7%) and T. rubrum (8.3%), was within the susceptibility range (0.015-0.25 mcg/mL). At day 14, 8/13 (61.5%) participants achieved a mycological response, 2/13 participants (15.4%) had a mycological failure and 90% showed a clinical response. CONCLUSION: COVID-19 pandemic affected patient recruitment and follow-up, so the findings call for a careful interpretation. Nevertheless, this real-world study reconfirmed the clinical efficacy and microbial susceptibility to itraconazole for the fungi causing dermatophytosis in India. TRIAL REGISTRATION: Trial registration number: Clinicaltrials.gov NCT03923010.

Addition of oral fexofenadine to topical therapy leads to a significantly greater reduction in the serum interleukin-31 levels in mild to moderate paediatric atopic dermatitis.

BACKGROUND: Recent evidence has suggested that oral antihistamines could have a beneficial role in atopic dermatitis (AD) because of their anti-inflammatory action. AIM: To evaluate the effectiveness of adding an oral second-generation, nonsedating, H1-receptor antihistamine (fexofenadine) to topical treatment in AD. METHODS: In this prospective randomized study, 50 patients with a diagnosis of mild to moderate AD were recruited and randomized into two groups: Group A was given appropriate topical treatment (topical tacrolimus 0.03-0.1% ointment once daily along with topical fluticasone propionate 0.05% cream once daily, as well as paraffin-based emollients) combined with oral fexofenadine, while Group B was given appropriate topical treatment only. Both groups received the respective treatments for 8 weeks. RESULTS: There was no significant difference between the two groups in terms of the SCORing Atopic Dermatitis and the 5-dimensions Itch Scale at any of the time points (Weeks 2, 4 and 8). However, in the fexofenadine group, the level of serum interleukin (IL)-31 decreased significantly from baseline to Week 8 of treatment. CONCLUSIONS: Although we could not conclusively confirm the clinical efficacy of adding oral fexofenadine to topical treatment in AD, serological evaluation indicates that fexofenadine treatment can lead to significant lowering of serum IL-31 levels in patients with AD.

Severe epidermolysis bullosa/Kindler syndrome-like phenotype of an autoinflammatory syndrome in a child.

A 5-year-old boy presented with generalized cutaneous erosions, severe scarring, depigmentation and contractures affecting major joints. The lesions had initially affected his ears, nose, feet, and the genital and ocular mucosa, leading to significant depigmentation, scarring, contractures and mutilation. The whole of the trunk and limbs were involved at the time of presentation, with the exception of some islands of spared skin on the proximal thighs, legs, nipples and external genitalia. Electron microscopy revealed a split in the sublamina densa with the absence of anchoring fibrils, suggestive of dystrophic epidermolysis bullosa (EB). Immunofluorescence antigen mapping demonstrated a broad reticulate pattern of staining with collagen IV, VII, and laminin 332 in the floor of the blister, suggestive of Kindler syndrome. Next-generation sequencing revealed a de novo heterozygous missense mutation (a variant of unknown significance) in exon 22 of the phospholipase-C gamma 2 gene (PLCG2), which resulted in a substitution of serine by asparagine at codon 798 (p.Asp798Ser), a result that was validated using Sanger sequencing. The child was diagnosed with PLCG2-associated antibody deficiency and immune dysregulation (PLAID)/autoinflammation and PLCG2-associated antibody deficiency and immune dysregulation (APLAID) syndrome. The cutaneous and corneal erosions, inflammation and scarring of this magnitude, and the eventual result of death have not been described previously for the PLAID/APLAID spectrum previously. In conclusion, this was an unusual acquired autoinflammatory severe EB-like disease that may be associated with de novo PLCG2 mutation.

Paediatric morphoea: a holistic review. Part 2: diagnosis, measures of disease activity, management and natural history.

Paediatric morphoea is a debilitating fibrosing disorder of uncertain aetiology, affecting the skin and subcutaneous tissues. Defining optimum management strategies in paediatric morphoea remains an ongoing challenge, owing to the varied presentations and a relative paucity of paediatric-specific studies. We performed a literature search on PubMed, MEDLINE and Google Scholar, using keywords such as 'pediatric morphea', 'juvenile localised scleroderma' and 'juvenile systemic sclerosis'. Relevant studies, including randomized trials, reviews of standard current guidelines and original research articles, were selected and results analysed before summarizing them. In Part 1 of this review, we described the epidemiology, aetiopathogenesis and clinical classification; in this part, we discuss the diagnosis, markers of disease activity, management and natural history in paediatric morphoea.

Paediatric morphoea: a holistic review. Part 1: epidemiology, aetiopathogenesis and clinical classification.

Morphoea, also known as localized scleroderma, is a debilitating fibrosing disorder of uncertain aetiology, affecting the skin and subcutaneous tissues. Paediatric-onset disease is not uncommon and is associated with frequent relapses. The disease has complex pathogenetic mechanisms and multiple clinical subtypes, and affects children of all ages. Recent research has focused on elucidating the disease pathophysiology and identifying measures of disease activity. We performed a literature search on PubMed, MEDLINE and Google Scholar, using keywords such as 'pediatric morphea', 'juvenile localised scleroderma' and 'juvenile systemic sclerosis'. Relevant studies, including randomized trials, reviews of standard current guidelines and original research articles, were selected, and results were analysed before being summarized. In the first of this two-part review, we provide a bird's-eye view of the current literature concerning the epidemiology, aetiopathogenesis and clinical classification of paediatric morphoea; in Part 2, we review the diagnosis, markers of disease activity, management and natural history.

Contact sensitization to hair colours in acquired dermal macular hyperpigmentation: results from a patch and photo-patch test study of 108 patients.

BACKGROUND: Acquired dermal macular hyperpigmentation (ADMH) is an umbrella term including lichen planus pigmentosus, erythema dyschromicum perstans and pigmented contact/cosmetic dermatitis. OBJECTIVE: To establish contact sensitization to hair colours as an aetiological factor for ADMH. METHODS: Detailed clinical examination, skin biopsies, and patch and photo-patch testing with Indian standard series and patient's own cosmetic products were performed. RESULTS: Thirty-nine (36.1%) patients were found to demonstrate a positive patch/photo-patch test with 35/39 reacting to their own products (all were hair colours) and 16/39 reacting to antigens from commercial series (commonly paraphenylenediamine). Fourteen patients developed delayed hyperpigmentation on positive patch-test sites at 1 month. Higher mean age, symptomatic pigmentation (pruritus, burning and photosensitivity), hair margins involvement (outer surface, helix and lobule of ear; temples and preauricular area), ill-defined lesions, epidermal atrophy and epidermal melanization extending >3 layers were significantly common in patch-test-positive patients. Well-defined lesions, perioral involvement and associated lichen planus were clinical pointers towards patch-test negativity. CONCLUSION: Index study exemplifies that patch-test results have distinct clinical and histopathological correlates in ADMH. Hair dye contact sensitization appears to be an important aetiological factor in about one-third patients presenting with ADMH.