A case of pemphigoid gestationis with concurrent IgG antibodies to BP180, BP230 and type VII collagen

A 22‐year‐old primigravida had a pruritic, erythematous, bullous eruption on the skin during the 26th week of gestation. After delivery the eruption flared up. The diagnosis of pemphigoid gestationis was confirmed based on histopathological and immunofluorescence findings. The result of immunoblotting showed IgG autoantibodies which reacted against BP230 in epidermal extracts and 290 kDa type VII collagen in dermal extracts. The BP180 antibodies were also detected by an enzyme‐linked immunosorbent assay BP180NC16a diagnosis kit. Pulsed corticosteroid and cyclophosphamide resulted in a favourable response at the acute stage. The patient was cured in 2 years. The analysis of the patient's autoantibodies provides strong evidence for the involvement of epitope spreading in her autoimmune disease.     

Palmar Purpura: An Atypical Presentation of Childhood Dermatitis Herpetiformis

Abstract: Dermatitis herpetiformis (DH) is seen most commonly as a pruritic, papulovesicular eruption in young children or adolescents. Differentiation from other bullous diseases of childhood may be difficult. We report the first case of an adolescent in whom pruritic, palmar, purpuric macules and papules were the only manifestations of DH. The patient later developed typical vesiculobullous extensor lesions and symptomatic gluten-sensitive enteropathy (GSE). All lesions and GSE symptoms resolved with dapsone and a gluten-free diet. Our purpose is to illustrate an unusual presentation of pediatric DH.     

Trichodysplasia spinulosa in a child: Identification of trichodysplasia spinulosa‐associated polyomavirus in skin,serum, and urine

A 6‐year‐old girl with a history of chronic immunosuppression following small bowel and colon transplantation for tufting enteropathy presented with a diffuse, facial‐predominant eruption composed of pink‐to‐skin‐colored papules with central white dystrophic spicules. Histology from a punch biopsy and polymerase chain reaction (PCR) from plucked spicules confirmed a diagnosis of trichodysplasia spinulosa (TS). Additional molecular studies identified several strains of the trichodysplasia spinulosa–associated polyomavirus infecting multiple tissues of the patient, confirming the systemic nature of trichodysplasia spinulosa infections.     

Bullous Sweet's Syndrome After Granulocyte Colony‐Stimulating Factor Therapy in a Child with Congenital Neutropenia

We present the case of a 20‐month‐old boy with congenital neutropenia for which he was being treated with granulocyte colony‐stimulating factor (G‐CSF) who developed bullous Sweet's syndrome. Because of the challenging and extensive differential diagnosis of an acute bullous eruption in an immunocompromised child, we highlight the importance of a prompt and precise diagnosis before initiation of any systemic therapy in children with Sweet's syndrome.     

Human orf complicated by epidermolysis bullosa acquisita

Orf is a DNA parapoxvirus transmitted to humans by contact with infected goats and sheep. Many complications have been reported after orf infection, including erythema multiforme. A few cases of autoimmune bullous dermatosis complicating orf disease have been reported to date. They are usually characterized by tense blister eruptions with or without mucosal involvement; linear deposition of C3, IgG and/or IgA along the basement membrane; and negativity of indirect immunofluorescence analysis and enzyme‐linked immunosorbent assay (ELISA) (performed in four of 11 reported cases). These analyses have targeted antigens of bullous pemphigoid, mucous membrane pemphigoid or epidermolysis bullosa acquisita, except one case of mucosal pemphigoid with antilaminin‐332 antibodies. We describe the case of a patient who presented with an ulceration on his finger 10 days after direct contact with a lamb during Eid al‐Adha. Four weeks later he developed a severe tense blistering eruption associated with mucous membrane erosions. Indirect immunofluorescence analysis using the patient's serum revealed circulating antibasement membrane IgG that bound the dermal side of salt‐split skin. ELISA was positive for recombinant immunodominant NC1 domain of type VII collagen. We finally diagnosed epidermolysis bullosa acquisita complicating probable human orf infection.     

Bullous Lupus: An Unusual Initial Presentation of Systemic Lupus Erythematosus in an Adolescent Girl

Abstract: Bullous systemic lupus erythematosus is a subepidermal blistering disease that occurs only rarely in a subset of patients with systemic lupus erythematosus and even less commonly in pediatric patients. Autoimmunity in bullous systemic lupus erythematosus is characterized by the presence of circulating anti‐type VII collagen antibodies. We report here a case of a child whose initial systemic lupus erythematosus presentation was a diffuse bullous eruption.     

Widespread Bullous Fixed Drug Eruption Mimicking Toxic Epidermal Necrolysis

This paper is written to heighten awareness of the presence of the most severe form of fixed drug eruption. Two patients with a widespread bullous form of fixed drug eruption (FDE) were initially given the diagnosis of toxic epidermal necrolysis (TEN). Both gave a history of a previous widespread eruption from the responsible drug, each had biopsies consistent with fixed drug eruption, and most importantly, both had an uncomplicated course, with complete cutaneous reepithelialization within 10 days. These observations suggest that widespread bullous fixed drug eruption may portend a more favorable prognosis than TEN, thus stressing the potential importance of distinguishing the two diseases. A review of fixed drug eruption and possible means of differentiating the widespread bullous form from TEN are discussed.     

Ibuprofen-induced bullous leukocytoclastic vasculitis

A dramatic case of ibuprofen-induced bullous leukocytoclastic vasculitis (LCV) is described in a patient with a history of prior sensitization to ibuprofen, a common household nonsteroidal anti-inflammatory drug (NSAID) that has few reported adverse skin reactions. Bullous LCV is a relatively rare clinical presentation of LCV, which requires differentiation from other blistering diseases, including bullous erythema multiforme, bullous fixed drug eruption, linear IgA bullous dermatosis, and bullous pemphigoid. The distinctive histopathologic changes of leukocytoclastic vasculitis readily distinguish this bullous eruption from the others.     

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.     

Localized bullous pemphigoid induced by thermal burn

BACKGROUND: Induced bullous pemphigoid is known to be triggered by drugs and some physical agents. Six cases of bullous pemphigoid induced by thermal burns have been reported since 1991. CASE REPORT: We observed a bullous eruption around thermal burns of the left leg in a 89 year-old woman. Bullous pemphigoid was diagnosed by histological and immunohistochemical findings with a sub-epidermal blister and linear IgG and C3 deposition at the dermal-epidermal junction. Indirect immunofluorescence was negative. The eruption rapidly resolved with topical steroids. DISCUSSION: We describe a bullous pemphigoid induced by a thermal burn. This case is original because the eruption was localized only around the site of the burn and healed with topical steroids. In the literature, there are 6 other cases reported but only one localized. We discuss the hypothesis of asymptomatic bullous pemphigoid exacerbated by presentation of bullous pemphigoid antigen by thermal burns or self immunization against antigens altered by burns.     

Penicillamine-induced pemphigus foliaceus-like dermatosis. A case with unusual features, successfully treated by plasmapheresis

A case of a severe, widespread bullous dermatosis clinically resembling pemphigus foliaceus occurred during treatment with penicillamine hydrochloride in a patient with rheumatoid arthritis. Histologically, the disease showed changes compatible with pemphigus vulgaris as well as with a bullous drug eruption. Treatment by plasmapheresis proved to be effective in controlling the disease.     

Fixed drug eruption caused by mefenamic acid: a case series and diagnostic algorithms

Background: Fixed drug eruption is a fairly common drug‐induced hypersensitivity reaction of the skin and the mucous membranes, which is characterized by the re‐occurrence of the lesion(s) exactly on the previously involved sites after repeated administration. The pathogenetic mechanisms of this site‐specificity are not fully elucidated. Patients and Methods: We report on three cases of fixed drug eruption, including a non‐pigmenting generalized bullous fixed drug eruption, caused by mefenamic acid in its pure form. Results and Conclusion: Provocation tests with the assumed causative drug represent the gold standard for establishing the diagnosis and for identifying the culprit. Advantages and pitfalls of topical and systemic provocation tests as diagnostic approaches are discussed.     

Epidermolysis bullosa acquisita-like reaction associated with penicillamine therapy for sclerodermatous graft-versus-host disease

A case of a bullous eruption occurred in a patient being treated with penicillamine for sclerodermatous graft-versus-host disease following bone marrow transplantation. After 7 days of treatment with 150 mg penicillamine, a painful bullous eruption with accompanying purpuric lesions suddenly developed in previous sclerodermatous infiltrations. A diagnosis of epidermolysis bullosa acquisita-like eruption was made, and the patient was treated with drug withdrawal and administration of cyclosporine and methylprednisolone. Epidermolysis bullosa acquisita-like reaction is an extremely rare cutaneous side effect of penicillamine. The surprisingly early onset of this eruption in lesions of sclerodermatous graft-versus-host disease might have been due to severe immune alteration.     

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.     

A case of atezolizumab‐induced photodistributed bullous pemphigoid

Immunotherapy has revolutionized cancer therapy in recent years but is associated with unique immunologically mediated adverse effects. Immunotherapy‐induced bullous pemphigoid (BP) is an uncommon but established reaction that portends significant management implications as in most instances systemic treatment is required. We report a case of immunotherapy‐associated BP in a marked photodistribution, highlighting the diverse clinical presentations of this eruption.     

Association of a pediatric bullous eruption, cutaneous and muscular atrophy, hyperpigmentation and dysmorphism. A new entity?

We report the case of a young patient who presented with dysmorphism, bullous eruption of childhood, cutaneous and muscular atrophy and hyperpigmentation. Attempts were made to find out where this case fits in the nosological framework. This young boy without any particular family history was born with facial dysmorphism consisting of micrognathia, right microphtalmos, gothic palate and left facial palsy of the peripheral type. At the age of 2 years and 9 months, generalized fragility of the skin appeared in the form of a recurrent bullous eruption. The bullae left numerous atrophic and depigmented scars; they spared the mucosae, and there was no photosensitivity. At the same time, generalized skin atrophy developed: the subcutaneous venous network was abnormally visible and there was diffuse hyperpigmentation. The hair was fine, curly and thin. The teeth dystrophic and abnormally positioned. The cornea of the right eye was invaded by conjunctiva. The bullous eruption subsided when the child was about 6 years' old, but the skin atrophy became worse and was accompanied with amyotrophy of the limbs and retraction in flexion of the joints at the extremities. Chronic ulcerations were present on the lower limbs. Statural and ponderal growth, as well as mental development were normal. The biochemical examinations performed revealed no abnormality. Light and electron microscopy of the skin showed a normal dermis-epidermis junction, but the anchorage fibres were rarefied. The dermal connective tissue was abnormal, with thin collagen fibres and disorganized fibrillae. Fibroblasts were hypoplastic and numerous. The elastic network was meagre and elastic fibres had a slashed appearance. Biopsy of a palmar nodule showed cheloid-like lesions. The nosological discussion involved some congenital bullous diseases and certain forms of connective tissue dystrophia. Among the bullous diseases, congenital poikiloderma, as described by Weary and Kindler, is unaccompanied with amyotrophy, articular retraction or dysmorphism. According to Verret et al., the bulla is located at the dermis-epidermis junction. Recessive dystrophic epidermolysis bullosa seems to differ from our case in that skin atrophy is localized and amyotrophy, dysmorphism and disorders of pigmentation are absent. In addition, we found no histological evidence of collagenolysis. Mendes Da Costa's bullous dystrophy can be excluded, as there was no nanism, microcephaly or diffuse alopecia in our patient. Among connective tissue dystrophias, acrogeria Ehlers-Danlos type IV syndrome does not include bullous eruption, amyotrophy or articular retraction.(ABSTRACT TRUNCATED AT 400 WORDS)     

Linear IgA dermatosis in association with angioimmunoblastic T-cell lymphoma infiltrating the skin: A case report with literature review

Angioimmunoblastic T-cell lymphoma (AITL) is an aggressive form of peripheral T-cell lymphoma, characterized by systemic symptoms, diffuse lymphadenopathy, hepatosplenomegaly and immunodysregulation. Half of AITL is associated with cutaneous symptoms, but only few cases with bullous eruption have been described. Association with a linear IgA dermatosis is extremely rare. Linear IgA dermatosis can be idiopathic, or linked with drug intake or neoplastic disorders. Some cases of linear IgA dermatosis presenting as toxic epidermal necrolysis (TEN) have been described, most of them being drug induced. We here present the case of a 72-year-old man recently diagnosed with AITL who developed a bullous eruption, presenting as TEN. Histopathology showed deep cutaneous involvement of the lymphoma with a sub-epidermal blistering and direct immunofluorescence revealed a heavy IgA linear deposit on the dermal-epidermal junction. A diagnosis of linear IgA dermatosis associated with cutaneous involvement of an angioimmunoblastic T-cell lymphoma was made. Chemotherapy and corticosteroids allowed cutaneous improvement but the patient died of his lymphoma shortly after.     

Epidermolysis bullosa acquisita occurring in association with systemic lupus erythematosus

A 77-year-old retired male physician with a 6-year history of systemic lupus erythematosus (SLE) developed a mechanobullous eruption, the features of which were clinically and immunopathologically consistent with a diagnosis of‘classical’epidermolysis bullosa acquisita (EBA). As EBA shares immunopathological findings with a number of cases reported as the‘bullous eruption of SLE', the clinical findings commonly recognized as‘classical EBA’may, in patients with SLE, represent a specific subset of the bullous eruption of SLE rather than a separate diagnostic entity. There arc few reports in the literature describing classical EBA in patients with SLE. Findings in this patient add further support to the suggestion that EBA occurring in association with SLE, represents a subset of the bullous eruption of SLE, the clinical features of which may be modified by genetic susceptibility or disease activity.     

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.     

Bullous pemphigoid with a prolonged prodrome

Bullous pemphigoid is an autoimmune blistering skin disease of the elderly that may be preceded by a pruritic, urticarial or eczematous eruption. We report a case of bullous pemphigoid preceded by prodromal eczematous eruptions that lasted an unusually long time of 11 years. Elderly patients with persistent pruritic or eczematous eruption of unknown etiology should be carefully followed, as bullous pemphigoid may be a potential diagnosis.