Bullous pemphigoid (BP) induced by radiotherapy.

Radiotherapy and Oncology (Impact Factor: 4.52). 02/2007; 82(1):105. DOI: 10.1016/j.radonc.2006.11.013
Source: PubMed
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    ABSTRACT: Ionizing and ultraviolet radiations, as well as burns, can selectively damage and immunologically mark the cutaneous area they act on through direct and indirect mechanisms. After the causal event has disappeared, the affected skin district may appear clinically normal, but its immune behavior is often compromised forever. In fact, irradiated or burned skin areas undergo a destabilization of the immune control, which can lead to either a reduction of immunity (as suggested by the facilitated local occurrence of tumors and infections) or an excess of it (as suggested by the possible local onset of disorders with exaggerated immune response). In other words, these areas become typical immunocompromised districts (ICD). Also, in recall phenomena the damaged skin area usually behaves as an ICD with an exaggerated immune response toward a wide range of drugs (especially chemotherapeutic agents) that prove to be harmless on the undamaged skin surface. The occurrence of any skin disorder on an irradiated, photoexposed, or burned skin area can be defined as an isoradiotopic, isophototopic, or isocaumatopic response, respectively; however, the opposite may also occur when elsewhere generalized cutaneous diseases or eruptions selectively spare irradiated, photoexposed, or burned skin sites (isoradiotopic, isophototopic, and isocaumatopic nonresponse, respectively). The pathomechanisms involved in any secondary disorder occurring on irradiated or burned skin areas may be linked to locally decreased or altered lymph flow (with dysfunction of lymph drainage) on the one hand, and to fibrotic throttling or reduction of peptidergic nerve fibers (with dysfunction of neuroimmune signaling) on the other hand, resulting in a significant dysregulation of the local immune response. Future clinical observations and experimental investigations on radiation dermatitis, sunburns, and thermal or chemical skin injuries should shed new light on the mechanisms regulating regional resistance to infectious agents, local oncogenesis, and district propensity to dysimmune reactions.
    Clinics in Dermatology 10/2014; 32(5):660–669. · 2.34 Impact Factor
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    ABSTRACT: The term pemphigoids includes a group of autoimmune bullous diseases characterized by subepidermal blistering. Bullous pemphigoid (BP) is not only the most common disorder within the pemphigoid group, but also represents the most frequent autoimmune blistering disease in general. The onset and course of BP depend on a variable interaction between predisposing and inducing factors. HLA genes are the most significant genetic predisposition factor to autoimmunity mechanisms. Many studies show an association between HLA-DQβ1*0301 and distinct clinical pemphigoid variants. Imbalance between autoreactive T helper (Th) and T regulatory cells, toll-like receptor activation, and Th17/IL-17 pathway are the three possible autoimmunity triggers underlying BP. The pathomechanism of BP hinges on an autoantibody response toward structural components of the hemidesmosome (BP180 and BP230). The binding of autoantibodies leads to complement activation, recruitment of inflammatory cells, and release of proteolytic enzymes. The inflammatory cascade also may be directly triggered by activation of Th17 cells with no intervention of autoantibodies. The intervention of inducing factors in BP can be identified in no more than 15% of patients. Facilitating factors in genetically predisposed individuals are various (drug intake, physical agents, and viral infections). Drugs may act as triggers by either modifying the immune response or altering the antigenic properties of the epidermal basement membrane. Cases of induction of BP by physical agents (eg, radiation therapy, ultraviolet radiation, thermal or electrical burns, surgical procedures, transplants) are rare, but well-documented events. A contributing role in inducing BP has been suggested for infections, in particular human herpes virus (HHV) infections (cytomegalovirus, Epstein-Barr virus, and HHV-6), but also hepatitis B and C viruses, Helicobacter pylori, and Toxoplasma gondii. Unlike pemphigus, no dietary triggers have been suspected of being involved in the induction of BP. In all patients who have a diagnosis of BP, an environmental agent as a potential cause should always be considered, because the prompt discontinuation of it might result in rapid improvement or even cure of the disease.
    Clinics in dermatology 01/2013; 31(4):391-399. · 3.11 Impact Factor
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    ABSTRACT: : To better define the spectrum of neoplastic and inflammatory diseases that affect female breast skin and the nipple-areola complex, we searched an institutional dermatopathology database and identified 500 specimens of female "breast" (from consecutive records accessioned January 2009-March 2011), 143 specimens of "areola," 124 specimens of "nipple" (records from the latter 2 groups were from patients evaluated June 1992-March 2011), and 500 control specimens of "abdomen" (accessioned January 2010-March 2011). Most specimens obtained from breast skin (76%) represented melanocytic or epithelial proliferations (eg, nevi, seborrheic keratoses, and cysts), as did those from the abdomen, whereas many from the nipple (41%) and areola (60%) were of inflammatory dermatoses. A striking finding was eosinophilic spongiosis (ES) in most areola specimens with spongiotic dermatitis (78%); in contrast, ES was identified in 50% and 31% of spongiotic dermatitis specimens from nipple and breast skin, respectively. ES was associated with a clinical diagnosis of dermatitis in all patients except one (who had pemphigus). Metastatic breast cancer was identified in 28 of 767 specimens (4%), including 6 of 124 (5%) from the nipple. Five of 124 specimens from the nipple (4%) and 1 of 143 from the areola (1%) showed Paget disease. All but one patient with Paget disease showed acantholytic features and none had tissue eosinophils. Abnormalities categorized as complications of radiotherapy, including mild fibrosis and vascular ectasias, morphea, and angiosarcoma, constituted a minority of cases. Our data and the literature indicate that few disorders specifically affect breast skin, but the nipple-areola complex should be approached with a different set of diagnostic considerations.
    The American Journal of dermatopathology 05/2013; 35(3):289-307. · 1.30 Impact Factor