A study of benign chronic bullous dermatosis of childhood and comparison with dermatitis herpetiformis and bullous pemphigoid occurring in childhood

Eighteen patients with benign chronic bullous dermatosis of childhood were studied and the findings compared with those of dermatitis herpetiformis (twenty-two cases) and bullous pemphigoid (five cases) beginning in childhood. The patients with benign chronic bullous dermatosis of childhood had a moderately pruritic bullous eruption with maximal involvement of the pelvic and perioral regions which tended to occur at an earlier age than either dermatitis herpetiformis or bullous pemphigoid. In contrast to dermatitis herpetiformis one-third of the cases with benign chronic bullous dermaiosis of childhood went into remission. Evidence of coeliac disease was only found in the dermatitis herpetiformis group. Surprisingly both diseases shared HLA-B8. A linear BMZ band of IgA was detected on direct immunofluorescence in all but one of the cases with benign chronic bullous dermatosis of childhood and circulating antibodies were detectable in two-thirds. Routine histopathology was of little value in distinguishing between benign chronic bullous dermaiosis of childhood and dermatitis herpetiformis or bullous pemphigoid. Several paradoxes have yet to be explained before it can be determined whether benign chronic bullous dermatosis of childhood is a variant of dermatitis herpetiformis or linear IgA disease.     

Chronic Bullous Dermatosis of Childhood in Singapore

Five cases of chronic bullous dermatosis of childhood (CBDC), a rare bullous disease, are presented. Also known as linear IgA bullous dermatitis, it has significant immunofluorescence findings. Treatment with dapsone, 50 mg daily, is recommended.     

Juvenile dermatitis herpetiformis and chronic acquired bullous disease in children

Eight children with chronic acquired bullous disease were studied by direct immunofluorescence. Multiple jejunal biopsies were examined as well as tissue antigens (HLA-andthcDRw₃ B cell allo-antigen). Juvenile dermatitis herpetiformis (JDH) was diagnosed in seven patients and chronic bullous disease of childhood (CBDC) in one. The use of these different laboratory techniques may make it possible to differentiate more clearly between the different chronic acquired bullous diseases seen in childhood.     

Epidermolysis bullosa acquisita in childhood – a case mimicking chronic bullous dermatosis of childhood

Epidermolysis bullosa acquisila (EBA) is rarely reported in childhood, hut we mm describe a 6-year-old Korean girl with the condition. She presented with multiple tense bullae annularly distributed on the perioral, periorbital and genital areas, and was successfully treated with dapsoae. The clinical and histological features were similar to those of chronic bullous dermatosis of childhood. We review seven previously reported childhood EBA cases and contrast their features with those of adult EBA. We suggest that some childhood EBA is different from the adult form and shares features with chronic bullous dermatosis of childhood. Epidermolysis bullosa acquisita (EBA) is an acquired, chronic, subepidermal bullous disease,¹ characterised by IgG autoantibodies reacting with type VII collagen of the fibrils beneath the basement membrane Zone of the stratified squamous epithelium.^2,3 Its clinical and epidemiological features are not fully understood, and only a few cases in children have so far been reported.^4–8     

Childhood cicatricial pemphigoid with linear IgA deposits

Four cases showing a unique combination of scarring conjunctivitis and widespread blistering, commencing in childhood are described. In all cases linear deposits of IgA were present at the dermo-epidermal junction in association with low titres of circulating IgA antibasement membrane zone antibody in three cases. The relationship of this entity to chronic bullous disease of childhood, cicatricial pemphigoid and linear IgA disease of adults is as yet unclear and it may be a distinct bullous dermatosis.     

Linear IgA Bullous Dermatosis in a Neonate

Abstract: A newborn black boy had two facial blisters at birth that progressed to bullous lesions over the trunk, genitals, extremities, and oral and tracheal mucosa. A biopsy specimen demonstrated a subepidermal bulla with mixed eosinophilic and neutrophille, inflammatory infiltrate. Direct immunofluorescence showed linear IgA, IgG, and C3 depositions along the basement membrane zone, consistent with a diagnosis of childhood linear IgA bullous dermatosis (chronic bullous dermatosis of childhood). The skin disease was controlled with combined prednisone and dipsone. This is the youngest reported patient with the disease. Linear IgA bullous dermatosis should be considered in the differential diagnosis of blistering diseases of the newborn, and immunofluorescence should be performed on a skin biopsy specimen.     

Bullous diseases of childhood

Although the bullous diseases of childhood are rare, their differential diagnosis is vast. A logical approach to these disorders exists, and a careful history and examination of a patient will usually lead to a correct diagnosis. Confirmation of this diagnosis may depend on sophisticated laboratory procedures including immunofluorescence, electron microscopy, and tissue culture. This article reviews the clinical features, ultrastructural findings, and treatment of the various inherited forms of epidermolysis bullosa and the acquired bullous diseases, including chronic bullous disease of childhood, dermatitis herpetiformis, bullous pemphigoid, and pemphigus.     

Annular bullous diseases

Annular bullous dermatoses represent an etiologically diverse group of cutaneous phenomena that present with a figurate morphology in association with vesicles and bullae. This group of diverse conditions consists of bullous pemphigoid; pemphigoid gestationis; epidermolysis bullosa simplex, Dowling-Meara type; linear immunoglobulin A bullous dermatosis; chronic bullous disease of childhood; anti-p200 pemphigoid; subcorneal pustular dermatosis; and immunoglobulin A pemphigus. Astute examination of clinical, histopathologic, and serologic features is crucial in distinguishing these bullous dermatoses. We review the clinical presentation, pathophysiology, histopathology, and treatments for each bullous annular disease to aid physicians in their recognition, diagnosis, and management.     

IgA linear dermatosis of childhood (chronic Bullous disease of childhood)

Of twenty-seven cases of subepidermal blistering disease of children twelve corresponded clinically, histologically and immunologically to dermatitis herpetiforms of adults, six to bullous pemphigoid, and eight to chronic bullous disease of childhood (CBDC), i.e. IgA linear dermatosis. This latter disease seems to be a distinct entity, different from both dermatitis herpetiformis and bullous pemphigoid, and is characterized immunopathologically by linear IgA deposits at the basement membrane zone. These cases usually do not show intestinal involvement and respond well to combined treatment with sulphones and corticosteroids, whereas sulphones or sulphapyridine alone are, even in very high doses, not sufficient for full control of the disease. CBDC or IgA linear dermatosis of childhood may be regarded as a counterpart of IgA linear dermatosis of adults.     

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.     

Chronic Bullous Disease of Childhood with IgG Predominance: What is the Locus Standi?

Linear IgA disease (LAD) is an acquired, autoimmune, subepidermal, blistering disease, characterized by linear deposition of IgA along the dermoepidermal junction on immunofluorescence. Some cases known as 'mixed immunobullous disease' show weak staining with other immune reactants like IgG, IgM or C3. We report a rare case of a child having typical manifestations of LAD (chronic bullous disease of childhood), but with IgG predominance rather than IgA. Obviously it is improper to term this as linear IgA disease. Such cases are reported in literature as variants of LAD, with a multitude of terms like mixed immune bullous disease, linear IgG / IgA disease, linear IgA / IgG disease, and so on. In view of the tremendous confusion that these multiple terms cause in the absence of any practical benefit, we propose that the broad term 'chronic bullous disease of childhood' be applied to all childhood cases, irrespective of the nature of the immune deposits.     

Erythromycin therapy in bullous pemphigoid: possible anti-inflammatory effects

Two patients with bullous pemphigoid treated with erythromycin demonstrated improvement. This response suggests that erythromycin may be a fairly safe supplemental drug which may allow a lower dose of systemic steroid, or may be of benefit when used alone or in combination with topical steroids in treating patients who are not ideal candidates for a systemic steroid. Erythromycin appears to have a significant anti-inflammatory effect, and possible mechanisms for this effect are proposed. One of the patients was a 4 1/2-year-old girl whose disease manifested several unusual features, making diagnosis difficult. A brief summary of differential immunofluorescent findings in childhood bullous diseases is also presented.     

Linear IgA disease with haemorrhagic pompholyx and dapsone-induced neutropenia

A case of haemorrhagic pompholyx occurring in a 29-year-old man with linear IgA disease is described. There were several features in our patient that are usually seen in chronic bullous disease of childhood. Treatment with dapsone cleared the eruption but induced a progressive yet reversible neutropenia.     

Is Erythromycin an Effective Treatment for Chronic Bullous Disease of Childhood? A National Survey of Members of the British Society for Paediatric Dermatology

Abstract:  Chronic bullous disease of childhood is the commonest acquired blistering disorder of children. Erythromycin has been reported to be beneficial for this condition. A three question survey was e-mailed to all members of the British Society for Paediatric Dermatology to assess the incidence, preferred treatments and experience of oral erythromycin in treating chronic bullous disease of childhood. A second, more detailed questionnaire was sent to members who had used erythromycin. Forty patients were reported to have been treated over the previous 2 years. The preferred treatment was dapsone. Erythromycin alone had been used in five children as first-line oral treatment. In three of these patients the initial improvement was graded as either "good" or "complete resolution." This benefit was only sustained in one child, with the other two relapsing between 4 and 12 weeks. In a further eight children, erythromycin had been used with other oral agents. In five of these children, erythromycin was associated with long-term benefit. These results suggest that erythromycin is unlikely to produce sustained improvement in chronic bullous disease of childhood when used as a sole first-line agent. However, erythromycin can cause an initial improvement, which may be useful whilst awaiting results of diagnostic tests and may confer benefit when used with other systemic treatments.     

Bullous pemphigoid in childhood

A young child with clinical presentation and histology like chronic bullous dermatosis of childhood and immunopathology of bullous pemphigoid is being reported to make the readers aware of its existence in Indian population and to stress the importance of immunopathology in bullous disorders of childhood.     

Chronic bullous dermatosis of childhood.

A case of chronic bullous dermatosis of childhood in a 3-year-old boy is described. Immunoflourescence tests were negative and biopsy of the jejunal mucosa showed marked villous atrophy. The dermatosis was brought under control by a combination of diaminodiphenylsulphone and systemic steroids. The relationship with other bullous eruptions of childhood such as dermatitis herpetiformis and bullous pemphigoid is discussed.     

Linear IgA Bullous Dermatosis of Childhood with Autoantibodies to a 230 kDa Epidermal Antigen

Abstract: Linear IgA bullous dermatosis (LABD) is an autoimmune, subepidermal disease defined on the basis of direct immunofluorescence findings. However, more recent techniques used to study bullous dermatoses suggest that LABD may be heterogeneous. A patient with LABD of childhood (chronic benign disease of childhood, CBDC) was studied by indirect immunofluorescence on salt-split skin and by Western blot in an attempt to characterize the involved autoantigen. This young girl's periorificial (mouth, genitalia), erythematovesicular lesions were diagnosed initially as herpes simplex. Histologic examination revealed eosinophilic spongiosis, suggesting the diagnosis of an autoimmune blistering disease. Direct immunofluorescence showed an exclusive linear IgA deposit at the dermoepidermal junction. Indirect immunofluorescence revealed circulating IgA autoantibodies that reacted with the epidermal side of saltsplit skin; these reacted by Western blot with a 230 kDa epidermal antigen, as in bullous pemphigoid. This case, fulfilling the diagnostic clinical and direct immunofluorescence criteria for LABD/CBDC, seems to represent IgA bullous pemphigoid. It further underscores the nosologic heterogeneity of LABD, which probably includes, apart from bullous pemphigoid, epidermolysis bullosa acquisita and cicatricial pemphigoid.     

CHRONIC BULLOUS DISEASE OF CHILDHOOD—ASPECTS OF MANAGEMENT*

Five cases of chronic bullous disease of childhood are reported. The management of these cases is discussed and the use of sulphapyridine or dapsone in preference to oral corticosteroids is advocated.     

Chronic bullous disease of childhood following Epstein—Barr virus seroconversion: a case report

We report the case of a 3-year-old boy with classical chronic bullous disease of childhood (CBDC) arising after recent Epstein—Barr virus seroconversion following infectious mononucleosis. The patient also had small red cells and decreased levels of circulating IgA. He received combined treatment with dapsone and prednisone with good results Our report is the first of CBDC preceded by Epstein—Barr virus seroconversion. The virus may have had an initial immunopathogenic role in the genesis of the bullous eruption.     

Benign chronic bullous dermatosis in childhood with involvement of the mouth mucosa

We report on a rare case of benign chronic bullous dermatosis of childhood (BCBDC) in a 5-year-old girl. In addition to the characteristic symptoms such as abrupt onset of the disease, tense blisters, predominant affection of the face, and later imitation of impetigo, we observed uncommonly extensive involvement of the oral mucosa. Whereas histopathology was not diagnostic, direct immunofluorescence could verify BCBDC as it showed linear deposits of IgA along the basement membrane zone. This case gives rise to the discussion of the clinical entity and nomenclature of BCBDC in relation to similar bullous diseases of childhood and adults.