ABSTRACT
The skin manifestations of monoclonal(M)-proteinemia are rare and present in patients with monoclonal gammopathy of undetermined significance (MGUS), smoldering plasma cell myeloma (SMM) and multiple myeloma (MM). In this study, we reported 4 cases with M-proteinemia-related rare skin lesions, including pyoderma gangrenosum (PG), erythema elevatum diutinum (EED), cutis laxa (CL) and lichen myxedematosus(LM). These skin lesions are specific, where the potential mechanism was immune-mediated paraneoplastic syndrome rather than direct plasma cell infiltration. Anti-plasma cell treatment was effective in treating skin lesions. The clinical outcome of MM-related skin changes was correlated to tumor control, whereas the prognosis of MGUS or SMM related skin lesions was favorable. Skin involvement in M-proteinemia is extremely rare and less well-known, which greatly impairs quality of life. The diagnosis and treatment of these 4 cases support the need for futher study.
ABSTRACT
A 61-year-old man presented with a 3-year history of erythematous firm nodules on the hands and feet. Histopathological findings of the lesional skin revealed perivascular and diffuse neutrophilic infiltrations on the upper and mid-dermis. Increased and dilated blood vessels were observed in the upper dermis. Fibrinoid necrosis of the vessel walls was unremarkable, but endothelial swelling and scant red blood cell (RBC) extravasation were noted. Fibrosis and sclerosis of collagen fibers were noted on the deep dermis. Results of laboratory examinations, including complete blood count (CBC), routine chemistry, c-reactive protein (CRP), syphilis and human immunodeficiency virus (HIV) tests, and serum immunoglobulin electrophoresis, were all negative or within normal limit. A diagnosis of erythema elevatum diutinum was made based on the clinical and histological findings. The patient was treated with prednisolone, dapsone, colchicine, and intralesional injection of triamcinolone and showed slight improvement after treatment for 8 months.
Subject(s)
Humans , Middle Aged , Blood Cell Count , Blood Vessels , C-Reactive Protein , Chemistry , Colchicine , Collagen , Dapsone , Dermis , Diagnosis , Electrophoresis , Erythema , Erythrocytes , Fibrosis , Foot , Hand , HIV , Immunoglobulins , Injections, Intralesional , Necrosis , Neutrophils , Prednisolone , Sclerosis , Skin , Syphilis , TriamcinoloneABSTRACT
@#Erythema elevatum diutinum (EED) is a rare condition believed to be a form of chronic recurrent leukocytoclastic vasculitis possibly secondary to vascular immune complex deposition. The disease is characterized by symmetrical, red, brownish-purple, and yellow papules, plaques, and nodules distributed mainly over the extensor surfaces of the extremities. We report a 61-year-old male with an atypical presentation of such disease as a giant warty lesion on the heels. Histologically, a spectrum from leukocytoclastic vasculitis to vessel occlusion and dermal fibrosis is seen in EED. These histological findings were present in the histopathological reading of the patient which established its diagnosis and further ruled out verruca vulgaris. The disease is associated with many disease entities, which include human immunodeficiency virus, malignant conditions, chronic infection, and autoimmune and connective tissue disorders. None of these conditions was present in the patient as manifested in the history, physical, and laboratory examinations. However, the patient has a low hemoglobin and a G6PD deficiency which makes him a bad candidate for dapsone therapy which is the main treatment for EED. Tetracycline, niacinamide and plain vaseline + salicylic acid were given initially for 4 weeks but no improvement was noticed. It was then shifted to 10mg intralesional corticosteroid and urea paste 40%. Niacinamide still was given. There was a marked thinning of the lesions. The medications were continued and were slowly tapered. More improvement of the lesions was observed.
Subject(s)
Glucosephosphate Dehydrogenase Deficiency , NiacinamideABSTRACT
Erythema elevatum diutinum (EED) is a rare, chronic inflammatory skin disease of unknown etiology. Clinical findings involve symmetrical livid-erythematous to red-brown papules and plaques or nodules. The skin lesions are located over extensor surfaces and often over joints. Histological findings are characterized by leukocytoclastic vasculitis and dense neutrophilic infiltration in the early stages of the lesions and by fibrosis in the late stages. EED may be associated with several hematological disorders, infectious diseases, immunological disorders, and certain malignancies. We report on a 59-year-old man with EED associated with multiple myeloma.
Subject(s)
Humans , Middle Aged , Communicable Diseases , Erythema , Fibrosis , Joints , Multiple Myeloma , Neutrophils , Skin , Skin Diseases , VasculitisABSTRACT
Erythema elevatum diutinum (EED) is emerging as a specific HIV-associated dermatosis which can be easily misdiagnosed as Kaposi's sarcoma or bacillary angiomatosis. Until now, no case of HIV-associated EED had been reported in Korea. We report a case of EED in a 49-year-old man with HIV infection. The patient was diagnosed with HIV-infection and treated with a combination of anti-retroviral agents and dapsone. Two years after the start of treatment the lesion had regressed.
Subject(s)
Humans , Middle Aged , Angiomatosis, Bacillary , Anti-Retroviral Agents , Dapsone , Erythema , HIV , HIV Infections , Korea , Sarcoma, Kaposi , Skin Diseases , Vasculitis, Leukocytoclastic, CutaneousABSTRACT
El eritema elevatum diutinum es una dermatosis neutrofílicacrónica poco frecuente, en la cual se han descritomúltiples asociaciones patológicas.Se presenta un caso clínico de esta entidad en asociacióncon enfermedad celíaca, que, según la revisiónbibliográfica que efectuamos, sería el primer trabajo en laliteratura de habla hispana.Destacamos el rol del dermatólogo en la detección deuna enfermedad sistémica previamente desconocida...
Erythema elevatum diutinum is a rare chronic neutrophilicentity, linked with many other diseases.We report a case associated with coeliac disease,which is according to our search, the first such publicationin spanish. We stress the role of the dermatologist in thedetection of a previously unrecognized systemic disease...
Subject(s)
Female , Celiac Disease , Erythema , Elbow , Knee , Necrosis , VasculitisABSTRACT
El eritema elevatum diutinum (EED) es una forma infrecuente de vasculitis leucocitoclásica cutánea crónica de etiología desconocida. Desde el punto devista clínico se observan lesiones papulosas, placas y nódulos persistentes, de color eritematovioláceo, pardo o amarillento, localizadas en forma simé-trica en áreas de extensión de extremidades especialmente sobre las articulaciones. A nivel histopatológico se evidencia una vasculitis leucocitoclásica neutrofílica acompañada de fibrosis concéntrica. Se asocia a diversas entidades, entre ellas enfermedades infecciosas, hematológicas, colagenopatías y neoplasias malignas.Presentamos a una mujer de 22 años de edad con EED asociado a crioglobulinas en suero...
Erythema Elevatum Diutinum (EED) is an uncommon chronic cutaneous leukocytoclasticvasculitis of unknown etiology. Clinically, it consist of persistentbilateral, symmetrical and periarticular erithematous and purpuric, brownishor yellowish papules, plaques and nodules usually over the dorsal aspectof the joints. Histologic findings. neutrophilic leukocytoclastic vasculitis andconcentric fibrosis.It has been associated with infectious diseases, hematological disorders, collagendiseases and carcinomas.We report a 22 years old woman with EED and cryoglobulinemia...
Subject(s)
Child , Erythema , Skin Diseases , Biopsy , Cryoglobulins , Vasculitis , Vasculitis, Leukocytoclastic, CutaneousABSTRACT
Herein, we report a case of an 8-year-old girl with dapsone hypersensitivity syndrome (DHS) that occurred during the treatment of erythema elevatum diutinum. She had fever, gross hematuria, and malaise for three weeks after initiation of dapsone therapy. Five days after stopping dapsone treatment, she returned to the emergency clinic because of high fever, emesis, diarrhea, upper respiratory symptoms, and worsening of exanthematous eruptions. A diagnosis of DHS was made, and it improved with oral prednisone. We recommend that pediatric patients who are treated with dapsone need to be observed carefully for the development of DHS.
Subject(s)
Child , Humans , Dapsone , Diarrhea , Emergencies , Erythema , Fever , Hematuria , Hypersensitivity , Prednisone , Vasculitis, Leukocytoclastic, Cutaneous , VomitingABSTRACT
Eritema elevatum diutinum es una enfermedad cutánea poco frecuente y de curso crónico recurrente. Se caracteriza por pápulas, placas y nódulos de color rojo-violáceo de distribución acral y simétrica sobre las superficies extensoras. Un hallazgo histopatológico característico es la vasculitis leucacitoclástica. El tratamiento de elección es la dapsona, A continuación se describen dos casos de Eritema Elevatum Diutinum, el primero en un paciente adulto y el segundo en una niña de 15 años, ambos con estudio histopatológico compatible y buena respuesta al uso de difenildiazona (dapsona).
Erithema elevatum diutinum is a rare cutaneous disease. It usually has a chronic course and is characterized by purple red papules, plaques o nodules with an acral and symmetrical distribution. The main histological feature is o leucocytoclostic vasculitis. Treatment of choice is dapsone. We describe two cases of erithema elevatum diutinum, a male adult patient and a 15 year old girl. Both cases present characteristic histological findings and good response to dapsone.
Subject(s)
Humans , Male , Adolescent , Female , Middle Aged , Erythema/pathology , Vasculitis, Leukocytoclastic, Cutaneous/pathology , Diagnosis, Differential , Dapsone/therapeutic use , Erythema/diagnosis , Erythema/drug therapy , Vasculitis, Leukocytoclastic, Cutaneous/diagnosis , Vasculitis, Leukocytoclastic, Cutaneous/drug therapyABSTRACT
El eritema elevatum diutinum (EED) es una dermatosis crónica, poco frecuente, de causa desconocida, que al comienzo presenta histopatológicamente una vasculitis leucocitoclásticade la piel y luego evoluciona hacia una fibrosis. Presentamos una paciente con lesiones dermatológicas características y generalizadas de esta rara entidad, en la que se obtuvo una buena respuesta con la sulfonoterapia.
Erythema Elevatum Diutinum (EED) is a rare chronic cutaneous condition of unknow origin, that initially presents histological as leucocytoclastic vasculitis of the skin and later resolves with fibrosis. We present a patient of this uncommon condition with typical cutaneous lesions and widespread involvement. The disease was initially misinterpreted on clinical examination. Dapsone therapy was followed by a remarkable recovery.
Subject(s)
Humans , Female , Middle Aged , Erythema/diagnosis , Diagnosis, Differential , Histological Techniques , Skin Diseases/complicationsABSTRACT
Erythema elevatum diutinum(EED) is a rare chronic skin disease characterized by red, purple, and yellowish papules, plaques, and nodules that are usually distributed acrally and symmetrically over extensor surface. It is characterized histologically by a leukocytoclastic vasculitis. Although no treatment has shown a complete response, dapsone is reported to be effective to EED. We report a rare severe case of EED with good response to dapsone treatment.
Subject(s)
Dapsone , Erythema , Skin Diseases , VasculitisABSTRACT
Erythema elevatum diutinurum is a relatively rare disease of unknciwr etiology. It is charaterized by persistent erythematous plaque and nodule that are usually affected symmetrically over extensor surface of extremities. Our patient, a 16-year-old female was referred for evaluation of asy nptomatic papules and nodules on extensor aspects of the hands, legs and buttocks. She complained of arthralgia on the knees. The laboratory values were all within normal limits with the exception of slight elevation of white blood cell counts and erythrocyte sedimenation rate. A skin biopsy specimen we obtained from the nodular lesion of the leg that showed preclominantly perivascular neutrophilic influration with occasional lymphocytes and unclear dusts. She was treated with 75mg/day of dapsone. Shin lesions were much improved after ten days. Eight weeks later, the lesion had almost resolved. After the discontinuation of dapsone, new lesions developed on the legs. Retreatment with dapsone resulted it a dramatic response of her lesions after three weeks.
Subject(s)
Adolescent , Female , Humans , Arthralgia , Biopsy , Buttocks , Dapsone , Dust , Erythema , Erythrocytes , Extremities , Hand , Knee , Leg , Leukocyte Count , Lymphocytes , Neutrophils , Rare Diseases , Retreatment , SkinABSTRACT
We report a case of erythema elevatum ditinum in a 6-year-old girl. Th symmetrically distributed skin lesions consisted of elevated purplish annular plaques and nodules on the elbows, knees, ankles and buttocks, and some of the lesions showed vesicles, hemorrhagic crusts, and necroses. Biopsy specimens from the knee and ankle revealed typical leukocytoclastic vasculitis. Ve performed a Multites CMI, a test for delayed r.utaneous hypersensitivity, in which the patient showed positive reaction to streptococcal antigen(Lancefield group C). We tried oral prednisolone and dapsone seperately and could see a remarkable response to both drugs.
Subject(s)
Child , Female , Humans , Ankle , Biopsy , Buttocks , Dapsone , Elbow , Erythema , Hypersensitivity , Knee , Necrosis , Prednisolone , Skin , VasculitisABSTRACT
We report a case of 77-year old male with erythema elevatum diutinum, associated with pancytopenia, which developed on the unusual sites. Erythematous or brownish nodules developed on the face, neck, trunk, upper extremities and tongue. Histopathological examination of skin and tongue lesions showed perivascular infiltration of neutrophils, lymphocytes and nuclear dusts, and deposition of fibrinoid material within and around the vessel walls. His skin lesions improved slightly with systemic corticosteroid therapy.