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the following: Lichen sclerosus Squamous cell hyperplasia (with and without atypia) (localized neurodermatitis) Primary irritant dermatitis Fixed drug eruption Reiter disease Lupus erythematosus Darier disease Aphthosis and Behçet disease Pyoderma gangrenosum Plasma cell vulvitis Vulvar vestibulitis Blistering diseases involving the vulvar region include the following: Familial benign chronic pemphigus (Hailey-Hailey disease) Pigmentary changes involving the vulvar region include the following (...) estrogen. [ ] The use of oral contraceptives might be a contributing factor by increasing the sensitivity of the vestibular mucosa. [ ] Blistering diseases Familial benign chronic pemphigus (Hailey-Hailey disease) Hailey-Hailey disease is a rare autosomal dominant acantholytic disorder due to a mutation on band 3q21-q24 and is characterized from late adolescence or adulthood by recurrent eruptions of vesicles and blisters typically located on the neck, axillae, and groin. Intrinsic desmosomal fragility
coincide with exacerbations of the colonic disease. Lesions in the colon consist of areas of hemorrhage and ulceration, along with abscesses. Cutaneous involvement consists of similar ulcerations that may arise on the buttocks, abdomen, thighs, and face, although in rare cases patients may develop pyoderma vegetans. [ , ] In the oral cavity, aphthous ulcers or angular stomatitis occurs in as many as 5-10% of patients, although hemorrhagic ulcers can occur. [ ] Rarely, patients can develop pyostomatitis (...) vegetans (PSV), the oral counterpart of pyoderma vegetans. Also see . Crohn disease Crohn disease is an idiopathic inflammatory disorder that can involve the entire GI tract with transmural inflammation and noncaseating granulomas. The prevalence of Crohn disease varies significantly between populations. In North America, the incidence of Crohn disease can be as high as 20.2 cases per 100,000 population. [ ] Although formerly considered a disease of Western nations, the incidence is rising in Asia
Pellagra Pemphigus vulgaris Pleural effusion Porphyria cutanea tarda Pregnancy Psoriatic arthritis Reiter syndrome Sarcoidosis Scleroderma Shell nail syndrome Syphilis Yellow nail syndrome Dermatologic diseases in onycholysis are as follows: Pachonychia congenita Congenital ectodermal defect Pemphigusvegetans Congenital abnormalities of the nail Neoplastic disorders in onycholysis are as follows: Squamous cell carcinoma (of nail bed) Carcinoma (lung) Exogenous factors in onycholysis Nonmicrobial
diseases, it should be considered when any cutaneous eruption or mucosal lesion is undiagnosed, particularly if patients have any of the following: Generalized lymphadenopathy Lesions on the palms or soles Condyloma lata Risk factors (eg, HIV, multiple sex partners) Clinically, secondary syphilis may be mistaken for a , , , , , , or, particularly, . Condyloma lata may be mistaken for , , or pemphigusvegetans; scalp lesions may be mistaken for or idiopathic . Secondary syphilis is excluded
Cyclophosphamide pulses with oral prednisolone in the treatment of pemphigus: a pilot study. An open labeled clinical trial aimed at assessing the efficacy and safety of pulse intravenous cyclophosphamide with daily oral prednisolone in the treatment of pemphigus was carried out. Twenty-six patients (12 men, 14 women; mean age, 48.4 years), comprising 25 cases with pemphigus vulgaris and 1 with pemphigusvegetans (< 10% body surface area involvement) who did not achieve adequate control (...) of 2.1 and 3.6 months. Three weeks to 8 months later, 9 patients had recurrences of activity on tapering/withdrawal of prednisolone, mainly in the oral mucosa. Side effects of treatment included amenorrhea (3 patients), microscopic hematuria (3) which cleared with co-administration of mesna, vomiting (1), weight gain (10), gastritis (1), and cataract (2). It is concluded that treatment with monthly intravenous cyclophosphamide boluses along with daily oral prednisolone clears lesions of pemphigus
rate than younger groups (P < 0.001). The majority (82%) of cases were vulgaris/vegetans, and no significant differences were found in 10-year survival for type of pemphigus (P > 0.05). The patients who had been treated with corticosteroids alone had longer median survival times than those who had been treated with corticosteroids plus azathioprine (P < 0.001). A total of 11 patients died; the median follow-up time for those still alive was 5.9 years (range 2-12 years). Estimated survival at 2, 6 (...) Determination of survival and hazard functions for pemphigus patients in Kerman, a southern province of Iran. Pemphigus has in the past been associated with a high mortality rate. However, with the discovery of corticosteroids, patient median survival has improved. Our purpose was to assess median survival after confirmed diagnosis of pemphigus in patients in Kerman, a southern province of Iran.All patients who were either admitted to the hospital or treated as outpatients in Kerman from 22
Pemphigus and Pemphigoid in Domestic Animals: An Overview Pemphigus and pemphigoid are uncommon dermatological entities in domestic animals and of a presumed autoimmune nature. In one form or another, they have been reported in the dog, cat, horse and goat. Although these diseases are considered to be bullous dermatoses, the clinical presentation may vary from ulcerative to exfoliative to proliferative depending on the individual condition. Currently, four variants of pemphigus are recognized (...) (vulgaris, vegetans, foliaceus, erythematosus) and two of pemphigoid (bullous, cicatricial) although cicatricial pemphigoid has not yet been conclusively demonstrated in animals. Diagnosis is based on history, clinical signs, histopathology and immunopathology. Therapy must be immunosuppressive to be effective and is palliative rather than curative.
Pemphigusvegetans occurring in a split-thickness skin graft. Pemphigusvegetans, a variant of pemphigus vulgaris, most commonly occurs in the flexural area.To describe an unusual case of pemphigusvegetans occurring in a skin graft recipient site and to discuss the possible etiology.We present a 41-year-old man who developed vegetating plaques from the graft recipient site of his left leg for 8 months.Based on the histopathologic findings of a skin biopsy, this case was diagnosed as pemphigus (...) vegetans. The patient's condition was successfully treated with systemic corticosteroids and acitretin.Our case is unique in its presentation of pemphigusvegetans shortly after a split-thickness skin graft. Physicians should be aware of this entity while differentiating cutaneous lesions arising from a skin graft.
Pemphigusvegetans of the oral cavity. Pemphigusvegetans, a variant of pemphigus vulgaris, constitutes a rare form of all pemphigus cases, and oral involvement is common. Two clinical subtypes of pemphigusvegetans exist, characterized initially by flaccid bullae and erosions (Neumann) or pustules (Hallopeau). Both subtypes subsequently develop into hyperpigmented vegetative plaques with pustules and hypertrophic granulation tissue at the periphery.We report three cases of pemphigusvegetans