Effect of clarithromycin in a case of infantile bullous pemphigoid.

Although rare, cases of infantile or childhood bullous pemphigoid are increasingly being reported in the literature. Treatment challenges, which are amplified in infancy, necessitate balancing efficacy and avoiding long-term risks. In this report, clarithromycin was successfully used to establish and maintain disease remission, offering insights into its immunomodulatory effects, making it a compelling steroid-sparing choice with a favorable side effect profile.

Genodermatoses with behavioural sequelae.

Children with genodermatoses are at an increased risk of developing behavioural disorders which may impart lasting damage on the individual and their family members. As such, early recognition of childhood mental health disorders via meticulous history taking, thorough physical examination, and disorder-specific testing is of paramount importance for timely and effective intervention. If carried out properly, prompt psychiatric screening and intervention can effectively mitigate, prevent or even reverse, the psychiatric sequela in question.

Infantile Hemangiomas.

Infantile hemangiomas (IHs) are the most common benign vascular tumors of childhood. They develop during the first few weeks of life and naturally progress by proliferating over several months before they involute and resolve; this renders them inconsequential in many cases, but sometimes IHs can have detrimental consequences on function and disfigurement. Hence, systemic propranolol has become a crucial element in IH management, alongside various other medical, procedural, and surgical options that aim to promote their quicker resolution and prevent and alleviate complications.

A Novel Pathogenic CDH3 Variant underlying Heredity Hypotrichosis Simplex detected by Whole-Exome Sequencing (WES)-A Case Report.

Background: Heredity Hypotrichosis Simplex (HHS) is a rare non-syndromic disease form of Hypotrichosis Simplex (HS) characterized by progressive hair follicle (HF) miniaturization. It is usually inherited in an autosomal dominant manner. The differential diagnosis of HHS and the treatments remain challenging despite recent advancement.

Treatments for Non-Syndromic Inherited Ichthyosis, Including Emergent Pathogenesis-Related Therapy.

The term 'inherited ichthyosis' refers to a heterogeneous group of mendelian disorders of cornification that involve the integument with varying degrees of scaling. The management of ichthyosis poses a challenge for most physicians. Treatment options proposed in the literature include moisturizers, topical keratolytics, topical and systemic vitamin D analogues, and topical and systemic retinoids; however, some of these modalities are less reliable than others.

Eosinophilic ulcer of the oral mucosa: An update on clinicopathologic features, pathogenesis, and management.

Eosinophilic ulcer of the oral mucosa is a benign, reactive, self-limited lesion. Its pathogenesis is unclear, and it clinically manifests as a punched-out ulcer with surrounding indurated border, raising suspicion for a possible malignancy. On histology, an acute and chronic dense inflammatory infiltrate can be appreciated, which can extend to the deep underlying tissue (including skeletal muscle).

Primary Localized Cutaneous Amyloidosis of Keratinocyte Origin: An Update with Emphasis on Atypical Clinical Variants.

Amyloid is a protein derived from at least 20 different substances. Once misfolded, it results in a group of cutaneous and systemic conditions. Primary localized cutaneous amyloidosis of keratinocyte origin is a very common subtype that can manifest either as lichen or macular amyloidosis, lacking systemic involvement.

Genodermatoses with hearing impairment.

Hearing loss is a prominent feature in multiple genodermatoses. Underappreciation of auditory deficits can misdirect proper diagnosis by the treating dermatologist. This review reviews the anatomic, developmental, and embryologic aspects that characterize the ear and summarizes genodermatoses that have aberrant auditory findings.

Imatinib-induced eruptive nevi.

Imatinib mesylate is a tyrosine kinase inhibitor used in the treatment of a variety of oncological conditions. It has been associated with a spectrum of pigmentary cutaneous side effects. Despite the well-known association of eruptive melanocytic nevi with the use of other tyrosine kinase inhibitors, this phenomenon has not been linked to the administration of imatinib.

Osteoclast-Like Giant Cells: Focus on Entities Relevant to Dermatopathology and Underlying Pathogenesis.

Osteoclast-like giant cells (OLGCs) resemble osteoclasts with their abundant cytoplasm and well-developed organelles. OLGCs are characteristic features of giant cell tumor of the tendon sheath and giant cell tumor of soft tissue but they have also been described in numerous other cutaneous conditions. The diagnostic and prognostic significance of the presence of OLGCs is unknown.

Neuroendocrine Differentiation of Skin Tumors: A Comprehensive Review.

Neuroendocrine differentiation is characterized by endocrine and neuronal features with prominent dense secretory granules and neuropeptides. Neuroendocrine differentiation of skin tumors is of unknown clinical significance. Nonetheless, the acknowledgment of this line of differentiation is important to prevent diagnostic pitfalls and subsequent inappropriate management.

Acute Coronary Syndrome in a Male with Elevated Anti-Cyclic Citrullinated Peptide and no Evidence of Longstanding Rheumatoid Arthritis.

A 55-year-old male, previously known to be healthy, presented to the emergency department with a 30-minute history of chest pain radiating to the upper extremities. Vital signs were within normal limits. Four days prior to this presentation, the patient presented for acute onset of polyarthritis and morning stiffness.

SuPAR, a potential inflammatory mediator in psoriasis pathogenesis.

Psoriasis is an inflammatory skin disorder that is strongly associated with the metabolic syndrome. The sole reliance on clinical examination to guide prognostication and treatment is insufficient at best; accurate diagnostic and prognostic psoriatic molecular biomarkers are needed. Soluble urokinase plasminogen activator receptor (suPAR) has been implicated in inflammation.

Malar rash in a young child with neurodevelopmental delay: a quiz.

-A 14-month-old boy born to consanguineous parents presented to our Dermatology Department with a 6-month history of a malar eczematous rash that worsens with sun exposure. He had butterfly-shaped, hyperpigmented exfoliating plaques, preceded by blister formation (figure 1). He was also noticed to have enophthalmos, a pinched nose, microcephaly and a cachectic physique.

A novel TRAF3IP2 variant causing familial scarring alopecia with mixed features of discoid lupus erythematosus and folliculitis decalvans.

Discoid lupus erythematosus (DLE) is an autoimmune disorder with a poorly defined etiology. Despite epidemiologic gender and ethnic biases, a clear genetic basis for DLE remains elusive. In this study, we used exome and RNA sequencing technologies to characterize a consanguineous Lebanese family with four affected individuals who presented with classical scalp DLE and generalized folliculitis.

Water-related dermatoses.

Water-related dermatoses are a spectrum of diseases that are associated with water exposure. They result either from the direct influence of water or after injury from water inhabitants. In this review, clinical entities that manifest after water exposure (aquagenic pruritus, aquagenic urticaria, and aquagenic wrinkling of the palms) will be discussed with particular focus on the reported pathogenesis.

SuPAR, an emerging biomarker in kidney and inflammatory diseases.

Soluble urokinase plasminogen activator receptor (suPAR) is a circulating form of a physiological and pathophysiological important cell surface receptor, implicated in inflammation. Recent studies showed that suPAR is a promising biomarker, useful for diagnosis, assessment and prognosis of several diseases. This review summarises the majority of preliminary studies and analyses the significance and the clinical application of suPAR in various clinical conditions.