Bullous pemphigoid related to PUVA therapy: two further cases

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 and multiple sclerosis: a report of two cases with ELISA test

We report two patients with longstanding multiple sclerosis (MS) who developed vesicles and bullae consistent with the diagnosis of bullous pemphigoid (BP). Both patients showed linear IgG at the dermal-epidermal junction, located on the epidermal side of patients' skin previously treated with 1M NaCl. In the two cases, the ELISA test was positive for the extracellular fragment of BP 180. However, the indirect immunofluorescence test (IIF) was repeatedly negative. Therapy either with prednisone plus dapsone or prednisone alone was initiated and the disease was controlled after 23 and 15 months of therapy, in patients 1 and 2, respectively. However, the first patient had a flare-up 2 months after treatment was stopped. The association of MS and BP has been described previously in 35 cases. We compare our two cases with the 25 patients previously reported in detail in the literature. We emphasize the role of the ELISA test in establishing the diagnosis of BP.     

Bullous pemphigoid developing during systemic therapy with chloroquine

Bullous pemphigoid has been reported to be induced or precipitated by systemic therapy with several drugs, including penicillamine, captopril, frusemide and ampicillin. We report an African male patient with sarcoidosis who was prescribed chloroquine for progressive dyspnoea. After 3 months he developed generalized pruritus which evolved into a widespread bullous eruption with acral targetoid lesions resembling erythema multiforme. The histological and immunofluorescence findings were diagnostic of bullous pemphigoid. The atypical clinical features of this case resemble the phenotype that has been noted in previous reports of drug-induced bullous pemphigoid.     

Granulomatous secondary syphilis coinciding with PUVA treatment in a patient with psoriasis: an apparent failure of PUVA therapy

A 39-year-old man is described who has been suffering from psoriasis for many years. Because of the extensive involvement of the skin he was treated with PUVA therapy, initially with a good result. A second course of PUVA treatment was unsuccessful. Histopathological examination of the (persisting) skin lesions at this time showed clear granulomatous infiltration, in addition to psoriatic changes. Based on history, serological and dark-field examination, a secondary syphilis was diagnosed. The possible influence of PUVA therapy is discussed.     

Bullous pemphigoid induced by PUVA therapy IS THIS THE AETIOLOGY OF THE ACRAL BULLAE PRODUCED DURING PUVA TREATMENT?

Among 24 psoriatic patients treated with PUVA therapy, five developed bullae. Direct and indirect immunofluorescence studies supported the diagnosis of bullous pemphigoid in one patient. In the others, the blisters were a phototoxic acral eruption.     

Bullous pemphigoid in a patient with a C4 deficiency

A 53-year-old man with bullous pemphigoid with an extremely low level of C4 in the serum is reported.     

Acantholytic dyskeratotic epidermal naevus in a patient with guttate psoriasis on PUVA therapy

A 79-year-old male with psoriasis developed a zosteriform acantholytic dyskeratotic epidermal naevus during PUVA therapy. The eruption was successfully treated with etretinate 0.75 mg/kg/day and a subsequent course of PUVA with the affected area covered prevented a recurrence of the naevus.     

Bullous pemphigoid arising in a patient with acquired perforating dermatosis

A middle‐aged Japanese man who had been on haemodialysis treatment for diabetic nephropathy developed multiple itchy papules and nodules, which were histopathologically diagnosed as acquired perforating dermatosis. Two years later he developed oral lesions and subsequently numerous erosive plaques with necrotic crusts on the trunk and extremities. Histopathology of a papule showed a parakeratotic plug intermingled with basophilic, necrotic debris and collagen bundles, along with penetration of collagen bundles across the epidermis and subepidermal blister. Immunoblotting studies revealed IgG autoantibodies in the patient's serum, which reacted with the C‐terminal and the NC16a domains of bullous pemphigoid (BP)180, indicating presence of BP. We searched the literature and found no other cases of an autoimmune blistering disease occurring in association with a perforating disorder. Possible injury to the basement membrane zone induced during the process of transepidermal elimination might be involved in the pathogenesis of the pemphigoid disease.     

DNA repair elicited by UVB during PUVA therapy for psoriasis

Summary The skin of patients receiving psoralen and UVA (PUVA) therapy for psoriasis is exposed to trace amounts of UVB radiation emitted by PUVA irradiators in addition to UVA. DNA repair activity was measured using autoradiography in the uninvolved skin of PUVA-treated patients in order to determine whether 8-methoxypsoralen (8-MOP) plus UVA elicits repair, inhibits the skin repair response to UVB, or protects epidermal-cell DNA from UVB damage by promoting a tan. Epidermal-DNA repair activity was observed in 27 out of 37 patients following the first PUVA treatment. Phototesting with multiples of the initial UV dose elicited a linear increase in repair activity. Glass-filtered radiation failed to stimulate repair, indicating that the reaction was due to UVB, not to 8-MOP plus UVA. The same amount of repair activity was observed in the skin of patients irradiated either before or after 8-MOP ingestion, demonstrating that the drug did not interfere with the response of the skin to UVB. At clearing, however, the repair activity was never greater than that elicited at the initial treatment and was often undetectable despite a tenfold increase in UV exposure. It is proposed that DNA damage should be measured to determine whether epidermal cells are entirely protected from UVB radiation at the completion of therapy.     

Bullous pemphigoid in a patient with suspected non-Herlitz junctional epidermolysis bullosa

A 56-year-old man with lifelong trauma-induced blisters, nail dystrophy and dental enamel hypoplasia presented with a new spontaneous blistering eruption. Clinicopathologically, he had evidence of both an inherited and an acquired blistering disorder: non-Herlitz junctional epidermolysis bullosa (nHJEB) and bullous pemphigoid (BP). HIstological examination of a skin biopsy found reduced (but not absent) collagen XVII in nonlesional skin, in vivo bound anticollagen XVII antibodies in perilesional skin, and prominent eosinophils in perilesional and lesional skin, with subepidermal blistering. Circulating anticollagen XVII antibodies were also present. Treatment with oral corticosteroids and mycophenolate mofetil led to clinical control of the BP but had no effect on the mechanobullous blistering. Our patient is unusual in that his skin retains some labelling for collagen XVII rather than having the complete absence of immunoreactivity expected in patients with generalized nHJEB. Moreover, we were unable to identify any pathogenic mutations in the COL17A1 gene encoding collagen XVII (or in other EB-associated basement membrane genes). It is plausible that the long-term consequences of basement membrane disruption in our patient, perhaps associated with atypical inherited COL17A1 pathology, might result in a conformationally altered and more immunogenic protein with the subsequent development of anticollagen XVII antibodies and BP as a secondary pathology.     

A biological approach in a patient with psoriasis and bullous pemphigoid associated with losartan therapy

Several cases of psoriasis associated with bullous disorders have been reported, and it is clearly recognized that bullous pemphigoid (BP) is the most common bullous disorder observed in association with psoriasis, especially after ultraviolet (UV)B and psoralen UVA therapy. Moreover, other medications have been repeatedly reported to induce bullous diseases, especially pemphigus vulgaris. We report for the first time a case of BP possibly induced by losartan, an angiotensin II antagonist, in a patient with a severe psoriatic background. Angiotensin II type 1 receptor antagonists belong to a new class of drug for hypertension or congestive heart failure with established efficacy and few side-effects. Coexistence of psoriasis vulgaris with bullous diseases represents also a difficult therapeutic challenge. This rare case of psoriasis and generalized BP triggered by a sartan drug was treated with rituximab and etanercept.