ABSTRACT
Resumen El granuloma actínico es una dermatosis granulomatosa poco frecuente, que se observa en adultos de mediana edad con antecedentes de fotoexposición. Su patogénesis no es clara; siendo la teoría más aceptada la radiación solar como factor disparador. Se caracteriza por placas anulares con atrofia central y bordes eritematosos elevados que son similares a los observados en el granuloma anular. Se presenta el caso clínico de una paciente de 71 años, con placas anulares en antebrazos compatibles con granuloma actínico. Se comunica el caso por lo infrecuente de esta entidad y se realiza una revisión bibliográfica.
Abstract Actinic granuloma is a rare granulomatous dermatosis, seen in middle-aged adults with a history of photoexposure. Its pathogenesis is not clear; the most accepted theory being solar radiation as a triggering factor. It is characterized by annular plaques with central atrophy and raised erythematous borders that are similar to those seen in granuloma annulare. The clinical case of a 71-year-old patient with annular plaques on the forearms compatible with actinic granuloma is presented. The case is reported due to the infrequent nature of this entity and a bibliographical review is carried out.
ABSTRACT
A 48-year-old lady presented with multiple, asymptomatic papules on upper trunk and upper extremities of 4 years duration. She gave a history of similar lesions/papules which used to heal earlier also, in a couple of weeks/months with atrophy. She had consulted many physicians and dermatologists without any relief. Biopsy from a papule showed dense nodular collection of foamy histiocytes in dermis in close proximity of neurovascular bundle. Special stain revealed absent elastic fibres in areas with infiltration. The case was diagnosed as lepromatous leprosy with elastolysis. Unusual features observed in the patient were predominant involvement of sun-exposed areas and atrophy. These signs and symptoms in the patient probably, mislead clinicians away from the diagnosis of leprosy.
ABSTRACT
Describimos el cuadro clínico de un paciente, que presenta placas y pápulas con aspecto de piel arrugada en papel de cigarrillo, localizadas en abdomen. El estudio histopatológico muestra ausencia de fibras elásticas, en una banda que compromete la porción media de la dermis. La elastolisis de la dermis media es una afección infrecuente, adquirida, idiopática o secundaria a un proceso inflamatorio previo, caracterizada por la pérdida focal de tejido elástico en la dermis media y sin compromiso extra cutáneo.
We describe a patient presenting plaques and papules -like wrinkled cigarette paper-, located in abdomen. Histopathological study shows absence of elastic fibers in a band that compromised the middle portion of the dermis. Mid dermal elastolysis is a rare, acquired idiopathic condition characterized by focal loss of elastic tissue in the middle dermis without extra cutaneous involment.
ABSTRACT
Se describe el caso clínico de una lactante de 18 meses de edad, quien fue atendida en la consulta externa del Hospital Oftalmológico "Amistad Argelia-Cuba" de la Wilaya de Djelfa en la República Democrática y Popular de Argelia, por caída de los párpados. En el examen físico se observó facies tosca, piel laxa, arrugada, caída e hiperextensible, con grandes pliegues, así como aspecto de envejecimiento, blefarocalasia y ectropion. No se detectaron alteraciones musculoesqueléticas, hernia inguinal o umbilical, ni otras manifestaciones gastrointestinales, genitourinarias o cardiorrespiratorias. Por las características clínicas se concluyó que la paciente presentaba cutis laxa.
The clinical case of an infant aged 18 months is described, who was attended in the Outpatient Department from "Amistad Argelia-Cuba" Ophthalmology Hospital of the Wilaya of Djelfa in the People's Democratic Republic of Algeria due to eyelid drooping. On physical examination coarse facies, hiperextensible and sagging, wrinkled lax skin with large folds were observed, as well as aging appearance, blepharochalasis and ectropion. There were not musculoskeletal changes, groin or umbilical hernia, or other gastrointestinal, genitourinary or cardiopulmonary manifestations. Taking into account clinical characteristics it was concluded that the patient presented with cutis laxa.
ABSTRACT
A 38-year-old woman presented with a 20-year history of yellow papules and band-like atrophy on the right neck. The lesions developed slowly and were asymptomatic. There was no history of long-term sun exposure or family history of similar diseases. Skin examination revealed multiple irregularly sized yellow papules and plaques on the right anterior neck following cleavage lines, multiple pin cap-sized perifollicular papules on the posterior right neck. Well-defined band-like atrophic patches with fine wrinkling were observed in the whole right neck, giving an aged appearance. The skin of the left neck was nearly normal. Pathological examination of biopsy specimens from the yellow papules showed a normal epidermis, scant lymphohistiocytic and melanophage infiltrates around the vessels in the superficial dermis without solar degradation. The collagen bundles in the mid dermis were slightly thickened and arranged tightly in parallel to the skin surface with the absence of inflammatory infiltrate. Verhoeff-van Giesen's staining confirmed a nearly complete absence of elastic fibers in the mid dermis as well as obvious swelling and breakage of resident scant elastic fibers. Von Kossa's staining was negative. Based on the above findings, the diagnosis was made as unilateral mid-dermal elastolysis.
ABSTRACT
Fibroelastolytic papulosis of the neck (FEPN) encompasses a spectrum of two disorders that were previously reported as pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE) and white fibrous papulosis of the neck (WFPN). The clinical presentation of FEPN is asymptomatic to mildly pruritic whitish-yellow papules that may coalesce into cobblestone patterned plaques that resemble pseudoxanthoma elasticum (PXE). The histology is characterized by a decrease or loss of elastic fibers in the papillary dermis and this is sometimes accompanied by a minimal or nodular increase of dermal collagen fibers. We report here on a 28-year-old Korean man with asymptomatic, multiple, skin-colored to slightly yellowish, match-head sized, cobblestone-patterned papules on the neck, and these were histologically consistent with FEPN and the papules showed slightly increased dermal collagen associated with decreased and fragmented elastic fibers, elastin and tropoelastin. The pathogenesis of FEPN in this case might have been related with mild dermal inflammation, followed by fragmentation, elastolysis and increased dermal collagen.
Subject(s)
Adult , Humans , Collagen , Dermis , Elastic Tissue , Elastin , Inflammation , Neck , Pseudoxanthoma Elasticum , TropoelastinABSTRACT
Pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-like PDE) is a rare acquired elastolytic disorder that clinically resembles pseudoxanthoma elasticum. The pathogenesis of this disease is uncertain, but it seems to be related to intrinsic aging, abnormal elastogenesis, a genetic predisposition and exposure to ultraviolet radiation. It generally develops on the sides of the neck and supraclavicular regions in elderly persons. Histologically, the characteristic features are the loss of elastic fibers in the papillary dermis and no solar elastosis. We report here on a case of PXE-like PDE in a 26-year-old female whose skin lesion developed 9 years previously. Despite the preferential occurrence at an old age, our patient was young.
Subject(s)
Adult , Aged , Female , Humans , Aging , Dermis , Elastic Tissue , Genetic Predisposition to Disease , Neck , Pseudoxanthoma Elasticum , SkinABSTRACT
Fibroelastolytic papulosis is proposed as the diagnostic term for cases previously identified as pseudoxanthoma elasticum-like papillary dermal elastolysis and white fibrous papulosis of the neck. Clinical presentation of fibroelastolytic papulosis is asymptomatic to mildly pruritic whitish-yellow papules that may coalesce into cobblestone- patterned plaques resembling pseudoxanthoma elasticum. Histology is characterized by normal, decreased, or absent elastic fibers in the papillary dermis sometimes accompanied by a thickening of collagen fibers. We report a 60-year-old woman with flesh to yellowish colored papules with cobblestone appearance, which were histologically consistent with fibroelastolytic papulosis.
Subject(s)
Female , Humans , Middle Aged , Collagen , Dermis , Elastic Tissue , Neck , Pseudoxanthoma ElasticumABSTRACT
Pseudoxanthoma elasticum-like papillary dermal elastolysis is a disorder of the dermis, characterized by clinical features similar to pseudoxanthoma elasticum and the pathologic features of elastolysis limited to the papillary dermis, such as sun-protected, intrinsically-aged skin. A 64-year old woman presented with a 7 year history of a localized skin lesion on the back of her neck. Physical examination revealed skin to yellow colored, non-follicular grouped uniform papules, sized 5~8 mm, and coalescent plaques localized below the hair line. Histopathologic and immunohistochemical findings of decrease and elastolysis of the elastic fibers in the papillary dermis confirmed the diagnosis. In this article, we report a case of pseudoxanthoma elasticum-like papillary dermal elastolysis which presented with typical clinical and pathologic features.
Subject(s)
Female , Humans , Middle Aged , Dermis , Diagnosis , Elastic Tissue , Hair , Neck , Physical Examination , Pseudoxanthoma Elasticum , SkinABSTRACT
Pseudoxanthoma elasticum (PXE)-like papillary dermal elastolysis (PDE) is an unusual acquired elastolytic disorder that clinically resembles PXE but shows partial or complete loss of the elastic network in the papillary dermis. PXE-like PDE is characterized by the multiple yellowish nonfollicular papules, small in diameter, on the antecubital fossae, flexor aspect of the forearms, supraclavicular region, sides of the neck, and lower abdominal area. The multiple papules coalesce to form plaques with a cobble stone appearance resembling "plucked chicken skin". No systemic involvement has been noted in this disorder. Histopathologically, Hematoxylin-eosin stain shows nearly normal finding, but elastic stain shows partial or complete loss of elastic fibers focally in the papillary dermis. We report a case of pseudoxanthoma elasticum-like papillary dermal elastolysis with typical clinical and pathological features.
Subject(s)
Chickens , Dermis , Elastic Tissue , Forearm , Neck , Pseudoxanthoma ElasticumABSTRACT
Annular atrophic lichen planus(AALP) is an uncommon variant of lichen planus that results from elastolytic activity of inflammatory cells with formation of areas of localized acquired cutis laxa. The annular configuration of the lesions resulted from peripheral enlargement with simultaneous central clearing. Histologically, it shows the feature of lichen planus in the active border of the lesion and a pattern of resolved lichen planus in its center. Elastic fibers had been destroyed in the papillary dermis both in the border and in the center of the lesion, resulting in an atrophic appearance of the lesion. We report a case of annular atrophic lichen planus which has never been reported in Korea.
Subject(s)
Cutis Laxa , Dermis , Elastic Tissue , Korea , Lichen Planus , LichensABSTRACT
Fibroelastolytic papulosis of the neck(FEPN) was introduced by Balus et al in 1997. Before this term was used, white fibrous papulosis of the neck(WFPN) and pseudoxanthoma elasticumlike papillary dermal elastolysis(PDE) had differently been used. These two disease entities had clinical similarities such as asymptomatic, white to yellow discolored, papular eruptions on the neck in elderly persons. As names implicated, however, they showed variable histological findings, for examples, fibrosis in WFPN or elastolysis in PDE or both in FEPN. Taken into ac-count together, instead of using WFPN or PDE separately, FEPN might be preferred to describe these two kinds of skin diseases. We report a case of a 36-year-old male patient with papules compatible with FEPN, whose biopsy mainly showed elastolysis.
Subject(s)
Adult , Aged , Humans , Male , Biopsy , Fibrosis , Neck , Skin DiseasesABSTRACT
Using SEM, we have observed surface structures of atherosclerotic lesions of human aortas obtained from autopsies ranging from 59 to 84 years of age (5 males and 4 females). We have found four major interesting features on the lumenal surface of the aortas: 1) blood cells including leukocytes adhering to the endothelial surface, 2) a de-endothelialized surface showing both elastogenesis and elastolysis, 3) abundant cholesterol-ester crystals in extracellular spaces, and 4) cave-like structures possibly suggesting new capillarization in the thrombotic atherosclerotic plaques. We concluded that SEM has a great value in revealing more interesting surface structures if morphological studies are previously done in detail so that the characteristic shapes can be identified, and perhaps then meaningful interpretations can be made on the mechanism of human atherogenesis.
Subject(s)
Aged , Aged, 80 and over , Humans , Male , Aorta/ultrastructure , Aortic Diseases/pathology , Arteriosclerosis/pathology , Microscopy, Electron, Scanning , Middle AgedABSTRACT
We have examined 24 human aortas aged 46 ~ 90 years obtained from autopsies. Most exhibited gross lesions of some degree on the lumenal surface. Using hot alkaline treatment (0.1 N NaOH) at 70 ~ 75degreeC for 5 hours, we extracted and quantitated elastin portions from the aortic wall in 3 different segments (UTA = upper thoracic aorta, LTA = lower thoracic aorta, AA = abdominal aorta). We have found UTA had 70.6% +/- 1.39 (SE), LTA 61.6% +/- 1.94 (SE), AA 49.2% +/- 1.84 (SE) elastin respectively based on wet weight. The differences between segments are statistically significant (p < 0.05, 0.025). However, there is no significant correlation between the age of the patients and the relative amounts of elastin in each segment. We have also observed the structure of elastin in the internal elastic lamina (IEL) and tunica media (TM) with SEM (scanning electron microscopy), and discovered that the IEL shows various forms of elastolysis-broken sheets, discontinuity, various sizes of lumps, vesicles, and possible newly formed elastin in the aortic lesions (Song and Roach submitted to YMJ). From these studies we conclude that elastin in the aortic wall remains well balanced quantitatively with age in spite of evidence suggesting vigorous degeneration and regeneration in the atherosclerotic lesions.