No abstract available.
Cushing Syndrome* ; Female ; Immunization, Passive* ; Pemphigoid Gestationis*

Herpes gestationis is a rare pregnancy-associated autoimmune bullous disease resembling bullous pemphigoid in both clinical and immunohistopathological aspects. Generally it may be improved spontaneously or easily controlled by topical or low-dose corticosteroid. Very rarely, however, severe and long-standing cases that did not respond to high-dose corticosteoid therapy were reported. We present a case of an unusual severe herpes gestationis in a 26-year-old woman that was resistant to high-dose corticosteroid and/or dapsone therapy. But the lesions responded dramatically to low-dose cyclosporine.
Adult ; Cyclosporine* ; Dapsone ; Female ; Humans ; Pemphigoid Gestationis* ; Pemphigoid, Bullous

Bullous pemphigoid(BP) and herpes gestationis(HG) are subepidermal bullous diseases which show clinical and immunological similarities. Both diseases show immune deposits along the basement membrane zone and their autoantibodies bind a common antigenic site within the non-collagenous stretch of the 180 kDa BPAG2 ectodomain. Besides its association with pregnancy, HG has some characteristic features that distinguish it from BP. The serum of patients with HG often contains an IgG that avidly fixes complement, and showes IgG1 subclass predominance. We report here two cases of non-pregnant young women presenting clinical and histological features of bullous pemphigoid or herpes gestationis. The immunopathology, IgG subtyping and immunoblotting studies showed that the autoantibodies in the patients were the characteristic ones of herpes gestationis. The patients might be a subtype of BP that have characteristics of autoantibodies of HG patients.
Autoantibodies* ; Basement Membrane ; Complement System Proteins ; Female ; Humans ; Immunoblotting ; Immunoglobulin G ; Pemphigoid Gestationis* ; Pemphigoid, Bullous* ; Pregnancy

Pemphigoid gestationis, also known as herpes gestationis, is a rare blistering disease associated with pregnancy, caused by autoantibody to the basement membrane component. It occurs in the second or third trimester of pregnancy or directly after delivery, as erythematous, urticarial papules, and plaques on the whole body, which aggravate to vesicles and bullae with severe pruritus. Histopathologically, subepidermal blisters and eosinophilic spongiosis are shown, and linear deposition of C3 in the dermoepidermal junction is seen on direct immunofluorescence assay. We present the case of 35-year-old woman who was diagnosed with pemphigoid gestationis, with the involvement of the uncommon site of the face.
Adult ; Basement Membrane ; Blister ; Eosinophils ; Female ; Fluorescent Antibody Technique, Direct ; Humans ; Pemphigoid Gestationis* ; Pemphigoid, Bullous* ; Pregnancy ; Pregnancy Trimester, Third ; Pruritus

Herpes gestationis(HG) characterized by the development of autoantibodies to the basement membrane is a pruritic bullous dermatosis of pregnancy and the postpartum period. HG is diag nosed on the basis of clinical findings, and a direct or indirect immunofluorescent test including an assaying for the denmonstration of IgG autoantibody in the serum. We report a case of herpes gestationis in Korea which showed a typical clinical and histopathologic findings accuring in a 30-year old primiparous patient. We could confirm the diagnosis in this case by in vitro C3 staining, and immunoblot analysis which revealed the presence of antibodies in her sertim against 180kD antigen of epidermal protein.
Adult ; Antibodies ; Autoantibodies ; Basement Membrane ; Diagnosis ; Female ; Humans ; Immunoglobulin G ; Korea ; Nose ; Pemphigoid Gestationis* ; Postpartum Period ; Pregnancy ; Skin Diseases

BACKGROUND: Bullous pemphigoid is a rare autoimmune bullous disease characterized by subepidermal bulla. The serum of these patients contains detectable autoantibodies which bind to the hemidesmosome of the basement membrane zone. It is well known that there are two bullous pemphigoid antigen molecules, 230KD and 170KD protein. Serum studies in Caucasian patients show that 70-80% of the patients recognize 230KD antigen while 10-30% recognize 170KD antigen, In contrast, in herpes gestationis, which is an autommune-mediated bullous disease of pregnancy, 90% of the patients recognize 170KD and 10% recognize 230KD antigen, The autoantibody of herpes gestationis(HG factor) has a strong complement binding capacity and it may share the same epitope as the antibody of bullous pemphigoid patients which recognize 170KD antigen. OBJECTIVE: The purpose of this study was to characterize the clinical and histological manifestations of Korean patients with bullous pemphigoid and to characterize the autoantibodies of Korean bullous pemphigoid patients by immunoblotting. We also wished to compare the characteristics of the antibodies with that of American bullous pemphigoid patients, and to elucidate the hypothesis that the bullous pemphigoid autoantibody against 170KD protein has the same strong complement binding capacity as the herpes gestationis autoantibody. MEHTODS: We investigated the clinical and histological characteristics of 9 Korean patients and also performed a complement binding capacity and immunoblotting study on the sera of 9 Korean patients and 16 American patients. RESULTS: 1. Korean bullous pemphigoid patients clinically showed polymorphic skin eruption in addition to tense bullae. They frequently showed pruritic erythematous patches and urticarial plaques. Histologically, infiltration of subepidermal bullae with eosinophils, neutrophils and lymphocytes were observed in all patient's specimen, in which eosinophils were the most predominant cells, Uncommonly, eosinophilic spongiosis, vaculoar degeneration of basal cells were observed. These observations did not have any particular characteristics or racial differences compared to those patients reported in Western literature. 2. In the immunoblotting study of Korean bullous pemphigoid patients, 7 of 9 sera(785) recognized 230KD antigen and, also, 7 of 9 sera(78%)recognized 170KD antigen, In contrast, 15 of 16 American patients sera (94%) recognized 230KD antigen and 6 of 16 patients sera(38%) recognized 170KD antigen. The high incidence of the autoantibody against 170KD in Korean patients shows possible racial differences in autoantibody formation. 3. There was no relationship between the types of autoantibodies typed by immunoblotting and the complement binding capacity. In other words, autoantibodies against 170KD antigen do not carry the same potential as autoantibodies of herpes gestationis for the complement biding capacity, CONCLUSION: The above results suggest tha there may be racial difference in bullous pemphigoid autoantibodies between Korean bullous pemphigoid patients and American patients. In conclusion, We conclude that 170KD bullous pemphigoid antibodies do not always have the same strong complement binding as herpes gestationis antibody.
Antibodies ; Autoantibodies ; Basement Membrane ; Complement System Proteins* ; Eosinophils ; Female ; Hemidesmosomes ; Humans ; Immunoblotting* ; Incidence ; Lymphocytes ; Neutrophils ; Pemphigoid Gestationis ; Pemphigoid, Bullous ; Pregnancy ; Skin ; Transcutaneous Electric Nerve Stimulation

The specific dermatoses of pregnancy represent a heterogeneous group of pruritic skin diseases that have been recently reclassified and include pemphigoid (herpes) gestationis, polymorphic eruption of pregnancy (syn. pruritic urticarial papules and plaques of pregnancy), intrahepatic cholestasis of pregnancy, and atopic eruption of pregnancy. They are associated with severe pruritus that should never be neglected in pregnancy but always lead to an exact work-up of the patient. Clinical characteristics, in particular timing of onset, morphology and localization of skin lesions are crucial for diagnosis which, in case of pemphigoid gestationis and intrahepatic cholestasis of pregnancy, will be confirmed by specific immunofluorescence and laboratory findings. While polymorphic and atopic eruptions of pregnancy are distressing only to the mother because of pruritus, pemphigoid gestationis may be associated with prematurity and small-for-date babies and intrahepatic cholestasis of pregnancy poses an increased risk for fetal distress, prematurity, and stillbirth. Corticosteroids and antihistamines control pemphigoid gestationis, polymorphic and atopic eruptions of pregnancy; intrahepatic cholestasis of pregnancy, in contrast, should be treated with ursodeoxycholic acid. This review will focus on the new classification of pregnancy dermatoses, discuss them in detail, and present a practical algorithm to facilitate the management of the pregnant patient with skin lesions.
Adrenal Cortex Hormones ; Cholestasis, Intrahepatic ; Female ; Fetal Distress ; Fluorescent Antibody Technique ; Histamine Antagonists ; Humans ; Mothers ; Pemphigoid Gestationis ; Pemphigoid, Bullous ; Pregnancy ; Pregnancy Complications ; Pruritus ; Skin ; Skin Diseases ; Stillbirth ; Ursodeoxycholic Acid

BACKGROUND: Autoimmune bullous diseases (ABDs) are a rare significant group of dermatoses that pose great challenges to the dermatologist. So far, few epidemiological surveys for the whole spectrum of ABDs have been completed in Korea, though many epidemiological survey have focused on single ABDs. OBJECTIVE: The aim of this study was to evaluate the incidence and clinical findings of patients in the Chonnam Province of Korea with ABDs over a 5 year period. METHODS: A retrospective analysis was conducted on 52 ABDs patients from 2002 to 2006. ABDs were diagnosed chiefly by histopathologic findings, immunofluoresence (IF), and immunoblotting with epidermal and dermal human foreskin extract. For the exact diagnosis of ABDs, several additional diagnostic methods such as indirect IF with salt-split normal skin, immunoblotting (IB) with normal keratinocyte cells, enzyme-linked immunosorbent assay (ELISA) with recombinant proteins, immunoprecipitaion (IP) were also performed. RESULTS: Out of 52 patients diagnosed with ABDs during the study period, pemphigus vulgaris was observed to be the commonest ABD (38.5%) followed by bullous pemphigoid (29.8%), pemphigus foliaceus (17.8%), epidermolysis bullosa acquisita (5.8%), paraneoplastic pemphigus, pemphigoid gestationis (3.8%), and linear IgA bullous dermatoses (1.9%). CONCLUSION: This study showed the incidence of the ABDs in Chonnam-Gwangju province area were very low (52 cases over 5 years) and was stationary over this period. Pemphigus vulgaris and bullous pemphigoid were common diseases among many ABDs. The incidence of ABDs in Korea as a whole needs to be investigated.
Enzyme-Linked Immunosorbent Assay ; Epidemiologic Studies ; Epidermolysis Bullosa Acquisita ; Female ; Foreskin ; Humans ; Immunoblotting ; Immunoglobulin A ; Incidence ; Keratinocytes ; Korea ; Pemphigoid Gestationis ; Pemphigoid, Bullous ; Pemphigus ; Recombinant Proteins ; Retrospective Studies ; Skin ; Skin Diseases ; Skin Diseases, Vesiculobullous

Herpes Gestationis is a rare polymorphous, vesiculobullous disease of pregnancy and puerperium. It is characterized by rapidly progressive skin eruption heraled initially by severe generalized pruritus. Erythematous papules and urticaria like plaques rapidly appear followed by grouped, tense, vesiculobullous eruptions. A 23 year-old pregnant woman was admitted to our departrnent of dermatology hecause of erythematous patches with pruritic vesiculobullous and pustular eruption on the whole body since third week of conception, Biopsy specimen of bullous skin lesion showed spongiosis of epidermal cells, mild necrosis of basal cells, subepidermal bulla, edema, of papillary derrnis and perivascular infiltration of inflammatory cells. Ultrastructural findings showed mild necrosis of basal cells, bulla above or below basement membrane zone and partial separation of basal larnina. Direct immunofluorescences of bullous lesion and normal appearing skin demonstrated deposition of IgG in linear pattern along basement rnembra,ne zone. After treatment with systemic corticosteroid and antihistamin, the lesions were cleared, leaving residual hyperpigmentation, and she delivered normal female baby.
Basement Membrane ; Biopsy ; Blister ; Dermatology ; Edema ; Female ; Fertilization ; Fluorescent Antibody Technique, Direct ; Humans ; Hyperpigmentation ; Immunoglobulin G ; Necrosis ; Pemphigoid Gestationis* ; Postpartum Period ; Pregnancy ; Pregnant Women ; Pruritus ; Skin ; Transcutaneous Electric Nerve Stimulation ; Urticaria ; Young Adult