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Bullous Lichen Planus

Rare LP


Presentation

  • We present an unusual case of bullous lichen planus arising in the skin graft donor site of a psoriatic patient. To our knowledge, such an association has not been reported to date.[ncbi.nlm.nih.gov]
  • We present two patients who were originally thought, on the basis of clinical and histopathologic criteria, to have bullous pemphigoid.[ncbi.nlm.nih.gov]
  • METHODS: In this retrospective chart review, we analyzed nine consecutive familial pedigrees of FBLP with 36 affected individuals who presented to the Department of Dermatology at the Wuhan Union Hospital, a tertiary referral hospital in central China[ncbi.nlm.nih.gov]
  • At present patient is in remission and following up with us on regular basis.[jemds.com]
  • Nail LP presents in 10 to 25 percent of LP patients and tends to present as roughness, vertical ridges or cracks, and thinning of the nail. This can eventually lead to scarring of the nail.[rarediseases.org]
Anhidrosis
  • Sometimes degeneration (atrophy) of the skin may occur as lesions persist (atrophic LP) and some patients experience an absence of sweating due to degeneration of sweat glands (anhidrosis).[rarediseases.org]
Receding Hairline
  • When and if hair loss does occur, it can involve small patchy areas of the scalp (atrophic cictrical alopecia) or cause a receding hairline (frontal fibrosing alopecia). Due to scarring, this hair loss is permanent if untreated.[rarediseases.org]
Incontinence
  • (a) Appearance of subepidermal clefting due to extensive basal cell vacuolation and lymphocytic infi ltrate hugging the epidermis (H and E, 10) (b) High power view revealing clefting, pigment incontinence and dermal melanophages (H and E, 40) Click here[ijdvl.com]
  • […] typically show histopathologic features indistinguishable from those of lichen planus: orthokeratosis, hypergranulosis, irregularacanthosis, basal cell hydropic degeneration with cytoid body formation, dense chronic inflammatory infiltration and pigment incontinence[jemds.com]
  • […] found at the lower dermis and superficial epidermis.[ 20 ] Vacuolar degeneration of the basal layer is typical and results in separated spaces between the epidermis and the dermis, known as "Max-Joseph spaces".[ 21 ] Often there is presence of pigment incontinence[ncbi.nlm.nih.gov]

Treatment

Prognosis

  • Cutaneous and mucosal lichen planus: a comprehensive review of clinical subtypes, risk factors, diagnosis, and prognosis. The Scientific World Journal. Volume 2014, Article ID 742826. Manousaridis I, Manousaridis K, Peitsch WK & Schneider S W.[rarediseases.org]
  • It resembles bullous pemphigoid, but carries a better prognosis.[medcaretips.com]
  • Prognosis and outcome The prognosis for spontaneous remission of erosive vulvovaginal lichen planus is poor. Despite a number of available therapies, significant control of symptoms and restoration of sexual function remain difficult.[cancertherapyadvisor.com]
  • Prognosis [ 1 ] Spontaneous resolution of cutaneous lesions usually occurs over a period of 6 to 12 months but hypertrophic lesions can last for years. Oral lesions usually have a chronic and progressive course and may never completely resolve.[patient.info]

Etiology

  • Etiology Etiology is unknown. The documents contained in this web site are presented for information purposes only.[orpha.net]
  • Etiology of Periodontal Diseases (Part 4) provides a more comprehensive background in periodontal anatomy, physiology, and pathogenesis.[books.google.com]
  • [Full text - PDF] ABSTRACT Lichen planus is a chronic mucocutaneous disease, inflammatory condition, autoimmune nature and unknown etiology, in which an aggression is produced by the T lymphocytes against the basal cells of the epithelium of the buccal[medigraphic.com]
  • DIFINETION CHRINIC INFLAMMATORY DISEASE OF UNKNOW ETIOLOGY CHARECTRISED BY ------ SHINY VIOLACEOUS ITCHY FLAT TOPPED PAPULAR LESIONS MORPHOLOGICALLY & HISTOLOGICALLY CHARECTERISED BY -------- Download Presentation An Image/Link below is provided (as is[slideserve.com]
  • No antibodies have been detected against 230kDa BP antigen, collagen type VII or laminin 7.26 The etiology of BLP remains obscure.[docksci.com]

Epidemiology

  • Summary Epidemiology Prevalence is unknown and only a few cases, both sporadic and familial, have been described in the literature. Clinical description The disease manifests during childhood or adolescence.[orpha.net]
  • Lichen planus: A clinical and epidemiological study. J Dermatol 2000;27:576-82. 2. Harjai B, Mendiratta V, Kakkar S, Koranne RV. Childhood lichen planus pemphigoides-A rare entity. J Eur Acad Dermatol Venereol 2006;20:117-8. [ PUBMED ] 3.[ijdvl.com]
  • Epidemiology The exact prevalence of BLP remains unknown. The disease is usually sporadic.[ncbi.nlm.nih.gov]
Sex distribution
Age distribution

Pathophysiology

  • "Pathophysiology, etiologic factors, and clinical management of oral lichen planus, part I: facts and controversies". Clinics in Dermatology. 28 (1): 100–8. doi : 10.1016/j.clindermatol.2009.03.004.[en.wikipedia.org]
  • Image Credit Pathophysiology of Lichen Planus The exact cause of lichen planus is not known, although it is immunologically mediated. The initiating antigen is unclear.[medcaretips.com]
  • Vulvar lichen sclerosus: Pathophysiology and treatment. Am J Clin Dermatol 2004;5:105-25. [ PUBMED ] 4. Iraji F, Schofield O.[e-ijd.org]
  • Nikolsky's observations were later confirmed by Lyell in 1956, who described a Nikolsky's sign in patients with toxic epidermal necrolysis. [9] Pathophysiology The pathophysiology associated with Nikolsky's sign is the acantholysis [8] i.e., loss of coherence[jiaomr.in]

Prevention

  • The information presented here is not intended to diagnose, treat, cure or prevent any disease. Read disclaimer.[gulfdoctor.net]
  • IMAGES Lichen Planus See a picture of lichen planus and other skin eruptions See Images Is it possible to prevent lichen planus? Since the cause of lichen planus is unknown, there are no reliable options to prevent it.[medicinenet.com]
  • Further development of scars and adhesions can be prevented with regular use of dilators as demonstrated in a number of prospective studies. Coating the dilator with either steroid ointment or vaginal estrogen cream is recommended.[cancertherapyadvisor.com]
  • Treatment to help prevent osteoporosis (calcium and vitamin D supplements, bisphosphonates) should be initiated if the systemic steroid will be given for more than one month.[patient.info]

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