1/6. Initial presentation and fatal complications of linear iga bullous dermatosis in the larynx and pharynx.Two cases of linear iga bullous dermatosis initially presenting as ulcerative lesions in the larynx and pharynx are reported. It was difficult to diagnose and treat the lesions, but they were finally diagnosed from the histopathological findings of accompanying skin lesion specimens. One of the patients required a tracheostomy due to increased airway stenosis by a laryngeal lesion. Despite general corticosteroid administration this could not be completely resolved, although partial opening of the glottis was observed, and the patient died of accidental tracheostomy tube complications during home care. Although there are no reports of this disease in the otolaryngological field, these rare diseases involving the skin and entire body should be considered in the differential diagnosis of laryngeal and pharyngeal ulcerative lesions, including airway stenosis. Furthermore, simple and safe procedures for relieving airway stenosis should be selected for rare and difficult-to-diagnose airway disease, prior to the final diagnosis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/6. lichen planus pemphigoides.lichen planus pemphigoides is a rare disease in which blistering occurs in association with lesions of classical lichen planus. We describe a patient with lichen planus pemphigoides and discuss the evidence for there being one disease rather than the coincidence of bullous pemphigoid and lichen planus.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/6. Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) after a diagnostic echogram. Report of two cases.We reported two cases of subcorneal pustular dermatosis (Sneddon-Wilkinson disease) which developed several hours after performance of an echography. To the best of our knowledge, there are as yet no reports in the literature relating performance of echography with the appearance of this rare disease. The possible explanation of the relation between the dermatologic disorder and the performance of echography is discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/6. Oesophageal involvement in benign mucous membrane pemphigoid.Benign mucous membrane pemphigoid is a relatively rare disease characterised by the presence of bullous lesions and erosions of the mucous membranes leading to scarring. Oral and conjunctival mucosae are most frequently affected. Occasionally, the oesophagus is involved and two radiological patterns, webs and smooth strictures, are seen.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/6. Congenital erosive and vesicular dermatosis with reticulated, supple scarring: a neutrophilic dermatosis.Congenital erosive and vesicular dermatosis with reticulated, supple scarring is a rare disease seen at birth with blisters that become eroded and crusted and then heal with characteristic scars. We report the seventh case in the American literature, and the first description of biopsy specimens of early lesions. The acute skin lesions were characterized by sterile, neutrophilic infiltrates in the dermis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/6. Acquired Blaschko dermatitis: acquired relapsing self-healing Blaschko dermatitis.Acquired Blaschko dermatitis is a rare disease with acquired unilateral relapsing inflammatory linear lesions along Blaschko's lines. Histopathology reveals spongiotic dermatitis. "Blaschkite de l'adulte" and "acquired relapsing self-healing Blaschko dermatitis" have been suggested as names for this condition. Our patient was a 27-year-old man with a 6 month history of repeated, unilateral, slightly pruritic, discrete and grouped erythematous papules and papulovesicles on the left side of the upper limbs and trunk along Blaschko's lines. Histologic examination showed subacute spongiotic dermatitis. The condition showed excellent improvement after treatment with systemic corticosteroid for 2 months. Only a few cases have been reported. We propose a new designation and describe a patient who represents the first reported case of acquired Blaschko dermatitis in korea.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |