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Persistent erythema and edema of the midthird and upper aspect of the face (morbus morbihan): evidence of hidden immunologic contact urticaria and impaired lymphatic drainage.

BACKGROUND: A persistent erythema and edema of the midthird and upper aspect of the face, which bears some resemblance to Melkersson-Rosenthal syndrome and rosaceous lymphedema, has been characterized as morbus morbihan (MM) by French dermatologists. The disease of yet unknown cause starts with recurrent facial edema of short duration, which ultimately leads to persistent swelling after a period of weeks or months. METHODS: We recruited 6 patients with MM and acquired their history, routine blood tests, and individual UV light tolerability. To check for contact allergies the allergen patch test and the open epicutaneous patch test were performed. To objectify the skin conditions laser Doppler flowmetry and 20-MHz ultrasound were used. Five patients with similar symptoms, but with definitely transient facial erythema and edema caused by proven contact urticaria on cosmetics served as a comparison group. RESULTS: In all patients, routine blood tests and UVA/UVB light tests showed no pathologic results. Observations of 6 patients with MM revealed the common feature of a clinically relevant immunologic contact urticaria caused by various cosmetic ingredients, which could be diagnosed in all of them. Delayed resorption of the acute edema and prolonged inflammation were shown by laser Doppler flowmetry and 20-MHz ultrasound in the affected skin areas in patients with MM after induction of immunologic contact urticaria by a cosmetic ingredient. Strict avoidance of cosmetics yielded a remarkable clinical benefit in the follow-up examinations. CONCLUSIONS: We conclude that recurrent and possibly subclinical inflammation caused by immunologic contact urticaria in conjunction with a locally pre-existing lowered lymphatic drainage plays a crucial role in the evolution of MM.

Adult↗

Methotrexate-induced bullous acral erythema in a child.

Chemotherapy induced acral erythema (CIAE) is an uncommon and dramatic reaction to high-dose chemotherapy. It is characterized by symmetrical, well-demarcated, painful erythema of the palms and soles which may progress to bullae formation and desquamation. Prompt recognition and discrimination from more serious conditions such as graft-vs-host disease or toxic epidermal necrolysis is essential. This paper describes the case of a 12-year-old boy who developed CIAE after high-dose methotrexate treatment and discusses the important clinical, histopathological, and therapeutic features of this condition.

Antimetabolites, Antineoplastic↗

Sternal erythema: a distinctive postsurgical eruption.

BACKGROUND/OBJECTIVE: Wound complications constitute a diverse array of surgical and dermatologic entities. We sought to describe the clinical and pathologic attributes of a series of patients in whom a distinctive thoracic surgical wound eruption developed after coronary bypass grafting. METHODS: We performed a detailed chart review, including history, surgical procedure notes, and biopsy findings, obtained from 3 patients; we examined the data and reconciled them with conventional etiologies in an attempt to discern a shared diagnosis and pathogenesis. RESULTS: The patients were white men (59, 68, and 73 years of age) in whom roughly symmetrical asymptomatic erythema developed within the borders of their sternal thoracotomy wounds between 1 and 2 years after open heart surgery. The cutaneous findings consisted of macular erythema and poikiloderma that blanched with external pressure. Biopsy findings yielded epithelial atrophy with capillary telangiectases. CONCLUSION: Although cellulitis, dermal hypersensitivity to surgically implanted hardware including sternal wire, and an anatomic variant of costal fringe remain as possible diagnoses, an additional consideration includes a form of postsurgical reflex sympathetic dystrophy.

Aged↗

[Necrolytic migratory erythema associated with glucagonoma].

Glucagonoma is a rare pancreatic tumor that is usually associated with a syndrome that includes diabetes, anemia, weight loss and skin lesions in the form of necrolytic migratory erythema. We present the case of a patient with malignant glucagonoma treated with surgery and octreotide, which manifested with skin lesions. The discussion will review the physiopathology, other causes of necrolytic erythema, diagnosis and differential diagnosis and treatment.

Erythema↗

The pathogenesis of vascular changes in erythema nodosum-like lesions of Behçet's syndrome: an electron microscopic study.

The probable sequence of microvascular changes affecting endothelial cells in erythema nodosum-like lesions was examined by electron microscopy in 18 patients with typical Behçet's syndrome. Dark endothelial cells in the superficial and deep dermis exhibited degeneration with contraction changes. Consequently, degenerated endothelial cells showed nuclear and cytoplasmic condensation, vacuolization in the cytoplasm, and rich ribosomes. Moreover, blood vessels which were enveloped by cuffs of lymphocytes simultaneously exhibited both endothelial cell hypertrophy and endothelial cell necrosis. The implied associations between perivascular lymphocytic cuffs and blood vessels exhibiting endothelial cell alterations indicate that microvascular changes in erythema nodosum-like lesions may be closely associated with the delayed-type hypersensitivity reaction.

Behcet Syndrome↗

Antibiotic therapy in early erythema migrans disease and related disorders.

Between December 1978 and July 1985, we used various antibiotics for the treatment of 97 adult patients with early erythema migrans disease (EMD). Six patients with borrelial lymphocytoma (BL) and 20 with acrodermatitis chronica atrophicans (ACA) were treated similarly. Follow-up was for a median of 20, 14, and 12 months, respectively. The erythema migrans and all associated symptoms resolved within a median of 3 weeks (0.5-18.4), BL within 7 weeks (4-16), and ACA partly or completely within several months. A Jarisch-Herxheimer (-like) reaction was observed in 8 patients with EMD. Fourteen patients with EMD and one with ACA developed an exacerbation of symptoms or new manifestations between the 2nd and 20th day, and 28 patients with EMD and one with ACA continued to have or acquired various symptoms greater than or equal to 3 weeks after initiation of therapy. Arthralgia, neurologic and constitutional symptoms, and in one instance a slight pulmonary interstitial edema developed in EMD. More severe initial illness was a risk factor for the development of later symptoms in EMD. Retreatment was more often necessary in ACA than in EMD. A patient with ACA had a recurrence after 5 1/2 years. IgG antibody titers rose at least fourfold in 5 patients with ACA and in 1 with EMD despite therapy. We tentatively recommend minocycline or high doses of parenteral penicillin for the treatment of these disorders.

Acrodermatitis↗

A case of contact dermatitis, erythema multiforme, and toxic epidermal necrolysis.

A young adult female patient, on prolonged corticosteroid therapy for nephrotic syndrome, developed erythema multiforme and toxic epidermal necrolysis following a double exposure to a locally applied perfume. We believe that this route of exposure should be re-emphasized. Toxic epidermal necrolysis, or the scalded skin syndrome, has become a well-recognized entity which may be divided into three distinct subgroups: the staphylococcal scalded skin syndrome, nonstaphylococcal or drug-induced scalded skin syndrome, and idiopathic. We present a patient who developed a dermatitis on her chest and abdomen after using a spray cologne, which resulted in the development of erythema multiforme with progression toxic epidermal necrolysis and, ultimately, her death.

Adrenal Cortex Hormones↗

HLA-B15 association with erythema multiforme.

Erythema multiforme (EM) is a cutaneous reaction pattern which follows numerous infections, drugs, neoplastic and inflammatory disorders in some individuals. We undertook a prospective study of thirty-eight HLA specificities of the -A, -B, and -C series in 16 Caucasian patients with EM and in 140 local Caucasian controls. Seven of 16 patients (44%) with EM and 5 of 9 patients (55%) with EM following herpes simplex infections possessed the HLA-B15 antigen, compared to 7% of local controls and 11.6% of the 1980 WHO Workshop Caucasian controls. Both associations were highly significant (p = 0.0125 and p = 0.02) when corrected for 38 HLA antigens. This is the first reported HLA association for erythema multiforme, a disease which may be a host-specific immune response to various antigens, determined in part by genes linked to HLA-B15.

Erythema Multiforme↗

Photosensitive recurrent erythema multiforme.

A case report of photosensitive recurrent erythema multiforme occurring in a 31-year-old man is presented herein. Lesions clinically and histologically resembling erythema multiforme were reproduced with light testing. The patient's lesions appeared to respond to both oral prednisone and hydroxychloroquine.

Adult↗

Cutaneous immunofluorescence study of erythema multiforme: correlation with light microscopic patterns and etiologic agents.

Direct immunofluorescence microscopy was positive in 88% of forty-one skin biopsy specimens from thirty-four patients with the clinical diagnosis of erythema multiforme. The most common finding, present in 67% of positive specimens, was the cytoid body, or fluorescent keratinocyte, which stained most often with IgM (homogeneously) or with C3 (speckled). Other findings included basement membrane zone (BMZ) fluorescence, primarily with fibrinogen and C3, and vascular fluorescence, most commonly C3 in a granular pattern. Correlation of direct immunofluorescent and light microscopic findings revealed that (1) the fluorescent keratinocyte was prevalent only in epidermal and mixed patterns, correlating with the eosinophilic necrotic keratinocyte by light microscopy, and (2) vascular fluorescence was most prominent in dermal forms. Herpes simplex-associated erythema multiforme showed exclusively a mixed histologic pattern, whereas the drug-related form was primarily epidermal.

Adolescent↗

Potential hazards in phototherapy with ultraviolet radiation arising from variation in dose required to produce erythema.

Phototherapy using 290-320 nm ultraviolet radiation (ultraviolet B) is an effective form of treatment for several skin diseases and is especially useful for psoriasis. Technical advances have made it more economical and convenient to quantify ultraviolet radiation. Ten volunteers received a series of exposures from three ultraviolet B sources to illustrate how variation in the spectral properties of phototherapy devices can lead to unanticipated and potentially severe sunburn reactions. The corresponding minimal erythema dose was then calculated with the use of irradiance data measured with two commercially available photometers. Variation in minimal erythema dose among the three sources tested indicates the need to characterize the emission spectrum of sources and the spectral sensitivity of the photometer used to measure its output.

Erythema↗

Erythema nodosum leprosum: nature and extent of the cutaneous microvascular alterations.

Skin biopsy specimens from four patients with erythema nodosum leprosum, when examined as Epon-embedded, 1-micron sections, exhibited a necrotizing vasculitis involving capillaries, venules, and small-to-medium arteries and veins. In the superficial dermis, affected venules and capillaries showed endothelial cell enlargement and focal necrosis associated with perivascular infiltrates of lymphocytes. In the deep dermis and subcutaneous tissue, affected venules, arterioles, and arteries exhibited endothelial cell necrosis and matted fibrin in the vessel walls associated with perivascular infiltrates of neutrophils. Throughout the dermis, mononuclear phagocytes with vacuoles containing numerous fragmented organisms were observed. By electron microscopy, electron-dense material resembling immune complexes was observed in the walls of these vessels. These observations support the concept that erythema nodosum leprosum is an immune complex-mediated necrotizing vasculitis involving capillaries, arterioles, arteries, venules, and veins.

Adult↗

Erythema multiforme in a patient with T cell chronic lymphocytic leukemia.

A patient with T4+ (helper-inducer) T cell chronic lymphocytic leukemia developed an erythema multiforme-like eruption, the diagnosis of which was supported by routine light microscopic findings. Immunopathologic studies using monoclonal antibodies demonstrate that despite an overwhelming majority of leukemic T4+ cells in the peripheral blood and dermal infiltrate, the predominant cells in the epidermal infiltrate are T8+ (cytotoxic-suppressor) cells. These findings are different from those seen in epidermotropic T cell leukemic infiltrates and are similar to those previously reported in erythema multiforme. Thus it is likely that the leukemic T4+ cells are participating in this cutaneous hypersensitivity reaction along with residual, normal T8+ cells.

Aged↗

Treating erythema chronicum migrans of Lyme disease.

The efficacy of antibiotic treatment of 117 patients with erythema chronicum migrans of Lyme disease was evaluated in terms of the necessity for retreatment and the prevention of the late manifestations of Lyme disease. Fifty-six patients with a minor form of the illness did not require retreatment and did not develop late manifestations following antibiotic treatment. Three pregnant patients were included in this group. Fourteen of sixty-one patients with a major form of the illness required retreatment, and five developed posttreatment late manifestations of Lyme disease consisting of Bell's palsy and persistent joint pain. Although the preferred antibiotic for treating erythema chronicum migrans of Lyme disease has not been conclusively established, tetracycline and penicillin proved effective. The use of probenecid plus penicillin may be of benefit to patients with the major form of the illness.

Adult↗

Annular erythema: a possible association with primary Sjögren's syndrome.

In four patients with primary Sjögren's syndrome a distinct annular erythema developed, which was characterized by a wide, elevated border (which can be likened to a doughnut ring) and central pallor. Histologically, there was a coat sleeve-like infiltration of lymphocytes around blood vessels and nuclear debris in the connective tissue. Vasculitis or epidermal changes suggestive of lupus erythematosus were not observed, and there was no immunoglobulin deposition along the basement membrane zone. This annular erythema may be a cutaneous manifestation of Sjögren's syndrome.

Adult↗

Erythema dyschromicum perstans. Immunopathologic studies.

Immunopathologic studies of a patient with clinical and histologic evidence of erythema dyschromicum perstans revealed Ia antigen expression on epidermal keratinocytes, pronounced OKT4 and OKT6 staining of epidermal dendritic cells, and dermal infiltration of T lymphocytes of both helper-inducer (OKT4) and suppressor-cytotoxic (OKT8) phenotypes--a pattern commonly associated with lichen planus. These findings, taken in conjunction with positive IgG staining on colloid bodies, suggest the possibility that erythema dyschromicum perstans and lichen planus have similar disease processes.

Adolescent↗

Epidermal autoantibodies in erythema multiforme.

We report a case of refractory erythema multiforme associated with atypical epidermal autoantibodies. The patient was a 63-year-old woman with clinical and histologic features of erythema multiforme and a large mediastinal T cell lymphoma. Immunofluorescence studies disclosed high serum titers of cell surface antibodies against epidermal keratinocytes in a pattern typical of pemphigus vulgaris. The epidermal antigen reacting with these circulating antibodies was characterized by incubating 14C-labeled keratinocytes with serum. The patient's serum pattern precipitated multiple proteins keratinocytes antigens), but not the 130,000- and 85,000-dalton polypeptides characteristic of pemphigus vulgaris. Thus autoantibodies reacting selectively with non-pemphigus vulgaris epidermal keratinocyte antigens may be the cause of a false-positive immunofluorescence test for pemphigus vulgaris.

Aged↗