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A 35-year-old woman developed an orf infection of the fingers after contact with an infected goat. Following the primary orf infection, erythema multiforme developed and was controlled with prednisone. The association between erythema multiforme and orf is discussed; also, it is noted that orf is another viral condition that should be added to the agents that can initiate erythema multiforme.
Erythematous, scaling patches over the face, torso, and extremities developed in a 2-month-old female infant. The facial lesions enlarged and became confluent, while the torso and leg lesions enlarged and assumed an annular configuration. Spores of Pityrosporum ovale and Pityrosporum orbiculare were found in large numbers in potassium hydroxide preparations from these lesions, a finding confirmed by culture. Clotrimazole ointment was applied, and the lesions resolved within one week, leaving depigmentation. The findings in this case and those of erythema gyratum atrophicans transiens neonatale resembled each other except for the demonstration of Pityrosporum spores. We assume that the disorder in our patient may represent superficial gyrate erythema (erythema annulare centrifugum) induced by Pityrosporum spores.
Erythema dyschromicum perstans (EDP) is a cutaneous pigmentary disturbance originally considered to be a variant of erythema perstans. The nosologic identity of EDP has been challenged repeatedly as the number of patients with concurrent EDP-like eruptions and lichenoid disorders has increased. In this report, we describe a woman who had EDP for two years before the onset of classic lichen planus (LP). The active lesions of LP gradually evolved into typical ashy-gray macules of EDP. This case provides further support for the concept that EDP and EDP-like conditions should be classified, in certain instances, as erythema dyschromicum variants of LP.
A tender diffuse erythema developed in a 47-year-old man who had psoriasis when he was treated with either suberythemogenic oral methoxsalen photochemotherapy or ultraviolet (UV)-B phototherapy and intramuscular methotrexate (MTX). Phototests with middle- and long-wavelength UV fluorescent lamps indicated that the sequence and timing of each therapeutic exposure were important to the development of the erythema. Histologic examination of the erythematous area demonstrated an increased number of pyknotic keratinocytes (sunburn cells) in the upper epidermis. It is possible that MTX prevented the repair of DNA damage caused by UV exposure, leading to the appearance of the sunburn cells and erythema. Care must be exercised when using UV radiation and MTX in combination.
During the past ten years we have seen two or more episodes of erythema nodosum develop in five patients in association with either pregnancy or the ingestion of female hormones. No alternative causes of erythema nodosum were found in these patients. The occurrence of erythema nodosum in association with hormonal change suggests that female hormones can, under some circumstances, be directly responsible for development of the disease.
The protean manifestations of erythema multiforme range from minor variants through Stevens-Johnson Syndrome, bullous erythema multiforme, and toxic epidermal necrolysis. Herpes simplex virus is a common etiologic agent for minor varieties particularly if they are recurrent. Certain drugs also cause severe varieties of erythema multiforme. Although no form of therapy is recognized as generally effective, many are used in treating this group of diseases. Local wound care is also important.
BACKGROUND AND DESIGN: Specific humoral and cell-mediated immune responses play an important role in the pathogenesis of Lyme borreliosis. Several previous studies demonstrated that a specific cellular immune response to Borrelia burgdorferi can occur independently of a diagnostic humoral response. Little is known about T-cell reactivities against B burgdorferi in early and late cutaneous manifestations of Lyme borreliosis. We studied the lymphoproliferative response of peripheral blood mononuclear cells to B burgdorferi antigen from 99 patients (25 with erythema migrans, 16 with acrodermatitis chronica atrophicans, 13 with lymphadenosis benigna cutis, and 45 with localized scleroderma) and 21 control subjects. The results are expressed as a stimulation index (SI) (mean count per minute of triplicate cultures with stimulant divided by mean count per minute without stimulant). The serum samples from all patients and control subjects were tested for antibodies to B burgdorferi by indirect immunofluorescence assay. RESULTS: The 21 healthy seronegative controls had an SI of 3.3 +/- 2.0 (mean +/- SD). Compared with that of control subjects, the SIs were significantly elevated in patients with erythema migrans (9.8 +/- 9.1), acrodermatitis chronica atrophicans (11.8 +/- 8.2), and lymphadenosis benigna cutis (7.2 +/- 6.2). The 45 patients with localized scleroderma had elevated proliferative responses, with an SI of 6.5 +/- 7.3, but these responses did not significantly differ from those of controls. Elevated titers of antibodies to B burgdorferi were present in six (24%) of 25 patients with erythema migrans, five (38%) of 13 patients with lymphadenosis benigna cutis, and 13 (29%) of 45 patients with localized scleroderma. All 16 patients with acrodermatitis chronica atrophicans had markedly elevated antibody titers. CONCLUSIONS: Our findings show that a significant lymphoproliferative response to B burgdorferi occurs in the majority of patients with cutaneous manifestations of Lyme borreliosis. The lymphocyte proliferation assay may be of diagnostic value in patients in whom Lyme borreliosis is strongly clinically suspected and who have nondiagnostic levels of antibodies against B burgdorferi.
Results from both animal and human studies have indicated that inflammatory skin reactions such as the flare response to histamine prick test involve a neurogenic regulatory component. It is still unknown to which degree inflammation induced by ultraviolet (UV) radiation may be mediated by the central nervous system. To investigate this, the effect of hypnotic suggestions to increase and decrease the response to UVB radiation on erythema and cutaneous blood flow was investigated in 10 highly hypnotizable subjects. The results showed a significant effect of hypnotic suggestions on cutaneous blood flow compared with the responses of a control group. For erythema no conclusive evidence was found. The results indicate the possibility of separate regulatory mechanisms behind central nervous system influence on UVB-induced erythema and skin blood flow respectively, and further investigations are needed.
Erythema nodosum is an uncommon skin eruption frequently associated with underlying infections, systemic diseases, or drug toxicity. We report the case of a 27-year-old aviator with three occurrences of erythema nodosum attributed to streptococcal pharyngitis. We review the causes of erythema nodosum to emphasize the importance of a thorough investigation to rule out serious underlying disease prior to returning an aviator to flight status.
The purpose of this retrospective clinicopathologic study was to investigate the presence of eosinophils in oral and skin lesions of erythema multiforme. Seventeen cases were selected which fulfilled clinical and histopathologic criteria for the disease. Twelve out of 13 sections taken from skin biopsies and 4 out of 5 sections taken from oral lesions contained eosinophils in varying densities. The occurrence of eosinophils in the lesions of erythema multiforme is parallelized and discussed with the existence of certain clinical and histopathologic aspects. Therefore, we suggest that eosinophils occur in cutaneous and oral lesions of erythema multiforme and are occasionally numerous.
BACKGROUND AND DESIGN: To conduct a prospective case-control study about causative factors of severe bullous erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis, we needed to define criteria for classifying the cases and standardize the collection of data so that cases could be reliably diagnosed according to this classification. Based on review of case histories and photographs of patients, a group of experts proposed a classification based on the pattern of erythema multiforme-like lesions (categorized as typical targets, raised or flat atypical targets, and purpuric macules) and on the extent of epidermal detachment. An atlas illustrating this classification that included photographs and schematic drawings was developed. We compared the evaluations of 28 cases by four nonphysicians relying on the atlas with the evaluations of the same cases by five experts not using the atlas to determine the usefulness of this atlas for classifying cases according to our nosologic schema. RESULTS: The following consensus classification in five categories was proposed: bullous erythema multiforme, detachment below 10% of the body surface area plus localized "typical targets" or "raised atypical targets"; Stevens-Johnson syndrome, detachment below 10% of the body surface area plus widespread erythematous or purpuric macules or flat atypical targets; overlap Stevens-Johnson syndrome-toxic epidermal necrolysis, detachment between 10% and 30% of the body surface area plus widespread purpuric macules or flat atypical targets; toxic epidermal necrolysis with spots, detachment above 30% of the body surface area plus widespread purpuric macules or flat atypical targets; and toxic epidermal necrolysis without spots, detachment above 10% of the body surface area with large epidermal sheets and without any purpuric macule or target. Using the atlas, the nonexperts showed excellent agreement with the experts. CONCLUSION: This study suggests that an illustrated atlas is a useful tool for standardizing the diagnosis of acute severe bullous disorders that are attributed to drugs or infectious agents. Whether the five categories proposed represent distinct etiopathologic entities will require further epidemiologic and laboratory investigations.
A patient with acute promyelocytic leukemia is presented. During treatment with all-trans-retinoic acid, erythema nodosum developed. Treatment with all-trans-retinoic acid was continued, the erythema nodosum resolved with steroid treatment, and a complete remission was attained. This case is the first report of erythema nodosum associated with all-trans-retinoic acid.
Eight cases of Sjögren syndrome (SjS) with anti-SS-A and anti-SS-B antibodies are reported. They developed erythema annulare centrifugum-like annular erythema which mainly appeared on the face. Laboratory tests showed similar serologic changes. RA factor, speckled type antinuclear antibody, anti SS-A & SS-B antibodies (DID & ELISA) were observed but complements and anti-double stranded DNA antibodies were not detected. In spite of mild sicca symptoms, sialogram, lip biopsy, Shirmer test and Rose Bengal staining showed typical changes of SjS. It is considered that the recurrent annular erythema is a specific skin manifestation of SjS with anti SS-A/SS-B antibodies.
Three infant boys with a centrifugal annular erythema mainly consistent with erythema annulare centrifugum, developing a few weeks after birth, are described. The lesions disappeared before the age of 6 months, without atrophy, and during this period the infants were otherwise healthy. This group is considered to belong to one of three types of reactivity in infants associated with or expressed as a connective tissue disease, especially lupus erythematosus, in the mother and child or in either. In type 1, signs and symptoms of systemic lupus erythematosus are or will be present in the mother and the child displays discoid lupus erythematosus lesions at birth or soon after. In type 2, the mother has the same signs and symptoms as in type 1 but the child develops a centrifugal annular erythema 3-6 weeks after birth. In type 3, discoid lupus erythematosus is present at an early stage in the infant, while the mother is healthy. This type may represent an early onset of lupus erythematosus in the infants.
The development of cellular hypersensitivity, as measured by lymphocyte blastogenic and skin tests assays, was correlated with intensification of lesion erythema in guinea-pigs infected with Trichophyton mentagrophytes. Development of lymphocyte blastogenic responses to trichophytin was assayed using forty infected animals and twenty controls; blastogenic conversion occurred between 9-11 days post spore application. The onset of cellular hypersensitivity was assayed in vivo in twenty infected guinea-pigs by skin testing at intervals after infection; positive responses occurred between 9-11 days post spore application which coincided with the time of blastogenic conversion. Clinical characteristics of lesions were followed in a group of twenty-four animals: lesions first appeared on day 7; erythema intensified until day 10; lesions reached maximum size by day 13; and alopecia began on day 17. Since cellular hypersensitivity (positive skin test and lymphocyte blastogenesis) develops at the same time as lesions reach maximum erythema, cellular hypersensitivity appears to be responsible for the intense inflammatory response associated with ringworm infections.
The clinical and histopathological classification of erythema exudativum multiforme major (EEMM), Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are difficult, due to the lack of clear-cut criteria. Based on a new clinical classification, 149 of 219 (68%) histopathological specimens, from a total of 534 patients with EEMM, SJS and TEN, have been reviewed. A comparison was made with the clinical picture, and any past history of infection or drug intake. All patients had been included in the German Registry of Severe Skin Reactions between April 1990 and December 1993. No differences could be found between the biopsies examined and the total number of histopathological specimens, concerning clinical diagnosis, gender and age. Sections from 28 of 149 specimens were not diagnostic or were too old to be properly evaluated. In nine cases, other diagnoses were proposed. One hundred and eleven of the histological slides with the diagnosis of EEMM (n = 16), SJS (n = 34) and TEN (n = 61), were classified as epidermal type of erythema multiforme. In these 111 slides, necrotic keratinocytes could be found, ranging from individual cells to confluent epidermal necrosis. The epidermo-dermal junction showed changes ranging from vacuolar alteration up to subepidermal blisters. The dermal infiltrate was superficial and mostly perivascular. It was sparse in SJS and TEN, and more pronounced in EEMM. Oedema in the papillary dermis was evident occasionally in all clinical groups. In 59 of 111 cases (53%), at least one eosinophil was present in the dermis. In 11 of 111 (10%), more than 10 eosinophils per field could be seen. Eosinophils were less common in the patients with the most severe forms of TEN, in whom there was detachment of more than 30% of the skin surface area. No differences in the history for drug intake, or for infection with Mycoplasma pneumoniae, herpes simplex and other organisms, could be detected between patients with or without eosinophils in their skin sections. This dermatopathological study of patients with EEMM, SJS and TEN indicates that the epidermal type of erythema multiforme is the pathological correlate for these diseases.
BACKGROUND: We review the literature and report a case of refractory erythema elevatum diutinum associated with IgA paraproteinemia that was successfully controlled with intermittent plasma exchange (PLEX). OBSERVATIONS: Typical lesions of erythema elevatum diutinum developed in a 72-year-old patient with IgA paraproteinemia; the condition predictably flared whenever IgA levels reached a threshold of 8 g/L. After 8 years of unsuccessful treatment with various agents, we instituted a trial of PLEX during an acute flare. Following 6 exchanges over a period of 2 weeks, the IgA level decreased from 8 to 2 g/L and the skin lesions cleared. Three weeks later, new skin lesions developed and the IgA level had rebounded from 2 to just over 8 g/L. A second course of PLEX was administered, with excellent results, and a 3-month course of oral chlorambucil (2 mg/d) was initiated. The patient's condition remained in clinical remission for 10 months. Over the ensuing 9 years, she suffered 11 further flares, each of which was associated with IgA levels of 8 to 10 g/L and each responding dramatically to 3 to 5 PLEXs followed by a consolidative dose of intravenous cyclophosphamide (250-500 mg). CONCLUSION: We believe that PLEX may have an important role in the management of severe erythema elevatum diutinum associated with monoclonal paraproteinemia refractory to other therapy.
Sunbed use was studied in relation to phenotype, erythema, sunscreen use and skin disease. The study population comprised 14-19 year-old Stockholm adolescents in 60 randomly selected classes, with 1252 students providing information. More than half (57%) reported sunbed use > or = 4 times during the previous year. Skin type III dominated (64%). Excessive exposure (> or = 10 times/year) was not correlated to skin type. Sunscreens were most commonly used by sunbed users. Of all sunbed users, 44% reported erythema. Adolescents with acne/seborrhoca, eczema or psoriasis used sunbeds more than others without skin diseases. The proportion with sunbed erythema (44%) indicates an unrecognized susceptibility to artificial ultraviolet radiation (UVR) among adolescents. The association between high exposure to UVR and sunscreen use stresses the importance of sunscreens being used as supplementary protection, not as a tool for tanning.