Linear arrangement of neutrophils along the Basal layer in bullous pemphigoid: a unique histological finding

Bullous pemphigoid is an inflammatory autoimmune subepidermal bullous disease with distinct immunohistological features. We report an unusual case of a 59-year-old woman with a bullous eruption whose lesional skin biopsy showed a subepidermal blister with a linear arrangement of neutrophils, mimicking linear IgA bullous dermatosis. However, direct immunofluorescence studies demonstrated IgG and C3 linear deposition along the basement membrane zone, compatible with bullous pemphigoid. We suggest that bullous pemphigoid should therefore be considered in the differential diagnosis of neutrophil-rich subepidermal bullous diseases along with dermatitis herpetiformis and linear IgA.     

Eccrine syringofibroadenomatous hyperplasia in a patient with bullous pemphigoid: a case report and review of the literature

A 76-year-old man with multiple erythematous lesions on his palms and soles which appeared following bullous pemphigoid (BP) is the subject of this case report. The lesions were not raised above the normal skin level, and there were no nodules on the erythematous lesions. The lesions had the histologic appearance of eccrine syringofibroadenoma. This condition is considered to be not a true tumor but a hyperplasia of eccrine sweat ducts following recurrent subepidermal blister formation in BP.     

大疱性Sweet综合征合并克罗恩病一例

患者,男,65岁.因腹泻1个月,全身红斑、大疱、血疱5天伴发热入院.皮肤组织病理示:表皮角化过度,局部表皮缺失,表皮下疱形成,真皮乳头高度水肿,真皮浅中层多数中性粒细胞浸润.结肠组织病理特征符合克罗恩病改变.结合临床及病理表现诊断为大疱性Sweet综合征合并克罗恩病.治疗:给予甲泼尼龙40 mg/d,发热逐渐消退,皮疹...     

寻常性银屑病并发成人型线状IgA大疱性皮病

报告l例寻常性银屑病并发成人型线状IgA大疱性皮病.患者男,36岁.因全身红色斑疹伴白色鳞屑反复发生20年.躯干、双上肢出现环状排列的水疱10d伴瘙痒就诊.皮损组织病理检查:表皮下水疱,疱内、真皮浅层和真皮乳头见中性粒细胞、嗜酸性粒细胞浸润;皮损周围皮肤直接免疫荧光显示基膜带Iga、IgG呈带状沉积;取患者血清行BP180NC16A(大疱性类天疱疮18 000抗原的近膜片段)-ELISA检查显示阴性;以盐裂正常人皮肤为底物,取患者血清行间接免疫荧光检查显示IgA、IgG呈带状沉积在真皮侧.诊断为寻常性银屑病并发成人型线状TgA大疱性皮病.     

Autoimmune vesicles on the face and neck. A variant of Brunsting-Perry type localized bullous pemphigoid?

We report a case of blistering disease presenting a unique distribution of vesiculobullous lesions on the face and neck which is similar to Brunsting-Perry type of localized bullous pemphigoid (BP). Histopathology of a lesional skin biopsy demonstrated a subepidermal blister. Direct immunofluorescence demonstrated a strong linear deposition of IgG and IgA to the basement membrane zone, and a faint staining for C3. However, circulating antibodies were not detected by indirect immunofluorescence and immunoblotting. And the patient did not develop atrophic scars and was a relatively young woman. This case might be a variant of Brunsting-Perry type of localized BP or localized epidermolysis bullosa acquisita, presenting the clinical heterogeneity of subepidermal blistering diseases.     

Mast cells in developing subepidermal bullous diseases: emphasis on tryptase, chymase and protease inhibitors.

The possible involvement of mast cell tryptase and chymase in subepidermal bullous diseases was studied enzyme-histochemically in specimens from erythematous and vesicular skin and from non-involved skin of patients with dermatitis herpetiformis, bullous pemphigoid, erythema multiforme, infective bullous eruption and linear IgA dermatosis. Patients with pemphigus were biopsied for comparison. The immunoreactivity of chymase inhibitors, alpha1-proteinase inhibitor (alpha1-PI) and alpha1-antichymotrypsin (alpha1-AC), in mast cells was demonstrated using the sequential double staining method. Tryptase-positive mast cells were unchanged or only slightly increased in number in erythematous lesions and slightly decreased in blistering skin compared with healthy-looking skin. Only occasionally were mast cells seen in apparent contact with the basement membrane zone. Chymase-positive mast cells and the chymase/tryptase ratio steadily decreased during the development of the lesions in each subepidermal bullous disease. The percentage of alpha1-PI+ and/or alpha1-AC+ mast cells increased simultaneously, which could explain the disappearance of chymase activity. Similar results were obtained regardless of the bullous disease. The results were also similar in pemphigus, which is an intraepidermal bullous disease. In conclusion, these results show significant alterations in mast cell chymase and protease inhibitors in a range of different bullous diseases, suggesting mast cell involvement. The apparent inactivation of chymase could be due to the action of chymase inhibitors detected in numerous mast cells. However, these alterations probably reflect general inflammation rather than a specific reaction in a certain bullous disease.     

A subepidermal blistering disease with histopathological features of dermatitis herpetiformis and immunofluorescence characteristcs of bullous pemphigoid: a novel subepidermal blistering disease or a variant of bullous pemphigoid?

A 64-year-old man presented with a bullous eruption which clinically and histopathologically resembled dermatitis herpetiformis. However, direct immunofluorescence analysis showed IgG deposits at the basement membrane zone, indicating a relationship with bullous pemphigoid or epidermolysis bullosa acquisita. Indirect immunofluorescence studies on salt-split skin showed binding of IgG mainly on the dermal side of the blister. Immunoblot analysis revealed a novel 200 kDa dermal antigen that could be associated with a major pathogen in this blistering a disease. The histopathological similarity to dermatitis herpetiformis and the immunofluorescence findings indicating bullous pemphigoid or epidermolysis bullosa acquisita seem typical of a distinct subepidermal blistering disease characterized by this 200 kDa antigen. However, the pathogenetic role of autoantibodies against this antigen should be further elucidated before confirming whether this case represents a novel subepidermal blistering disease or a special variant of bullous pemphigoid.     

Disseminated cicatricial pemphigoid in a child and in an adult. Ultrastructural diagnostic criteria and differential diagnosis with special reference to acquired epidermolysis bullosa

Summary The first case of an infant affected with a rare, disseminated variant of benign cicatricial pemphigoid is described, showing the same ultrastructural features of initial blister formation as an adult patient. These consist in edematous changes within the superficial dermis caused by vesiculation or dissolution of cellular and noncellular connective tissue elements, coalescing into subepidermal blisters. Differential diagnosis excludes other nonhereditary bullous disorders because of the ultrastructure of the dermo-epidermal junction in nascent blisters and in perilesional skin. In spite of evident clinical, histological, and immunohistological similarities as well as controversial and confusing immunological studies, acquired epidermolysis bullosa can be clearly separated from our case by a diagnostic hallmark on the electron-microscopical level, i.e., band-like IgG depositions beneath the basal lamina. This is demonstrated in comparing the two cases of disseminated cicatricial pemphigoid with three patients suffering from acquired epidermolysis bullosa, thus providing evidence that disseminated cicatricial pemphigoid and acquired epidermolysis bullosa are two distinct nosologic entities.     

Bullous pemphigoid associated with dermatomyositis and colon carcinoma

It has been suggested that both bullous pemphigoid (BP) and dermatomyositis are associated with internal malignancies. However, there are no reports of the coexistence of these three conditions in one patient. We report the first case, to our knowledge. of coexistent BP, dermatomyositis and colon carcinoma. An 81-year-old Japanese woman was diagnosed with dermatomyositis based on muscular weakness and tenderness of the extremities, increased levels of circulating muscle enzymes and histological inflammatory changes in skeletal muscle and skin. Carcinoma of the sigmoid colon was detected during screening for internal malignancy; she was therefore surgically treated. Ten days after the operation, several large bullous eruptions appeared on her extremities. From the histopathological, immunofluorescence microscopy and BP180 ELISA study findings, we diagnosed the bullous eruptions as BP. Coexistence of BP with dermatomyositis or colon carcinoma is rare. Although the reason why our case exhibited these three disorders is not understood, it is suggested that these conditions may be associated with epitope spreading.     

Severe dermatomyositis with pronounced generalized subcutaneous edema and dysphagia: A rare manifestation of a highly active disease

Severe subcutaneous edema is rare in dermatomyositis (DM). Such patients usually have a highly active disease, which requires aggressive treatment. To date, there are 14 reported cases of generalized edema secondary to adult DM. We described a severe case of DM manifesting generalized edema, oropharyngeal dysphagia, and dysarthria. A 44-year-old female presented with a typical rash of DM, proximal muscle weakness, and marked swelling of the limbs and face. The findings in the skin biopsy, muscle enzymes, and electromyography were consistent with DM. No internal malignancy was detected. After a brief initial response to oral dexamethasone, the patient experienced a sudden worsening of muscle weakness with dysarthria and an inability to swallow even saliva. A magnetic resonance imaging study revealed edema of the subcutaneous tissue and muscles. The symptoms improved gradually in 2 months after intravenous pulse corticosteroid therapy. Generalized subcutaneous edema is a very rare manifestation of DM that can occur as a presenting symptom. It appears to be a hallmark of a severe DM that requires prompt and aggressive treatment. Additional cases are needed to establish guidelines for treatment of this rare variant.     

Sweet's syndrome with bullous lesions

We present a case of Sweet's syndrome with atypical lesions, characterized by erythematous plaques, vesicles and bullous lesions. Skin lesions in patients with an underlying malignancy are more frequently atypical and with vesicular, bullous or even ulcerative characteristics, in addition to the typical plaques and nodules. However, the case presented is not associated with malignancy, despite the fact that these processes, particularly hematologic ones, should be suspected.     

Case with Brunsting–Perry‐like localized subepidermal blister formations and immunoglobulin G antibodies against unidentified basement membrane zone antigen

Brunsting–Perry type bullous pemphigoid is defined by the blister formation limited to the head and neck, and autoantibodies to type VII collagen are detected in several cases. However, the pathomechanisms and autoantigens in this condition remain unknown. We report a 20‐year‐old female patient with a more than 2‐year history of recurrent tense blisters localized on the face with no distinct atrophic scar formation. The patient had neither extensive sun exposure nor a history suggestive of contact dermatitis. Oral betamethasone was effective on the skin lesions. Histopathology revealed subepidermal blister formation with dermal infiltrates of neutrophils. Although direct and indirect immunofluorescence tests detected immunoglobulin G antibodies to the basement membrane zone (BMZ), no known dermal or epidermal autoantigens were detected in immunoblot analyses. Therefore, this case may be a rare variant of Brunsting–Perry type localized bullous pemphigoid with autoantibodies to an undetermined BMZ antigen.     

Immunoelectron microscopic observations in a case of linear IgA bullous dermatosis of childhood

A 16-month-old boy exhibited vesicobullous skin lesions on the lower part of his abdomen, in his genital region, and on the buttocks and extremities. The eruptions presented small, tense bullae with or without erythematous changes of the surrounding skin, some of which clustered around the resolving lesions. Histologic examination of the eruptions showed subepidermal, ultrastructurally suprabasal laminar blister formation with infiltration of the polymorphonuclear cells and large mononuclear cells in the blister cavity. Immunoelectron microscopy revealed IgA deposits at the basilar surface of the basal keratinocytes of the lesional skin, as well as the presence of circulating IgA-class antibodies reacting to the basilar surface of the hemidesmosomes. The eruptions responded well to a combination therapy of small doses of a corticosteroid agent and a sulfonamide. There were neither clinical nor laboratory findings suggesting dermatitis herpetiformis. This case, diagnosed as linear IgA bullous dermatosis of childhood by immunofluorescence microscopy, ultrastructurally had a pathogenic site close to that observed in bullous pemphigoid.     

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.     

DERMATOMYOSITIS: A CLINICAL OF TWENTY-THREE CASES IN HONG KONG

SUMMARY.— Twenty-three cases of dermatomyositis are reported of which 12 (52%) were associated with internal malignancy. In the patients over 40 years of age, malignant diseases were encountered in 69%, while only one of 7 cases under 40 had associated malignancy. Nasopharyngeal carcinoma accounted for 75% of the malignant disease.
A distinctive skin eruption, consisting of hyperkeratotic, follicular, erythematous papules, is described. On the face, trunk and limbs, the papules tended to become confluent. Along the tendons and over the bony prominences on the backs of the hands and feet, the eruption was usually arranged in a linear fashion. There was alopecia of the scalp. On the palms and soles were hyperkeratotic papules or plaques. The occurrence of this form of eruption in dermatomyositis may be influenced by racial factors.     

Polymorphic pemphigoid. Is it still worth using this terminology?

The term polymorphic pemphigoid has been used in the literature as a variant of bullous pemphigoid. But this term is imprecise and now obsolete, since patients with linear IgA dermatosis have been reported under this terminology. The patients who develop an atypical subepidermal bullous disease with clinical and histological features of both bullous pemphigoid and dermatitis herpetiformis may actually be classified into three groups: (1) vesicular variant of bullous pemphigoid; (2) linear IgA dermatosis, and (3) mixed subepidermal bullous disease.     

Acrokeratosis paraneoplastica with bullous lesions associated with esophageal squamous cell carcinoma

Acrokeratosis paraneoplastica (Bazex syndrome) is characterized by an acral eruption with a psoriasiform appearance, which usually presents simultaneously with an underlying neoplasm. We describe the case of a 64-year-old male who presented with a two-month history of pruritic, flaky, erythematous lesions on the palms, backs of the fingers and toes and pinnae, accompanied by bullous lesions. The patient also reported progressive dysphagia in the last six months, and general wasting with a loss of 15 kg. The digestive endoscopy revealed a squamous cell carcinoma in the proximal esophagus. The histopathological study of the bullous lesions showed the presence of a subepidermal bulla, and direct immunofluorescence revealed granular deposits of IgG, IgA and C3 in the basal membrane of the healthy perilesional skin. These clinical/pathological findings support the existence of an immunological pathogenic mechanism related to this entity.     

Human Herpesvirus‐8: A Useful Marker for Distinguishing Kaposi Sarcoma and Kaposi Sarcoma‐like Pyogenic Granuloma

Malignant mesothelioma presenting as a primary umbilical tumor is not reported in the English literature, to our knowledge. We present a 63‐year‐old woman with a painless and initially skin colored nodule in the umbilical area, which within one month became erythematous. The performed biopsy revealed an infiltrating glandular and ductal proliferation involving the dermis, subcutaneous fat and underlying skeletal muscle embedded in a loose and mucinous stroma. The tumor cells were of medium size with round to oval nuclei and prominent nucleoli. Mitoses were conspicuous. The tumor cells showed a positive reaction for kermix, EMA, CK5/6, calretinin and CK7 and a negative reaction for CK20, CEA, Ber‐Ep4, S100 protein, TTF‐1, PRP, ERP, and Leu‐7. Since calretinin is both a sensitive and specific marker of reactive and neoplastic mesothelial cells, a diagnosis of malignant mesothelioma, epithelioid variant, was made. The performed exploratory laparotomy showed no evidence of malignancy and wide excision of the umbilical area showed no evidence of residual tumor. Follow‐up examinations of the patient with repeated CT scans for the past two years were negative for recurrent or metastatic disease. We conclude that this malignancy likely developed in a setting of umbilical abnormalities associated with mesothelial remnants.     

Periorbital edema as the presenting sign of dermatomyositis

BACKGROUND: Periorbital edema can occur in dermatomyositis, which is characterized by symmetric macular erythema, Gottron's papules, Gottron's sign, periungual telangiectasia, heliotrope rash, and poikilodermatous macules on the shoulders, arms, or upper back (shawl sign). CASE REPORT: We report the case of an 81-year-old man with dramatic periorbital edema. It was not until he was hospitalized with dysphagia 6 months after developing the edema that the diagnosis of dermatomyositis was considered. RESULTS: Laboratory tests, skin biopsy, and electromyography resulted in a diagnosis of dermatomyositis. CONCLUSIONS: Periorbital edema may appear as the presenting cutaneous manifestation of dermatomyositis.