Wells syndrome following vaccination: A pediatric case with positive patch test to gelatin.

We report a 12-month-old boy with a skin eruption that developed 15 days after receiving the measles, mumps, rubella (MMR), pneumococcal, and meningococcal vaccines, consistent with the diagnosis of Wells syndrome. Patch testing showed a positive reaction to gelatin, which is used as a stabilizer for both live and inactivated vaccines. Gelatin was only present in the MMR vaccine.

Medication-Related Osteonecrosis of the Jaw in a Patient with Nonuremic Calciphylaxis.

A 62-year-old man was referred to the emergency department of our hospital with pain and swelling in the left mandibular region that had evolved over the previous 2 months (Figure 1). His medical history included nonuremic calciphylaxis and systemic sclerosis (Figure 2). Since the diagnosis of the nonuremic calciphylaxis 5 years before, the patient had been treated with intravenous (IV) sodium pamidronate 60 mg per week for 11 months, without improvement, followed by IV sodium thiosulfate 25 mg twice a week for 18 month. During year 3 of treatment, the calciphylaxis lesions reappeared, and IV sodium pamidronate 60 mg per week was reintroduced to the patient's treatment. The patient remained with double treatment for the next 2 years, but 3 months before the patient's presentation, the IV treatment had been suspended due to an absence of peripheral venous access.

Nonuremic Calciphylaxis: Four Cases Associated with Autoimmune Diseases.

Calciphylaxis is a rare and severe syndrome of vascular calcification with unclear pathogenesis. This disease mainly affects patients with end-stage renal disease; however, it also has been described in nonuremic patients. We report four cases of nonuremic calciphylaxis (NUC) with ulcerated lesions associated with autoimmune disease. These cases support the literature suggesting that bisphosphonates and sodium thiosulfate are effective drugs in the treatment of calciphylaxis regardless of renal function.

Piebaldism and neurofibromatosis: state of knowledge.

Despite progress in understanding the molecular basis, the diagnosis of neurofibromatosis 1 (NF 1) is based on clinical criteria, established by the National Institute of Health (NIH) Consensus Conference in 1987. The association of NF1 and piebaldism has been reported, but some authors disagree with this co-occurrence. In the light of present knowledge, we highlight that both entities might co-exist in the same patient.

Recurrent Wells' syndrome associated with allergic asthma exacerbation.

Wells syndrome is an inflammatory eosinophilic dermatosis of unknown pathogenesis characterized by clinical polymorphism, a suggestive but nonspecific histopathologic traits, usually with a recurrent course and inconstant response to therapy. It seems to be an unspecific hypersensitivity reaction in response to various exogenous and endogenous stimuli, such as insect bites, infections, drug eruption or underlying internal disorders. We present a patient with allergic asthma and atopic dermatitis in whom a skin eruption developed in the sequence of allergic asthma exacerbation, which was clinically and histologically consistent with the diagnosis of eosinophilic cellulitis. The authors discuss the probability of a common pathogenesis and the role of IL-5. To our best knowledge this is the first pediatric case where this association is reported.

Erythropoietic protoporphyria: a family study and report of a novel mutation in the FECH gene.

Erythropoietic protoporphyria (EPP) is a rare inherited disorder of heme biosynthesis mostly caused by a deficient activity of the enzyme ferrochelatase (FECH), and consequent accumulation of protoporphyrin (PP) in various tissues. Clinical manifestations include a childhood onset, cutaneous photosensitivity and, sometimes, hepatobiliary disease. We report a 16-year-old male with EPP characterized by acute episodes of painful photosensitivity since early infancy, permanent changes in the photoexposed skin, microcytic anemia, thrombocytopenia, and mild hepatic dysfunction. His 18-year-old sister presented less acute symptoms with no chronic changes. Lesional biopsy disclosed perivascular deposition of PAS positive hyaline material. Rimington-Cripps test was positive and PP erythrocyte levels were >9,000 µg/L (N<1,600), but normal in their parents and younger brother. Genetic studies in both patients and their mother revealed heterozygosity for a novel mutation (c.1052delA) in FECH gene of both children, and heterozygosity for the hypomorphic allele IVS3-48T>C in all of them. This confirms the "pseudodominant" inheritance pattern usually observed, explained by the combined presence of a disabling FECH mutation and a common intronic polymorphism affecting the counterpart allele (IVS3-48T>C). Phenotypic heterogeneity for this genotype explains the divergent clinical presentation. This is the first description of a Portuguese family with EPP characterized at the molecular level.

Herp zoster at the site of infliximab infusion: case report.

Worldwide, many patients have been treated with tumor necrosis factor-α (TNF-α) antagonists for indications that include chronic inflammatory diseases such as rheumatoid and psoriatic arthritis, inflammatory bowel disease and others. Since their approval, concerns regarding safety have been raised. Increased susceptibility to bacterial infections, especially due to intracellular bacteria like Mycobacterium tuberculosis that is responsible for the most serious complications associated with this treatment. Viral infections are less frequently reported but probably relatively common, representing an important cause of morbidity to remember. Varicella zoster virus is one of the most frequently implicated viruses. We present the case of a 20-year-old man with Crohn's disease under infliximab treatment who developed herpes zoster at the site of infliximab's 7th and 9th infusion.

Severe onychodystrophy caused by allergic contact dermatitis to acrylates in artificial nails.

Acrylic resin monomers, especially acrylates and methacrylates, are well-known sensitizers responsible for allergic contact dermatitis mainly in the occupational setting. The most frequently affected professionals are dentists, orthopedic surgeons, manicurists, painting industry and fiberglass workers. The authors report the case of a 39-year-old healthy woman, a secretary, who developed severe onychodystrophy of all fingers, 1 week after the application of sculptured acrylic nails.

Antineutrophil cytoplasmic antibody (ANCA)-positive cutaneous leukocytoclastic vasculitis induced by propylthiouracil confirmed by positive patch test: a case report and review of the literature.

A 43-year-old female with antiphospholipid syndrome and Graves' disease developed a cutaneous leukocytoclastic vasculitis associated with antineutrophil cytoplasmic antibody (ANCA) against myeloperoxidase (MPO-ANCA) and proteinase-3 (PR3-ANCA), whilst treated with propylthiouracil (PTU). The skin lesions were progressively resolved after withdrawal of PTU and treatment with oral steroids. Patch testing with PTU at 1%, 5%, and 10% in petrolatum was positive at 48 h. Despite positive ANCA titers after 1 year of follow-up, the patient maintains complete clinical remission. PTU is a common antithyroid drug, which has been known to induce ANCA-positive vasculitis. Although most patients with this rare side effect have a good outcome, some fatal cases have been reported. Therefore, patients treated with PTU should be carefully followed and monitored, not only for their thyroid state but also for early detection of potential serious complications of this drug. Early diagnosis and prompt cessation of PTU therapy are essential to improve the outcome. Also key aspects of PTU-induced ANCA-positive vasculitis are reviewed.

[Uncommon causes of leg ulcers: investigative approach and therapeutics]. / [In Process Citation]

Chronic wounds of the lower limbs are a major public healthcare problem affecting 1 percent of the adult population and 3 to 5 percent of people older than 65 years. These numbers are rising in the western population as a result of increased life expectancy and increased risk factors for atherosclerotic occlusion, such as smoking, obesity, and diabetes mellitus. This very debilitating condition, which reduces significantly the quality of life, causes social discomfort and generates considerable cost, not only to the patient but also to the society. Treating chronic leg ulcers is always a challenge. Over the last years, the treatment of this condition has been given more attention because of the frequent ineffectiveness of the methods used, the awareness of the psychological and social impact, and the recognition of the risks of malignancy in these wounds. Determining the etiology is an essential factor for directed and more effective wound care. Although 90 percent of ulcers are of vascular origin, the list of other possible causes responsible for the other 10 percent is extremely long. In this review, the authors focus on the differential diagnosis of chronic leg ulcers and the impact of directed treatment in the prognosis of this condition.

Diffuse Large B-Cell Lymphoma in the Setting of Chronic Lymphocytic Leukemia.

A 77-year-old man was referred to our department with a 6-month history of a painless, rapidly enlarging tumor on the left leg. Fourteen years ago, he was diagnosed with chronic lymphocytic leukemia (CLL), and he had presented with lymphocytosis and multiple lymphadenopathies (Rai stage II). Both bone marrow aspiration and peripheral blood immunophenotyping identified a monoclonal B-cell population expressing surface CD5, CD20, CD23, CD43, and IgG kappa. At that time, he underwent eight courses of treatment with oral fludarabine, followed by complete remission, and he had been clinically stable ever since (Rai stage 0).

Leg ulcer and thigh telangiectasia associated with natural killer cell CD56(-) large granular lymphocyte leukemia in a patient with pseudo-Felty syndrome.

Clonal disorders of large granular lymphocytes (LGL) represent a rare spectrum of biologically distinct lymphoproliferative diseases originating either from mature T cells or natural killer cells. Both subtypes can manifest as indolent or aggressive disorders. We report a 77-year-old woman with rheumatoid arthritis, splenomegaly, and neutropenia who developed a painful leg ulcer refractory to treatment and thigh telangiectatic lesions. Because of the association of rheumatoid arthritis, splenomegaly, and nonspecific neutropenia, the diagnosis of Felty syndrome was initially made. Further investigation allowed the diagnosis of a CD56(-) natural killer-cell LGL leukemia and documented skin infiltration by natural killer cells. Cutaneous manifestations of LGL leukemia have been rarely reported. This report of pseudo-Felty syndrome with CD56(-) LGL leukemia, presenting with a leg ulcer and telangiectasia, enhances the role of dermatology in the diagnosis of hematologic neoplasia.

Docetaxel-induced acral erythema and nail changes distributed to photoexposed areas.

Docetaxel is a semisynthetic taxane used in the treatment of several malignancies. A 60-year-old male patient suffering from a non-small cell lung cancer with bone metastasis was under treatment with docetaxel and developed acral erythema involving photoexposed areas. Onycholysis and melanonychia were also observed. Drug-induced acral erythema usually involves the palms and soles, but it may occur in more atypical areas such as the backs of the hands and feet and also the face, as occurred in this patient. The nail changes are usually observed after several cycles of docetaxel, but tend to resolve gradually over several weeks despite continued treatment. Early recognition of skin and nail changes in the setting of docetaxel treatment, especially with an unusual presentation, is of paramount importance not only for patient reassurance, but also to avoid unnecessary withdrawal of drug treatment.

Nonpigmented fixed drug eruption induced by esomeprazole.

A 56-year-old white woman developed a distinctive skin eruption over her mammary, lumbosacral, and pubic areas 2 weeks after the start of esomeprazole therapy for dyspeptic symptoms. Skin biopsy disclosed a spongiotic dermatitis with predominantly lymphocytic dermal infiltrate. Treatment with a tapering dose of corticosteroid and withdrawal of the suspected drug led to a rapid resolution of the eruption without residual dyschromia. Patch testing with esomeprazole 2% in petrolatum was negative at 48 and 72 hours but became positive on day 6. Oral-controlled provocation test induced the reappearance of the lesions over the mammary areas, confirming the putative involvement of this drug. Therefore, the patient was diagnosed as having a nonpigmented fixed drug eruption associated with esomeprazole. This compound is a proton-pump inhibitor developed as the S-isomer of omeprazole to improve its pharmacokinetic properties. Reports of cutaneous reactions to proton-pump inhibitors are quite common, but reports of such reactions to esomeprazole are rare, which demonstrates the need for higher clinical awareness and knowledge of reactions to these drugs.

Porphyria cutanea tarda induced by tamoxifen.

Porphyria cutanea tarda (PCT) results from a decrease in the activity of uroporphyrinogen decarboxylase. In the sporadic form, the decrease in the activity is restricted to the liver and is generally related to alcohol, estrogens, iron overload, hepatitis C infection, and halogenated aromatic hydrocarbons. We describe the development of porphyria cutanea tarda in a 53-year-old woman one year after breast cancer surgery and the initiation of treatment with tamoxifen. No additional drugs were prescribed. After tamoxifen was discontinued, a gradual clinical and laboratorial improvement was noticed suggesting a causative role of the drug. There are many reports discussing tamoxifen side-effects, but there are only three case reports in the literature that describe tamoxifen as a probable trigger of porphyria cutanea tarda. In this report, the potential porphyrinogenicity of tamoxifen and clinical implications are the targets of our discussion.

[A cutaneous infection by Mycobacterium chelonae in a patient with rheumatoid arthritis]. / Infecção cutânea por Mycobacterium chelonae numa doente com artrite reumatóide.

There are no pathognomonic findings for cutaneous infection caused by Mycobacterium chelonae. The type and duration of therapy varies considerably among reports and no single antibiotic is considered the treatment of choice. A 61-year-old patient, suffering from rheumatoid arthritis (treated with metotrexate and salazopyrine), presented with violaceous nodules of the right leg that had been evolving for 6 months. She was underwent several skin biopsies. Tissue culture of the last showed an atypical mycobacteria, identified as M. chelonae. Despite improvement after a two-week course of treatment with clarithromycin, a switch to ciprofloxacin was made because of gastrointestinal intolerance. After 3 months, only slight improvement of the lesions was achieved and clarithromycin was reintroduced; significant clinical improvement occurred by the third month. Clarithromycin was continued a further two months until the patient quit on her own and. no recurrence was observed. Infections caused by M. chelonae frequently occur in the setting of immunological impairment. Contaminated water is the natural reservoir, but we were unable to establish the source of contamination. As was previously described, there was a significant delay between clinical presentation and diagnosis. Thus, a high index of suspicion and multiple biopsies with culture are of paramount importance to confirming the diagnosis.

Piebaldism and neurofibromatosis type 1: family report.

Piebaldism is a rare disorder present at birth and inherited as an autosomal dominant trait. It results from a mutation in the c-kit proto-oncogene and is associated with a defect in the migration and differentiation of melanoblasts from the neural crest. Clinical manifestations and phenotypic severity strongly correlates with the site of mutation within the KIT gene. Here we report a 3-year-old boy and his 33-year-old father with leukoderma and poliosis associated with clinical criteria for Neurofibromatosis type 1. Genetic study of both revealed a p.Gly610Asp mutation in the KIT gene. This familiar mutation has not yet been reported in the literature. There are rare reports of piebaldism in association with neurofibromatosis type I.

Unilateral laterothoracic exanthem and primary Epstein-Barr virus infection: case report.

Unilateral laterothoracic exanthem is a self-limited disease that occurs most commonly in children. It is characterized by unilateral exanthem, often in axillary region. The etiology is unknown, but a viral agent is suspected. We report a 1-year-old white girl with unilateral laterothoracic exanthem associated with Epstein Barr virus infection, suggesting this virus has a possible etiologic role.

Epidermolytic hyperkeratosis with palmoplantar keratoderma in a patient with KRT10 mutation.

We report the case of a 12-year-old girl presenting at birth with erythroderma, erosions and blisters scattered over the integument. By the age of 3 she presented generalized hyperkeratotic plaques with a cobblestone pattern and a pungent odour, most prominently around flexures, scalp and palmoplantar areas. Clinical, histological and ultrastructural findings confirmed the diagnosis of epidermolytic hyperkeratosis (EHK). Molecular genetic analysis revealed a mutation in the KRT10 gene. Treatment with oral acitretin was attempted but it was discontinued due to hepatic dysfunction and marked desquamation and blistering. EHK is a rare autosomal dominant disorder of keratinization, caused by mutations in either the KRT1 or KRT10 genes. Although palmoplantar keratoderma is typically found in patients with KRT1 mutation, our patient presents EHK with palmoplantar involvement and KRT10 mutation. Moreover, a poor response to systemic retinoids was observed, contrary to what is expected in patients with KRT10 mutation. Even though management is usually unsatisfactory, some patients with this lifelong and serious condition may experience improvement with age.