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Erythema elevatum diutinum (EED) is a rare chronic skin disease with cutaneous vasculitis, characterized by the absence of systemic vasculopathy. We describe a 79-year-old white woman who has been followed with the diagnosis of EED for 27 years. Our patient has a previously unreported combination of EED and acro-osteolysis. After reviewing possible reasons for the association, her vasculitis seems the most likely factor.
Necrolytic migratory erythema (NME) is an uncommon condition classically associated with high plasma levels of circulating glucagon and a glucagonoma. We report a patient with cirrhosis who showed clinical and histological features of NME. Investigation revealed normal glucagon levels without evidence of glucagonoma. Serum zinc levels were below the normal range and zinc supplementation resulted in rapid and complete resolution of the eruption.
OBJECTIVE: To clarify the mechanism of epidermal cell death in erythema multiforme. DESIGN: Retrospective study. SETTING: Academic referral center. PARTICIPANTS: Nine patients with Stevens-Johnson syndrome (SJS), 9 patients with Hebra disease (EMH), and 5 healthy volunteers. INTERVENTIONS: Biopsy specimens were obtained from the border of the fresh lesions before treatment. Control specimens were obtained from normal skin. MAIN OUTCOME MEASURES: Histopathological epidermal apoptosis detected with nuclear DNA fragmentation and counts of dermal immunoreactive perforin-positive infiltrates were compared between SJS and EMH. RESULTS: Eight patients (89%) with SJS showed clear apoptosis with keratinocyte DNA fragmentation. All SJS samples had intensive perforin-positive dermal infiltrates. Only 3 patients (33%) with EMH showed apoptotic change, and it was to a far less extent with far less dermal perforin-positive infiltrates. Control specimens showed no apoptotic cells in the epidermis or expression of perforin in the dermis. CONCLUSIONS: Perforin mediates apoptosis in the pathogenesis of the epidermal cell changes in SJS but not in EMH. In addition to the differences in clinical severity and histopathological conditions, our findings indicate a pathogenic difference between SJS and EMH.
A 20-year-old woman developed erythema of the lower legs and swelling of both parotid glands. Her lung function parameters were initially unremarkable, and only discrete pulmonary-hilar changes were visible on the chest X-ray, and acute sarcoidosis was considered as one of the possible differential diagnoses. The paper describes in detail the diagnostic approach and the establishment of the differential diagnosis. The first attempts at therapy with the non-steroidal antiinflammatory drug diclofenac were unsuccessful, but treatment with glucocorticoids that was thereupon tried proved effective.
Four cases of erythema chronicum migrans occurred within a one-month period in southeastern Connecticut. The syndrome may include advancing erythematous rash stemming from an apparent insect bite, hyperesthesias, myalgias, malaise, fever, lymphadenopathy, and, rarely, meningitis. Treatment with penicillin, the tetracycline, or, in our experience, erythromycin usually results in prompt resolution.
OBJECTIVE: In relatively few cases have perinatal factors been included as risk factors for allergy development. Delivery has not been considered as a possible influential factor in allergy development. To identify risk factors in allergy development, we have included erythema toxicum neonatorum (ETN). PATIENTS AND METHODS: We have prospectively studied 356 newborns that were followed for a period of two years. Characteristics of the delivery, such as the pregnancy, instrumental delivery, circular cord, ETN, number of vesicles, day of presentation, season of birth, maternal and cord blood IgE levels and cord blood eosinophils and the development allergies during the two year follow-up period were included. RESULTS: ETN was seen in 25.3% of the children. The histopathology study of vesicles showed eosinophils. There was a significant difference between males and females (61.9% versus 38.1%, respectively). Cord blood IgE levels were not related to ETN, except in situations of allergy from 0.9 IU in cord blood or from 20 IU at six months of age (p < 0.05). CONCLUSIONS: ETN is related to delivery characteristics, instrumental, circulars, amniotic alteration or fall in arterial pH < 7.24. In 84.2% of allergy manifestations during the first two years of life, ETN or a low pH was seen at birth, with atopic dermatitis being those that displayed ETN (85.7%).
"Early onset sarcoidosis" is a chronic granulomatous disease occurring in children younger than 5 years of age, and characterized by a classic symptom triad consisting of skin, eye and joint lesions, with on rare occasion pulmonary involvement. The disorder often goes unrecognized because of its rarity and, since polyarthritis and uveitis are the predominant symptoms, most of these children are misdiagnosed as having juvenile chronic arthritis (JCA). A child with erythema nodosum at 7 months of age, later diagnosed as JCA and definitively recognized as "early onset sarcoidosis" is reported. This case shows that, whenever possible, a biopsy showing the typical picture of sarcoid granulomas is crucial to distinguish these clinical conditions.
A 21-year-old woman who had been taking several kinds of analgesics to treat dysmenorrhea developed episodic attacks of a purpuric macular eruption and a burning sensation on unexposed areas of the upper chest and back where she had sustained severe sunburn eight months earlier. Target-like lesions developed on these areas after intake of Bufferin, a combination of aspirin and dialuminate. After the eruptions had abated following systemic administration of a corticosteroid agent, a challenge test was performed, using a quarter of a tablet of Bufferin. The patient developed a temporary burning sensation and a erythematous color on the previously sunburned skin. We diagnosed this case as a drug eruption due to Bufferin showing erythema exsudativium multiforme with a photo-recall-like phenomenon. In our case, skin tests would be useful to confirm the causal drug.
Cutaneous larva migrans is an intensely pruritic serpiginous eruption caused by the dog or cat hookworm. Often, the disease is self-limiting and no other significant pathology develops; however, a significant localized inflammatory response to the nematode is extremely common. We present a case of cutaneous larva migrans in which a systemic inflammatory process ensued that was characteristic of erythema multiforme. We discuss possible mechanisms of this complication and review the literature.
Erythema multiforme (EM) is a hypersensitivity reaction that occurs mainly after exposure to certain medications or in the setting of infection, most commonly that due to herpes simplex virus. Rare cases of EM have been reported after allergic contact dermatitis due to various substances. There has been one case in the literature of EM following Rhus contact dermatitis. We report three patients who developed EM after allergic contact dermatitis due to poison ivy. In all three patients, targetoid lesions developed primarily on the palms and soles, either after a brief course of prednisone or during its taper. Two of the patients have had more than one episode of EM after poison ivy dermatitis. Although EM has been described after allergic contact dermatitis due to a variety of antigens (nickel being the most common), there is only one report in the literature of EM following Rhus contact dermatitis. Given the prevalence of allergic contact dermatitis due to poison ivy, this may be an under-reported complication.
We describe a patient with a streptococcal myositis/fasciitis and toxic shock syndrome following an intramuscular injection with diclofenac. A patient complaining of sore throat and headaches for two days and fever up to 38.5 degrees C for one day consulted her family physician. 75 mg of diclofenac were injected intramuscularly for symptomatic treatment. On the next day massive pain at the injection site and a generalized erythema occurs and fever up to 38.5 degrees C persists. She is admitted to the local hospital for suspected abscess formation. Despite rapid antibiotic treatment a septic shock develops. The patient is transferred to a tertiary care hospital. An extensive debridement is performed and the antibiotic regimen changed to high dose penicillin and clindamycin. The association of life threatening diseases due to Group A streptococci and non-steroidal anti-inflammatory drugs (NSAID) is well documented by several case reports. We believe there is no longer any need for intramuscular injections of NSAID. The rare but severe complications preclude further use of the intramuscular dosage in view of the availability of oral alternatives.
The differentiation of graft-verus-host disease (GVHD) from erythema multiforme (EM) presents a common diagnostic challenge in skin biopsy specimens from patients who have received patients allogeneic bone marrow transplants. The presence of gastrointestinal involvement might be the only way to make a diagnosis of GVHD in these cases. In the absence of liver function tests, gastrointestinal biopsy, or molecular techniques such as microsatellite DNA analysis, the presence of intraepidermal bile pigment might prove helpful in elucidating hyperbilirubinemia and allowing a pathologist to favor a diagnosis of GVHD over EM. Routinely processed archival tissue from 50 cases of GVHD (42 Caucasian and 8 of unknown race) and 50 cases of EM (31 Caucasian and 19 of unknown race) was examined for pigmentation. Intraepidermal pigmentation was stained for bile pigment and melanin. Among the intraepidermal EM lesions, 4 (8%) stained for intracorneal melanin, but none stained for bile pigment. Among the intraepidermal GVHD lesions, 8 (16%) stained for intracorneal melanin, but 3 (6%) stained for intracorneal bile pigment. In addition, 13 (26%) GVHD lesions and 9 (18%) EM lesions showed melanosis with melanin in all layers of the epidermis as well as within papillary dermal melanophages. Thus, when presented with a differential diagnosis of GVHD versus EM, the presence of intraepidermal bile pigment might suggest liver involvement and a diagnosis of GVHD.
The dermatologic disorders in polyglandular autoimmune disease (PGAD) type 1 (previously called chronic mucocutaneous candidiasis) are nail dystrophy, vitiligo, and alopecia. A patient with PGAD and erythema annulare centrifugum (EAC) is presented. This association has not been reported previously. EAC may be related to occult or low-grade Candida infection in PGAD.
A 36-year-old Caucasian woman arrives in your office, her chief complaint being vulvar pain and discomfort of 2 years' duration. She says that the onset of vulvar erythema and pain was gradual, beginning with mild tingling discomfort and eventually developing into dyspareunia and pain whenever she attempted to insert tampons, wore tight-fitting pants, or went horseback riding. The patient denies having vaginal discharge, change in menstrual pattern, or dysuria. She says she complained of the problem to several previous clinicians, who recommended anti-infective treatment for candidal vulvovaginitis, bacterial vaginosis, and recurrent cystitis, yet the symptoms persisted. One physician suggested a psychiatric evaluation, but the patient refused. She switched soap, shampoo, and even underwear fabric in an unsuccessful effort to ameliorate the symptoms. Over the past 6 months she has become increasingly frustrated with this problem.
Necrolytic migratory erythema (NME) is generally associated with glucagonoma. It waxes and wanes by successive relapses and remissions. The clinical and microscopical diagnosis is complex. In addition to glucagonoma treatments, the administration of corticoids, aminoacids, zinc or essential fatty acids can be helpful. There exist several etiological hypotheses for NME. These are based on modifications of pancreatic enzyme activities and on variations of aminoacids, fatty acids, zinc or glucagon concentrations.
Reported herein are 13 borderline lepromatous (BL) or subpolar lepromatous (LLs) patients who presented with or developed delayed-type hypersensitivity (DTH) reactions after initiation of antibacterial therapy, but who subsequently developed erythema nodosum leprosum (ENL), the DTH to ENL group. During the same time, three LLs patients had ENL followed by relapse-associated DTH, a significant (p < 0.05) difference in sequence of the two conditions. The DTH to ENL group had statistically significant higher biopsy indexes at the time of diagnosis of the DTH reaction compared with two DTH control groups, 7 multibacillary patients presenting with DTH reactions and 15 BL or LLs who developed DTH reactions after starting treatment but had no ENL. DTH-associated histologic changes were less well developed in the DTH to ENL group than in either of the two control groups. In the DTH to ENL group, 77% required prednisone in addition to thalidomide to achieve a complete remission in contrast to only 10% of 21 ENL clinical controls. In the DTH to ENL group, the classical histologic ENL pattern was present in only 31% of these patients, in contrast to 88% of 33 ENL histologic controls. In 9 of 9 of the DTH to ENL patients studied, after the ENL remitted, Mycobacterium leprae-sonicate-stimulated lymphocyte transformation tests gave stimulation indexes within the range of our tuberculoid (TT) and borderline tuberculoid (BT) patients, in contrast to absent responses in 6 ordinary, longterm-treated patients who had had ENL.
The clinical histories of twenty children hospitalized for erythema multiforme (EM) were reviewed. Three forms of the illness were defined according to clinical criteria: minor (EMm), intermediate (EMt) and major (EMM). Previous infections were associated with the process in 30% of the cases, administration of drugs also in 30%, and a combination of the two factors in 30% of the subjects. No specific etiology was found in two cases (10%). Respiratory tract symptoms and general discomfort preceded the EMM form, and evidence of digestive organ involvement was frequent in the EMm form. Erythrocyte sedimentation rate was usually high. Postinflammatory sequelae occurred in only two cases, and one subject died in the first 24 hours.
The aim of the study was to evaluate erythema migrans (EM) prevalence and treatment in north-eastern Poland during the last 25 years. The material for the study consisted of data sent to the Department of Dermatology by physicians from fifteen regional out-patient clinics. They were collected according to specially prepared questionnaires. The data were analysed with respect to the total number of EM cases, patients' age, seasonal prevalence of the disease, dynamics of its prevalence and treatment methods in successive years. Of the 262,421 case histories, retrospectively analysed 1235 EM cases (0.4%) were found. The age of the patients varied from 4 to 78 years. The highest incidence was observed between August and October (57.7% of all cases). The annual number of cases increased from only a few, in the years 1969-76, to over 436 in 1993. Penicillin or erythromycin were preferred for the treatment of EM before 1981, tetracycline or ampicillin between 1982-90, and amoxicillin or doxycycline in the last three years. Our results showed a significant increase in the number of EM cases in the dermatological clinics of north-eastern Poland in recent years. The treatment of the disease applied at present, complies with the generally accepted guidelines.