Presentation
There exist reports on the involvement being present in up to six generations. [scielo.br]
We describe a patient presenting in association with psoriatic erythroderma. A 27-year-old male presented with generalized erythema and scaling all over body for 1 month. [ncbi.nlm.nih.gov]
A 54-year-old woman with a previous history of ovarian adenocarcinoma presented with painful edema of the distal half of the fifth left finger. [jrheum.org]
A case of atypical localized scleroderma presenting with pseudoainhum: treatment with tranilas.t, an anti-fibrotic agent. Acta Derm Venereol. 1996; 76:162. [revista.spdv.com.pt]
We describe the first suchcase in a 25-year-old woman who presented witha painful constricting band of the fifth toe. [escholarship.org]
Entire Body System
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Streptococcal Infection
Familial as well as acquired forms of the disease have been reported. ( 9) Pityriasis rubra pilaris (PRP) has been reported in association with autoimmune disorders like Grave’s disease, (10) HIV infection, internal malignancies, Streptococcal infections [jemds.com]
Familial as well as acquired forms of the disease have been reported. [9] Pityriasis rubra pilaris (PRP) has been reported in association with autoimmune disorders like Grave's disease, [10] HIV infection, internal malignancies, Streptococcal infections [go.galegroup.com]
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Inflammation
Iloprost was started, with resolution of the inflammation of the fingers and improvement of RP. Sustained stabilization of the fibrotic constrictive pseudoainhum over a 3-year followup period was observed. Figure 1. [jrheum.org]
With respect to complications, the following has been described: fungal and/or bacterial infection in the fissures and folds of the joints, occasioning a characteristic odor; inflammation and pain, which makes walking and manual work difficult and even [scielo.br]
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Pathologist
certified Specialist by Brazilian Society of Dermatology (SBD) IV Medical pathologist, Head of clinic, Adjunct Professor of Pathological Anatomy, Santa Casa de Sao Paulo Medical Faculty V Student-physician, CHPBG VI Head Physician, Padre Bento Hospital [scielo.br]
Cardiovascular
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Vascular Disease
There was no clinical evidence to suggest peripheral vascular disease. The patient could not recall how long the painless, asymptomatic lesions had been present. She reported no trauma to the toes or feet. Figure. [jaoa.org]
Skin
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Hyperkeratosis
Skin biopsy showed marked compact hyperkeratosis with a normal granular layer, mild-to-moderate acanthosis, and mild perivascular lymphocytic infiltration. [ijdvl.com]
Histopathological examination of a punch biopsy specimen obtained from the dorsum of affected foot showed compact hyperkeratosis and papillomatosis in a church spire manner with preserved granular cell layer. [ijpd.in]
Chronic trauma, infection, hyperkeratosis, decreased vascular supply, and impaired sensation may produce excessive fibroplasia in a susceptible host. 4 Dent et al described impaired blood supply to the foot proximal to the groove at the plantar digital [accessmedicine.mhmedical.com]
Chronic trauma, infection, hyperkeratosis, decreased vascular supply, and impaired sensation may produce excessive fibroplasia in a susceptible host. [jemds.com]
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Pachyonychia
Hereditary causes include pachyonychia congenita, Mal de Meleda, mutilating keratoderma, 6 lamellar ichthyosis, 7 and psoriasis. 8 – 10 Nonhereditary diseases include vascular anomalies as seen in Raynaud disease, diabetes mellitus, linear scleroderma [accessmedicine.mhmedical.com]
Hereditary causes include pachyonychia congenital and mutilating keratodermas like Mal de Meleda.[ 4, 5 ] Nonhereditary disease includes vascular abnormalities (Raynauds disease, diabetes mellitus and linear scleroderma) systemic sclerosis, sensory changes [ncbi.nlm.nih.gov]
Among the causes responsible for acquired pseudoainhum are palmoplantar keratoderma, Olmsted syndrome, pachyonychia congenita, erythropoeitic protoporphyria, infections like leprosy, trauma, cold injury, neuropathy and systemic sclerosis. [2] Congenital [ijpd.in]
Hereditary causes include pachyonychia congenita, Mal de Meleda, Unna-Thost, Vohwinkel, Papillon-Lefevre syndrome, (3,4) mutilating keratoderma, lamellar ichthyosis. [jemds.com]
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Papule
Punch biopsy was done from hyperkeratotic papules with prominent follicular openings and sent for histopathological examination. [jemds.com]
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Follicular Plugging
There was marked orthokeratosis, focal parakeratosis, papillomatosis, acanthosis and follicular plugging with focal hypergranulosis. Dermis showed sparse superficial perivascular lymphocytic infiltration. [jemds.com]
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Follicular Hyperkeratosis
After three weeks, there was significant improvement of follicular hyperkeratosis and keratoderma of both palms and soles. Patient is still under regular follow-up. DISCUSSION Constricting bands are classified as ainhum and pseudoainhum. [jemds.com]
Workup
Whenever in the presence of pseudoainhum, a detailed workup, including autoimmune disorders screening, is mandatory in order to rule out possible associated syndromes or underlying diseases. [revista.spdv.com.pt]
Other Pathologies
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Lymphocytic Infiltrate
Skin biopsy showed marked compact hyperkeratosis with a normal granular layer, mild-to-moderate acanthosis, and mild perivascular lymphocytic infiltration. [ijdvl.com]
Dermis showed sparse superficial perivascular lymphocytic infiltration. All the histopathological features were consistent with pityriasis rubra pilaris. After checking the fasting lipid profile, the patient was started on Cap. [jemds.com]
Treatment
There is no medical treatment which can halt the progression in Ainhum Steroid injections and salicylic acid ointment may reduce pain Surgery may release the constricting band Ainhum. [learningradiology.com]
Keloid pathogenesis and treatment [review]. Plast Reconstr Surg. 2006; 117 (1):286-300. doi:10.1097/01.prs.0000195073.73580.46. [CrossRef] [PubMed] Figure. [jaoa.org]
We alsodescribe her treatment course, which consisted ofa failed z-plasty, the traditional therapeutic optionfor acute pseudoainhum, and report the success ofsubsequent full thickness skin graft, suggesting thebenefit of this procedure as a therapeutic [escholarship.org]
The patient studied in this paper initially received treatment with oral antibiotics and local care at the focus of infection on his right foot. [scielo.br]
Treatment of the underlying disease may sometimes reverse the process. Codes ICD10CM: L94.9 – Ainhum SNOMEDCT: 19988008 – Pseudoainhum Best Tests Subscription Required References Subscription Required Last Updated: 09/02/2015 [visualdx.com]
Prognosis
Prognosis Outcome is related to the stage in ainhum when the disease is diagnosed. Pain may be severe in ainhum and in pseudoainhum. [emedicine.medscape.com]
Etiology
The Journal of foot and ankle surgery : official… 2016 Pseudoainhum is a disorder of unknown etiology that is usually congenital and nonprogressive. [semanticscholar.org]
MD * Case Reports Abstract Author Information Authors Pseudoainhum is a rare condition of unknown etiology that produces digital constricting rings, most commonly on the small fingers. [journals.lww.com]
5 syphilis, 6 leprosy, 7 cicatrices, 8 metabolic diseases, 9 syringomyelia, peripheral neuritis, 10 parasites, 11 keratoderma hereditaria mutilans, 12 ancylostomiasis, 13 a family disease, 14 and congenital anomaly, 15 as well as the result of known etiological [jamanetwork.com]
Etiology and Pathogenesis The pathogenesis of ainhum has not been clearly elucidated. [plasticsurgerykey.com]
Ainhum is a disease of unknown etiology, common in middle aged African males accustomed to walking barefoot. [ncbi.nlm.nih.gov]
Epidemiology
Epidemiology Ainhum (dactylolysis spontanea) is traditionally a disease of middle-aged African males accustomed to going barefoot. 1 In the tropic and subtropic climates, its incidence has been reported as between 0.015% and 2% of the population. 2 This [plasticsurgerykey.com]
Intralesional injection of corticosteroids is also helpful. [2] Epidemiology [ edit ] Ainhum predominantly affects black people, living in West Africa, South America and India. [en.wikipedia.org]
Pathophysiology
When associated with chronic trauma and infection of the extremities, the pathophysiology may be identical to true ainhum. ... [accessmedicine.mhmedical.com]
When associated with chronic trauma and infection of the extremities, the pathophysiology may be identical to true ainhum. Clinical Findings Ainhum usually affects the fifth toe; it may be unilateral, but 75% of the cases are bilateral. [plasticsurgerykey.com]
There are three pathophysiological categories of pseudoainhum- (1) congenital constricting bands are caused by the umbilical cord; (2) constriction by external forces, such as hairs or threads, which are generally factitial; and (3) constricting bands [jemds.com]
Pathophysiology In true ainhum, dactylolysis of a toe (most commonly, but not always, [4, 5] the fifth toe) most likely is triggered by trauma; however, the true cause remains unknown. The trauma may be related to walking barefoot in the tropics. [emedicine.medscape.com]
Prevention
Congenital pseudoainhum cannot be prevented and can lead to serious birth defects. [4] Treatment [ edit ] Incisions across the groove turned out to be ineffective. [en.wikipedia.org]
Treatment with oral retinoids is more beneficial in the prevention of teeth destruction if started early during eruption and maintained during development of the permanent teeth. [8] Further, oral rehabilitation with newer orthodontic procedures such [idoj.in]