Complement-fixing abilities and IgG subclasses of autoantibodies in epidermolysis bullosa acquisita

Epidermolysis bullosa acquisita (EBA) is an autoimmune-mediated subepidermal bullous disease in which the target of the autoantibodies is type VII collagen, a major component of anchoring fibrils. The purpose of this study was to evaluate the complement-fixing abilities and IgG subclass distribution of autoantibodies in EBA, and to also attempt to investigate the relation between inflammation, complement fixation and IgG subclass distribution in EBA patients. Only 2 sera of 18 patients (11%) showed weak complement-fixing abilities. IgG1 and IgG4 were the most frequently and intensely stained IgG subclasses in EBA sera. We could not find any relationship between the clinico-pathologic types, complement-fixing abilities and IgG subclasses in EBA. These results suggested that complement activation may not be a key factor of bulla formation in EBA.

Two Cases of Multiple Pilomatricoma / 대한피부과학회지

Pilomattiaoma, aften called calcifying epithelioma of Malherbe, is a benign tumor originating from the outer root sheath cell of the hair follicle and extending into the hair matrix, Pilomatricoma usually occurs as a single, asymptomatic dermal or subcutaneous nodule. Multiple lesions are quite unueuel, comprising only 2-3.5% of cases. We report two patients with multiple pilomatricoma.

Two Cases of Bullous Pemphigoid Showing the Characteristics of Herpes Gestationis Autoantibodies / 대한피부과학회지

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.

A Case of Impetigo Herpetiformis Terminating in Fetal Death / 대한피부과학회지

Impetigo herpetiformis is a form of pustular psoriasis that occurs during pregnancy and may be life-threatening. The pathogenesis and etiology of impetigo herpetiformis is not clear, but several reports have associated onset of this disorder with hypocalcemia, hypoparathyroidism, and the use of oral contraceptives. The typical lesions are erythematous patches that are studded with tiny superficial pustules particularly coalescing at their margins causing pain, and a burning sensation. Laboratory findings include an elevated erythrocyte sedimentation rate, increased peripheral blood leukocyte counts, hypoalbuminemia, and in severe cases, hypocalcemia. The histopathology is the same as that of pustula psoriasis. Our patient, a 31 year-old female (Intra Uterine Pregnancy 28 weeks), was admitted to our hospital due to generalized skin eruption and severe constitutional symptoms. Her clinical features, laboratory and histopathologic findings were compatible with that of impetigo herpetiformis. In spite of our all efforts, intrauterine fetal death was noted at her 33rd week of pregnancy. We report a case of impetigo herpetiformis terminating in fetal death with the review of literature.

Clinicopathological Characteristics of Erythema Multiforma: A Comparison of Palmoplantar Erythema Multiforme with Erythema Multiforme Involving Other Siers / 대한피부과학회지

BACKGROUND: Erythema multiforme is an inflammatory disorder of the skin which may be characterized by erythematous papules, plaques, vesicles, and 'target-like' annular lesions. Mucosal lesions may occur. Symmetric irivolvement of the extrernities, especially the dorsurn of the hands and feet, and face is the classic pattern of cutaneous distribution. Palmoplantar lesions of erythema multiforme are relatively uricornmon. OBJECTIVE: Our purpose was to investigate the clinical and histopathological characteristics of erythema multiforme involving the palm and sole. METHODS: Thirty one cases of erythema multiforme involving the palm and sole, and thirty cases involving other sites wer collected and analysed with particular reference to epidemiological data, type and distribution of the skin lesion and histopathological characteristics. RESULTS: 1. The median ages of both groups were the early thirties. There were no sexual predominances. 2. The clinical features of erythema multiforme involving the palm and sole were relatively mild and showed atypical skin lesions including macules and papules compared with erythema multiforme involving the other sites. 3. The histopathological changes of the palmoplantar group were also mild, especially the epidermal changes which included keratinocytes necrosis, subepidermal blisters, focal parakeratosis, extensive epidermal necrosis and xocytosis etc. 4. The most important and consistent histopathological finding of the palmoplantar erythema multiforme group was lymphocyte tagging along the basernent membrane, which was found consistently apart from one case. CONCLUSION: We investigated the clinical and histopathological features of erythema multiforme involving the palm and sole. They showed mild clinical features and atypical skin lesions cornpared with other group. The histopathological spectrum of this group was diverse, and reflected the early stage of pathomecha nisms of erythema multiforme. Among them, lyrnphocyte tagging was the most important and censistent finding in palmoplantar erythema multiforme.

A Case of Weber-Cockayne Epidermolysis Bullosa Simplex - Ultrastructural Findings / 대한피부과학회지

Epidermolysis bullosa simplex (EBS) is a group of autosomal dominantly inherited genetic disorders characterized by blistering due to mechanical- stress-induced degeneration of basal epiderrnal cells. Recently, it was discovered that EBS is induced by keratin 5 and 14 gene mutations. Weber Cockayne (W-C) EBS is the mildest type, with blistering concentrates primarily on palar and plantar regions, and basal cell cytolysis by keratin filament perturbations is present. Herein we report a case of W-C EBS with its ultrastructural findings. Electron microscopy showed cytolysis and separation of the basal epidermal cells, mainly at the subnuclear cytoplasm. The cyto- plasm of basal cells showed edema, loosening and intact rnitochondria. Besides the cytoplasmic changes, the nucleus also showed lytic degeneration. Characteristically, dense condensation of tonofilarnent was observed, which suggests that W-C EBS is. also a disorder of keratin.

Hypereosinophilic Syndrome on the Extremities Associated with Abnormal Peripheral Blood Circulation / 대한피부과학회지

Hypereosinophilic syndrome is characterized by marked eosinohia and eosinopbilic infiltrition of various organ-systems without any identifiable underlyin sathologic conditions such as hypersensitivity, drug eruption, parasitic infestat,ion, malignant tuinc, and autoimrnune and infectious d iseases. A 31 year-old male had weight loss, abnormal peripheral cirrultiction both extremities and two distinct skin lesions. One showed rice sized, erythematous follicular popules on both extremit.ies and trunk and the other showed a walnut sized, gangrenous ulcer on the left 2nd finger tip. The patients skin lesions and circulation defect. improved after retrnent with steroid and the blood eosinophilia returned to normal.

Cutaneous T Cell Lymphoma Involving Subcutaneous Tissue / 대한피부과학회지

T-cell lymphomas primarily involving subcutaneous tissue and minicking panniculitis have rarely been described. In addition, some cases may have been orviously reported as malignant histiocytosis. Some of the affected patients have developed a hemohagocytic syndrome. A 44-year-old wornan showed multiple, recurrent, ill-defined, sarble sized, erythematous, tender subcutnaeous nodules on the trunk and extremities. Histopathological findings levealed the infiltration of hyperchrorriatiatypical large lymphocytes and nurnerous beanbag-like cells in the subcutaneous fat. The infitrted cells were positively stained with Leucocyte common antigen, UCHL-1 antigen, Lysozyme, that ot with CD20. Bone marrow biopsy showed mild erythreid hyperplasia and erythorophagocytic. hitiocytes. These histopathological features were consistent with cutnaeous T cell lymphoma involvnig the subcutaneous tissue.

A clinical and pathological study of Becker's nevus / 대한피부과학회지

BACKGROUND: Beckers nevus is a benign lesion that may be congental or acquired, macular or papular, hairless or hypertrichotie. OBJECTIVE: Our pcrpose was to describe the clinical findings of Eeckers nevus and to establish a method for the histologic diagnosis of Beckers nevus. MEHTODS: We reviewed PF skin biopsies and their clinical finding from 28 patients with Beckers nevus during a 10 year period from January 1981 to January 1991. Results : The results were summarized as follows. A Clinical characteristics 1. Sex distribution showed of male predominence with male female a ratio of 1.5:1. Age of onset was predominent in less that 5 years old and puberty. 2. The lesions were located on the trunk(24 cases), head & neck(8 cases), extremities(6 cases), and were repsilateral in 26 cases, bilateral in 2 cases. 3. The lesions were accompanied by hair in 17 cases(61%). The shaies of the lesions were reticulated (75 %), patch(18%), mottled(7%). 4. Hairs were distributed as follows : abdomen(100%), scapula(100 %), extremities (83%), cheek(80 %), neck(67 %), chest(40%). All lesions of the face had follicular papules. 5. Associated skin diseases were : smooth muscle hamartoma(3 cases), striae distensa(2 cases), steroid acne(2 cases), pigmented nevus(2 cases), and verruca plana(1 case). B. Histopathologic characteristics 1. The lesions were accompanied with plugs in 21 cases, which were follicular, acrosyringeal, epidermal. 2. Elongation of the rete ridge and hyperpigmented basal cells were observed in the epidermis in all cases. 3. The shape of the rete ridge in Beckers nevus has its characteristic appearance, which is single, bridging and reticulated. Single, bridging and reticulated shapes cexisted in 17 cases(61%). 4. Inflammatiry cell infiltrations were present in all of the upper drmis, in which melanophages(21 %), hair follicles(89%) and smooth muscle hamartoma(3cases) w ere observed CONCLUSION: These findings indicated that the shape of the rete ridge in Beckers nevus has its characteristic appesrance and can be useful for the diagnosis of Beckers nevus.

Three cases of earty dyskeratoma / 대한피부과학회지

Warty dyskeratoma is a rare, well-circumscribed epidermal tumor that frequently aries as a single lesion on the skin of the head or neck of middle aged or older persons. Its histopathological features include : suprabasilar clefting with villous proliferations of the connective tissue papillae into the clefts ; acantholytic and dyskeratoic epithelial cells,basilar hyperplasia, and hyperleratosis. We report three cases of warty dyskeratoma describing clinical and histochemical fea tures.