Search

Pellagra is a nutritional disease caused by the deficiency of niacin. It is a clinical syndrome characterized by four "D's": diarrhea, dermatitis, dementia and ultimately death. We describe a case of pellagra as the initial presentation of congenital duodenal diaphragm., (Copyright © 2012. Published by Elsevier Masson SAS.)

The old saying, 'a stitch in time saves nine' is particularly true in the management of eczema. Early diagnosis and the recognition of an underlying cause can mean that more simple measures, such as moisturizers, may be sufficient to keep eczema under control, while the identification of an allergic stimulus can forestall further problems. Equally, being aware of what action to take when a course of treatment is ineffective, and having the ability to teach parents and families to realize when they need extra help, may allow changes to be made that will restore control of the condition more quickly. An understanding and empathetic ear may make all the difference when a patient is having to come to terms with eczema. This article discusses the aetiology and symptoms of different types of eczema, and summarises the range of available options for the management of this often disruptive condition.

The most common clinical presentations of hand eczema are atopic hand dermatitis, pompholyx, and contact dermatitis (irritant contact dermatitis [ICD], allergic contact dermatitis [ACD]). The diagnosis of hand dermatitis is determined by a review of the patient's medical history, a physical examination including other body sites as well as the hands, and a thorough overview of the patient's daily activities with emphasis on occupation and hobbies. Irritant contact dermatitis usually is diagnosed by the absence of a positive patch test result; however, patch testing is essential in confirming a clinical diagnosis of ACD by identifying the allergens to which the patient has been sensitized. Treatment includes topical and/or systemic corticosteroids to reduce inflammation and ceramide-containing moisturizers to repair the skin's barrier function. Topical calcineurin inhibitors may be alternatives to topical corticosteroids. The most important step in the management of hand eczema is prevention with physical protective products (e.g., gloves) or barrier protection creams.

It has been suggested that chronic eczematous eruptions of the elderly could be associated with chronic drug exposure. To determine the drugs associated with these eruptions, we conducted a case-control study on 102 cases and 204 controls. Cases were consecutive patients older than 60 years presenting with an eczematous eruption that had evolved continuously or recurrently for more than 3 months without a reliable cause. Two controls were matched to each case on age, sex, in/outpatient origin, and center. Information about drug exposure was obtained from patients and their pharmacists. Drug use for more than 3 months within the year preceding the eruption was compared between cases and controls. An association was found between calcium channel blockers (CCB) and eczema, with a matched OR (odds ratio) of 2.5 (95% CI (confidence interval): 1.3-4.6). To ascertain the course of patients after CCB withdrawal, two ancillary studies were performed on 74 patients with eczematous eruptions from our department before the case-control study period, and on 101 patients registered in the French "Pharmacovigilance" database. Healing of these eruptions after CCB withdrawal occurred in 83 and 68% of these cases, respectively. The long-term use of CCB is a risk factor for chronic eczematous eruptions of the elderly.

The skin, as one of the most important barriers of the human body, protects the inner homeostasis from the harmful environmental influences as well as physical, chemical and biological factors. When the impact of these factors exceeds the tolerance and reproducing capacity of the skin, pathological alterations will develop. If follows from this that dermatology can surely be considered to be a part of environmental medicine. Eczematous diseases are mostly pathological pictures of varied mechanisms developing as a result of environmental influences (irritants, contact allergens, microbes). Since their clinical appearance is similar, it is a serious professional challenge to diagnose them. In this article we present the clinical features, provoking factors of these skin diseases as well as therapeutical possibilities.

The guidance in this report is for evaluation and treatment of patients with complications from smallpox vaccination in the preoutbreak setting. Information is also included related to reporting adverse events and seeking specialized consultation and therapies for these events. The frequencies of smallpox vaccine-associated adverse events were identified in studies of the 1960s. Because of the unknown prevalence of risk factors among today's population, precise predictions of adverse reaction rates after smallpox vaccination are unavailable. The majority of adverse events are minor, but the less-frequent serious adverse reactions require immediate evaluation for diagnosis and treatment. Agents for treatment of certain vaccine-associated severe adverse reactions are vaccinia immune globulin (VIG), the first-line therapy, and cidofovir, the second-line therapy. These agents will be available under Investigational New Drug (IND) protocols from CDC and the U.S. Department of Defense (DoD). Smallpox vaccination in the preoutbreak setting is contraindicated for persons who have the following conditions or have a close contact with the following conditions: 1) a history of atopic dermatitis (commonly referred to as eczema), irrespective of disease severity or activity; 2) active acute, chronic, or exfoliative skin conditions that disrupt the epidermis; 3) pregnant women or women who desire to become pregnant in the 28 days after vaccination; and 4) persons who are immunocompromised as a result of human immunodeficiency virus or acquired immunodeficiency syndrome, autoimmune conditions, cancer, radiation treatment, immunosuppressive medications, or other immunodeficiencies. Additional contraindications that apply only to vaccination candidates but do not include their close contacts are persons with smallpox vaccine-component allergies, women who are breastfeeding, those taking topical ocular steroid medications, those with moderate-to-severe intercurrent illness, and persons aged < 18 years. In addition, history of Darier disease is a contraindication in a potential vaccinee and a contraindication if a household contact has active disease. In the event of a smallpox outbreak, outbreak-specific guidance will be disseminated by CDC regarding populations to be vaccinated and specific contraindications to vaccination. Vaccinia can be transmitted from a vaccinee's unhealed vaccination site to other persons by close contact and can lead to the same adverse events as in the vaccinee. To avoid transmission of vaccinia virus (found in the smallpox vaccine) from vaccinees to their close contacts, vaccinees should wash their hands with warm soapy water or hand rubs containing > or = 60% alcohol immediately after they touch their vaccination site or change their vaccination site bandages. Used bandages should be placed in sealed plastic bags and can be disposed of in household trash. Smallpox vaccine adverse reactions are diagnosed on the basis of clinical examination and history, and certain reactions can be managed by observation and supportive care. Adverse reactions that are usually self-limited include fever, headache, fatigue, myalgia, chills, local skin reactions, nonspecific rashes, erythema multiforme, lymphadenopathy, and pain at the vaccination site. Other reactions are most often diagnosed through a complete history and physical and might require additional therapies (e.g., VIG, a first-line therapy and cidofovir, a second-line therapy). Adverse reactions that might require further evaluation or therapy include inadvertent inoculation, generalized vaccinia (GV), eczema vaccinatum (EV), progressive vaccinia (PV), postvaccinial central nervous system disease, and fetal vaccinia. Inadvertent inoculation occurs when vaccinia virus is transferred from a vaccination site to a second location on the vaccinee or to a close contact. Usually, this condition is self-limited and no additional care is needed. Inoculations of the eye and eyelid require evaluation by an ophthalmologist and might require therapy with topical antiviral or antibacterial medications, VIG, or topical steroids. GV is characterized by a disseminated maculopapular or vesicular rash, frequently on an erythematous base, which usually occurs 6-9 days after first-time vaccination. This condition is usually self-limited and benign, although treatment with VIG might be required when the patient is systemically ill or found to have an underlying immunocompromising condition. Infection-control precautions should be used to prevent secondary transmission and nosocomial infection. EV occurs among persons with a history of atopic dermatitis (eczema), irrespective of disease severity or activity, and is a localized or generalized papular, vesicular, or pustular rash, which can occur anywhere on the body, with a predilection for areas of previous atopic dermatitis lesions. Patients with EV are often systemically ill and usually require VIG. Infection-control precautions should be used to prevent secondary transmission and nosocomial infection. PV is a rare, severe, and often fatal complication among persons with immunodeficiencies, characterized by painless progressive necrosis at the vaccination site with or without metastases to distant sites (e.g., skin, bones, and other viscera). This disease carries a high mortality rate, and management of PV should include aggressive therapy with VIG, intensive monitoring, and tertiary-level supportive care. Anecdotal experience suggests that, despite treatment with VIG, persons with cell-mediated immune deficits have a poorer prognosis than those with humoral deficits. Infection-control precautions should be used to prevent secondary transmission and nosocomial infection. Central nervous system disease, which includes postvaccinial encephalopathy (PVE) and postvaccinial encephalomyelitis (or encephalitis) (PVEM), occur after smallpox vaccination. PVE is most common among infants aged < 12 months. Clinical symptoms of central nervous system disease indicate cerebral or cerebellar dysfunction with headache, fever, vomiting, altered mental status, lethargy, seizures, and coma. PVE and PVEM are not believed to be a result of replicating vaccinia virus and are diagnoses of exclusion. Although no specific therapy exists for PVE or PVEM, supportive care, anticonvulsants, and intensive care might be required. Fetal vaccinia, resulting from vaccinial transmission from mother to fetus, is a rare, but serious, complication of smallpox vaccination during pregnancy or shortly before conception. It is manifested by skin lesions and organ involvement, and often results in fetal or neonatal death. No known reliable intrauterine diagnostic test is available to confirm fetal infection. Given the rarity of congenital vaccinia among live-born infants, vaccination during pregnancy should not ordinarily be a reason to consider termination of pregnancy. No known indication exists for routine, prophylactic use of VIG in an unintentionally vaccinated pregnant woman; however, VIG should not be withheld if a pregnant woman develops a condition where VIG is needed. Other less-common adverse events after smallpox vaccination have been reported to occur in temporal association with smallpox vaccination, but causality has not been established. Prophylactic treatment with VIG is not recommended for persons or close contacts with contraindications to smallpox vaccination who are inadvertently inoculated or exposed. These persons should be followed closely for early recognition of adverse reactions that might develop, and clinicians are encouraged to enroll these persons in the CDC registry by calling the Clinician Information Line at 877-554-4625. To request clinical consultation and IND therapies for vaccinia-related adverse reactions for civilians, contact your state health department or CDC's Clinician Information Line (877-554-4625). Clinical evaluation tools are available at http.//www.bt.cdc.gov/agent/smallpox/vaccination/clineval. Clinical specimen-collection guidance is available at http://www.bt.cdc.gov/agent/smallpox/vaccination/vaccinia-specimen-collection.asp. Physicians at military medical facilities can request VIG or cidofovir by calling the U.S. Army Medical Research Institute of Infectious Diseases (USAMRIID) at 301-619-2257 or 888-USA-RIID.

Splenomegaly, when present as a manifestation of sarcoidosis, may cause pressure symptoms and hypersplenism. Literature reports of giant splenomegaly are few in number and describe the use of aggressive intervention with systemic steroids and splenectomy. However, the natural history of splenic sarcoidosis is not well-defined, particularly the potential for spontaneous remission as seen in pulmonary sarcoidosis. In the case described here, a spontaneous remission of giant splenomegaly from sarcoidosis was observed. Careful follow-up appears to be appropriate management for at least some of these patients.

Background: Recurrent cutaneous T-cell lymphoma (CTCL) is managed with a variety of modalities. Repeat treatment with additional courses of total skin electron beam therapy (TSEBT) has not been formally evaluated., Objective: Our purpose was to evaluate the efficacy and toxicity of additional TSEBT for recurrent CTCL., Methods: A total of 14 patients were treated with TSEBT and received at least two courses, with five of those patients receiving a third course. Patients were offered additional TSEBT if they suffered recurrence despite other therapy if the extent of the recurrence precluded localized radiation. The median follow-up was 36 months., Results: The median dose for the entire group was 57 Gy. Thirteen patients (93%) achieved a complete response (CR) after the initial course. After the second course, 12 patients (86%) had a CR; of the five patients who underwent a third course, three (60%) achieved a CR. The median disease-free interval after the first course of therapy for those with a CR was 20 months and 11.5 months after the second course. Median survival after the second course was 15 months. All patients had xerosis, pruritus, desquamation, mild erythema, epilation, and anhidrosis of the skin., Conclusion: Patients with recurrent CTCL recalcitrant to other forms of therapy or too diffuse for treatment with localized radiation fields are candidates for additional TSEBT. This therapy is effective and well tolerated with an acceptable risk profile.