Case Report and Review of the Literature: Bullous Skin Eruption After the Booster-Dose of Influenza Vaccine in a Pediatric Patient With Polymorphic Maculopapular Cutaneous Mastocytosis.

Vaccination is a well-known trigger for mast cell degranulation in subjects affected by mastocytosis. Nevertheless, there is no exact standardized protocol to prevent a possible reaction after a vaccine injection, especially for patients who have already presented a previous vaccine-related adverse event, considering that these patients frequently tolerate future vaccine doses. For this reason, we aim to share our experience at Meyer Children's University Hospital in Florence to raise awareness on the potential risk for future vaccinations and to discuss the valuable therapeutic strategies intended to prevent them, taking into account what is proposed by experts in literature. We describe the case of an 18-month-old female affected by a polymorphic variant of maculopapular cutaneous mastocytosis that presented an extensive bullous cutaneous reaction 24 hours after the second dose (booster dose) of inactivated-tetravalent influenza vaccine, treated with a single dose of oral corticosteroid therapy with betamethasone (0.1 mg/kg) and an oral antihistamine therapy with oxatomide (1 mg/kg/daily) for a week, until resolution. To the best of our knowledge, in the literature, no documented case of reaction to influenza vaccine in maculopapular cutaneous mastocytosis is described. Subsequently, the patient started a background therapy with ketotifen daily (0.05 mg/kg twice daily), a non-competitive H1-antihistamine, and a mast cell stabilizer (dual activity). A non-standardized pharmacological premedication protocol with an H1-receptor antagonist (oxatomide, 0.5 mg/kg) administered 12 hours before the immunizations, and a single dose of betamethasone (0.05 mg/kg) together with another dose of oxatomide (0.5 mg/kg) administered 2 hours before the injections was followed to make it possible for the patient to continue with the scheduled vaccinations. Indeed, no reactions were subsequently reported. Thus, in our experience, a background therapy with ketotifen associated with a premedication protocol made by two doses of oxatomide and a single dose of betamethasone was helpful to make possible the execution of the other vaccines. We suggest how in these children, it could be considered the idea of taking precaution when vaccination is planned, regardless of the kind of vaccine and if a dose of the same vaccine was previously received. However, international consensus needs to be reached to manage vaccinations in children with mastocytosis and previous adverse reactions to vaccines.

Between fire and ice: refractory hypothermia and warmth-induced pain in inherited erythromelalgia.

Inherited erythromelalgia (IEM) is a well-described pain disorder caused by mutations of sodium channel Nav1.7, a peripheral channel expressed within dorsal root ganglion and the sympathetic ganglion neurons. Clinically, IEM is characterised by paroxysmal attacks of severe pain, usually in the distal extremities, triggered by warmth or exercise. Pain is not adequately treated by existing pharmacological agents. Individuals with IEM classically cool their limbs for relief, in some cases resulting in tissue injury. We describe a patient from a family with IEM due to the L858F mutation of Nav1.7 who presented with refractory hypothermia due to overcooling. This presentation of refractory hypothermia necessitating warming strategies, complicated by severe warmth-induced pain, posed a substantial therapeutic challenge. We report our experience in overcoming hypothermia lasting 3 weeks in a child with IEM, discuss possible pathophysiological mechanisms underlying this unusual complication and suggest potential therapeutic interventions.

Calidad de vida dermatológica en ancianos frágiles comunitarios / Dermatological quality of life in community-dwelling frail elderly people

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Early Description of Diet-Induced Blistering Skin Diseases in Medieval Persia: Avicenna's Point of View.

Pemphigus is an autoimmune blistering skin disease that is strongly associated with different environmental factors. Among these, nutritional factors are considered to trigger pemphigus; however, their role may be underestimated. Investigated more recently in conventional medicine, this causative bond between dietary factors and blistering skin diseases was mentioned by Persian scholars such as Avicenna a thousand years ago. Avicenna, a well-known Persian physician and philosopher, who could be considered a pioneer in dermatology, discussed skin diseases in a chapter in The Canon of Medicine. He accounted for some nutritional triggers for skin blisters (mentioned as "hot swellings"), such as onion, garlic, leek, pepper, and wine. His precise description of causative factors based on principles of traditional Persian medicine (TPM) is appreciable and might well lead us to find more efficient ways for the prevention and treatment of blistering skin diseases.

Dermatosis ampollosa IgA lineal de la infancia y «el collar de perlas» / Linear Immunoglobulin A Dermatosis of Childhood With the String of Beads Sign

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Treatment of subepidermal immunobullous diseases.

The subepidermal immunobullous diseases are a group of autoimmune blistering disorders of the skin and mucous membranes that share the common features of autoantibody deposition and blister formation at the dermal-epidermal junction or basement membrane. This group includes bullous pemphigoid, linear IgA disease, dermatitis herpetiformis, and epidermolysis bullosa acquisita, among others. Although these disorders share some common features, each disease is unique in its clinical presentation, histopathology, and immunofluorescence patterns, which allows for accurate diagnosis and disease-specific treatment strategy. Treatment of these disorders is complex and requires expert knowledge of disease pathogenesis. We review common treatment approaches for each of these disorders.

Estudio de las dermopatías que se presentan con mayor frecuencia en el pie del deportista / Study of the most frequent dermal foot lesions in athletes

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Cytokine expression patterns associated with systemic adverse events following smallpox immunization.

Vaccinia virus is reactogenic in a significant number of vaccinees, with the most common adverse events being fever, lymphadenopathy, and rash. Although the inoculation is given in the skin, these adverse events suggest a robust systemic inflammatory response. To elucidate the cytokine response signature of systemic adverse events, we used a protein microarray technique to precisely quantitate 108 serum cytokines and chemokines in vaccine recipients before and 1 week after primary immunization with Aventis Pasteur smallpox vaccine. We studied 74 individuals after vaccination, of whom 22 experienced a systemic adverse event and 52 did not. The soluble factors most associated with adverse events were selected on the basis of voting among a committee of machine-learning methods and statistical procedures, and the selected cytokines were used to build a final decision-tree model. On the basis of changes in protein expression, we identified 6 cytokines that accurately discriminate between individuals on the basis of adverse event status: granulocyte colony-stimulating factor, stem cell factor, monokine induced by interferon-gamma (CXCL9), intercellular adhesion molecule-1, eotaxin, and tissue inhibitor of metalloproteinases-2. This cytokine signature is characteristic of particular inflammatory response pathways and suggests that the secretion of cytokines by fibroblasts plays a central role in systemic adverse events.

Epidemic bullous impetigo in a nursery due to a nasal carrier of Staphylococcus aureus: role of epidemiology and control measures.

From September 14, 1981 to February 28, 1982, an epidemic of bullous impetigo caused by a penicillin/tetracycline resistant strain of Staphylococcus aureus, phage type 3A/3C, occurred in a newborn nursery in Louisville, Kentucky. Twenty of 1,181 (1.7%) infants at risk developed disease during the six-month epidemic period. Clinically all case-infants had bullous impetigo skin lesions. One infant developed staphylococcal septicemia. No infant died. An epidemiologic investigation identified a nurse as having significantly greater contact with case-infants than control-infants (p = 0.0013). She was also found to be a nasal carrier of the epidemic strain. Infection control measures appeared to decrease infant-to-infant transmission via the hands of non-colonized nurses, but did not affect transmission from the nurse carrying the epidemic strain to infants. No cases of bullous impetigo have occurred since this nurse was temporarily removed from the nursery for treatment.