Concomitant psoriasis and bullous pemphigoid: coincidence or pathogenic relationship?

Psoriasis vulgaris and bullous pemphigoid represent two clinically well-characterized, chronic, inflammatory skin diseases. The concomitant occurrence of these two entities in a patient is rare, and the pathogenic implications of this phenomenon are unknown. We describe a 55-year-old woman with a 25-year history of plaque-type psoriasis who presented with disseminated tense bullae. Skin biopsies showed the typical histologic and immunohistochemical traits of bullous pemphigoid, and she had circulating immunoglobulin G (IgG) antibodies against the basement membrane zone, specifically the BP180 antigen. The bullous eruption was successfully treated with oral methylprednisolone and dapsone. Bullous pemphigoid is the autoimmune blistering disease that has most often been associated with psoriasis. Forty cases have been described in the literature. Classical psoriasis and psoriasis associated with bullous pemphigoid are identical. The pathogenic relationship between psoriasis and bullous pemphigoid is unclear. It has been postulated that the autoimmune process responsible for bullous pemphigoid lesions may be induced by ultraviolet light therapy, topical corticosteroids, and/or the inflammatory processes that occur in psoriasis. Immunomodulatory therapy may positively influence shared as well as distinct inflammatory mechanisms in patients who have psoriasis and bullous pemphigoid.     

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.     

Erythema gyratum repens-like figurate eruption in bullous pemphigoid

A patient with bullous pemphigoid developed a bizarre transient figurate erythematous eruption which clinically resembled erythema gyratum repens in the absence of any demonstrable underlying malignancy. An unusual feature was the eruption of fresh bullae within the erythematous areas, biopsy of which revealed the characteristic histological and immunofluorescence changes of pemphigoid. The relationship between the annular erythemas and the auto-immune bullous disorders is discussed.     

Figurate erythema: an unusual presentation of the nonbullous phase of bullous pemphigoid

BACKGROUND AND OBJECTIVE: Bullous pemphigoid, an autoimmune disorder, most commonly presents as a bullous eruption in patients over the age of 60. There may be a prodromal urticarial or papular eruption that evolves to bullae. CONCLUSION:We report a 46-year-old woman with a figurate erythema presentation of bullous pemphigoid.     

Bullous pemphigoid manifesting as dyshidrotic eczema and prurigo nodularis

A 63-year-old woman presented simultaneously with two rare forms of bullous pemphigoid. Initially she had a dyshidrotic palmoplantar eczema. Repeated epicutaneous patch tests ruled out a type IV allergic reaction to common allergens. Six months later the patient developed disseminated pruritic nodules. After 4.5 years she then demonstrated scattered bullae within erythematous plaques indicative of bullous pemphigoid. A skin biopsy showed linear IgG and IgM as well as C3 deposits at the dermo-epidermal junction and circulating autoantibodies against the basement membrane could be found in serum. Accordingly, the diagnosis of bullous pemphigoid was made. In retrospect, the vesicular palmoplantar eczema and pruritic nodules could be interpreted in this case as early manifestations of bullous pemphigoid. We recommend that in patients with persistent pruritic skin lesions, which are resistant to standard therapy, bullous pemphigoid should be excluded by adequate immunological examinations.     

Adult linear IgA bullous dermatosis: a polymorphic disorder

We report on two patients with unusual forms of adult linear IgA bullous dermatosis. One was a middle-aged woman who had targetoid lesions and bullae on her trunk and extremities. This patient first presented with lesions that clinically resembled erythema multiforme, but these evolved into a widespread eruption with bulging, elongated bullae. Examination of a biopsy specimen showed changes compatible with dermatitis herpetiformis and bullous pemphigoid. Findings on immunofluorescence studies showed deposition of linear IgA at the basement membrane zone. The second patient was an elderly woman with intensely pruritic vesicles whom we classified as having vesicular pemphigoid, until the linear IgA band on direct immunofluorescent test results became the predominant immunofluorescent finding. These cases are reported because of their unusual clinical presentations. The mechanism for the targetoid lesions in the first patient is discussed.     

Lokalisiertes bullöses Pemphigoid,getriggert durch ein Erysipel

Localized bullous pemphigoid is a seldom subtype of bullous pemphigoid. We report the case of a 63-year-old man who developed tense bullae on an erythematous area on the left calf after two episodes of erysipelas. Using histological and direct immunofluorescence studies localized bullous pemphigoid provoked by erysipelas was diagnosed. The patient was successfully treated with topical steroids. Clinicians should be aware of this differential diagnosis to avoid unnecessary antibiotic treatments.     

Bullous dermatosis and systemic lupus erythematosus in a 15-year-old boy.

A 15-year-old boy had a bullous eruption suggestive of bullous pemphigoid and established systemic lupus erythematosus (SLE). Direct immunofluorescence studies of the bullae and adjacent skin revealed the linear deposition of IgG and complement localized to the basement membrane zone. Indirect immunofluorescence examination of the serum failed to reveal circulating basement membrane zone antibodies. The differential diagnosis of the bullous eruption is reviewed, and the problem of diagnosis in cases of coexistent bullous disease and SLE is discussed.     

Oesophageal involvement in bullous pemphigoid

An elderly patient with bullous pemphigoid who had extensive oesophageal bullae is described. The diagnosis was confirmed by routine histopathological examination and immunofluorescence studies. These large oesophageal bullae may have caused massive gastrointestinal haemorrhage in this patient.     

Systemic lupus erythematosus presenting as a bullous eruption in a child

An 8-year-old girl presented with a generalized bullous eruption clinically resembling bullous pemphigoid or chronic bullous disease of childhood. Further study revealed immunopathologic findings seen in patients with epidermolysis bullosa acquisita or bullous systemic lupus erythematosus (SLE). Although she did not fulfill the American Rheumatism Association (Atlanta) criteria for SLE at her presentation, one year later she went on to do so. As well as being the youngest patient reported with bullous SLE, our patient is noteworthy because the bullous eruption was the initial manifestation of her SLE. Bullous SLE should be considered in the differential diagnosis of children presenting with generalized bullous eruptions.     

Bullous pemphigoid with pemphigus type antibodies in vivo. 2 cases

Pemphigus vulgaris, whether of the vulgaris or foliaceus variety, and bullous pemphigoid (BP) are two groups of auto-immune bullous diseases which in most cases can easily be differentiated on the basis of clinical, histological and, mainly, immunopathological data. Like cicatricial pemphigoid, BP may be accompanied with circulating pemphigus-like antibodies (PLA) which are not detected in vivo by direct immunofluorescence (IF). However, a true pemphigus-BP association, as reported first by Chorzelski et al., is exceptional. Two cases of BP immunolabelled with pemphigus-like antibodies at direct IF are reported, raising a discussion on this particular association. The first case concerns a 62-year old man presenting with extensive psoriasis treated with salicylated vaseline and topical corticosteroids. The patients was admitted for a disseminated, symmetrical and pruriginous bullous eruption made up of tense bullae on healthy and psoriatic skin or on an urticarial background, without Nikolsky's sign. Pathological examination of a recent bulla showed subepidermal detachment without acantholysis. Direct cutaneous IF revealed linear labelling of the basement membrane zone with IgG, C3 and C1q, and labelling of the inter-cellular substance of the epidermis with IgG. Indirect IF on O+ human skin demonstrated antibodies of the pemphigoid type (1/128) and of the pemphigus type (1/64). Standard laboratory examinations only showed moderate blood eosinophilia (950/mm3) and a rise in total IgE. Under systemic corticosteroid therapy (prednisone 1 mg/kg/day) and azathioprine (2 mg/kg/day) the bullae rapidly disappeared.(ABSTRACT TRUNCATED AT 250 WORDS)     

Bullous pemphigoid associated with mantle cell lymphoma

BACKGROUND: Bullous pemphigoid has developed in association with different types of malignant diseases, including a few cases of B-cell lymphoproliferative disorders. However, the paraneoplastic significance of this association is still controversial. OBSERVATIONS: We describe a 39-year-old patient who presented with a bullous eruption and generalized lymphadenopathy. The results of histologic, immunofluorescence, and antigenic studies confirmed the diagnosis of bullous pemphigoid. The histopathologic and immunophenotypic features of a lymph node biopsy specimen were consistent with mantle cell lymphoma. There was total resolution of the mucocutaneous lesions when mantle cell lymphoma went into remission. CONCLUSION: The age of the patient and the concomitant appearance and simultaneous remission of both diseases strongly suggest that bullous pemphigoid was a paraneoplastic phenomenon in the present case.     

Bullous lichen planus: diagnosis by indirect immunofluorescence and treatment with dapsone

Bullous lichen planus may be confused with other subepidermal bullous dermatoses, especially if bullae arise on normal-appearing skin. We present two patients who were originally thought, on the basis of clinical and histopathologic criteria, to have bullous pemphigoid. However, results of standard indirect and direct immunofluorescence assays did not support the diagnosis of bullous pemphigoid. Further investigation using the indirect autologous immunofluorescence assay revealed deposits of immunoglobulins in the stratum granulosum. Moreover, the indirect allogeneic immunofluorescence assay (using papular lichen planus lesional substrates from different patients) was positive for the same pattern, confirming the diagnosis of bullous lichen planus. Bullous lichen planus with bullous pemphigoid-like histologic features can be differentiated from bullous pemphigoid on the basis of the indirect autologous and allogeneic immunofluorescence assays for circulating antigranulosum antibodies. Both patients were treated with systemic corticosteroids. One patient went into remission with this therapy alone; the other patient had a favorable response when dapsone was given with systemic corticosteroids. The suggestion that there is a subset of bullous lichen planus with bullous pemphigoid-like histologic features that responds to dapsone needs to be explored.     

Bullous pemphigoid-like lesions induced by phenacetin. Report of a case and an immunopathologic study

An 84-year-old man with chronic eczema and ischemic heart disease had bullous pemphigoid-like lesions develop following the oral administration of phenacetin. The diagnosis was confirmed by routine histopathologic studies and by immunofluorescent and electron microscopy. Since rechallenge with phenacetin resulted in bulla formation, phenacetin was considered to be the causative agent. This patient showed several atypical clinical features, compared with patients with idiopathic bullous pemphigoid, including no associated erythematous macules, a positive Nikolsky's sign, painful superficial ulcers, and nonpruritic bullae. Histopathologically, there was lack of polymorphonuclear leukocyte infiltrate. The immunopathologic characteristics, however, were quite typical for those found in bullous pemphigoid. The antigenic sites for the autoantibodies from our patient were, at least in part, different from those of idiopathic bullous pemphigoid.     

Epidermolysis Bullosa Acquisita Localized to the Face

A 39-year-old woman had a three-year history of recurrent bullous eruption localized to her left cheeck. The diagnosis of epidermolysis bullosa acquisita was confirmed by means of direct immunofluorescence and direct immunoelectron microscopic studies performed on the perilesional salt-split skin. Topical corticosteroid treatment reduced pruritus and bullae formation. This case of localized epidermolysis bullosa acquisita on the face is reminiscent of Brunsting-Perry cicatricial pemphigoid. We also review the previously reported cases of localized epidermolysis bullosa acquisita.     

Bullous subacute cutaneous lupus erythematosus

We describe a 59-year-old woman, with a history of autoimmune disease and disseminated uterine leiomyosarcoma, who developed a photoaggravated, blistering skin eruption. An initial rash, at the outset of treatment with chemo- and radiotherapy, resembled erythema multiforme. Review of the original skin biopsy showed it to be subacute cutaneous lupus erythematosus. There were no systemic symptoms or signs to suggest systemic lupus erythematosus. The much later photoaggravated rash consisted mainly of bullae and eventual epidermal denuding which resembled toxic epidermal necrolysis. We propose that the clinical and histological diagnosis is one of bullous subacute cutaneous lupus erythematosus in a patient with no other features of systemic lupus erythematosus.     

Pemphigus with pemphigoid-like presentation, associated with squamous cell carcinoma of the tongue

A 53-year-old woman presented with an inoperable squamous cell carcinoma of the tongue associated with tense large bullae consistent with bullous pemphigoid, preceded by a prodrome of urticarial plaques. The histological findings showed a regenerating subepidermal blister with eosinophils and no acantholysis. Direct immunofluorescence study, however, showed positive staining for IgG and C3 throughout the epidermis consistent with pemphigus. The blistering eruption had no mucosal involvement and responded to low dose corticosteroids. Our patient may represent another presentation of a 'paraneoplastic pemphigus spectrum'.     

Coexistence of lichen planus and bullous pemphigoid (an immunofluorescence study of a "lichen pemphigoides") (author's transl)]

A 35 year old black man presented with a generalized eruption of lichen planus; subsequently tense blisters appeared within the lichenoid lesions and on clinically normal skin. Histopathological characteristics of lichen planus were present in the papules, and those of bullous pemphigoid were seen in the bullae taken from non-lichenoid skin. Direct immunofluorescence studies revealed immunological characteristics of lichen planus in skin and mucosal lesions of L. P. Bound IgG and beta1 C/beta1 A with tubular patterns were detected at the dermo-epidermal junction in all the skin fragments (clinically normal skin, bullous lesions lichenoid skin and mucous lesions). Indirect immunofluorescence studies showed at several intervals that the patient had circulating antibasement membrane zone antibodies (IgG; titres 1/50). This is the third published case in which immunofluorescence studies have established the "pemphigoid" nature of some bullous lichen planus. These findings are in favour of an immune disorder in lichen planus.     

Bullous pemphigoid, figurate erythema and generalized pigmentation with skin thickening in a patient with adenocarcinoma of the stomach

A 52-year-old woman with bullous pemphigoid developed a bizarre figurate erythematous eruption and generalized pigmentation and velvety skin thickening. Later on, these cutaneous signs were found to be associated with an underlying adenocarcinoma of the stomach. Double-diffusion precipitation and indirect immunofluorescence, however, failed to demonstrate circulating antibodies against the tumor tissue in our patient.