The author reports two cases of Bullous pemphigoid (BP) with neurofibroma (NF)-like histopathological change. The two patients without neurofibromatosis type 1 (NF1) presented with several bullae on their trunk. Based on the results of positivity for anti-BP180 antibody, direct immunofluorescence, and histopathological findings, they were diagnosed with BP. Histologically, another lesion in the dermis, which was composed of spindle cells with wavy nuclei, collagen fibers, and mast cells, was located close to the bulla. Immunohistochemically, the spindle cells were diffusely positive for S-100 protein and CD34, and weakly positive for epithelial membrane antigen in certain foci. These findings were considered to be "NF-like" histopathological change. This is the first two cases of BP with NF-like histopathological change in patients without NF1.
A prospective study of 77 consecutive patients with bullous diseases was done to study the pattern and natural history. Pemphigus was the commonest with 45 patients (59%) followed by pemphigoid with 21 patients (27%). Pemphigoid was more common in Indians than in other ethnic groups and its age of onset was a decade later than pemphigus. Unusual immunofluorescent findings in both diseases are discussed. Six of the 7 patients with dermatitis herpetiformis had linear IgA in the dermo-epidermal junction and the classical papillary IgA deposits were absent. Ultrastructural findings of pemphigoid and dermatitis herpetiformis confirmed earlier reports. Chronic bullous dermatoses of childhood was seen in 4 patients, all of whom had total remission within one year of onset disease.
A case of bullous pemphigoid with extra-oral and intraoral lesions in a 60-year-old female is reported. Diagnosis is based on histopathology and direct immunofluorescence, and the treatment regime described included oral prescription of prednisolone and topical application of a mixture of fluocinonide ointment and triamcinolone acetonide in Orabase on the oral lesions.
The characteristics of three primary bullous diseases, pemphigus, pemphigoid, and dermatitis herpetiformis, seen in this country, probably reflecting this region, are discussed and compared to those reported in the literature in the West. One hundred and forty-eight patients with bullous diseases were seen over a period of 15 years. The criteria for confirmation of clinical diagnosis were the findings of the direct immunofluorescent test. Pemphigus vulgaris was the commonest bullous disease. The incidence of bullous pemphigoid was highest in the Indians of Malaysian origin, and they are also more likely to develop pemphigus vulgaris than any other ethnic group. Linear IgA type formed the entire group of dermatitis herpetiformis. The granular type was not seen at all. The patients were treated with prednisolone alone or together with methotrexate or azathioprine. Dapsone alone controlled the dermatitis herpetiformis. The known association between pemphigus and other diseases with immunologic disturbances was not found in this study. The natural history of the bullous disease as seen in the follow-up patients is described. Deaths in pemphigus and pemphigoid were more due to either complications of steroid therapy or unassociated diseases than the primary disease itself. Introduction of immunofluorescence as a diagnostic tool in pemphigus and extension of this facility to other bullous diseases has led to detailed characterization of these diseases as seen in the West; however, publications in English dealing with epidemiologic aspects of bullous diseases in this region are rare.(ABSTRACT TRUNCATED AT 250 WORDS)
BACKGROUND: An association of bullous pemphigoid with neurological disorders has been reported. The objectives of this study were to review the clinical characteristics of patients with bullous pemphigoid and compare the association between bullous pemphigoid and various neurological disorders and comorbidities.
METHODS: This was a retrospective case-control study involving 43 patients with bullous pemphigoid and 43 age-, sex- and ethnicity-matched controls.
RESULTS: There was a statistically significant association between bullous pemphigoid and neurological disorders [Odds Ratio (OR) = 3.5, 95% Confidence Interval (CI) 1.3 to 9.2, p=0.011 and adjusted OR=3.5, 95% CI 1.2-10.3, p=0.026], in particular for dementia (p=0.002). Although stroke was more common among patients with bullous pemphigoid, this association was not statistically significant with OR of 1.9 (95% CI 0.7 to 5.2) and adjusted OR of 2.1 (95% CI 0.6 to 7.2). Similarly both ischaemic stroke (OR 1.5, 95% CI 0.5 to 4.2) and haemorrhagic stroke (OR 1.5, 95% CI 0.2 to 9.7) were more common. Other neurological disorders more common among patients with bullous pemphigoid were Parkinson's disease and epilepsy. Dyslipidaemia was significantly less common among patients with bullous pemphigoid (OR 0.4, 95% CI 0.1 to 0.9, p=0.033).
CONCLUSION: A combination of an inflammatory process, prothrombotic state and endothelial activation leads to an increased frequency of neurological disorders among patients with bullous pemphigoid. Thus, a holistic approach to patient care, including screening for dementia and control of comorbidities, should be practised as bullous pemphigoid affects more than just the skin.
Bullous pemphigoid (BP) has a recognized association with solid organ tumors, but is relatively rare in hematological malignancies. We report a 67-year-old male who developed BP after being diagnosed with myelodysplastic syndrome and refractory anemia with excess of blast (RAEB). Skin biopsy elucidated sub-epidermal bulla using direct immunofluorescence, revealing linear C3 and IgG deposits along the basement membrane. His BP was recalcitrant to the conventional treatment and only responded to a combination of high dose oral prednisolone and azathioprine. The relative refractory nature of his condition and concurrent RAEB supports a paraneoplastic nature.