Paraneoplastic Pemphigus Associated with a Malignant Thymoma: A Case of Persistent and Refractory Oral Ulcerations Following Thymectomy

Paraneoplastic pemphigus is a rare, life-threatening autoimmune mucocutaneous blistering disease associated with underlying neoplasia, commonly lymphoproliferative tumors. Herein we report a case of paraneoplastic pemphigus with a unique autoantibody profile associated with a malignant thymoma. A 56-year-old female patient presented with relapsing oral ulcerations accompanied by erythematous papules and patches on her extremities for 2 months. Skin and mucosal biopsies identified interface dermatitis with lichenoid lymphocytic infiltration in the upper dermis. Immunoblotting and enzyme-linked immunosorbent assays revealed that the patient had multiple autoantibodies against desmoglein 1, desmocollin 1, 2, 3, laminin gamma-1, envoplakin, and periplakin. The skin lesions completely healed following thymectomy and systemic corticosteroid therapy, but the oral ulcerations persisted through a follow-up period of over 2 years.

A Case of Hailey-Hailey Disease with a Novel Nonsense Mutation in the ATP2C1 Gene

No abstract available.

A Case of Pemphigus Herpetiformis with Only Immunoglobulin G Anti-Desmocollin 3 Antibodies

Pemphigus represents a group of autoimmune blistering diseases caused by autoantibodies against desmogleins (Dsgs), a class of desmosomal cadherins. Recently, several pemphigus patients only with desmocollin (Dsc) 3-specific antibodies have been reported. Here, we report a case of pemphigus herpetiformis (PH), where only anti-Dsc3-specific antibodies but not anti-Dsg antibodies were detected. A 76-year-old woman presented with a 3-year history of blister formation. Physical examination revealed pruritic erythemas with vesicles on the trunk and legs, but no lesions of the oral mucosa. A skin biopsy specimen revealed intraepidermal blister containing neutrophils, eosinophils, and lymphocytes. Direct immunofluorescence (IF) showed immunoglobulin G (IgG) and complement 3 (C3) depositions on the keratinocyte cell surfaces. Indirect IF showed IgG anti-keratinocyte cell surface antibodies. These findings hinted at a diagnosis of pemphigus. However, repeated enzyme-linked immunosorbent assays (ELISAs) for both anti-Dsg1 and 3 antibodies proved to be negative. Immunoblotting of normal human epidermal extracts revealed Dsc antibodies, and recently established ELISAs using human Dsc1-Dsc3 recombinantly expressed in mammalian cells detected anti-Dsc3 antibodies. Based on these clinical, histopathological, and immunological findings, the patient was diagnosed as PH with only anti-Dsc3 antibodies. Treatment with corticosteroid prednisolone and steroid-sparing agent dapsone accomplished complete clinical remission of the patient.

Shikonin Promotes Skin Cell Proliferation and Inhibits Nuclear Factor-κB Translocation via Proteasome Inhibition In Vitro / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Shikonin is a major active chemical component extracted from Lithospermi Radix, an effective traditional herb in various types of wound healing. Shikonin can accelerate granulomatous tissue formation by the rat cotton pellet method and induce neovascularization in granulomatous tissue. The purpose of the study was to investigate its mechanism of action in human skin cells.</p><p><b>METHODS</b>MTS assay was used to measure cell growth. The collagen type I (COL1 ) mRNA expression and procollagen type I C-peptide (PIP) production were detected by real-time quantitative reverse transcription-polymerase chain reaction and enzyme-linked immunosorbent assay, respectively. Immunofluorescence and western blot analyses were carried out to investigate nuclear factor-κB (NF-κB) signaling pathway. Cell-based proteasome activity assay was used to determine proteasome activity.</p><p><b>RESULTS</b>In this study, we found that 10 μmol/L shikonin stimulated the growth of normal human keratinocytes and 1 μmol/L shikonin promoted growth of human dermal fibroblasts. However, shikonin did not directly induce COL1 mRNA expression and PIP production in dermal fibroblasts in vitro. In addition, 1 μmol/L shikonin inhibited translocation of NF-κB p65 from cytoplasm to nucleus induced by tumor necrosis factor-α stimulation in dermal fibroblasts. Furthermore, shikonin inhibited chymotrypsin-like activity of proteasome and was associated with accumulation of phosphorylated inhibitor κB-α in dermal fibroblasts.</p><p><b>CONCLUSIONS</b>These results suggested that shikonin may promote wound healing via its cell growth promoting activity and suppress skin inflammation via inhibitory activity on proteasome. Thus, shikonin may be a potential therapeutic reagent both in wound healing and inflammatory skin diseases.</p>

Coexistence of Psoriasis and Bullous Pemphigoid in Three Cases / 대한피부과학회지

Psoriasis vulgaris and bullous pemphigoid represent 2 clinically and histologically distinct, chronic inflammatory skin conditions. The concomitant occurrence of these 2 diseases is rare, and the pathogenic relationship between psoriasis and bullous pemphigoid remains unclear. The development of bullous pemphigoid in patients with psoriasis is considered to be related to treatments for psoriasis, especially ultraviolet therapy. However, some recent reports have suggested that an immunologic or biochemical association between these two diseases plays a role in the pathogenesis. Herein, we report 3 cases of bullous pemphigoid occurring in patients with psoriasis, and we discuss the possible pathogenic mechanisms of an association between psoriasis and bullous pemphigoid.

A Case of Mitral Valve Stenosis and Tricuspid Valve Regurgitation Accompanied by Metal Allergy Treated with Mitral Valve Replacement and Tricuspid Valve Annuloplasty / 日本心臓血管外科学会雑誌

A 66-year-old woman who had percutaneous mitral valve commissurotomy 12 years before was admitted complaining of dyspnea on effort. Echocardiography showed severe mitral stenosis and regurgitation, and moderate tricuspid regurgitation associated with atrial fibrillation. Based on her past history we suspected allergy to metal, and skin patch tests showed a positive reaction to zinc, manganese, nickel, cobalt, dichromate, stainless steel, titanium alloys, and nickel-chromium-cobalt alloys. We selected an artificial organ which would not cause an allergic reaction. The St. Jude Medical standard cuff mechanical valve was the only compatible prosthetic valve. Anterolateral right thoracotomy, instead of median sternotomy, was selected. Mitral valve replacement with a 27-mm St. Jude Medical standard cuff mechanical valve and tricuspid valve annuloplasty with a 27-mm Duran flexible band were performed. Her postoperative course was uneventful. She is doing well without any allergic symptom 18 months after the surgery.

Morphological changes of roof of subacromial bursa in patients with rotator cuff tear / 中华创伤杂志(英文版)

<p><b>OBJECTIVE</b>To investigate the morphological changes of the roof of the subacromial bursa (SAB) and its involvement extent after rotator cuff tear.</p><p><b>METHODS</b>In the experimental group, the roof of SAB was obtained from 30 cases of rotator cuff tear both at the tear site and a site 2.5-3.0 cm distal to the tear site during rotator cuff repair. In the control group, the roof of SAB was obtained from the exposed site of recurrently dislocated shoulder or fractured humeral shaft of 8 cases. The specimens were stained with hematoxylin and eosin and observed under a transmission electron microscope. The cell number was quantitated through counting the blue-stained nucleus in SAB with a computer image analysis system.</p><p><b>RESULTS</b>The number of cells increased significantly in the roof of SAB in the experimental group compared with that of the control group. However, no difference of the bursal reaction was found among the type of rotator cuff tear, the bursa thickness and the presence of fluid in the bursa. The great majority of cells were type B cells observed under the transmission electron microscope.</p><p><b>CONCLUSIONS</b>The increase in cell number in the roof of SAB in the experimental group is a reactive increase rather than an inflammatory process and the involvement of SAB is not limited in extent. The change of the roof of SAB is a secondary reaction to the rotator cuff tear.</p>