Chronic Bullous Dermatosis Of Childhood Research Articles

Clinical features of chronic bullous dermatosis of childhood

Chronic bullous dermatosis of childhood (CBDC) is a rare autoimmune subepidermal blistering disease, which can develop following vaccination or medication, or with an autoimmune condition or illness, among other causes. To identify and better understand the clinical features of CBDC by performing a systematic review, in line with the Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines. Eligible studies included publication since 1980, CBDC diagnosis, case studies, subjects aged < 18 years, clinical features and no language restriction. A database search was conducted including Embase, MEDLINE and Scopus on 14 July 2021 (see Appendix for search terms). Data were assessed for risk of bias. Jamovi was used for statistical analysis. Age and sex were compared with mucocutaneous involvement, cutaneous involvement, other symptoms, human leucocyte antigen type and lesion descriptions. After removing duplicate references using Endnote, 351 papers were identified, of which 91 met the inclusion criteria. These papers included 130 cases of CBDC: 110 children and 20 neonates. The ratio of male : female patients was 19 : 1 for neonates and 74:55 for children. χ² analysis with 1 degree of freedom showed that CBDC in neonates was associated with facial (χ²(1) = 9.67, P < 0.01), mouth (χ²(1) = 31.0, P < 0.001), upper airway (χ²(1) = 52.7, P < 0.001), oesophageal (χ²(1) = 34.6, P < 0.001), ophthalmic (χ²(1) = 6.27, P = 0.01) involvement and with respiratory distress (χ²(1) = 22.7 P < 0.001). CBDC in children was associated with genital (χ²(1) = 3.96, P < 0.05), arm (χ²(1) = 6.99, P < 0.01) and leg (χ²(1) = 7.03, P < 0.01) involvement. CBDC in male patients was associated with facial (χ²(1) = 7.01, P < 0.01), scalp (χ²(1) = 5.96, P < 0.02) and perianal (χ²(1) = 7.22, P < 0.01) involvement. Neonates with CBDC are more likely to have a mucocutaneous distribution of lesions, whereas children are more likely to have cutaneous lesions. The limitations of this study include selection bias, and the small neonate sample size makes the study unrepresentative of the population. The review highlights the need for further research into the clinical features of CBDC in neonates.

Diagnostic challenge: chronic bullous dermatosis of childhood

Chronic bullous dermatosis of childhood is a rare acquired autoimmune disease characterized by subepidermal blistering disease of childhood with linear deposition of IGA on immunofluorescence. A 3-year-old female child presented with lesions mimicking Hand foot mouth disease which gradually progressed to isolated multiple bullous pruritic lesions all over the body. The absence of a characteristic string of pearl appearance, the histopathological findings, and the response to immunosuppressants lead us to the diagnosis of pox like lesions. Histopathology revealed parakeratosis with increased eosinophils. The child finally responded to a combination of cyclosporine and steroids. Although CBDC is common in lower trunk and genitalia it can also present as generalized bullous lesions. Here the current study reports a case of chronic bullous dermatosis of childhood that presented as a diagnostic challenge.

A 50-Year-Old Male with Dapsone Hypersensitivity Syndrome

Dapsone, diamino-diphenyl sulfone, is an antibacterial and anti-inflammatory drug which is used worldwide for treating many diseases, such as leprosy, dermatitis herpetiformis, linear IgA bullous dermatosis, chronic bullous dermatosis of childhood, bullous eruption of systemic lupus erythematosus, erythema elevatum diutinum, leukocytoclastic vasculitis, polyarteritis nodosa and other kinds of vasculitis, prurigo nodularis, nodulocystic acne, cutaneous mycetoma, pustular psoriasis, malaria, pneumocystis carinii pneumonia etc. Since it is widely used, the adverse effects of this drug attract the attention of doctors from different specialities. Dapsone can cause several adverse effects, the most serious one is idiosyncratic systemic hypersensitivity syndrome, namely dapsone hypersensitivity syndrome (DHS), which is potentially fatal, characterized by fever, facial edema with infiltrated papules, generalized papulopustular or exanthematous rash which may extend to exfoliative dermatitis, eosinophilia, lymphadenopathy, hematologic involvement and organ involvement such as hepatitis, nephritis, pneumonitis, encephalitis, myocarditis occurring after 3–6 weeks of drug therapy. Here we have described a case of dapsone hypersensitivity syndrome developed after dapsone therapy for prurigo nodularis. The case is being reported to emphasize the need for timely diagnosis and prompt treatment of this rare complication for successful outcomes. Physicians should be aware of this infrequent but potentially fatal severe form of adverse reaction that can mimic other conditions.J Enam Med Col 2018; 8(2): 108-113

Clinicopathological Study of 55 Cases of Immunobullous Diseases in Jordan

Background: Immunobullous skin diseases are a serious group of skin disorders with variable presentations that need to be diagnosed early for better prognosis. A proper clinical and histopathological evaluation is needed for correct diagnosis, so it is an important issue to share experience and to highlight this hot spot in dermatology. Objective: To have a clinical and histopathological insight into the immunobullous skin diseases as a group to show their distribution in our region and to share experience for proper early diagnosis. Material and Methods: All cases of immunobullous skin diseases that had been diagnosed clinically and histopathologically at referral centers in Jordan, Amman (King Hussein Medical Center, Jordan University hospital) and in private practice from Jan. 2000 to Jan. 2012 were included in this retrospective study. All cases were analyzed clinically and histopathologically. Hematoxylin and Eosin stained sections were used for histopathological analysis. Immunoflourscence study was done on 16 cases only and Tzanck smear examination after staining with Giemsa stain was done on 5 cases. Results: From Jan. 2000 to Jan. 2012 a total number 55 cases of different immunobullous skin diseases had been diagnosed clinically and histopathologically from a total number of 2156 skin biopsies that had been performed for different reasons and examined during that period of time. There were 21 male patient and 34 female patients with M:F ratio 1.2: 2. Age range of the patients is 4-85 yr. The distribution of different immunobullous diseases in decreasing frequency was; bullous pemphigoid 24 patients (44%), Pemphigus vulgaris 14 patients (25%), Pemphigus folaceous 5 cases (9%), Pemphigoid gesatationis 4 patients (7%), dermatitis herpetiformes 3 patients (5%) chronic bullous dermatosis of childhood 2 patients (8%), pemphigus erythematosus, one patient (4%), linear IgA dermatosis, one patient (4%), and lichen planus Pemphigoides one patient (4%). Histopathological features were conclusive in most of the cases. Tazanck smear test was a helpful test in cases of pemphigus group. The study reveals a rare form of pemphigus vulgaris as pemphigus with scalp lesions only. Rare histopathological features of bullous pemphigoid including cell poor blister and festooning were also seen. Conclusion: Immunobullous skin diseases are serious skin diseases that affect all age groups. Proper clinical and histopathological examination is essentially conclusive for diagnosis. In some cases immunoflourscence study is needed for diagnosis. Simple bedside test like Tzanck smear test is helpful in some cases

An unusual guest in the panel of differentials!

Scabies is a common infestation in children with the varied presentation. Unusual types of clinical presentation of scabies are encountered generally in patients with immunosuppression either due to disease conditions or external medications in the form of immunosuppressant. This poses a diagnostic dilemma and a therapeutic challenge to the treating physician. Reporting of atypical presentation of scabies manifestations widens the horizon and increases the chances of rapid diagnosis and appropriate treatment. We hereby report a case of 6-month-old child of scabies presenting with a cluster of jewel appearances of the lesions masquerading a chronic bullous dermatosis of childhood like picture.

Direct immunofluorescence of skin and hair in pemphigus

Background: The outer root sheath of the anagen and telogen hair is structurally similar to the epidermal layer of the skin. Hence Direct Immunofluorescence(DIF) demonstrating Antibodies to Desmoglein 1 and 3 in Pemphigus group of autoimmune bullous diseases would show a similar pattern in skin & hair. Aim: To compare DIF of skin and telogen hair in pemphigus group of patients in clinically active cases and those in remission. Method: 30 consecutive diagnosed cases of Pemphigus vulgaris (PV) (both active cases and remission) were selected for the prospective study. Other blistering diseases –Dermatitis herpitiformis, Chronic Bullous Dermatosis of childhood and vasulitis were excluded. Skin biopsy and telogen hair obtained by combing were subjected to Direct Immunoflurescence. (Fluorescein Isothyocyanate) FITC –conjugated rabbit antihuman IgG was used. The samples were viewed under fluorescence microscope using a blue filter (490 nm). Green fluorescence was observed. The pattern of staining & intensity of staining were compared. Results: 30 cases of Pemphigus included 22 cases of Pemphigus vulgaris(PV), 5 cases of Pemphigus Foliaceous(PF) and 3 of pemphigus erythematosus(PE). Pemphigus Vulgaris and Pemphigus Foliaceous patients with active disease showed strong ICS(Intercellular substance) deposits in both skin and hair. 37.5% and 50% of Pemphigus Vugaris and Pemphigus Foliaceous in remission showed weak deposits in hair only. All cases of Pemphigus Eryhtematosus showed only ICS pattern with no (BMZ) Basement membrane zone deposits. Conclusion: Direct Immunoflurescence of skin and hair are comparable in pattern and intensity. Telogen hair is a useful tool in remission period as it is easily accessible and picks up potential cases likely to develop relapse and therefore requiring close follow up. Keywords: DIF, Telogen hair, Skin, Pemphigus, Disease activity

IgA LINEAR BULLOUS DERMATOSIS IN CHILDHOOD

IgA linear bullous dermatosis, also known as chronic bullous dermatosis of childhood, is an autoimmune disease which may be idiopathic or drug-induced. The disease affects children and adults. We present a 4 years old girl with itchy polymorphic eruptions. The skin rash was presented by bullous-erosive rosette-like lesions with reddish-brown crust in the center, distributed on the skin of the face, trunk and extremities. The vesicles were filled with serous and hemorrhagic content. Laboratory examinations were within normal values according the age. Histopathological examination of the lesional skin revealed sub epidermal blister. Direct immunofluorescence of perilesional skin demonstrated linear deposition of IgA in the basement membrane. Systemic treatment with Methylprednisolon and Claritromycin was applied with satisfactory effect. The patient is under observation.

Autoimmune basement membrane and subepidermal blistering diseases

Autoimmune mucocutaneous blistering diseases (ABDs) represent a group of conditions that manifest with blisters on the skin and/or mucous membranes. Bullous pemphigoid (BP) is the most common autoimmune mucocutaneous blistering disease. In BP, the location of the blisters is subepidermal and the oral involvement is rare. Variants of BP have been described, including pemphigoid vegetans; however, this disease is not completely characterized. The majority of ABDs have blisters and/or vesicles, that are often pruritic, and manifest autoantibodies to diverse proteins. These proteins include 1) hemidesmosomal plaque proteins(ie, BP230, plectins), 2) transmembrane proteins such as BP180 and α6β4-integrin, which are connected via laminin 332 to type VII collagen and 3) currently uncharacterized 105 kDa and 200 kDa molecules. Other ABDs include drug-induced linear IgA disease, bullous systemic lupus erythematosus (BSLE), dermatitis herpetiformis (DH), cicatricial pemphigoid (CP; also termed mucous membrane pemphigoid), lichen planus pemphigoides (LPP), pemphigoid gestationis (PG), herpes gestationis(HG), chronic bullous dermatosis of childhood (CBDC) and the localized forms of CP, such as Brunsting-Perry pemphigoid. The diagnosis of ABDs requires clinical data; skin biopsies (in 10% buffered formalin) for hematoxylin and eosin (HE IgG, IgM, IgA, other immunoglobulins, complement components and fibrinogen may be detected. Indirect immunofluorescence (IIF) yields antibody titer data; the titers usually correlate with disease activity and with ELISA. Linear epitopes are commonly studied by using an immunoblotting (IB) assay. Topical and systemic corticosteroids remain as mainstays of therapy in ABDs; however, multiple other immunosupressors and/or “steroid sparing agents” such as azathioprine have been demonstrated to be of therapeutic value. In the IgA mediated dermatoses, dapsone is often helpful; in addition, liver and blood testing (including G6PD levels) is indicated. The prognosis depends on each case; rapid diagnosis avoids complications and assists in maintaining a good quality of life for each patient.

Unusual clinicopathological and immunological presentation of chronic bullous dermatosis of childhood (linear IgA dermatosis)

Linear IgA bullous dermatosis is a rare sulfone-responsive subepidermal blistering disorder of unknown etiology in which smooth linear deposits of IgA are found in the basement membrane zone. Chronic bullous dermatosis of childhood is equivalent to linear IgA disease of adulthood and is characterized by an abrupt onset of large, widespread and tense bullae on a normal or erythematous base. In this case, we describe an unusual presentation of chronic bullous dermatosis in a 14-month-old Saudi girl. Histopathological examination revealed subepidermal cell poor blisters with linear deposition of IgA, IgG, IgM, and C3 along the dermoepidermal junction. The unusual clinical, histopathological and immunofluorescence findings in this patient are discussed, with an account on the differential diagnosis in such cases along with a detailed review of the relevant literature.

Case report: an example of international telemedicine success

An 8-month old girl presented to the Angkor Hospital for Children in Siem Riep, Cambodia with fevers, bilateral eye discharge and an extensive body rash. The rash consisted of large, fluid-filled bullae and significant desquamation. The patient was admitted to the hospital and given intravenous cloxacillin for presumed bullous impetigo. Despite treatment with antibiotics, the skin lesions did not improve and the fevers continued. Telemedicine consultations were initiated by email between Angkor Hospital for Children and paediatric specialists in the USA. Several diagnoses were entertained throughout the subsequent collaborative dialogue. Ultimately, teleconsultation led to a diagnosis of chronic bullous dermatosis of childhood (CBDC), a rare sub-epidermal blistering disease. The child was started on appropriate medications. Within 24 hours, the lesions showed significant improvement and fevers resolved. By enabling advice from distant providers on diagnosis and treatment of paediatric patients, telemedicine may improve health care in developing countries.

PL1 Subepithelial bullous diseases – topic overview

Subepithelial bullous diseases comprise the group of mucocutaneous autoimmune blistering diseases characterized by subepithelial separation and the deposition of immunoglobulin and complement against several antigens along the basement membrane zone (BMZ). This result in spectrum of diseases that affect skin, oral mucosa, and other mucosal membranes and include bullous pemphigoid (BP), mucous membrane (cicatricial) pemphigoid (MMP), linear IgA disease (LAD), and chronic bullous dermatosis of childhood (CBDC). The most common clinical features are oral erosions, desquamative gingivitis and conjunctival fibrosis, as well as skin lesions, predominantly in older female population. The heterogeneity of clinical presentation and diversity of target autoantigens have contributed to difficulties in characterizing this condition immunologically. In addition to the clinical presentation and a subepithelial vesicle or bullae on routine histologic analysis, the diagnosis is based on direct and indirect immunofluorescence studies. The nature of the disease is determined by the target antigens in the epithelium and BMZ such as antigen 180 (BP180), antigen 230 (BP230), laminin 5, and beta 4 integrin. Circulating IgG and IgA antibodies bind to different epitopes of BP180. The use of salt‐split skin substrate enables differentiation between epidermal and dermal ’binders’. Since the antigen and the antibody titer appear to have direct relationships with the disease severity, and a combination of clinical finding and antibody titer provides valuable prognostic data, these investigations should be carried out routinely. Clinicians should recognize clinical spectrum of SBD, the histopathologic and immunopathologic characteristics, the differential diagnosis, the treatment, and the natural history of the disease. Involvement of oral medicine specialists, dermatologists, ophthalmologists, otolaryngologists and gastroenterologists contribute to early diagnosis and will aid in providing SBD patients with the highest quality of care.

Allergic skin reactions in the tropics

Cutaneous allergic reactions are relatively common in tropical countries, with some caused by the tropical environment and others by changes in the traditional living conditions combined with poverty. HIV infection is a major contributory factor. A large number of reactions are eczematous. Atopic dermatitis is on the increase together with contact allergic reactions. Available products are often of inferior quality and contain irritants and allergens that may be forbidden elsewhere in the world. The extensive and uncontrolled use of drugs and indigenous medications together with an increase in HIV prevalence leads to drug eruptions such as the Stevens-Johnson syndrome and toxic epidermal necrolysis. Poverty plays an important role in the prevalence of insect bites and resulting papular urticaria and possibly in acropustulosis infantum. Many unexplained environmentally induced blistering diseases occur, ranging from phytophotodermatitis to fogo selvagem and from pemphigus foliaceus to chronic bullous dermatosis of childhood. This article describes the allergic conditions that occur in the tropics. Special attention is given to those cases of eczema and eruptions that are specific to tropical countries. Available treatments are also discussed.

Response of chronic bullous dermatosis of childhood to a combination of dapsone and nicotinamide

Response of chronic bullous dermatosis of childhood to a combination of dapsone and nicotinamide

The Shed Ectodomain of Collagen XVII/BP180 Is Targeted by Autoantibodies in Different Blistering Skin Diseases

Collagen XVII/BP180, an epidermal adhesion molecule, exists as a full-length transmembrane protein and as a soluble 120-kd ectodomain that is shed from the keratinocyte surface by furin-mediated proteolysis. Despite a number of studies on autoantibody targets in blistering skin diseases, it has remained unclear whether the physiologically shed ectodomain of collagen XVII plays a role as an autoantigen. Here we isolated the authentic, soluble form of human collagen XVII and showed that it is an autoantigen recognized by IgG and IgA autoantibodies in different blistering skin diseases and is, in some cases, the preferential target. The ectodomain was isolated from the epidermis, keratinocyte media, amniotic fluid, and pemphigoid blister fluid, and autoantibodies affinity-purified with this ectodomain bound to the proximal surface of the epidermis in normal skin but not in collagen XVII-deficient skin. The antibody reactivity was not dependent on the native conformation or the N-glycosylation of the soluble ectodomain, but was abolished by collagenase treatment. Sera of 81 patients with a clinically active blistering skin disease were reacted with full-length collagen XVII, the authentic soluble ectodomain, and recombinant fragments. In bullous and cicatricial pemphigoid, IgG reactive with full-length collagen XVII also recognized the soluble ectodomain. In linear IgA dermatosis and chronic bullous dermatosis of childhood, IgA targeted the soluble ectodomain more efficiently than the full-length protein. The use of recombinant fragments demonstrated that epitopes were present in several noncollagenous and collagenous subdomains of the molecule, and that a significant portion of the sera targeted Col15 domain, a hitherto unrecognized epitope region.

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

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

CHRONIC BULLOUS DERMATOSIS OF CHILDHOOD: DIFFERENTIATION FROM OTHER AUTOIMMUNE BLISTERING DISEASES IN CHILDREN

An 8‐year‐old girl presented with a 1‐week history of intensely pruritic vesicles and bullae on the trunk and extremities. The patient was otherwise in good health and was not taking any medications.Physical examination revealed multiple large, tense bullae on normal‐appearing skin on the trunk, extremities, and pelvic region. Some of the bullae were hemorrhagic, while others were grouped in circular clusters (Fig. 1A, B). The oral cavity and conjunctivae were spared. Complete blood cell count, electrolytes, serum urea nitrogen, creatinine, and liver function studies were normal.Histopathologic examination of perilesional skin demonstrated a subepidermal blister with neutrophils in the dermal papillae (Fig. 2). Direct immunofluorescence of the specimen showed heavy deposition of IgA in a linear pattern in the basement membrane zone (Fig. 3). Broad but faint deposits of IgM were also present. Blood obtained for indirect immunofluorescence revealed no circulating autoantibodies.The patient was diagnosed with chronic bullous dermatosis of childhood and treated with dapsone (2 mg/kg) with dramatic improvement. New blister formation and puritis were suppressed within several days. All lesions healed with mild hyperpigmentation but without scarring.

Successful treatment of chronic bullous dermatosis of childhood with colchicine

Successful treatment of chronic bullous dermatosis of childhood with colchicine

Linear IgA bullous dermatosis of childhood (Chronic bullous dermatosis of childhood)

Linear IgA bullous dermatosis of childhood (Chronic bullous dermatosis of childhood)

Chronic bullous dermatosis of childhood-clinical and immunological features seen in African patients

During the period 1985-88, 30 children with a chronic blistering dermatosis were studied. Of these 25 were found to have chronic bullous dermatosis of childhood (CBDC) and five had bullous pemphigoid (BP). No case of dermatitis herpetiformis (DH) was seen in the same period. Except for the difference in immunofluorescence (IMF) there were no definite clinical, histological or therapeutic differences between the two groups. All the children were Africans with the exception of one Indian girl. Their ages ranged from 1 year to 12 years with a mean of 5 years. The females outnumbered the males in a ratio of 3:2. All children had a generalized eruption consisting of large tense blisters arising on normal skin. The blisters were more profuse on the lower trunk, pelvic region and limbs. Face and scalp were also affected. Histological features of BP and DH were seen. Direct IMF in the CBDC patients showed linear deposits of IgA at the basement membrane zone (BMZ) while linear deposits of IgG were seen in the BP group. Complement and IgM were also seen in some cases in both groups. Sixty per cent of the CBDC patients showed IgA BMZ antibodies by indirect IMF. There were no symptoms or signs of malabsorption. Serum vitamin B12 and folate levels were normal. HLA studies showed the B-8 antigen in five of the 20 patients studied. Therapy was difficult in most cases. All patients haemolysed on therapeutic doses of dapsone, sulphapyridine and/or prednisone had to be added. Follow-up was generally poor as six patients failed to return after discharge from hospital.(ABSTRACT TRUNCATED AT 250 WORDS)

Use of 1M NaCl split skin in the indirect immunofluorescence of the linear IgA bullous dermatoses

We compared 1M NaCl split skin with intact skin as substrates for detection of circulating IgA anti-basement membrane (BMZ) antibodies in linear IgA dermatosis (LAD). The sera of 63 patients with LAD including 27 adults and 36 with chronic bullous dermatosis of childhood (CBDC) were examined. 62% of patients overall had circulating IgA anti-BMZ antibodies detectable on intact skin. 73% of patients had circulating antibodies detectable on 1M NaCl split skin as an additional 7 sera were positive. This was a statistically significant increase (p less than 0.01). The sera were mostly positive at a higher titre on the split skin when compared with intact skin. On routine indirect immunofluorescence (IIF) all positive sera produced linear fluorescence on the epidermal side of the split. Twenty serum samples were incubated with split skin overnight; 4 of these specimens exhibited linear fluorescence on the epidermal and dermal sides of the split after this prolonged incubation. These findings suggest that 1M NaCl split skin is a more sensitive substrate for detection of circulating IgA anti-BMZ antibodies in LAD, that these antibodies are heterogeneous and that the target antigen has an epidermal component.