Trichodysplasia spinulosaassociated polyomavirus uses a. Virusassociated trichodysplasia spinulosa vats is a cutaneous eruption of spiny papules predominantly affecting the face that is associated with a distinctive histologic picture of abnormally. Trichodysplasia spinulosa successfully treated with adapalene. Currently, the patient is being treated with topical cidofovir cream 1% with some improvement. Trichodysplasia spinulosa ts is a recently described rare disease of. Multiple minute digitate hyperkeratosisa peculiar entity. Ts is a rare follicular skin disease caused by human polyoma virus, which was discovered in 2010. Trichodysplasia spinulosaassociated polyomavirus tsv and. The diagnosis of trichostasis spinulosa ts can be made clinically without obtaining a biopsy specimen. Trichodysplasia spinulosa is characterized by active. Merkel cell polyomavirus, trichodysplasia spinulosa polyomavirus, human polyomavirus 6 and 7, and malawi polyomavirus are shed from the skin, and merkel cell polyomavirus, trichodysplasia spinulosa polyomavirus, human polyomavirus 6 and. Observations we report a case of ts in a 48yearold african american man after renal transplant. Polyomaviruses pyvs are ubiquitous viruses that infect a variety of hosts, e. Trichodysplasia spinulosa associated polyomavirus primerdesign ltdtm 150 tests genesig standard kit for general laboratory and research use only quantification of trichodysplasia spinulosa associated polyomavirus genomes.
Histopathology finds dilated and dysmorphic hair follicles and. Previous studies have shown strong mcvspecific tcell responses, while no data exist on tcell immunity against tsv. Histopathology of the affected skin showed distended. Biopsy from the left flank shows a infundibular plugging with keratotic debris, an aborted hair shaft hematoxylineosin stain, x 100 original magnification, and b enlarged, irre gular trichohyalin granules of the inner root. The recent discovery of genomic dna from a new polyomavirus named trichodysplasia spinulosaassociated polyomavirus in ts lesions and good clinical response to cidofovir strengthens the hypothesis of a viral etiology for the disease. Trichodysplasia spinulosaa rare complication in immunosuppressed patients it is a vital part of treatment regimes following organ and bone marrow transplants. Trichostasis spinulosa ts is a condition where instead of one hair protruding from a hair follicle, a bundle or bush of hair come out of a single follicle.
Trichostasis spinulosa frequently is discovered as an incidental finding, and often it is confused with keratosis pilaris or acne comedones. Trichostasis spinulosa lesions appear as numerous tiny open pores filled with multiple tiny short hairs, usually only visible with a magnifying glass. Trichodysplasia spinulosa successfully treated with. However, the use of immunosuppressive drugs has been shown to cause infections with common and unusual pathogens. Trichodysplasia spinulosa can be diagnosed clinically or by performing a skin biopsy. Trichodysplasia spinulosa ts is a recently described rare disease of immunosuppressed patients characterized by folliculocentric papules and keratin spicules. Pathology outlines virus associated trichodysplasia spinulosa. Media in category trichodysplasia spinulosa this category contains only the following file. Additional molecular studies identified several strains of the trichodysplasia spinulosa associated polyomavirus infecting multiple tissues of the patient, confirming the systemic nature of.
Trichodysplasia spinulosaassociated polyomavirus tspyv is a new member of the polyomaviridae that was discovered in 2010. Trichodysplasia spinulosa polyomavirus in respiratory tract of immunocompromised child article pdf available in emerging infectious diseases 249. Volume 2014, issue 465, part 2, septemberoctober 2014, page 18. This is a case report of an immunocompromised individual who presented with progressive alopecia, friable follicular spinous processes, and erythematous, indurated papules. May 22, 2007 trichodysplasia spinulosa is a cutaneous viral infection of immunosuppressed patients that causes abnormal hair follicle maturation. What is the treatment for trichodysplasia spinulosa. Neurofilamentis superior to cytokeratin 20 in supporting cutaneous origin for neuroendocrine carcinoma. Virusassociated trichodysplasia spinulosa vats is a cutaneous eruption of spiny papules predominantly affecting the face that is associated with a distinctive histologic picture of abnormally maturing anagen follicles with excessive inner root sheath differentiation and hyperkeratotic infundibula. Aug 05, 2016 trichodysplasia spinulosa ts is a recently described rare disease of immunosuppressed patients characterized by folliculocentric papules and keratin spicules. Trichodysplasia spinulosa also known by many other names, including viralassociated trichodysplasia spinulosa, viralassociated trichodysplasia, pilomatrix dysplasia and ciclosporininduced folliculodystrophy, although the last is a misnomer is a rare cutaneous condition that has been described almost exclusively in immunocompromised patients, usually organ transplant recipients, on. On the basis of knowledge regarding other polyomaviruses, we anticipated that infections with trichodysplasia spinulosaassociated polyomavirus tsv occur frequently and become symptomatic only. There was madarosis of the eyebrows and a leonine facies appearance. The disease is characterized by development of follicular papules and keratin spines spicules predominantly in the. Apart from ts being an extremely rare disease, little is known of its epidemiology.
Clinical appearance and histology of the trichodysplasia spinulosa. May 01, 2007 summary we report two boys with trichodysplasia spinulosa associated with chemotherapy for acute lymphocytic leukaemia. Stanoszeklm1, chan mp1,2, palanisamyn3, carskadons3. European perspective on human polyomavirus infection, replication and disease in solid organ. Trichodysplasia spinulosa was first coined and described in 1999 by haycox and colleagues. Trichodysplasia spinulosa is a rare disease that occurs in the setting of immunosuppression, associated with tolerogenic therapy used in allograft recipients or patients with hematologic malignancies. Each follicular plug may contain between 525 hairs and is usually less than 1 mm in size. The human trichodysplasia spinulosaassociated polyomavirus tspyv is. It is characterised by solitary or multiple patches of minute follicular papules, each centred by a horny spine, which may occur on any part of the body.
These are usually located over the nose and forehead, but can more rarely affect other areas of the body including the ears and trunk. Virusassociated trichodysplasia spinulosa request pdf. Clinically it is characterised by multiple centrofacial folliculocentric papules with spinous protuberances. Trichostasis spinulosa is a common but rarely diagnosed disorder of the hair follicles that clinically gives the impression of blackheads, but the follicles are filled with funnelshaped, horny plugs that are bundles of vellus hairs. The condition is highly associated with the tsassociated polyomavirus tspyv.
Seroprevalence of trichodysplasia spinulosaassociated. The specimen may be placed on a glass slide for microscopic. Trichodysplasia spinulosa ts is a rare condition primarily affecting recipients of solid organ transplants who are receiving immunosuppressive medication. Histology from a punch biopsy and polymerase chain reaction pcr from plucked spicules confirmed a diagnosis of trichodysplasia spinulosa ts. In this condition, there are numerous tiny open pores filled with multiple tiny short hairs, usually only visible with a magnifying glass. Histopathology found increased cellularity of the nucleated eosinophilic inner root sheath. In trichostasis spinulosa ts, clusters of vellus hairs become embedded within hair follicles, with resultant dark, spiny papules on the face or trunk. If the diagnosis is in doubt, a specimen may easily be obtained by removing a hair plug with a forceps or comedone extractor.
Background trichodysplasia spinulosa ts is a rare, disfiguring skin condition that affects immunosuppressed patients, universally involving the central face. The biology and clinical features of cutaneous polyomaviruses. Trichodysplasia spinulosa presents as an eruption of flesh coloured follicular papules each with a central keratin spine. Trichodysplasia spinulosa associated polyomavirus tspyv or human polyomavirus 8 was identified from patients with trichodysplasia spinulosa, a rare skin disease affecting the faces of. Trichodysplasia spinulosa associated polyomavirus tsv is a type of human polyomavirus that was first described in 2010 and is most closely related to bornean orangutan polyomavirus and mcpyv. Abnormally maturing anagen hair follicles no papillae, ectatic infundibula with excessive inner root sheath differentiation, and hyperkeratotic infundibula am j surg pathol 2005. Clinical appearance and histology of the trichodysplasia spinulosa patient.
Histopathology revealed absent hair shafts within enlarged follicles. Typical histopathological features include large and distended hair follicles, which are often plugged with keratin and other debris. Trichodysplasia spinulosa successfully treated with adapalene 0. The trichodysplasia spinulosaassociated polyomavirus. Trichodysplasia spinulosa follicular spines of multiple myeloma fsmm. Background trichodysplasia spinulosa ts is a rare, disfiguring skin condition. A total of 15 ts cases have been described, of which 3 were identified in 2010 911.
Trichodysplasia spinulosaa newly described folliculocentric viral infection in an. Trichodysplasia spinulosa is characterized by ac ve polyomavirus infec on. It is associated with trichodysplasia spinulosa, a rare skin disease only seen in immunocompromised and lymphocytic leukemia patients. Trichodysplasia spinulosa ts is a rare skin condition seen in immunosuppressed patients that is characterized by folliculocentric papules with central spiny spicules mainly on the face. The full text of this article hosted at is unavailable due to technical difficulties. Histopathologically, distorted and dilated anagen hair follicles were. Trichostasis spinulosa is a common but rarely diagnosed disorder of the hair follicles that clinically gives the impression of blackheads, but the follicles are filled with funnelshaped, horny plugs that are bundles of vellus hairs 768. Multiple minute digitate hyperkeratosis a peculiar entity as a paraneoplastic syndrome, resolving after successful treatment of the accompanying laryngeal carcinoma. Merkel cell carcinoma and tspyv causing trichodysplasia spinulosa, the last two being proliferative skin diseases.
A case with electron microscopic and molecular detection of the trichodysplasia spinulosaassociated human polyomavirus mark r. Currently, there is a dearth of case reports on this condition, and treatment for ts is not well. Electron microscopy was attempted, but all lesional tissue was already exhausted. Trichodysplasia spinulosa is a cutaneous viral infection of immunosuppressed patients that causes abnormal hair follicle maturation. The trichodysplasia spinulosa associated polyomavirus.
Trichodysplasia spinulosa ts is a folliculocentric and clinically papular. Lichen spinulosus is a clinically distinctive variant of keratosis pilaris. Our patients presented with widespread papules, some extruding a central keratin spicule, which were most prominent on the face. Otras medidas consisten en extraccion manual y cidofovir o valganciclovir topico.
First discovered in 2010, tspyv is associated with trichodysplasia spinulosa, a rare skin disease only seen in immunocompromised patients. Examination of skin biopsies using light microscopy and immunohistochemistry revealed pathologic changes of the follicular inner root sheath epithelium with dystrophic trichohyaline granules. Trichodysplasia spinulosa polyomavirus also known as trichodysplasia spinulosa associated polyomavirus, abbreviated tspyv or tsv is a member virus of human polyomavirus 8 that infects human hosts. Jan 31, 2017 in trichostasis spinulosa ts, clusters of vellus hairs become embedded within hair follicles, with resultant dark, spiny papules on the face or trunk.
Acta pathologica, microbiologica, et immunologica scandinavica 20 chapter 3. We report the case of an 82yearold woman with a history of treated nonhodgkin lymphoma and a recent diagnosis of stage iv colon cancer. Trichodysplasia spinulosa ts is a rare entity, characterized by a follicular digitate keratosis predominantly affecting the face and variable degrees of hair loss, most severely facial hair. Trichodysplasia spinulosaassociated polyomavirus tspyv. Esaa, ba,b purba gupta, mbbs,c and mary gail mercurio, mda rochester, new york introduction trichodysplasia spinulosa ts is a rare condition primarily affecting recipients of solid organ. Viral associated trichodysplasia trichodysplasia spinulosa. Exposure to the virus occurs at a very young age, and the overall prevalence in adults is around 70%. Trichodysplasia spinulosa polyomavirus also known as trichodysplasia spinulosaassociated polyomavirus, abbreviated tspyv or tsv is a member virus of human polyomavirus 8 that infects human hosts. The histopathology is distinct and treatment with antiviral agents appears to be the most effective. European perspective on human polyomavirus infection, replication and disease in solid organ transplantation h. Two cases of trichodysplasia spinulosa responsive to. Ac ve tspyv infec on in trichodysplasia spinulosa 63.
Summary we report two boys with trichodysplasia spinulosa associated with chemotherapy for acute lymphocytic leukaemia. May 01, 2014 trichodysplasia spinulosa is a rare polyomavirus. Trichodysplasia spinulosaassociated polyomavirus tsv is a type of human polyomavirus that was first described in 2010 and is most closely related to bornean orangutan polyomavirus and mcpyv. Specific detection of trichodysplasia spinulosaassociated. Skin inflammatory nontumor virus associated trichodysplasia spinulosa. On the basis of knowledge regarding other polyomaviruses, we anticipated that infections with trichodysplasia spinulosaassociated polyomavirus tsv occur frequently and become symptomatic only in immunocompromised patients. The characteristic histopathologic findings include dilated hair. Trichodysplasia spinulosa associated polyomavirus tsv, which exhibit high seroprevalence in general population, has been detected in trichodysplasia spinulosa ts skin lesions suggesting an etiological role for this disease. What are the signs and symptoms of lichen spinulosus.
Trichodysplasia spinulosa clinical description trichodysplasia spinulosa ts is a rare skin disease first reported by izakovic and colleagues in 1995. Pdf seroprevalence of trichodysplasia spinulosaassociated. She presented with clinical and histopathologic findings consistent. Trichodysplasia spinulosa ts is a rare disease of the skin seen in solid organ transplant patients receiving immunosuppressive therapy 15 and in lymphocytic leukemia patients 4,68. Usually ig in follicles, but one case demonstrated a low copy number of merkel cell polyomavirus mcv in a patient with fsmm keratosis pilaris 33 34. Trichodysplasia spinulosa ts is a unique clinical and histological entity described in immunosuppressed patients. We present a case of ts in a kidney transplant recipient that was successfully treated with adapalene 0. This results in elevated, dark spiny papules on the head, face usually the nose, and trunk. Trichodysplasia spinulosa is a rare polyomavirusassociated cutaneous eruption occurring in the setting of immunosuppression. New data point to the recently discovered tsassociated polyomavirus tspyv as the causative agent. European perspective on human polyomavirus infection. Jun 28, 2011 trichostasis spinulosa ts is a condition where instead of one hair protruding from a hair follicle, a bundle or bush of hair come out of a single follicle. Ultrastructurally, intranuclear viral particles consistent with polyoma virus are found. Nine distinct human pyv species have been identified so far,6, 7.
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