结节性类天疱疮一例

患者男,56岁.因全身红斑、丘疹、结节伴剧烈瘙痒1年于2010年3月16日来我院就诊,门诊以"结节性痒疹"收入院.患者1年前于"盐湖"洗浴后出现全身多发红色丘疹,瘙痒剧烈,搔抓后丘疹逐渐增大为直径约1 cm结节,部分表面破溃、出血.半年前背部及双侧大腿外侧部分结节表面曾有水疱出现,自行挤出疱液后水疱消退.先后就诊于多家医院,均诊断为结节性痒疹,服用止痒药、中药(具体药物不详)等治疗,皮疹无明显改善,瘙痒顽固.发病以来一般情况尚可,因瘙痒严重而影响睡眠,略感焦虑,余元不适.既往有痛风病史2年,余无特殊个人史、家族史.     

Pemphigoid nodularis: a case report

Several variants of bullous pemphigoid have been reported including pemphigoid nodularis. Patients with pemphigoid nodularis have clinical features of prurigo nodularis in combination with clinical or immunologic characteristics of bullous pemphigoid. We report the case of a 71-year-old woman with pemphigoid nodularis. The diagnosis was suspected clinically and established by positive indirect immunofluorescence (IIF) findings characteristic of pemphigoid. Results of direct immunofluorescence (DIF) testing were negative, which emphasizes the importance of conducting both DIF and IIF when pemphigoid nodularis is suspected.     

结节性类天疱疮1例

患者,女,72岁,全身丘疹、结节,伴剧痒1年余,头面部、四肢红斑、水疱1个月,无口腔水疱、糜烂.组织病理可见表皮下水疱,直接免疫荧光检查见IgG及C3于基底膜带呈线状沉积,间接免疫荧光示抗桥粒抗体弱阳性.诊断:结节性类天疱疮.     

Pemphigoid nodularis: a report of two cases

Pemphigoid nodularis is a rare variant of bullous pemphigoid characterized by the development of pruritic hyperkeratotic nodules. These nodules may be the presenting feature of the disease, and may precede the development of bullae by several years. The condition appears to be more common in females than males, and is often resistant to treatment. We report two definite cases and one possible case of pemphigoid nodularis, and review the literature relating to this disorder.     

结节性类天疱疮一例并文献复习

报道1例结节性类天疱疮并复习相关文献。患者，女，62岁。全身反复出现丘疹、结节伴瘙痒10年。查体：躯干、四肢可见暗红色丘疹、结节、斑块，未见水疱。实验室检查：IgE明显升高。组织病理示：表皮角化过度、角化不全，棘层增厚，真皮浅层血管周围轻度单一核细胞浸润。直接免疫荧光和间接免疫荧光均阴性。血清学检查示：抗BP180抗体>200 U/mL(0～20 U/mL)。诊断为结节性类天疱疮。予泼尼松、环磷酰胺治疗，继续随访中。     

Pemphigoid nodularis with IgA autoantibodies against the intracellular domain of desmoglein 1

Pemphigoid nodularis is a rare variant of bullous pemphigoid. We report a 49-year-old Japanese male with clinical and histopathological features of pemphigoid nodularis including circulating and in vivo-bound IgG antibasement membrane zone antibodies and IgA anti-intercellular antibodies. Although the precise molecular target of the IgG autoantibodies could not be determined, intriguingly, immunoblotting showed that the IgA in the patient's serum reacted with the intracellular domain of desmoglein 1, the target antigen in cases of pemphigus foliaceus. However, the IgA did not react with the extracellular domain of desmoglein 1 in sensitive enzyme-linked immunosorbent assay studies using a baculovirus system. These results suggest therefore that these IgA antibodies may possibly not be pathogenic. The mechanism for the production of different autoantibodies is unknown, but this case provides further illustration of the atypical skin immunoreactants often seen in this unusual subtype of bullous pemphigoid.     

Pemphigoid nodularis (non-bullous): a clinicopathological study of five cases

Pemphigoid nodularis is a rare clinical variant of pemphigoid characterized by overlapping clinical features of both prurigo nodularis lesions and pemphigoid-like blisters. In pemphigoid nodularis, the immunopathological findings are identical to those of bullous pemphigoid (BP). We describe five patients who presented with the typical clinical phenotype of prurigo nodularis, who were found to have circulating and tissue-bound antibasement membrane zone autoantibodies. By immunoelectron microscopy and Western immunoblotting studies, the circulating antibodies were shown to target the hemidesmosome and specifically the BP antigens 1 and 2 (BP180 and BP230). In contrast to the majority of reported cases, none of these patients has ever developed blisters. The role of antibasement membrane zone antibodies in the development of the eruption, or the role of the eruption in the development and persistence of autoantibodies, is not clear. These cases demonstrate that the presence of these antibodies is not sufficient for the development of blisters.     

Pemphigoid nodularis: a report of three cases and review of the literature

Many variants of bullous pemphigoid have been reported, some of which are clinically very atypical. One of the rarest variants is pemphigoid nodularis. We report three patients with this distinct entity. In all three cases patients initially presented with prurigo-like lesions and in one case nodular lesions preceded the onset of blistering by many months. Direct immunofluorescence was positive in all cases, with linear basement membrane zone deposition of IgG and C3. Circulating IgG antibasement membrane antibody was detected in two cases. and in these two patients on salt-split immunofluorescence the antibodies bound to the roof of the bullae.     

Letter: Lichenoid eruption induced by etanercept

Lichenoid drug eruption is an uncommon, but previously reported, side effect of anti-tumor necrosis factor therapy. The majority of these adverse events relate to infliximab. We report a patient who developed a lichenoid eruption on the back of her hands during etanercept therapy. She improved with topical treatment and discontinuation of the drug was not necessary. The physiopathological link between anti-TNF treatment and lichenoid eruptions remains unclear. It is important to realize that a lichenoid reaction pattern may occur during anti-TNF agent treatment.     

Panniculitis with crystals induced by etanercept subcutaneous injection

Panniculitis with lipid crystallization within adipocytes may be seen in several disorders, including crystal‐storing histiocytosis, gouty panniculitis, subcutaneous fat necrosis of the newborn, post‐steroid panniculitis, sclerema neonatorum, oxalosis and subcutaneous fungal infections by mucormycosis, zygomycosis or aspergillosis. Panniculitis at the sites of subcutaneous injection of drugs are frequent, but to our knowledge no crystals have been described in the drug‐induced panniculitis at the sites of subcutaneous injections. We report on a patient who developed a panniculitis with lipid crystallization at the site of etanercept injection.     

Thrombocytopenia possibly induced by etretinate in a psoriatic patient

A patient with psoriasis vulgaris developed multiple purpura on the extremities and hemorrhage at the oral mucosa and gingiva with marked thrombocytopenia. This thrombocytopenia was possibly induced by a retinoid agent, etretinate, from the clinical course and data. The total amount of etretinate administered was 2,410 mg over 191 days (41.9 mg/kg body weight, average 15.3 mg/day). A platelet transfusion partially restored the platelet count and the purpura and gingival hemorrhage disappeared approximately 10 days after the cessation of etretinate. However, the platelet count remains at 60-80 x 103/mm3 after two and a half years without etretinate therapy. Although there are only a few case reports of etretinate-induced thrombocytopenia, we should pay more attention to the peripheral platelet count during etretinate therapy.     

Multiple keratoacanthomas possibly induced by psoralens and ultraviolet A photochemotherapy

We report two patients who developed multiple keratoacanthomas while receiving photochemotherapy (PUVA). Both patients had received excessive doses of PUVA, which resulted in severe phototoxicity. The role of PUVA in the production of these lesions is discussed.