Bejeweled: Chronic bullous disease of childhood in a 2-year old treated with colchicine: A case report

@#Linear IgA bullous dermatosis, also known as chronic bullous disease of childhood when present in the pediatric age group, is a rare blistering disease more predominantly seen in females less than five years old. This case describes a 2-year old girl who presented with scattered, tense vesicles and bullae on an erythematous base forming the classic “cluster of jewels” appearance. This clinical picture is often mistaken as bullous impetigo, commonly seen in children, delaying diagnosis and prompt treatment. Histopathologic examination showed subepidermal blistering with a predominantly neutrophilic inflammatory infiltrate. The direct immunofluorescence studies revealed a linear band of IgA deposition in the basement membrane zone consistent with the diagnosis of CBDC. The patient was started on colchicine and oral prednisone at 1 mg/kg/day and complete resolution was achieved within two weeks of therapy.

Bullous hemorrhagic dermatosis due to enoxaparin use in a bullous pemphigoid patient

Adverse reactions of subcutaneous low molecular weight heparin or unfractionated heparin could be complications by bleeding, heparin-induced thrombocytopenia, drug-induced liver injury, osteoporosis, and cutaneous reactions. Heparin-induced skin lesions vary from allergic reactions like erythema, urticaria, eczema to intradermal microvascular thrombosis associated with heparin-induced thrombocytopenia. There is a rare cutaneous complication, called bullous hemorrhagic dermatosis. We experienced this rare case of the cutaneous complication caused by enoxaparin. Several tense bullous hemorrhagic lesions occurred after 3 days of enoxaparin in a known bullous pemphigoid patient who had aortic valve replacement surgery with a mechanical prosthesis. The bullous hemorrhagic lesions were regressed after the discontinuation of enoxaparin but recurred after re-administration. The lesions were controlled by the administration of systemic corticosteroid and alternative anticoagulant. To date, less than 20 cases have been reported worldwide. This is the first case of bullous hemorrhagic dermatosis induced by enoxaparin, a low-molecular-weight heparin in Korea. This is also the first case of bullous hemorrhagic dermatosis in a known bullous pemphigoid patient.

Assessment of the Quality of Life in Autoimmune Blistering Skin Disease Patients / 대한피부과학회지

BACKGROUND: Autoimmune blistering skin diseases such as pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid and epidermolysis bullosa acquisita substantially affect patients' daily life and psychosocial well-being. OBJECTIVE: The aim of this study was to evaluate the quality of life (QOL) in patients with autoimmune blistering diseases and to identify the factors that can influence their QOL by comparing them to healthy controls. METHODS: Forty patients with autoimmune blistering skin diseases and 40 healthy controls were interviewed using the Korean version of Skindex-29. The study assessed the clinical severity of the disease. RESULTS: The total, symptom, function, and emotion scores of Skindex-29 were significantly higher in patients with autoimmune blistering skin diseases (35.28, 40.78, 30.57, and 36.67, respectively) than in the healthy controls (6.90, 9.38, 5.47, and 6.60, respectively) (p<0.001). Higher disease severity had a negative correlation with QOL in patients with blistering skin diseases, and QOL was lower when patients had low levels of satisfaction with treatment. CONCLUSION: The results show that autoimmune blistering skin diseases can affect patients' QOL. In addition, disease severity and low satisfaction with treatment are important factors that reduce QOL. Development of new treatments should improve treatment efficacy and the QOL of patients with autoimmune blistering diseases.

A Comparative Study for the Titer of IgG Autoantibodies in the Serum and Urine of Patients with Bullous Pemphigoid, Epidermolysis Bullosa Acqusita, and Pemphigus / 대한피부과학회지

BACKGROUND: The immunoglobulins can be detected in the urine in normal people without any renal disease. According to recent articles, it is known that autoantibodies of IgG, which share the identical antigenic specificities with the serum autoantibodies, targeting some nuclear antigens and cutaneous basement membrane zone antigens could be detected in the urine in patients with systemic lupus erythematosus and autoimmune bullous diseases. OBJECTIVE: We examined the urine for the detection of autoantibodies in patients with autoimmune bullous diseases and compared the titers of IgG autoantibodies in the serum and urine in each patient. Patients and METHODS: Nine patients were included in this study. They were 3 patient-groups of bullous pemphigoid(BP), epidermolysis bullosa acqusita(EBA), and pemphigus foliaceus(PF). Each group consisted of 3 patients having circulating autoantibodies in the serum. For a semi-quantitation of the IgG autoantibodies in the serum and urine specimens in each group, the indirect immuno fluorescence(IIF) examinations were performed. RESULTS: Seven out of nine patients showed positive results of autoantibodies in urine specimen. In the BP and EBA group, IgG autoantibodies were detected in the urine of all six patients at low titers. In the PF group, one out of three patients showed IgG autoantibodies in the urine. The high antibody titers in the sera roughly correlated with the positivity/titer of the urine IgG autoantibodies. CONCLUSION: The IIF examination of the urine could be tried for a diagnosis of autoimmune bullous diseases in some patients with active autoimmune bullous dermatoses, espacially in cases with certain difficulties in collecting serum samples.

Frequencies of Serum IgA Autoantibodies and IgA-associated Clinical Features in Pemphigus, Bullous Pemphigoid and Epidermolysis Bullosa Acquisita / 대한피부과학회지

BACKGROUND: Pemphigus (P; pemphigus vulgaris, PV; pemphigus foliaceus, PF), bullous pemphigoid (BP) and epidermolysis bullosa acquisita (EBA) are autoimmune blistering skin diseases induced by IgG autoantibodies. In some cases of these diseases IgA autoantibodies can be detected at the lesional skin as well as in the sera. OBJECTIVE: The aims of this study were to examine the frequencies of circulating disease-specific IgA autoantibodies in addition to IgG, in P, BP and EBA; possible clinical relevancies in connection with the presence of IgA autoantibodies in the sera were also reviewed with the patients in each disease. PATIENTS/METHODS: Twenty new patients of the individual diseases who showed positive indirect immunofluorescence findings of IgG were selected. Immunoblotting and multi-step immunostaining were performed ('amplified alkaline phosphatase immunoblot' preparations [Bio-Rad BRL]) with patients' sera using tissue antigens of A431 cell culture-extracts. RESULTS: Among the above 20-patient groups in P, 7 cases (PV 3, PF 4) were positive for serum IgA autoantibodies. In BP and EBA, 4 cases and 16 cases were positive for circulating IgA autoantibodies, respectively. Between the positivity-rate found in each category, patients with EBA showed a significant high positive frequency of 80% as compared with others having P or BP (p>0.045). There were no statistically significant clinical correlations between the serologic findings of IgA-positivities and the clinical expressions of the diseases including mucous membrane involvements in each disease (p>0.3). CONCLUSIONS: The frequency-rates of dual expressions of autoantibodies, IgG and IgA, were relatively lower in patients with P (35%) and BP (20%) than those patients with EBA showing a significant high rate (80%). Clinical relevancies with the presence of serum IgA autoantibodies were not seen in this study, perhaps indicating further immunopathological and clinical evaluations might be necessary to define the role of IgA autoantibodies in each disease.

Diagnostic Usefulness of Immunoblot Assay in Autoimmune Bullous Dermatoses / 대한면역학회지

Immunologic or immunopathologic assays are neccesary for the diagnosis of autoimmune bullous dermatoses including pemphigus vulgaris (PV), bullous pemphigoid (BP), and epidermolysis bullosa acquisita (EBA). The objectives of this study is to compare the sensitivity and usefulness of indirect immunofluorescence 0F) with that of immunoblot assay using amplified alkaline phosphatase staining system in the diagnosis of the above diseases; detection of disease-specific IgG autoantibodies. We selected 4 patients in each bullous dermatosis of PV, BP, and EBA, who had serum levels of IgG autoantibodies at a titer of 1:80 or higher. In each three disease, 2 patients with negative serum antibodies or serum titer lower than 1:20, were also enrolled. Among the former 4-patient groups the titers of IgG antibodies found on indirect IF were in the range of 1:80 to 1:160, whereas the titers recognized by immunoblot assay were 1 or 2 dilutions higher in most of these patients. In the latter 2-patient groups, 4 out of the 6 cases revealed antibody-positive on immunoblot-staining membrane. The indirect IF can be performed easily and seems favorable in the aspect of cost-effectiveness. However, immunoblot assay with sensitive staining method would be warranted in cases of antibody-negative or atypical clinical variants of autoimmunebullous dermatoses to confirm the diagnosis.

Depositions of Complement Components and Their Inhibitors in Atuto - immune Dermatoses / 대한피부과학회지

The complement system is known to be involved in the pathogenesis of the skin lesions in pernphigus vulgaris, bullous pemphigoid, dermatitis herpetiformis, epidermolysis bullosa acquisita, and systemic lupus erythematosus. Authors examined the skin specimens of each disease cases, who did not show any evidence of complement deficiency, to determine the deposition of complement components(C4, C3, Chb-9) and their inhibitors(C4bp, Factor H, S-protein) by modified direct immunofluorescence. We also looked at the staining pattern and localization, for further insights of their pathobiologic contributions in each disease. The findings of deposits of complement components up to C9, as well as inhibitor proteins at the primary histopathologic sites, in the majority of those cases, may indicate that the complement system, to certain extent, involves the inflamrnatory reactions in these diseases. The co-localization of C5b-9 and S-protein could be regarded as the consequence of in situ formation of SC5b-9 complexs or as the result of non-lytic adsorbed complexes of fluid phase SC5b-9. The pathologic role of the complement seems to depend mostly on the complement-fixing biologic property and the amount of the tissue bound immune complexes, which are often heterogeneous to different diseases and among different patients.

Immunofluorescent Studies of Various Chronic Bullous Dermatoses / 대한피부과학회지

The accuracy and sensitivity of both direct and indirect immunofluorescence microseopy in diagnosing chronic bullous dermatoses were evaluated and compared in 11 cases of six different disease entities(pemphigus vulgaris, pemphigus vegetans, bullous pemphigoid, linear IgA bullous dermatosis, familial benign chronic pemphigus), which had been diagnosed clinically and by routine histopa thological studies. And the results obtained were as follows: 1) In 4 cases of bullous pemphigod, the direct IF of perilesional skin showed linear deposition of IgG and C2 along basement membrane zone(BMZ), whereas the indirect IF revealed negetive findings. 2) In 2 cases of pemphigus vulgaris, the direct IF showed deposition of IgG and C2 in intercellular substance(ICS) of perilesional epidermal tissue, and the indirect IF revealed auto-antibody to ICS(1:320) in one case. 3) In 2 cases of linear IgA bullous dermatosis, which were diagnosed as dermatitis herpetiformis by clinical and routine hisopathological findings, the direct IF of perilesional and uninvolved skin manifested linear deposition of IgA, IgM, C3 and F along BMZ, However, no immunofluorescence was detected by the indirect IF. 4) In a case of chronic bullous dermatosis of childhood, the direct IF of perilesional skin showed linear deplosition of IgA and IgM along BMZ. 5) In a case of pemphigus vegetans, the direct IF revealed no specific findings, while the inderect IF disclosed auto antibody positive to ICS(1:40). 6) In a case of familial benign chronic permphigus, no immunofluorescence was found by direct IF. These results indicate that both direct and indirect immunofluorescence micros-copy are valuable in diagnosing chronic bullous dermatoses and in understanding their immune pathogenesis.