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Acute disseminated epidermal necrosis types 1, 2, and 3: study of sixty cases.

Sixty patients with acute disseminated epidermal necrosis (ADEN) were hospitalized and carefully studied. They included thirty-nine patients with drug-associated Stevens-Johnson syndrome, five patients with drug-associated Lyell's syndrome, and sixteen patients with transitional ADEN. On the basis of growing evidence of an association between erythema multiforme major and drugs and between Lyell's syndrome and drugs, and because of the existence of transitional cases, a unitary hypothesis for this group of cases is proposed. Considering the lack of precise definitions and the confusing current terminology, we define and propose the following terms: ADEN type 1 for drug-associated Stevens-Johnson syndrome, ADEN type 2 for drug-associated transitional cases, and ADEN type 3 for drug-associated toxic epidermal necrolysis, or Lyell's syndrome. The most frequent underlying diseases in our patients were seizures, and the most frequently suspected cause of ADEN was the use of anticonvulsants. All our patients were treated with supportive therapy; none received corticosteroids. The general mortality rate was 15%. The recognition of ADEN type 2 (transitional) has important prognostic and therapeutic implications.

Adolescent↗

Intercellular complement C3 binding in normal skin of patients without pemphigus.

We report here direct immunofluorescence studies of normal skin biopsies that exhibited only complement C3 staining in intercellular areas of epithelium with a view toward evaluating the significance of such findings. During a 5-year period, 11,000 skin biopsy specimens were examined for in vivo binding of immunoglobulins, fibrin, and complement C3 by means of defined direct immunofluorescence methods. Four of ten patients demonstrating intercellular C3 deposits alone had drug-related reactions or erythema multiforme, and four were subsequently shown to have a connective tissue disease. Pemphigus was ruled out in all ten cases in these retrospective studies. These observations indicate that the finding of intercellular C3 deposits in the absence of IgG in normal skin is not a sign of pemphigus but, rather, a sign either of an unusual type of drug reaction or, possibly, of some connective tissue disease. However, the finding of intercellular C3 plus IgG (with or without other immunoglobulins) is a sign of pemphigus. The mechanism of C3 deposition without immunoglobulins is not clear.

Adult↗

Bullous systemic lupus erythematosus: an unusual clinical course and detectable circulating autoantibodies to the epidermolysis bullosa acquisita antigen.

Bullous systemic lupus erythematosus is a newly recognized form of systemic lupus erythematosus characterized by a skin eruption clinically and histologically resembling dermatitis herpetiformis and responsive to dapsone. We report on a patient with bullous systemic lupus erythematosus who initially presented with lesions clinically resembling erythema multiforme, experienced exacerbation of her disease with dapsone, and had detectable circulating autoantibodies to the epidermolysis bullosa acquisita antigen.

Adolescent↗

Mucocutaneous complications of intraarterial 5-bromodeoxyuridine and radiation.

5-Bromodeoxyuridine (BUDR), a halopyrimidine thymidine analogue, is incorporated into the DNA of dividing cells and causes photoradiosensitization. Twenty-five patients with malignant astrocytomas were treated with continuous intracarotid BUDR radiosensitization and radiotherapy for 8 1/2 weeks. Unique dose-limiting mucocutaneous complications were encountered. Ipsilateral facial dermatitis with epilation of eyebrows and eyelashes, ocular irritation, and bilateral nail dystrophy developed in all patients. Less common reactions included oral ulceration in six patients, body exanthem on the trunk in five, and atypical erythema multiforme major in one.

Alopecia↗

Stevens-Johnson syndrome limited to multiple sites of radiation therapy in a patient receiving phenobarbital.

Stevens-Johnson syndrome (SJS) is a severe cutaneous eruption that most often appears as an adverse reaction to a medication. There have been 21 reported cases of atypical erythema multiforme, toxic epidermal necrolysis, and SJS arising in patients receiving radiation therapy in addition to phenytoin, phenobarbital, or carbamazepine. We report the second case of SJS resulting from concomitant phenobarbital and radiation therapy, in which the eruption was limited to the sites of radiation, which were multiple.

Biopsy↗

Acquired immune deficiency syndrome (AIDS) in the United States in 1986: etiology, epidemiology, clinical manifestations, and dental implications.

The acquired immune deficiency syndrome (AIDS) results from a lymphotropic retrovirus (HTLV-III) infection and is characterized by specific opportunistic infections and malignancies. The virus is transmitted primarily by semen and blood. Infection is limited principally to defined risk groups, i.e., homosexual men and intravenous drug users. Head and neck manifestations include cervical lymphadenopathy and Kaposi's sarcoma. Oral manifestations include Kaposi's sarcoma, candidiasis, hairy leukoplakia, precocious periodontal disease, xerostomia, herpes simplex, recurrent aphthae, erythema multiforme, and venereal warts. Although HTLV-III is present in saliva, there are no reported cases of transmission secondary to dental procedures. Appropriate precautions and techniques are recommended in treating patients at risk for AIDS.

Acquired Immunodeficiency Syndrome↗

Cutaneous manifestations of systemic mycoses.

Systemic fungal diseases are primary pulmonary diseases caused by the dimorphic fungal pathogens, Blastomyces dermatitides, Coccidioides immitis. Histoplasma capsulatum, or Paracoccidioides brasiliensis. Infection occurs after inhalation of the infectious form of the fungus and may be acute, self-limited, or subclinical. Primary cutaneous infection occurs only after traumatic implantation of the fungus and is unusual. Erythema nodosum or erythema multiforme may accompany the acute form of the disease. Other cutaneous manifestations represent disseminated disease and, as such, require systemic antifungal therapy. Because cutaneous lesions have occurred coincidentally with other cutaneous pathologies, emphasis should be placed on a complete clinical history, physical examination, and diagnosis by histopathology and culture.

Antifungal Agents↗

[Cutaneous vasculitis revealing a Mycoplasma pneumoniae infection].

INTRODUCTION: Mycoplasma pneumoniae is a bacterial agent that must be evoked when confronted with cutaneous vasculitis, notably within a context of fever and inflammation, and despite the absence of respiratory symptoms. OBSERVATION: A young 16 year-old boy was hospitalised for cutaneous vasculitis with fever but without respiratory symptoms. A recent M. pneumoniae sero-conversion was revealed. DISCUSSION: M. pneumoniae is an intra-cellular pathogen responsible for 20 to 35% of community-acquired pneumonia in adults. The absence of respiratory symptoms in M. pneumoniae infection is not uncommon. Extra-pulmonary complications of M. pneumoniae infections are frequent and varied, notably dermatological. Cutaneous vasculitis associated with M. pneumoniae is seldom found in the medical literature. It is reported as immune-complex -mediated vasculitis or Henoch-Schonlein purpura. Cutaneous eruptions are dominated by maculo-papular rashes and multiform erythema.

Adolescent↗

The effect of herpesvirus infection on the expression of cell adhesion molecules on cultured human dermal microvascular endothelial cells.

Cell-mediated immune response to herpes simplex virus (HSV) may be important in the pathogenesis of herpes keratitis, erythema multiforme or Behcet's disease. We examined whether herpesvirus infection regulates the expression of cell adhesion molecules on cultured human dermal microvascular endothelial cells (HDMEC) and the regulation of T-lymphocytes binding to HDMEC. The expression of intercellular adhesion molecule 1 (ICAM-1), vascular cell adhesion molecule 1 (VCAM-1), or E-selectin on HDMEC increased significantly after treatment with HSV-1, HSV-2, or measles virus on HDMEC. Anti-IL-1 alpha antibody or anti-TNF alpha antibody partially inhibited the expression of ICAM-1, VCAM-1, or E-selectin on HDMEC. The binding of T-lymphocytes to HDMEC increased significantly after the treatment of HSV-1 or measles virus on HDMEC. The binding of T-lymphocytes to HDMEC was significantly inhibited after 16 h of incubation following treatment with anti-ICAM-1 antibody, anti-IL-1 alpha antibody or anti-TNF alpha antibody to HDMEC. These study results suggest that HSV induces the increased expression of ICAM-1, or induction of VCAM-1 and E-selectin on HDMEC and that among these adhesion molecules, the expression of ICAM-1 on HDMEC mainly regulates the binding of T-lymphocytes to HDMEC. The data also suggest that IL-1 alpha or TNF alpha which was produced by HSV infected HDMEC may be related to these events.

Cell Adhesion↗

Diseases associated with photosensitivity.

Photosensitive disorders may be classified as those entirely caused by solar exposure and the photoaggravated disorders. Those in the former category include polymorphic light eruption, juvenile spring eruption, actinic prurigo, hydroa vacciniforme, solar urticaria, also chronic actinic dermatitis. Genodermatoses whose expression mainly depends on UV or light exposure include the DNA repair deficient disorders, some disorders of cornification, the Smith-Lemli-Opitz syndrome and porphyria. Examples of photoaggravated diseases include lupus erythematosus, erythema multiforme, atopic eczema, psoriasis, viral exanthemata, pemphigus, dermatitis herpetiformis and rosacea. Drugs and chemicals may interact with UV to induce photosensitivity. In many of these diseases the action spectrum is known or may be determined by phototesting. Recognition of the reaction patterns associated with the photodermatoses greatly assists clinical classification of the photodermatoses.

Bloom Syndrome↗

Autoimmune progesterone dermatitis and its manifestation as anaphylaxis: a case report and literature review.

OBJECTIVE: Autoimmune progesterone dermatitis is a rare cyclic premenstrual reaction to progesterone produced during the luteal phase of a woman's menstrual cycle with a variety of presentations including erythema multiforme, eczema, urticaria, angioedema, and progesterone-induced anaphylaxis. We present a case of progesterone-induced anaphylaxis and a review of literature focusing on its diagnosis and therapy. DATA SOURCES: We surveyed all the literature in English back to 1921 when the first case was published. First, we researched the terms progesterone anaphylaxis, autoimmune progesterone dermatitis, cyclic urticaria, using the PubMed resource. Then we included articles found within these publications' reference sections. STUDY SELECTION: We selected articles based on whether the cases described appeared to fit the description of the entity autoimmune progesterone dermatitis. All cases included had dermatologic reactions occurring during the luteal phase of the menstrual period, positive skin or intramuscular reactions to progesterone, and treatment amenable to anovulatory agents and/or hysterectomy with bilateral salpingo-oophorectomy. RESULTS: We found approximately 50 published cases of autoimmune progesterone dermatitis, and only nine known cases of its manifestation as anaphylaxis. These cases, including the case described by us, are summarized, and successful diagnostic and therapeutic approaches in the literature are reviewed. CONCLUSIONS: Autoimmune progesterone dermatitis is a rare entity associated with progesterone production of the luteal phase of a woman's menstrual cycle. It can be diagnosed using intradermal or intramuscular progesterone tests and can be treated by disrupting the ovulation cycle using specific medications or by oopherectomy.

Adult↗

Acute generalized exanthematous pustulosis.

Acute generalized exanthematous pustulosis (AGEP) is now a well-known clinical entity, characterized by acute onset with associated fever, and numerous non-follicular pin-head sterile pustules on erythematous background. The biopsy evidences subcorneal pustules resembling those of pustular psoriasis. However, polymorphic aspects such as pseudo-erythema multiforme purpuric lesions, and edema are often associated, and with the rapid self-healing course of this impressive pustulosis, allow the differential diagnosis with pustular psoriasis. Most cases of AGEP are drug induced, particularly by antibiotics and mainly beta-lactams. However, a number of other drugs, of which the list is increasing, may be responsible. Few cases are related to other causative factors such as viral infections or ultraviolet radiation.

Diagnosis, Differential↗

When to request immunofluorescence: practical hints.

This review highlights important considerations in obtaining good skin biopsy specimens to optimize results of direct immunofluorescence (IF) studies and also summarizes the various patterns of cutaneous IF deposition and their associated diagnoses. IF findings of immunobullous diseases, lupus erythematosus, vasculitis, lichen planus, and erythema multiforme are included. The uses of indirect IF studies are also reviewed including newer modifications that are valuable in helping to diagnose epidermolysis bullosa acquisita and paraneoplastic pemphigus.

Basement Membrane↗

Paraneoplastic pemphigus as the presenting symptom of a lymphoma of the tongue.

A patient is described who initially presented with an acrovesicular eczema which subsequently developed into erythema multiforme with histopathological features of bullous pemphigoid. Although the various laboratory studies pointed to the diagnosis of paraneoplastic pemphigus (PNP), the underlying neoplasm was not detected until 6 months later, when the biopsies of an oral lesion showed the presence of an underlying non-Hodgkin lymphoma.

Aged↗

The contribution of meibomian disease to dry eye.

The tear film lipid layer is the major barrier to evaporation from the ocular surface. A decrease in its thickness or functional integrity may cause evaporative dry eye (EDE). Obstructive meibomian gland dysfunction (MGD) is the most common cause of EDE and occurs as a primary disorder or secondary to acne rosacea, seborrheic or atopic dermatitis, and with cicatrizing conjunctival disorders, such as trachoma, erythema multiforme, and cicatricial pemphigoid. MGD may be an incidental finding in asymptomatic eyes, or it may be responsible for irritative lid symptoms in the absence of dry eye. MGD-dependent EDE is diagnosed on the basis of a defined degree of MGD in a symptomatic patient showing typical ocular surface damage in the absence of an aqueous tear deficiency. When MGD occurs in a background of aqueous tear deficiency (ATD), then an additional evaporative component may assumed, depending on the extent of meibomian obstruction. However, definitive criteria are not yet established. The clinical severity of dry eye is greatest when ATD and EDE occur together, particularly in Sjogren syndrome. A hypothesis is proposed to explain the steps leading to primary, simple MGD and subsequent EDE.

Journal Article↗

Allopurinol-induced severe hypersensitivity with acute renal failure.

A 62-year-old male was sent to the emergency room due to a high fever and generalized skin rash after taking allopurinol for 9 days. Physical examination was normal except for the generalized skin rash presenting with erythematous macules. Complete blood count showed leukocytosis with eosinophilia. Blood biochemistry showed impaired renal and hepatic function. Pathologic examination concluded that the skin rash was erythema multiforme. These findings met the diagnostic criteria for allopurinol-induced hypersensitivity syndrome (AHS). Our patient not only had the most common skin lesion but soon developed acute renal failure that required intermittent hemodialysis, despite rapid discontinuation of allopurinol and adequate hydration and steroid therapy. No other causes of acute renal failure were found. Renal impairment was the worst part of the patient's condition and he never completely recovered. AHS should be considered in the differential diagnosis of acute renal and hepatic failure in patients with evidence of allergy and recent use of allopurinol.

Acute Kidney Injury↗

Severe drug rashes in three siblings simultaneously.

BACKGROUND: Adverse reactions to drugs commonly occur sporadically. Certain individuals seem to have an increased susceptibility to develop reactions to multiple drugs. Genetic predisposition has not been elucidated. Our objective was to describe a case of three siblings who developed severe rashes to the same drug simultaneously. METHODS: Review of the patients' medical records for information on the clinical course, and comparing their serum immunoglobulin (Ig) levels initially and during follow-up. RESULTS: The rashes were compatible with erythema multiforme and Stevens-Johnson syndrome and developed in the three siblings within 1-3 weeks after the intake of thiabendazole. Follow-up of serum Ig levels did not show any particular pattern, except for an initial mild to moderate elevation in IgG, IgA and IgM. CONCLUSIONS: The occurrence of such severe cutaneous drug reaction in the three siblings to the same drug suggests a genetic predisposition to adverse drug reactions.

Antinematodal Agents↗