Psoriasis is associated with increased risk of bullous pemphigoid: A nationwide population-based cohort study in Taiwan.

Psoriasis may coexist with bullous pemphigoid (BP); however, no cohort studies have investigated the relationship between psoriasis and the risk of BP. This study aims to investigate the relationship between psoriasis and the risk of BP in Taiwan. This cohort study consists of 109 777 psoriatic patients and 109 777 matched non-exposed controls. Psoriatic patients diagnosed between 2002 and 2012 were identified from the Taiwan National Health Insurance Research Database. The age-, sex- and index date-matched non-exposed group was selected from the same database. The relationship between psoriasis and the risk of BP was investigated using Cox proportional hazards analyses. Psoriasis was significantly associated with an increased risk of BP (hazard ratio, 3.05; 95% confidence interval, 2.10-4.43; P < 0.001). The mean interval between the diagnoses of psoriasis and BP was 2.86 years, with the highest occurrence in the first year after psoriasis diagnosis, and gradually decreasing with each year of observation. Psoriatic patients with BP were significantly younger than BP patients in the non-exposed group (71.6 ± 13.9 vs 76.6 ± 7.7 years, respectively; P = 0.030). A higher proportion of patients with coexisting psoriasis and BP received phototherapy (20%). In conclusion, psoriasis was independently associated with a 3.05-fold increased risk of BP, and psoriatic patients with BP were younger, with over one-third of BP cases diagnosed in the first year after incident psoriasis. Therefore, clinicians treating patients with psoriasis may be aware of the possibility of the development of BP.

PUVA-induced Bullous Pemphigoid in Psoriasis.

The association between psoriasis vulgaris and bullous pemphigoid is due to the still unclear autoimmune process. The common disease site is the dermo-epidermal junction or basal membrane zone (BMZ), with specific alterations for both diseases. Photochemotherapy (PUVA) is one of the therapeutic modalities for psoriasis and can trigger production of autoantibodies against antigens in the BMZ in patients with subclinical bullous pemphigoid. Furthermore, PUVA therapy can alter the immunological milieu and hence can contribute to the expression of bullous pemphigoid in patients with psoriasis. We observed a bullous eruption compatible with bullous pemphigoid in a psoriatic patient treated with PUVA. We speculate that the cumulative dose of PUVA sufficient for triggering blister formation is individually determined.

Nutrition and bullous skin diseases.

Autoimmune and nonautoimmune bullous diseases can both be associated with significant morbidity and mortality. Although our understanding of the pathogenic mechanisms of these diseases has increased tremendously, there is still much to learn about the various factors affecting their onset, course, and therapy. In recent years, increasing information has been published about the effect of vitamins, minerals, and other nutrients on bullous skin diseases. Some factors are believed to be inducers (thiol and phenol-containing foods in pemphigus), whereas others are believed to be protective (antioxidants in cutaneous porphyrias). This contribution reviews the evidence in the literature of the role of various dietary factors in bullous diseases, including the nonautoimmune and the deficiency dermatoses. Additional studies and new investigations are needed to provide a better understanding of the specific associations of dietary factors with bullous diseases and better management for patients affected by these conditions.

[Bullous pemphigoid related to PUVA therapy: two further cases]. / Penfigoide ampolloso relacionado con tratamiento con PUVA: aportación de dos nuevos casos.

Bullous pemphigoid is an autoimmune disease that generally affects elderly people and is characterised by the development of subepidermal blistering. Although bullous pemphigoid is potentially photosensitive, its occurrence during the treatment course with PUVA, especially in patients with psoriasis, has exceptionally been described. The association of bullous pemphigoid and psoriasis gives rise to difficulties when initiating treatment and we consider that the use of methotrexate, with or without associated corticoids, is a good alternative in the management of such patients. We report two further cases of bullous pemphigoid related to PUVA therapy in patients with psoriasis.

Bullous pemphigoid in a patient with psoriasis during the course of PUVA therapy: study by ELISA test.

A 65-year-old woman had a history of deep vein thrombosis and depression. Psoriasis was diagnosed in 1986 and various topical and systemic therapies, singly or in combination, were prescribed: tar, topical corticosteroids, cyclosporine, etretinate, and methotrexate. Two courses of oral and one course of bath psoralen plus UVA (PUVA) therapy (cumulative dose, 467 J/cm(2)) and UVB (2.96 J/cm(2)) had been given. In January 1999, she developed a flare of generalized psoriasis. In May 1999, therapy with PUVA (8-methoxypsoralen) plus topical acetonide triamcinolone 0.1% was initiated. At the time, she was taking acenocoumarol, lorazepam, and hydroxyzine chlorhydrate. In August 1999, at session 30, when the dose of UVA was 9 J/cm(2), and the total dose was 205 J/cm(2), a bulla appeared on the dorsum of the toe and was controlled with topical antibiotics. Five further sessions of PUVA were given and a generalized itching bullous eruption appeared all over the body. PUVA was stopped and the patient was hospitalized. On physical examination, extensive psoriatic plaques plus vesicles and bullae on the normal skin and on psoriatic lesions were observed all over the body (Fig. 1). Histopathologic study of a lesion showed a subepidermal vesicle containing fibrin, neutrophils, and a few eosinophils. No sunburn cells were observed (Fig. 2). The direct immunofluorescence (DIF) test of perilesional uninvolved skin revealed immunoglobulin G (IgG) (Fig. 3) and C3 at the dermal-epidermal junction. The DIF study using the patient's skin, previously treated with 1 m NaCl, localized the IgG at both the epidermal and dermal sides of the basement membrane zone (Fig. 4). Bullous pemphigoid (BP) was diagnosed and therapy with prednisone (60 mg/day) was started. The disease was well controlled in 3 weeks. The dose of prednisone was tapered and stopped 20 months later, without any recurrence. Study of the antibodies by the indirect immunofluorescence (IIF) test, using monkey esophagus and guinea pig as substrate, was positive at a titer of 1/160 in September 1999. The titer decreased to 1/10 in January 2000, and was negative in July 2000. An enzyme-linked immunosorbent assay (ELISA) test, performed using the commercial kit MBL, which identifies antibodies directed against epitopes of the extracellular fragment NC16 of antigen 2 of BP, was positive at 15 U/mL (normal value, < 9 U/mL) in September 1999, and negative in July 2000 (Table 1).

Childhood bullous pemphigoid: report of a case with life-threatening course during homeopathy treatment.

Bullous pemphigoid (BP) is an autoimmune blistering disorder that may very rarely occur in childhood. We describe a 9-month-old child who developed bullous pemphigoid while she was being treated for presumptive atopic eczema with a homeopathic regimen comprising sulfur, mercury, cantharides, and Rhus (Toxicodendron). She had generalized bullae and a progressive worsening of her general condition with asthenia, dehydration, malnutrition. While the role of homeopathy in triggering the disease remains unclear, our observation attests to the potential life-threatening course of childhood BP in instances where appropriate treatment is withheld.

Bullous pemphigoid in a patient treated with UVA-1 phototherapy for disseminated morphea.

Bullous pemphigoid is an autoimmune disease of the skin characterized by the production of antibodies directed at structures of the basement membrane zone (BMZ) leading to subepidermal blisters. Several causative triggers have been described in the literature, among them UV light. Here, we report on a 73-year-old Caucasian female with disseminated morphea who developed blisters on her extremities after receiving whole-body UVA-1 phototherapy. The initial differential diagnosis of a phototoxic versus photoallergic reaction was ruled out as the lesions continued to spread after discontinuation of phototherapy. Histological and direct immunofluorescence examination showing a subepidermal blister and linear IgG deposits along the BMZ along with detection of circulating anti-BMZ antibodies led to the diagnosis of bullous pemphigoid. Immunosuppressive therapy resulted in regression of all blisters. After ruling out other possible causes, such as neoplasias or drugs, we conclude that UVA-1 has to be regarded as the most likely trigger of the disease.

PUVA-induced lichen planus pemphigoides.

A 72-year-old woman had suffered from parapsoriasis en plaque (large plaque type) controlled by topically applied psoralen ultraviolet A (PUVA) therapy. The parapsoriasis lesions gradually disappeared, but numerous tiny red papules with pruritus appeared over the forearms and lower legs 120 days after starting PUVA therapy. These papules developed to form violaceous plaques. Histological findings demonstrated the characteristics of lichen planus. Two months later, tense bullae developed on the plaques and on uninvolved skin of the limbs. These were subepidermal, with linear deposits of IgG and C3 along the basement membrane zone (BMZ) in immunofluorescence of peribullous skin, and immunodeposits of type IV collagen along the floor of the bullae. We therefore, diagnosed lichen planus pemphigoides (LPP). Using systemic and topical steroid therapy, the lesions rapidly resolved and there has been no recurrence. This case suggests that the combination of basal cell injuries caused by chronic inflammation and PUVA therapy could expose BMZ components to autoreactive lymphocytes and induce LPP.

Actualización en penfigoides: análisis de 55 casos / Update in pemphigoid: analysis of 55 cases

El pengigoide (PMB o penfigoide de las membranas mucosas PMM) es una enfermedad de las mucosas, piel o de piel y mucosas, de naturaleza autoinmune que se caracteriza por la formación de ampollas. En esta ocasión se presenta una revisión de 55 casos, realizada en el IREPO (Instituto de Referencia de Patología Oral) y en el Servicio de diagnósticos de la Escuela Dental, entre los años 1978 y 1998, en todos los pacientes el diagnóstico fue confirmado con biopsia

Bullous pemphigoid possibly induced by psoralen plus ultraviolet A therapy.

An elderly white man with a long history of psoriasis, previously treated with topical steroids and ultraviolet B (UVB), developed bullous pemphigoid shortly after starting psoralen plus UVA (PUVA) therapy. The eruption spread from the forearms to the arms and neck after phototherapy was discontinued. The clinical features of the 12 reported cases of PUVA-induced bullous pemphigoid are reviewed.

Delayed onset of bullous pemphigoid after PUVA and sunlight treatment of psoriasis.

The coexistence of bullous pemphigoid and psoriasis has previously been noted. In most patients, the bullous lesions start during PUVA or UVB therapy of psoriasis. We report on a psoriatic patient who developed bullous pemphigoid approximately three to four weeks after discontinuing PUVA treatment, and then three to four weeks after exposure to natural sunlight. Delayed induction of bullous pemphigoid has not been previously reported, and it raises the question of ultraviolet therapy inducing subclinical or latent bullous pemphigoid in psoriatic patients.