[Erythema dyschromicum perstans].
The case of a 4 1/2 year old girl with the typical clinical picture of erythema dyschromicum perstans is presented. A survey of the literature on this dermatosis is described.
SEARCH · Search PubMed
Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
The case of a 4 1/2 year old girl with the typical clinical picture of erythema dyschromicum perstans is presented. A survey of the literature on this dermatosis is described.
A case of lepromatous leprosy with erythema nodosum leprosum (ENL) undergoing treatment with dapsone, rifampin, and thalidomide developed focal tuberculoid granulomas in the ENL lesions. This is the first report known to the authors of a lepromatous leprosy patient in whom ENL and an upgrading reaction occurred simultaneously.
A survey of the relevant literature, with particular attention to clinical and immunological studies propounding pathogenetic theories, is followed by the presentation of 32 cases of erythema nodosum observed in infants examined at the Turin Paediatrics Clinic and Regina Margherita Hospital. The clinical laboratory data showed that streptococcal infection was hardly less significant than T. B. as the cause of this condition.
Fifty patients in the Negev area with erythema nodosum (EN) were studied retrospectively. The clinical picture and demographic characteristics did not differ markedly from those in the literature. In contrast to most European studies but like those from Egypt, we found streptococcal pharyngitis to be the major cause of EN. Conspicuously, sarcoidosis is a rare cause of EN in the Negev. The low incidence of EN in the Negev--2 cases/100,000 people per year--as opposed to Europe--14 cases/100,000 people per year--is hardly explained by the low occurrence of sarcoidosis in Israel. Other factors, unknown at present, may account for this low incidence.
Erythema Nodosum Leprosum (ENL) or Type II reaction is an immune complex syndrome seen in multibacillary leprosy. 20 patients with histological confirmation of ENL in leprosy were studied from 1982 to 1986. These patients had a range of clinical signs, from fever, tender dusky nodules, bullae, ulcers to lymphadenopathy, arthralgia and neuritis. The four major histological patterns are: a) classical pattern showing heavy infiltrations of neutrophils in three cases, b) sub-epidermal bulla pattern with marked oedema of the upper dermis, and collections of neutrophils in five cases, c) vasculitis pattern, affecting superficial and mid-dermal vessels, leading to epidermal necrosis, bulla formation and ulceration. Dilated vessels, congestion, lumenal fibrin clots and fibrinoid necrosis of vessels were seen, d) non-specific picture in nine cases with mild oedema, infiltration with neutrophils, and two cases with minimal reaction had chronic ENL with clinical vasculitis. All the five cases with vasculitis showed C1q, C3 and fibrinogen in the vessels. Comparing ENL reactions reported in Asia, our pattern is similar to that of Malaysians with the majority showing sub-epidermal oedema. Vasculitis is more common in India. Oedema with collagen necrosis as seen in acute ENL with iritis in New Guinea. The Lucio's phenomenon was not seen in any of the countries in Asia.
Recently, it has been suggested that reactive oxygen species (ROS) produced by activated polymorphonuclear leukocytes (PMNs) exert auto-oxidative tissue damage at the site of inflammation. In the present study, a possible role of ROS in the pathogenesis of erythema multiforme (EM) was investigated by determining the capacity of the sera from patients for generating ROS from PMNs. Significantly increased hydroxyl radical production was observed, which is one of the most potent ROS capable of causing tissue damage. This change was not observed when PMNs were incubated with sera from patients with bullous pemphigoid (BP) or inflammatory acne, indicating that this finding was not a common feature of immunologically mediated and/or inflammatory cutaneous disorders. Elevated C1q activities and depositions of immunoreactants in the blood vessels were also noticed in some patients. These findings suggest that ROS generated by PMNs are involved in the formation of cutaneous lesions of EM and that immune complexes (ICs) may provide an important mechanism in PMN activation.
In Lyme's disease (LD) as well as in the European form of erythema chronicum migrans (ECM), the etiologic agents are spirochetes. As fas as we know by now, these microbes are closely related but not identical. Consequently, LD and ECM should be regarded as closely related but not as identical diseases. The sera of our 21 patients suffering from acrodermatitis chronica atrophicans (ACA) contained elevated antibody titers directed against the etiological agent found in ECM. These findings strongly suggest that ACA is also induced by spirochetes-possibly by the same microbes found in ECM.
Cell-mediated immune (CMI) responses were measured in 39 lepromatous, 44 erythema nodosum leprosum (ENL), and 22 post-ENL patients. The leukocyte migration inhibition test was used to measure CMI responses to mitogen phytohemagglutinin-P (PHA), crossreacting antigen purified protein derivative (PPD) of tuberculin, and armadillo-derived whole and sonicate Mycobacterium leprae. "Early T" lymphocytes of the peripheral blood were also enumerated using the rosetting technique. Significantly enhanced immune responses (lower migratory indices) were found to whole M. leprae during ENL. Although responses were high with PHA and PPD, they were identical in all of the groups, indicating that during ENL reaction M. leprae-specific responses are enhanced. "Early T" lymphocytes also showed a significant increase in ENL reactions compared to lepromatous patients. However, there was no response to the leprolin skin test in ENL patients in contrast to the enhanced in vitro CMI responses.
During 2 years, 25 lepromatous patients were hospitalised in the Hansen complications room of Institut Marchoux. Between these patients, 9 developed a Necrotic Erythema Nodosum Leprosum. A review of the observed clinic effects is established and 3 types of signs are isolated and discussed: necrotic extension after big nodes on chest and arms, a punch crater complicating small nodes, and a sclerous xylodermia on arms and legs. The course of this complication is estimated about six months average, with pauses and relapses with general and subjectives symptoms. The final course shows side effects: anemia, denutrition, functional disabilities of joint movements and cutaneous straps. In the group of 9 patients, 3 died. We did not find relations between the necrotic phenomenon and therapy or occurred diseases. The best drug to stop the necrotic processus is thalidomide 400 mg daily and decrease 100 mg when the signs fall near normality. One or two mg/kg each day coricosteroides were tested: the effect is unconstant, the duration of action is shorter. Side effects occur rapidly and patient will become corticosteroïd dependent. The importance of bath with disinfectants is high. We did not observed surinfection.
Linear IgA dermatitis was diagnosed in a 13-year old girl with erythema annulare centrifugum (EAC) on the basis of the criteria laid down by Jablonska: vesiculo-bullous eruption with specific patterns on subsequent flare-ups, subepidermal vesicles and bullae with papillary eosinophilic abscesses in erythematous areas, positive linear IgA antibody response at direct immunofluorescence in the lamina basal, absence of intolerance to gluten and responsiveness to sulfapyridine and dapsone. This patient was followed up for 10 years. During the first 5 years any attempt at withdrawing dapsone resulted in quick relapse which always remained responsive to that drug. After 5 years discontinuing dapsone was no longer followed by relapse, and the girl was considered clinically cured. Yet direct immunofluorescence in healthy skin remained positive for 2 years after treatment was stopped, as has previously been reported. At the age of 23, after 5 years without treatment the patient remained cured. This case demonstrates that linear IgA dermatitis is one of the causes of EAC. Autoimmune bullous diseases, such as pemphigus with eosinophilic spongiosis, bullous pemphigoid and dermatitis herpetiformis, are known to present as EAC. Direct cutaneous immunofluorescence is necessary to the aetiological diagnosis of EAC.
A young woman had recidivating herpes with erythema multiforme for several years. When conventional therapy failed to correct this condition she was treated with Transfer Factor (TF). Immunologic anomalies which had been registered previously became normal after the first application of TF. The skin lesions disappeared after the fourth dose. Six months later a new skin manifestation with immunologic alterations became apparent. They disappeared after two doses of TF. A year and a half later the patient has no lesions and the immunologic parameters, which were monitored throughout this period, are normal.
17 patients with acute and chronic symptoms of Borrelia burgdorferi infection are described. These cases, which were diagnosed within a short period of time at the Männedorf-Zürich hospital, suggest that Erythema migrans disease is likely to be frequent in Switzerland as well as elsewhere.
A study of 64 patients with erythema nodosum (EN), including 53 females, showed sarcoidosis diagnosed in 65% of the cases to be the principal cause of EN. It was followed by drug disease (10%), tuberculosis (3%), pregnancy or labor (5%), tumors (3%) and other underlying diseases (Behcet's diseases, streptococcal infection, histoplasmosis, nonspecific aorto-arteritis, chronic active hepatitis). The cause of EN could not be identified in 5% of patients only. Two or three possible etiologic factors were identified in 17%. Sarcoidosis combined with EN regressed without treatment in 82% of cases. Corticosteroid therapy was only given in cases of pulmonary lesion or other systemic manifestations. Purposeful investigation is believed to be capable of establishing etiology of EN in virtually all cases.
Three cases of Erythema Elevatum Diutinum are reported and a revision of the literature about this subject is made. The authors described extensively the general and cutaneous clinical picture. They precise the histological characteristics and mention other dermatological proceses from which this entity must be differentiated. Finally the ethiology and treatment of this clinical picture are discussed.
A distinctive acral erythema developed in four patients with myelogenous leukemia, subsequent to blood transfusions and intensive chemotherapy with cytarabine. The clinical and histopathologic features of the eruption were suggestive of a drug-induced toxic eruption. To our knowledge, only one previous similar case has been reported in the literature. For patients in whom this self-limited condition develops, reassurance should serve as the mainstay of therapy.
A 29-year-old woman from Trinidad experienced the rapid onset of extensive lesions characteristic of erythema dyschromicum perstans following an x-ray study using orally administered contrast. Eleven months later a skin biopsy specimen showed few epidermal changes but extensive incontinence of melanin pigment and marked dilatation of lymphatics in the superficial dermis. Results of a systemic evaluation were normal. The available data on this disease are analyzed and conclusions offered regarding its nature and causes.
We report a 31-year-old male patient suffering from Sweet's syndrome. The particularities of the case are the long period between onset of the disease and establishment of the diagnosis-the cutaneous lesions having persisted all the time, and the marked persistence of postlesional erythemas showing positive improvement by means of selective ultraviolet phototherapy (SUP).
A 79-year-old man with severe generalized pruritus and erythema annulare centrifugum as manifestations of his idiopathic hypereosinophilic syndrome slowly responded to long-term therapy with ketoconazole and dapsone. The skin lesions cleared within one month but the pruritus and eosinophilia required seven full months of therapy.