A 3-year-old boy presented with an unusual combination of indurated skin, sclerotic plaques with lichenification, and yellowish papules. Histopathology revealed diffuse dermal mucin deposits, and laboratory tests showed a positive throat culture for Group A streptococcus and elevated serum anti-streptolysin titers. An 10-day course of oral amoxicillin was associated with near-complete resolution of all dermatological findings within 4 months.
View Article and Find Full Text PDFAm J Dermatopathol
August 2022
Eccrine squamous syringometaplasia (ESS) has been associated with several conditions including morphea, linear scleroderma, and burns. It is yet to be reported in lichen sclerosus et atrophicus (LSA). We describe a bullous LSA plaque on the forearm of a woman with pre-existing genital LSA and vitiligo.
View Article and Find Full Text PDFKaposi's sarcoma-associated herpesvirus (KSHV), also familiar as human herpesvirus 8 (HHV-8), is one of the well-known human cancer-causing viruses. KSHV was originally discovered by its association with Kaposi's sarcoma (KS), a common AIDS-related neoplasia. Additionally, KSHV is associated with two B-lymphocyte disorders; primary effusion lymphoma (PEL) and Multicentric Castlemans Disease (MCD).
View Article and Find Full Text PDFAutosomal recessive congenital ichthyosis (ARCI) is a rare and heterogeneous skin cornification disorder presenting with generalized scaling and varying degrees of erythema. Clinical manifestations range from lamellar ichthyosis (LI), congenital ichthyosiform erythroderma (CIE) through the most severe form of ARCI, Harlequin ichthyosis (HI). We used homozygosity mapping, whole-exome and direct sequencing to delineate the relative distribution of pathogenic variants as well as identify genotype-phenotype correlations in a cohort of 62 Middle Eastern families with ARCI of various ethnic backgrounds.
View Article and Find Full Text PDFBackground: Periodic acid-Schiff (PAS) staining of nail clippings is an adjunct diagnostic tool for onychomycosis.
Objective: To detect histopathological findings as clues to the presence of PAS-positive (+) fungal elements in nail clippings.
Methods: Four hundred sixteen consecutive nail clippings suspected of onychomycosis were stained with hematoxylin and eosin, and with PAS stains.
Neonatal autoimmune subepidermal blistering disease is rare. Mucosal involvement is more common in neonatal linear immunoglobulin A (IgA) bullous dermatosis. We describe a neonate with subepidermal cutaneous blistering disease with severe laryngeal and esophageal involvement leading to acute respiratory distress.
View Article and Find Full Text PDFThe highest efficacy of oral propranolol is for infantile hemangioma (IH) in the proliferative phase. Evaluation of the effectiveness of oral propranolol is less established when it is administered in late infancy following the proliferative phase. We aimed to assess the clinical outcomes of pediatric patients managed by oral propranolol beyond the proliferative phase of IH.
View Article and Find Full Text PDFWe report a case of a patient who presented with bilateral chronic painful necrotic leg ulcers. A skin biopsy revealed histopathological findings compatible with calciphylaxis, a rare phenomenon accompanied by high morbidity and mortality. Treatment options are limited and are based mainly on case reports and small series, so further research is needed in this area.
View Article and Find Full Text PDFBackground: Several cases of folliculotropic mycosis fungoides, associated with immunosuppressive therapy, including calcineurin inhibitors, have been reported in solid organ transplant patients. We have encountered 3 patients on immunosuppressive therapy who developed follicular eruptions with folliculocentric infiltrates of nonatypical lymphocytes.
Objective: To characterize these follicular eruptions and review the literature.
Background: We have encountered three cases of follicular eruptions with folliculotropic infiltrates of non-atypical lymphocytes associated with anti-tumor necrosis factor alpha (TNF-α) therapy.
Methods: Three patients aged 15 to 56 years treated with anti-TNF-α therapy (one with adalimumab, and two with infliximab) developed follicular eruptions characterized histopathologically by folliculotropic lymphocytic infiltrates. These were studied clinically, histopathologically, immunophenotypically, and molecularly.
The development of T-cell lymphomas, granulomatous reactions, and autoimmunity has been observed in immunodeficiency due to milder forms of recombination activating gene (RAG) deficiency. A few cases of cutaneous clonal papulonodular CD8 lymphocytic infiltrates and cutaneous CD8 granulomatous T-cell lymphoma have been described in association with common variable immunodeficiency, and with X-linked agammaglobulinemia. We describe a 15-year-old girl with several autoimmune disorders and recurrent infections that presented with several nodules on her cheek.
View Article and Find Full Text PDFBackground: Periodic acid-Shiff (PAS) stain may help to diagnose fungal infection in biopsies from dermatoses of the palms and soles. It is questionable whether PAS stain should be used routinely or only when tinea is suspected clinically.
Methods: A total of 195 consecutive punch biopsies of dermatoses from the palms (90) or soles (105) were stained with PAS, regardless of the clinical differential diagnosis.
JAMA Dermatol
February 2019
Importance: The prevalence of mucosal involvement in bullous pemphigoid (BP) is inconsistent. Nonoral mucosal involvement was reported anecdotally in few patients with BP.
Objective: To evaluate the prevalence of mucosal involvement in patients with BP, and to characterize the subgroup of patients with mucosal lesions.
Background/objectives: Erosive oral lichen planus (LP) may be painful and debilitating. Symptomatic oral LP has been treated with a wide spectrum of topical and systemic therapies, but few have been evaluated in large series. Hydroxychloroquine is suggested to be effective in oral LP.
View Article and Find Full Text PDFThe specificity and the predictive values of indirect immunofluorescence (IIF) in real-life settings is yet to be firmly established. The natural history of patients with false-positive results has not been sufficiently elucidated. The primary aim of the current study is to evaluate the diagnostic value of IIF analysis on monkey esophagus in pemphigus, utilizing a large cohort arising from the real-life experience of a tertiary referral center.
View Article and Find Full Text PDFImportance: The association of bullous pemphigoid (BP) with the use of dipeptidyl-peptidase 4 (DPP-4) inhibitors among patients with diabetes has recently emerged. The risk of developing BP during treatment with new DPP-4 inhibitor agents like linagliptin is yet to be established. The clinical features and the prognostic outcomes of patients with DPP-4 inhibitor-associated BP are yet to be established.
View Article and Find Full Text PDFThere is little consensus regarding mortality data in bullous pemphigoid (BP). The aim of this study was to evaluate mortality among a relatively large cohort of Israeli patients with BP and to perform a meta-analysis synthesizing existing data on 1-year mortality rates of patients with BP. This retrospective cohort study of 287 patients diagnosed with BP between 2000 and 2015 compared the mortality of patients with BP with age- and sex-matched control subjects in the general population.
View Article and Find Full Text PDFStaphylococcus lugdunensis is an emerging pathogen in skin and soft tissue infections that was previously considered a commensal. The aim of this study was to elucidate the characteristics of skin infections by S. lugdunensis and its appropriate management, in a tertiary referral medical center.
View Article and Find Full Text PDF