Medicinal bioactivites and allergenic properties of pumpkin seeds: review upon a pediatric food anaphylaxis case report.

SUMMARY: Food allergy to pumpkin seed is considered very rare, and only some isolated case reports have so far been published. We report here a case of food anaphylaxis to pumpkin seed in an eight-year-old boy, who tolerated all other edible seeds, peanut and tree nuts, as well as pulp of different kinds of pumpkins and other fruits of the Cucurbitaceae family. From this observation, a review of the botanical, historical, medicinal and allergenic aspects of pumpkin and its seeds is proposed. With the advent of diets rich in omega-3 and omega-6 polyunsaturated fatty acids, edible seeds like pumpkin seed have been incorporated in the modern diet. Their incremental use in the food-processing industry might contribute to an increase in food allergy to pumpkin seed in the future.

[Granulomatous periocular eruption]. / Dermatite périoculaire granulomateuse.

BACKGROUND: Herein, we report a case of atypical periorificial dermatitis in a patient that had been receiving treatment for some time for atopic dermatitis. The specific feature of this rash was its periocular predominance with no perioral involvement, its clinical aspect and its histological picture evocative of sarcoidosis. PATIENTS AND METHODS: A 33-year-old man was being treated for a atopic dermatitis limited to the face and poorly responsive to dermal corticosteroids. Treatment was initiated with topical tacrolimus 0.1%. After 4 years, dependence on this treatment was noted, with daily application being needed to control the lesions. One year later, symmetric lesions were seen on the eyelids and periorbital regions; these were erythematous, micropapular and poorly delineated in a setting of oedema. Biopsy revealed epithelioid granulomatous inflammation, and, to a lesser degree, sarcoidal giant-cell features without caseous necrosis. Staging tests to identify systemic sarcoidosis were negative. Treatment with hydroxychloroquine at 400mg per day and discontinuation of topical tacrolimus resulted in complete remission of the lesions within 2 months. Hydroxychloroquine was discontinued after 6 months, and no relapses had occurred after 2 years of follow-up. DISCUSSION: Three diagnostic hypotheses may be posited for these granulomatous facial lesions. We opted for a diagnosis of granulomatous periorificial dermatitis despite the fact that exclusively periorbital involvement is rare (this condition is generally associated with perioral dermatitis). The second was that of pure cutaneous sarcoidosis, but the topography and clinical appearance of the lesions did not correspond to any of the cutaneous forms classically described. The third was that of tacrolimus-induced granulomatous rosacea, but the histological picture is different. CONCLUSION: The present case underscores the fact that a histological appearance of sarcoidosis on skin biopsy may be associated with perioral dermatitis.

Hereditary angioedema with normal C1 inhibitor and factor XII mutation: a series of 57 patients from the French National Center of Reference for Angioedema.

Hereditary angioedema (HAE) is a rare disease associated with either a quantitative or qualitative deficiency in C1-inhibitor (C1-INH) or normal C1-INH. HAE with normal C1-INH is associated in 20% of cases with mutations in the gene for factor XII (FXII) or FXII-HAE. A recent review described 41 families, including 14 German and 15 Spanish families. We have constructed a register of French patients and their characteristics. A national survey was launched through the French National Center of Reference for Angioedema (CREAK) to study the clinical, biological and therapeutic characteristics of patients with HAE linked to a mutation of FXII gene. Fifty-seven patients were identified from 24 different families. In most cases they were young women (mean age at diagnosis: 31 years, mean age at first symptom: 21 years, female/male ratio: 76%). Twenty-one per cent of the patients experienced angioedema attacks only during pregnancy or when on oestrogen contraception. Sixty-three per cent had attacks at all times, but they were more severe during these same periods. Male carriers of the mutation were more frequently asymptomatic than females (P = 0·003). C1-INH concentrate and icatibant were both effective for treating attacks. The prophylactic use of tranexamic acid led to a 64% decrease in the number of attacks. This is one of the largest series reported of HAE patients with FXII mutation. The therapeutic management appeared to be identical to that of HAE with C1-INH deficiency.

Idiopathic histaminergic angioedema without wheals: a case series of 31 patients.

Idiopathic histaminergic acquired angioedema (IH-AAE) is a common cause of recurrent angioedema without wheals. It is a mast cell-mediated disease thought to belong to the same clinical entity as chronic urticaria (CU). The objective of this study was to describe the clinical and epidemiological characteristics of IH-AAE patients. From 2014 to 2015, 534 patients were seen at our national reference centre for angioedema and/or urticaria. Among them, we identified 31 patients with idiopathic histaminergic acquired angioedema without wheals (IH-AAE). Thirty-one patients (15 men and 16 women) with a mean age of 50 years met the criteria for IH-AAE. The average delay in diagnosis was 6·3 years. A history of allergy was found in 12 patients (38·7%), nine suffering from allergic rhinitis. The mean duration of attacks was 28·1 h. The AE attack was located in the upper respiratory tract in 54·8% of cases (17 patients). A lingual location was found in 29% of patients. Men were more likely than women to have an upper airway involvement. No intubations or admissions to intensive care units were reported. The dosage of anti-histamines to control the symptoms was onefold the recommended dose in 51·6% of patients (16 patients), twofold in 32% (10 patients) and three-fourfold in 16·1% (five patients). IH-AAE is characterized by an important delay in diagnosis, a frequent involvement of the upper airway and a benign course during attacks. As in CU, a trial of up to fourfold dose of H1-anti-histamines may be necessary to control symptoms.

[Lethal Lyell's syndrome induced by fusidic acid]. / Syndrome de Lyell à l'acide fusidique oral d'évolution fatale.

BACKGROUND: Herein, we report the first case of toxic epidermal necrosis due to oral fusidic acid having a fatal outcome. PATIENTS AND METHODS: An 82-year-old woman was referred to our dermatology department for generalized bullous skin eruption. Clinical examination showed fever, oral and ocular ulcerations, and epidermal detachment involving more than 70 % of her body surface area together with a positive Nikolsky sign. Lyell's syndrome was diagnosed. Cutaneous histology showed total epidermal necrosis and a normal dermis. Oral fusidic acid had been prescribed 12 days earlier for a chronic sacral pressure sore. No other treatment had been introduced during the previous two months. The outcome was fatal within 24 hours. DISCUSSION: Fusidic acid is commonly used topically by dermatologists for limited staphylococcal skin infections. Oral treatment is rare and is recommended only for skin, bone or joint infections. This is the first reported case of toxic epidermal necrolysis due to oral fusidic acid. The French national drug safety monitoring register contains only one case in which fusidic acid was a possible culprit. CONCLUSION: Fusidic acid must be considered a potential source of serious cutaneous adverse reactions, particularly toxic epidermal necrolysis.

[Recurrent pyoderma gangrenosum-like ulcers induced by oral anticoagulants]. / Ulcérations récidivantes à type de pyoderma grangrenosum induites par les antivitamines K.

BACKGROUND: Other than the classic skin necrosis induced by oral anticoagulants (OAC) in patients with protein C and S deficiencies, other types of OAC induced-skin ulcers are little known. Herein, we describe an original case of recurrent pyoderma gangrenosum (PG)-like ulcers induced by OAC. PATIENTS AND METHODS: A 70-year-old female heart-transplant recipient presented deep, hyperalgesic and quickly-spreading necrotic ulceration of the right leg 6 weeks after starting oral anticoagulant therapy with fluindione. Histological analysis revealed dermal infiltrate containing polynuclear neutrophils, which accords with the histopathological diagnosis of leukocytoclastic vasculitis or PG. Infectious, autoimmune and thrombophilic causes were ruled out. Fluindione was withdrawn and the ulcer healed completely within a month. Six months later, right leg ulceration recurred two weeks after the patient resumed fluindione but healed within 1 month of discontinuation of the drug. An OAC from another chemical family (warfarin) was then introduced, with further recurrence of ulceration after 2 weeks of treatment. DISCUSSION: The chronology of events and the negativity of aetiological explorations allowed a diagnosis to be made of OAC-induced skin ulcer, a rare complication of which the pathophysiology is unclear. This is the first case of PG-like ulcers induced by OAC.

[Delayed-type hypersensitivity to heparin: diagnosis and therapeutic management]. / Hypersensibilité retardée aux héparines : diagnostic et prise en charge thérapeutique.

Heparin is widely used as an anticoagulant and is indicated in the prevention and treatment of thromboembolic disorders. Heparin-induced delayed-type hypersensitivity presents as eczematous lesions, either at the injection site or generally, and affects 7.5% of patients on heparin. This poses diagnostic and therapeutic issues, since an alternative anticoagulant treatment is essential and the risk of cross-reactivity may be as high as 80%, depending on the type of heparin used. If delayed-type hypersensitivity is suspected, heparin-induced thrombocytopenia must first be ruled out, and heparin should be stopped. Fondaparinux is currently the first-line alternative, with a risk of cross-reactivity estimated at only 10%. The switch from a low-molecular-weight heparin (LMWH) to another LMWH is no longer recommended. The use of unfractionated heparin, danaparoid or hirudin may be warranted in the event of recurrence with fondaparinux, and an immuno-allergological work-up is needed to specify the exact profile of cross-allergies.

Dermoscopy of lentigo maligna melanoma: report of 125 cases.

BACKGROUND: Lentigo maligna melanoma (LMM) is the most common subtype of melanoma on the face. Its presentation may be quite subtle, particularly in early stages, and delayed diagnosis is common. Few dermoscopic studies have been performed and the main dermoscopic features of LMM were defined by Stolz and coworkers in 2000. OBJECTIVES: To investigate classical as well as new dermoscopic features in a large series of LMM in a white-skinned population, in order to evaluate their diagnostic value. METHODS: One hundred and twenty-five consecutive histopathology-proven LMMs were analysed retrospectively based on medical records, clinical and dermoscopic photographs by three independent observers for the presence of 19 predefined criteria. RESULTS: At least one of the classical Stolz criteria was present in 87% of cases (hyperpigmented follicular opening, annular-granular pattern, pigmented rhomboidal structures, obliterated hair follicles). Three original criteria were also present at a relatively high frequency: increased density of the vascular network (58%), red rhomboidal structures (40%), target-like patterns (41%). Darkening at dermoscopic examination (when compared with naked-eye examination) was observed in 25% of lesions. Classical dermoscopic features of extrafacial melanoma (atypical pigment network, irregularly distributed globules, dots, streaks and pseudopods) and vertical growth phase-associated dermoscopic criteria (ulceration, blue papular areas and black structureless areas) were rarely seen. A large number of colours, pigmented rhomboidal structures, obliterated hair follicles and red rhomboidal structures were significantly more frequent in invasive LMMs. In contrast, in situ melanomas were more often associated with one or two colours and few distinctive dermoscopic features. CONCLUSIONS: We present herein, in a large series of LMM, confirmation of the diagnostic value of the classical Stolz dermoscopic criteria and describe four additional original criteria, mainly vascular. A correlation between the presence of some dermoscopic features and thicker tumoral invasion has also been demonstrated.

[Diffuse systemic sclerosis after occupational exposure to trichloroethylene and perchloroethylene]. / Sclérodermie systémique diffuse liée à une exposition professionnelle au trichloroéthylène et perchloréthylène.

BACKGROUND: Diffuse systemic sclerosis (DSS) is an autoimmune disease that is most often endogenous but which can also be induced by exogenous substances of occupational origin. PATIENTS AND METHODS: We report a case of DSS involving prolonged intermittent occupational exposure to solvents (trichloroethylene [TCE] and perchloroethylene [PCE]). The disease was rapidly fatal with cardiac arrest secondary to myocardial fibrosis. DISCUSSION: In the event of exposure to TCE/PCE, we suggest more systematic prevention and diagnosis of DSS.

[Pericarditis and pulmonary embolism. A difficult differential diagnosis and a confusing association]. / Péricardite et embolie pulmonaire. Un diagnostic différentiel difficile, une association déroutante.

The association of pericarditis and pulmonary embolism may be the source of diagnostic error and delay in the administration of anticoagulant therapy. Two cases are reported. Pericarditis occurred late in patients with severe, chronic pulmonary embolism with electrocardiographic changes of acute cor pulmonale. Two physiopathological mechanisms for this association have been proposed. The first, haemodynamic, suggests friction between the pericardium and distended right ventricle and pulmonary artery. The second, an immunological hypothesis, compares the association of pericarditis and pulmonary embolism to that of the Dressler syndrome after myocardial infarction. This assimilation would imply the constitution of an anatomical pulmonary infarction. It is not justifiable to accept this pathogenesis on the evidence of transient pulmonary opacities resulting from intra-alveolar haemorrhage or of linear opacities of pulmonary atelectasis secondary to hypocapnic pneumoconstriction which are radiological signs of anatomo-physiological stages of pre-infarction.