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Las glándulas de Bartolino, también conocidas como glándulas vestibulares mayores, son responsables de un gran número enfermedades ginecológicas de la vulva.; Los quistes y abscesos de la glándula de Bartolino pueden presentarse con síntomas diferentes y el manejo quirúrgico requerido debe ser marsupialización y no incisión y drenaje. Aunque se demostró que el procedimiento de incisión y drenaje es relativamente rápido y fácil de realizar y que la tasa de curación es rápida entre los pacientes que reciben este tipo de técnica quirúrgica, sin embargo se ha demostrado que la tendencia de recurrencias ha aumentado. Objetivo: realizar una revisión bibliográfica actualizada sobre el tema y su mejor manejo. Fuente de Datos: se realizó una búsqueda bibliográfica sistemática en la base de datos PubMed, Hinari y google académico desde el año 1985 al año 2020. Conclusiones: El mejor nivel de atención para las mujeres con quistes o abscesos de la glándula de Bartolino nunca podría lograrse sin la colaboración interprofesional.

Bartholin's glands, also known as greater vestibular glands, are two mucus-secreting glands located in the posterolateral wall of the vagina opening. The function of these glands is to produce mucus that moisturizes the vestibule of vagina. When the outflow path from the gland is blocked, a cyst forms. When the abscess is formed usually it is infections with a polymicrobial background.The most common symptoms of a Bartholin's gland cyst or abscess include pain in the vulva, swelling, difficulty walking, dyspareunia, and fever.The procedure of choice in this case is surgery. The operator can choose from among many possible options, among others there is simple incision and drainage of the cyst, marsupialization, a Word catheter, silver nitrate application, alcohol sclerotherapy, excision and laser CO2 and, as a last resort, surgical removal of the gland.The aim of this study is to review the most common surgical procedures used in Bartholin's gland cyst or abscess.

Leucorrhea is a female vaginal secretion. It is a mixture of vestibular glands, cervical glands, endometrial secretions, vaginal mucosal exudates, and shedding vaginal epithelial cells. Routine examination of leucorrhea is a common check in gynecology.

In the present study, a detailed histological description of the female reproductive tract of South American fur seal (Arctophoca australis) pups has been conducted. The uterine tube was covered by cuboidal to columnar epithelium; nerve fibers were present in the mesosalpinx and beneath the muscular layer. The uterus was bipartitus; the endometrial surface of the horns was lined by a simple cuboidal or columnar epithelium with deep tubular glands; caudally ("the transition area"), the epithelium changed to pseudostratified columnar, few tubular glands were present and the myometrium increased in width. A bistratified epithelium internally coated the uterine body, whereas it changed to cylindrical stratified epithelium with a highly vascularized lamina propria and a strong muscular layer in the cervix; no endometrial glands were observed in this region. From the transition area of the uterus to the vagina there were several nerve fibers and ganglia belonging to the uterovaginalis plexus. In the vestibule, hymenal folds were poorly developed; adnexa structures included the major vestibular glands and a neurovascular structure similar to the vestibular bulb. Minor vestibular glands were associated with the clitoris. The skin of the perineum was lined by a keratinized stratified epithelium, pigmented, with sebaceous glands, sweat glands and hair follicles. This is the first detailed histological description of the reproductive tract of South American fur seal pups, including the glandular adnexa and nerve structures. These results contribute to the reproductive biology in Pinniped species, and give a better understanding of the utero-placental perfusion mechanism during diving. Anat Rec, 2017. © 2017 Wiley Periodicals, Inc. Anat Rec, 300:600-613, 2017. © 2016 Wiley Periodicals, Inc.

EnglishTumors affecting Bartholin’s gland are considered rare in human medicine; there are few reports in the veterinary literature, with descriptions occurring only in cows. This article described the clinical and pathological findings associated with Bartholin’s gland adenoma in a goat. Clinically, a 7-year-old pregnant Saanen goat presented bilateral enlargement of the vulva that did not regress spontaneously after parturition. Grossly, these vulvar masses were multilobulated, contained cystic areas from which oozed a whitish fluid. Histopathology revealed an adenoma characterized by the proliferation of irregularly shaped neoplastic epithelial cells that formed tubular to glandular-like structures. These neoplastic cells demonstrated moderate anisokaryosis and evident nucleoli. The intratumoral proliferation index (PI) was estimated by immunoreactivity with the protein ki-67. Further, the glandular-like structures produced a Periodic Acid-Schiff positive secretion. A diagnosis of Bartholin’s gland adenoma was established due to the anatomic location of the neoplastic growths, the histopathological features, and the PI of the tumor. Key words: Histopathology; ki-67 protein; adenoma; major vestibular glands portuguesTumores que afetam a glândula de Bartholin sao considerados raros em humanos e ha poucos relatos na medicina veterinaria, sendo descrito somente em vacas. Este artigo descreve os achados clinicos e patologicos associados ao adenoma na glândula de Bartholin em uma cabra. O animal da raca Saanen, com sete anos de idade e gestante apresentava aumento de volume bilateral na regiao da vulva, que nao regrediu apos o parto. Macroscopicamente, a massa era multilobulada, com presenca de varios cistos preenchidos por uma secrecao esbranquicada. A histopatologia revelou um adenoma, caracterizado por proliferacao de celulas epiteliais de formato irregular que formavam estruturas tubulares semelhantes a glândulas. Estas celulas neoplasicas apresentavam anisocariose moderada e nucleolos evidentes. O indice de proliferacao celular intratumoral (PI) foi estimado por imunorreatividade a proteina ki-67. Alem disso, a secrecao produzida por essas estruturas do tipo glandular, foi positiva na coloracao do Acido Periodico-Schiff. O diagnostico de adenoma da glândula de Bartholin foi estabelecido pela localizacao anatomica dos tumores neoplasicos, caracteristicas histopatologicas e a PI do tumor. Palavras-chave: histopatologia; ki-67; adenoma; glândulas vestibulares maiores

The authors report a case of lymphoepithelioma-like carcinoma originating in the Bartholin's gland in a 52-year-old woman. The tumor showed distinct morphologic features including variably sized and shaped nodules and syncytial sheets of large epithelial cells that contained large round nuclei with vesicular chromatin and prominent nucleoli and that were permeated and surrounded by small, well-differentiated lymphocytes and plasma cells. Focally, the neoplastic nodules blended with residual acini of the Bartholin gland. In the transitional areas, there were few acini that evidenced squamous cell metaplasia with partial or complete replacement of mucinous cells by squamous epithelium that showed dysplastic changes. In addition, some ducts in the transitional areas manifested lymphocyte infiltration of their walls. The uninvolved residuum of the Bartholin gland was unremarkable except for a few ruptured ducts or acini with extravasated stromal mucin. Immunohistochemically, the tumor cells were positive for p16, as were acini with metaplastic and dysplastic changes. Neither Epstein-Barr virus nor human papillomavirus was identified in the lesional tissue by polymerase chain reaction. This seems to be the first full-documented report of lymphoepithelioma-like carcinoma originating in the Bartholin gland.

Objective The aim of this study was to investigate the dried secretion of human major vestibular glands in order to establish its crystallographic pattern and to compare the data with those obtained for other human genital biopolymers. Study design After air drying, samples were examined comparatively under transmitted and polarized light. At first sight, dehydrated vestibular fluid exhibits a fern-like crystallographic pattern very similar in appearance to those described in mid-cycle cervical mucus and bulbo-urethral fluid. Results Dendritic structures fill the central space of all preparations, prolonged by apparently amorphous peripheral fucus-like expansions. Spherulitic interdendritic crystalline microstructures (ICMs) can be considered a constant feature of dried vestibular exudate. In contrast with dendritic formations, fucus-like expansions and isolated spherulites are anisotropic under polarized light. Anisotropy appears to be the guise of a luminescent border lining the dendrites or bright nodules shining on a dark background. Conclusion The study confirms the close physico-chemical proximity of vestibular secretion, mid-cycle cervical mucus and bulbo-urethral fluid. However, if isotropic dendritic formations and anisotropic structures are grossly similar, the number and size of the anisotropic ICMs are typical of human vestibular secretion. The different patterns of ICMs observed in these three human biological hydrogels demonstrate differences in the salt concentrations.

Summary The presence of neuroendocrine (NE) cells producing biogenic amines and hormonal peptides has been investigated in the vestibular glands of the genital tracts of cows and pigs using immunohistochemistry. NE cells containing chromogranin A-, serotonin-, cholecystokinin- and somatostatin-immunoreactive material were found in both major and minor vestibular glands. Such cells were numerous, scattered in the acini and excretory duct epithelium, small in size and rounded, triangular or bipolar in shape. The function of the NE vestibular cells has been related to the secretory activity of the glands and to a sexual climax induction mechanism involving the stimulation of 5HT 3 receptors of vestibular nociceptor nerve fibers. The role of NE cells in small cell carcinomas of the vestibular glands is a topic for further investigation owing to possible parallelism between this type of tumor and the small cell carcinoma of the human prostate.

The vulva, which is the external genitalia of the female, collectively consists of the mons pubis, labia majora and minora, clitoris, vestibule, greater (Bartholin) and lesser vestibular glands, and vaginal opening (introitus). This chapter describes the gross and microscopic anatomy of the adult, as well as the embryological derivation of the region’s multiple components.

The vestibular gland and egg masses of Dendrodoris nigra (Gastropoda: Opisthobranchia: Nudibranchia) were investigated histologically and ultrastructurally. A detailed description of the ultrastructure of the gland and the egg masses is given, and the presence of symbiotic bacteria in the vestibular gland and identical-appearing bacteria in the egg masses is reported for the first time. Hypotheses about the function of these bacteria in the reproductive system of D. nigra are presented.

The authors report two examples of nodular hyperplasia of the Bartholin gland. Each occurred in the vulva, close to the introitus of women aged 33 and 49 years, who presented with slightly painful lesions that were clinically thought to be cysts. Grossly, both masses exhibited a multilobular architecture, were elastic, were gray in color, and measured 4 cm and 3.2 cm in greatest dimension. On microscopic examination, the lesions looked similar and exhibited an increased number of secretory acini, with maintenance of the normal duct-acinar relationship--features compatible with nodular hyperplasia. Rare dilated ducts were observed, and intraluminal inspissated secretions were occasionally seen. In one case, there were a focal mild inflammatory infiltrate, squamous metaplasia of larger ducts, and rare ruptured ducts with extravasated stromal mucin. Clonality analysis performed in one case revealed a monoclonal pattern, suggesting that the lesion may be a neoplastic process rather than simple reactive hyperplasia.

The topographical anatomy of the bulbo-clitoral organ was already partially covered in the first chapters, with its morphology and structure having been observed in situ, i.e. within the external feminine genitalia. However, several points remain to be clarified and very specific connections have yet to be studied. We will discuss the connections with the vestibule, labia and greater vestibular glands; the connections with the urethra, the vagina and the perineal membrane; and finally, the relations with the pelvic diaphragm (levator ani muscles).

The chromodond nudibranch Tyritma nobiiis, described originally by Bergh, 1898 from Chile, has been collected on the Atlantic coast of Patagonia (Argentina) We have made a redescription which includes the presence of a vestibular gland, absent in the Bergh's description, and provided information on the biology of the living animal* Tynnna nobihs was compared with other nominal species assigned to the genera Cadlina and Tyrinna. Tynnna pusae Marcus, 1959 was synonymised with T. nobdis, and Cadlina bumayi Ortea, 1988 was included in Tynnna. The presence of a ramifying vestibular gland in T nobiiis, was discussed in relation to phylogeny of the family Chromodorididae

Spindle cell epithelioma is a very rare benign tumour of the vagina, which contains epithelial and mesenchymal components and co-expresses the markers for both. It has its origin in the epithelial cells of the remnants of the vestibular gland. The presence of glandular structures and the pattern of immunostaining, help in the differentiation of these tumours from the other common vaginal tumours.

Vulvar vestibulitis is a recently described clinical condition of unknown etiology. Human papillomavirus (HPV) infection has been implicated as a causal pathogen by some investigators. Because reports on this entity are few, we studied the pathologic features of vestibulectomy specimens from 36 women with a clinical diagnosis of vulvar vestibulitis. The patients' ages ranged from 19 to 53 years (mean, 31 years). Bilateral excisions were performed in 34 patients and a unilateral excision in two. Minor vestibular glands or ducts were identified in 28 patients (78%). Chronic inflammation was present in all specimens. The inflammatory infiltrate consisted predominantly of T lymphocytes and plasma cells with smaller numbers of B lymphocytes. Lymphoid follicles were present in four patients, ulcers in two. The predominant site of inflammation was the squamous mucosa, especially the lamina propria, where the degree of inflammation was graded as mild in 11 patients, moderate in 21 patients, and severe in four patients. To a lesser degree, the inflammation also typically involved the periglandular/periductal connective tissue of the minor vestibular glands, where it was graded as mild in 25 patients and moderate in three patients. Foci of squamous metaplasia were observed in 27 of 28 patients with demonstrable vestibular ducts or glands. Nodular hyperplasia of a vestibular gland (so-called "adenoma") occurred in one patient. In six selected cases, fungi were not identified on silver stains. Morphologic findings of HPV infection, such as koilocytotic atypia or squamous dysplasia, were absent. In-situ hybridization studies for HPV types 6, 11, 16, 18, 31 and 35 were negative in all 36 cases. We conclude that (a) vestibulectomy specimens from patients with a clinical diagnosis of vulvar vestibulitis typically show a chronic inflammatory cell infiltrate that predominantly involves the mucosal lamina propria and periglandular/periductal connective tissue and (b) a pathogenetic role for HPV types 6, 11, 16, 18, 31 and 35 is not substantiated.

Examination of the vagina must include assessment of the minor vestibular glands and urethral meatus. Vaginal mucosal lesions related to papillomavirus infection and neoplasia are best discovered by the use of acetic acid wash followed by the application of Monsel's solution.

The hypothalamic peptide orexin A (oxA) binds specifically the G-protein–coupled orexin receptor 1 (ox1R). It is involved in many physiological functions including the regulation of food intake, sleep–wake cycle, arterial blood pressure, heart rate, and sexual behavior. The localization of oxA in adrenal glands, stomach, bowel, pancreas, and testis has recently been assessed. Here, we provide the first evidence for the expression of oxA and ox1R in the vestibular glands of mammalian genital tract. Anat Rec, 2009. copy; 2008 Wiley-Liss, Inc.

The presence of neuroendocrine (NE) cells producing biogenic amines and hormonal peptides has been investigated in the vestibular glands of the genital tracts of cows and pigs using immunohistochemistry. NE cells containing chromogranin A-, serotonin-, cholecystokinin- and somatostatin-immunoreactive material were found in both major and minor vestibular glands. Such cells were numerous, scattered in the acini and excretory duct epithelium, small in size and rounded, triangular or bipolar in shape. The function of the NE vestibular cells has been related to the secretory activity of the glands and to a sexual climax induction mechanism involving the stimulation of 5HT(3) receptors of vestibular nociceptor nerve fibers. The role of NE cells in small cell carcinomas of the vestibular glands is a topic for further investigation owing to possible parallelism between this type of tumor and the small cell carcinoma of the human prostate.

Carcinomas of the vulva are a relatively common gynecologic malignancy of the female genital tract and represent 3–5% of all diagnosed female genital malignancies. Squamous cell carcinoma comprises 90% of these tumors (Canavan and Cohen, 2002). Primary vulvar glandular neoplasms, on the other hand are rare, accounting for 1% of all malignant neoplasms of the vulva. Most of them are associated with extra-mammary Paget's disease (Doley et al., 2001). Vulvar adenocarcinomas may originate from adnexal structures, mammary-like glands, vestibular glands, and Bartholin's glands. More common neoplasms include papillary hidradenomas, Bartholin's carcinoma, and Paget's disease of the vulva. Adnexal adenocarcinomas of the vulva are exceedingly rare, accounting for less than 0.1% of all vulvar carcinomas and as a result the literature is limited. The diagnosis of primary vulvar adenocarcinoma remains difficult for pathologists and clinicians. We report a primary vulvar adenocarcinoma of adnexal origin and discuss the diagnosis as well as the treatment of this unusual and uncommon neoplasm.

The dependency of the secretory function of the feline major vestibular gland (Bartholin's gland) on the ovarian steroid hormones was studied histochemically. After estrogen (estradiol-17 beta or diethylstilbestrol), progesterone or a combination of these was administered to cats which were previously ovariohysterectomized, the major vestibular glands were removed, embedded in paraffin, sectioned, and stained by alcian blue, periodic acid-Schiff or peroxidase-labeled lectin (peanut agglutinin and wheat germ agglutinin). The vividly positive reactions to the stainings applied were observed in the secretory epithelial cells of the major vestibular glands in the estrogen treated animals. The dependency of the secretory function of the major vestibular glands on the estrogen was demonstrated in this study.

Sections of vulvar tissue containing minor vestibular glands (15 sections from 14 women with vulvar vestibulitis syndrome and six control sections from five asymptomatic women) were immunohistochemically stained with antibodies against chromogranin, synaptophysin, serotonin, and CXCR2. The degree of inflammation in vestibular tissue from women with and without vulvar vestibulitis syndrome was not significantly different. All vulvar minor vestibular glands contained neuroendocrine cells that expressed chromogranin to some degree. Only one case failed to express synaptophysin. The number of cells expressing chromogranin and synaptophysin was the same regardless of the degree of inflammation. However, moderate to severe inflammation was associated with a statistically significant increase in the number of cells expressing serotonin (p < 0.001) and CXCR2 (p < 0.02). It is concluded that neuroendocrine cells are present within minor vestibular glands of the vulva. The number of cells expressing the inflammatory mediator serotonin and CXCR2, the shared interleukin-8 receptor, are upregulated with inflammation. Chronic inflammation is a normal finding in vestibular tissue and does not serve as a histologic marker for vulvar vestibulitis.

A 33-year-old Japanese woman presented with a polypoid 2.5 x 2.5 x 1.9 cm mass located in the posterior wall of the lower vagina. Microscopically, the tumour was composed of benign epithelial and stromal-type elements. Predominant epithelial elements were mucinous glands with squamous metaplasia and islands of mature squamous epithelium. The stromal-type cells showed reticular or short fascicular patterns with a transition to the epithelial elements. There was no dual epithelial-myoepithelial combination in the glands as seen in so-called mixed tumours (pleomorphic adenomas) of the salivary gland. Immunohistochemically, the epithelial elements were strongly positive for cytokeratin, PKK1 and epithelial membrane antigen, while the stromal-type cells co-expressed PKK1 and vimentin. Staining for S-100 protein, muscle actin, alpha-smooth muscle actin, desmin, and CD34 was uniformly negative in the tumour cells. The DNA pattern was diploid. The patient is alive and well without recurrence for 50 months after excision. These results indicate that an epithelial cell proliferation, probably of the remnant vestibular gland, plays a major role in the development of mixed tumours of the vagina.

The external female genitalia include the mons pubis, labia majora and minora, prepuce, frenulum, clitoris, and vestibule. The orifices of the Skene and Bartholin glands, as well as those of the minor vestibular glands and the urethral meatus, open into the vestibule (Fig. 2.1). After adren-arche, the mons pubis and lateral aspects of the labia majora acquire increased amounts of subcutaneous fat and develop the coarse, curly surface hair characteristic of the adult. During adolescence, the labia acquire a characteristic hyperpigmentation and the clitoris undergoes some enlargement. The entire vulva, with the exception of the vulvar vestibule, is covered by keratinized, stratified squamous epithelium.95 The labia majora contain both smooth muscle and fat, whereas the labia minora are devoid of adipose tissue but are rich in elastic fibers and blood vessels.171 Within the lateral aspects of the labia majora, sebaceous glands associated with hair follicles are evident. Toward the medial aspects of the labia majora the hair follicles are absent and sebaceous glands open directly to the surface epithelium. Similar sebaceous gland elements are seen on the perineum posterior to the vestibule. The labia minora typically do not contain glandular elements, although sebaceous glands without hair follicles may be found within the interlabial sulcus and at the lateral base of the labia minora.

Vulvar vestibulitis syndrome is a constellation of symptoms and findings involving and limited to the vulvar vestibule that consists of: (1) severe pain on vestibular touch to attempted vaginal entry, (2) tenderness to pressure localized within the vulvar vestibule, and (3) physical findings confined to vulvar erythema of various degrees. Histopathologic findings are consistent with a chronic, nonspecific inflammatory response that is occasionally associated with metaplasia of the minor vestibular glands. The cause is likely multifactorial, and to date the syndrome has been seen in association with subclinical human papillomavirus, chronic recurrent candidiasis, chronic recurrent bacterial vaginosis, chronic alteration of vaginal pH, and the use of chemical and destructive therapeutic agents. Therapy is directed at elimination of these symptoms. When symptoms are unrelieved, a surgical approach consisting of vestibulectomy with vaginal advancement has a high rate of success.

Bartholin’s duct cyst is a sequel of occlusion of the main duct leading to the accumulation of secretion. It presents as a slightly tender, round to ovoid swelling at the lower part of labium majus. The size usually varies from 3 to 6 cm with an average of 5 cm. A larger cyst attaining 12.5 cm and weighing up to 183 g has been described (Aborjaily et al. 1958). The cyst is more commonly unilateral, but bilateral cysts are sometimes seen. Depending on whether the main or smaller ducts are obstructed, the cyst may be uni- or multilocular; the unilocular cyst usually results from main duct obstruction. A collar-button-type distension generally occurs proximal to the obstruction of the main duct (Tancer 1965). The common cause of obstruction is Bartholin adenitis due to gonococcal infections. Infections by other organisms have also been incriminated. Mechanical trauma can cause distortion of the duct and thereby hindrance to the outflow of secretions. Rarely, malignancy of the organ can manifest as a cystic swelling in elderly women. The posterior third of vulva is the usual site for these cysts.

Larval behavior and metamorphosis in Parasmittina nitida morphotype B from the Gulf of Mexico has been studied. The larvae have two basic types of movement: (1) a clockwise-counterclockwise movement about the aboral-oral axis of the lobular larval form resulting in either slow horizontal or rapid vertical movement, and (2) a directed horizontal movement of the creeping larval form, whereby either the oral lobe is pressed against the substrate or the aboral-oral axis is tilted forward. In both forms, the vibratile plume of the pyriform organ complex extends the leading edge of the larva. Metamorphosis was observed with Nomarski differential interference microscopy in living specimens and with scanning electron microscopy in fixed specimens. Polypide development— in particular, the formation and diminution of the nutritive mass, the differentiation of the polypide rudiment, diaphragm, vestibular glands, operculum, major components of the musculature and alimentary canal, and the early stages of astogenetic growth—is described. The tata ancestrula of this species is characterized by a frontal wall calcified distally to the aperture, which is surrounded by nine erect spines. The polypide feeds actively within seven to eight days after the onset of larval attachment and metamorphosis under laboratory conditions of 22°C.

A case of benign mixed tumor of the vulva and a case of carcinoma arising in a similar benign mixed tumor of the vulva are reported. Both mixed tumors were of salivary gland type and probably arose in the Bartholin's gland. Only three previous cases of benign mixed tumor of the vulva have been documented in the literature. Two arose in the vestibular glands, and the third arose in cutaneous sweat glands. No case of carcinoma arising in a benign mixed tumor of the vulva has been previously documented.

Intact multiparous bovine reproductive tracts at various stages of the oestrous cycle were examined post mortem. The mean (±SD) length of the vestibule was 115·6 ± 12·82, volume of the suburethral diverticulum 4·0 ± 1·33 ml, and weight of the major vestibular gland was 6·9 ± 2·58 g. 26·5% of reproductive tracts had macroscopically visible cysts along the major vestibular gland duct. At all stages of the oestrous cycle and at all positions examined, the vestibular epithelium was stratified squamous containing variable numbers of lymphocytes and polymorphonuclear leucocytes. Lymphatic nodules associated with the epithelium, seen throughout the vestibule, were most commonly observed around the clitoris. Histochemically, the branched mucus-secreting tubules of the major vestibular glands contained neutral, sialated and sulphated mucins.

Four examples of adenocystic (pseudoadenomatous) basal cell carcinoma of vestibular glands of vulva are reported. Three of these arose in the region of the Bartholin gland whereas the exact vulvar location of the fourth was not described but probably was from a major rather than a minor vestibular gland. The tumor is a histologic entiry and consists of small uniform basal cells with pseudoglandular and microcystic formations and usually considerable hyalinized acellular stroma. It pursues a slow insidious course, is prone to local recurrence and perineural spread, and has the ability to metastasize by vascular channels. The neoplasm should not be likened to mixed tumor of the salivary gland or confused with basal cell carcinoma of the skin, or adenocarcinoma of the vestibular glands.

Forty-one cases of vulvar vestibulitis syndrome are presented. All cases were studied to provide a histopathological description of this condition in terms of location, intensity, and characterization of the inflammatory response. Alterations of the normal histology of minor vestibular glands forming vestibular clefts are described. The association of this condition with other disease processes, and special studies done to elucidate an etiology, are discussed.