Immunofluorescence of Autoimmune Bullous Diseases

Autoimmmune bullous diseases of skin and mucosa are uncommon, disabling, and potentially lethal diseases. For a quick and reliable diagnosis immunofluorescence is essential. This article describes two variants of immunofluorescence. The direct method uses a skin or mucosal biopsy of the patient to detect in vivo bound antibodies. Indirect immunofluorescence uses patient’s serum and a substrate to visualize circulating autoantibodies. These two methods supplemented with advanced techniques allow reliable classification of autoimmune bullous diseases; not only the main entities pemphigus and pemphigoid, but also subclasses within these groups. This is important because prognosis and therapy vary among different variants of autoimmune bullous diseases.     

Lúpus eritematoso sistêmico bolhoso em gestante: relato de caso*

Systemic lupus erythematosus (SLE) can cause numerous skin lesions. Despite being rare, lupus-specifi c bullous lesions demonstrate characteristic clinical and immunopathological features and require differential diagnosis among numerous bullous conditions that may overlap with SLE. The present study presents a case of bullous systemic lupus erythematosus (BSLE) in a pregnant woman.     

Primary laryngeal and nasal septal lesions in pemphigus vulgaris.

Synchronous laryngeal and nasal septal lesions in pemphigus vulgaris are rare. The authors report a case of pemphigus vulgaris in an elderly man who was seen with lesions of the supraglottic larynx and nasal septum. The classic skin changes of pemphigus eventually developed, and the patient responded to daily treatments of 80 mg of prednisone. Steroids and immunosuppressive agents remain the treatment of choice, but a favorable prognosis is still dependent on early diagnosis and institution of therapy.     

Pemphigus Vegetans Occurring in a Split-Thickness Skin Graft

BACKGROUND: Pemphigus vegetans, a variant of pemphigus vulgaris, most commonly occurs in the flexural area. OBJECTIVE: To describe an unusual case of pemphigus vegetans occurring in a skin graft recipient site and to discuss the possible etiology. METHODS: We present a 41-year-old man who developed vegetating plaques from the graft recipient site of his left leg for 8 months. RESULTS: Based on the histopathologic findings of a skin biopsy, this case was diagnosed as pemphigus vegetans. The patient's condition was successfully treated with systemic corticosteroids and acitretin. CONCLUSION: Our case is unique in its presentation of pemphigus vegetans shortly after a split-thickness skin graft. Physicians should be aware of this entity while differentiating cutaneous lesions arising from a skin graft.     

Development of bullous pemphigoid during the haemodialysis of a young man: case report and literature survey

Haemodialysis is the most frequent form of renal replacement therapy (RRT) in patients with end‐stage renal disorder (ESRD). Patients with ESRD frequently develop skin problems, mainly xerosis, pruritus and hyperpigmentation, as well as bullous diseases, mainly porphyria or pseudoporphyria and, in some cases, bullous pemphigoid (BP). BP is the most common autoimmune sub‐epidermal blistering disease, and it predominantly affects elderly people. Clinically, BP is characterised by generalised pruritic, bullous eruptions and urticaria‐like lesions. Usually, BP is an idiopathic disorder; however, in some cases, underlying internal disorders are present, like diabetes or neurological disorders. Herein, we present a 33‐year‐old man with ESRD, maintained on haemodialysis, who developed BP. There are only six cases with BP provoked by the placement of a fistula for haemodialysis. BP in the current patient was confirmed by direct immunofluorescence (DIF) and indirect immunofluorescence using BIOCHIP. The patient responded promptly to tertracycline and 0·05% clobetasol propionate lesionally. However, the relationship between BP and the fistula for haemodialisys still remains unknown. It is highly likely that the skin injury associated with fistula placement was responsible for the alteration of the basement membrane zone (BMZ) and the stimulation of the immune system, leading to BP development. To explain the real role of fistula placement as a provocative factor in BP, other such cases are required for assessment.     

Rhabdomyolysis, acute renal failure, and multiple focal neuropathies after drinking alcohol soaked with centipede

Many Chinese like to drink alcohol soaked with creatures for promoting health. This study reports a 49-year-old male who presented with multiple focal neuropathies of the upper limbs, coagulopathy, erythematous swelling of the bilateral upper extremities and trunk with bullous skin lesions, and rhabdomyolysis associated with acute renal failure after drinking alcohol soaked with centipede. Soaking a centipede, Scolopendra subspinipes mutilans, in 53% alcohol, produced the wine. Supportive treatment was administered, and the skin lesions and renal failure improved with subsequent neurologic deficit during the week following initial presentation. Alcohol binge or immobilization was the likely cause of neuropathy, bullous skin lesions and rhabdomyolysis in the patient. However, there is a possibility that centipede venom also contributed to the illness in this patient.     

Bullous Lesions in a Patient with Systemic Lupus Erythematosus

Bullous eruptions in patients with lupus erythematosus can be difficult to diagnose as bullous lesions can develop in lupus-specific lesions, and primary blistering disorders can also occur. Additionally, these patients tend to have multiple co-morbidities making them more likely to be on many medications that can lead to bullous drug reactions. A thorough history, the clinical presentation, and histopathological findings along with direct immunofluorescence can be helpful in diagnosing most cases. The authors report the case of a woman with a long history of systemic lupus erythematosus who initially presented in their clinic for diagnosis and management of erythema dyschromicum perstans and one year later developed bullae in atypical targetoid lesions on the extremities and trunk. They discuss several blistering disorders that have been reported in patients with lupus erythematosus with a focus on features that help distinguish erythema multiforme, fixed drug eruption, and lupus erythematosus from Stevens-Johnson syndrome/toxic epidermal necrolysis. In the patient described herein, the authors favor a diagnosis of Stevens-Johnson syndrome, but the classification between erythema multiforme major and Stevens-Johnson syndrome/toxic epidermal necrolysis cannot be made in some cases.The diagnosis of bullous eruptions in patients with lupus erythematosus (LE) can be difficult to make as several different primary blistering disorders have been reported to occur in association with LE, including bullous pemphigoid, pemphigus vulgaris, dermatitis herpetiformis, epidermolysis bullosa acquisita, linear immunoglobulin A (IgA), porphyria cutanea tarda, Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN).1-3 Bullous lesions can also occur in erythema multiforme (EM). These conditions must be differentiated from the bullous lesions that can occur in cutaneous lesions of LE, which can be due to extensive vacuolar degeneration of the basement membrane (BM) or from antibodies to type VII collagen in bullous systemic lupus erythematosus (BSLE).4 Patients with LE also tend to have multiple comorbidities, making them more likely to be on multiple medications that can lead to bullous drug eruptions. To add to the difficulty, many of these conditions may mimic SJS/TEN, which can be associated with significant morbidity and mortality. A thorough history, the clinical presentation, and histopathological findings along with direct immunofluorescence (DIF) can be used to diagnose most cases, but there are some cases where a clear diagnosis cannot be made. The authors report a case of a patient with systemic lupus erythematosus (SLE) who presented with a bullous eruption and focus on a discussion of features that help differentiate fixed drug eruption (FDE), LE, and erythema multiforme (EM) from SJS/TEN.     

Subcutaneous gluteal metastasis from gastric cancer: case report and clinicopathologic analysis of 50 Japanese patients

Skin metastasis from gastric cancer is rare. We report a case of gluteal skin metastasis from gastric cancer. A 74-year-old woman underwent curative gastrectomy for advanced gastric cancer. Thirty-six months after surgery, follow-up abdominal computed tomography showed a subcutaneous gluteal tumor, and surgical extirpation was performed. Histopathologic examination of the resected tumor revealed poorly differentiated adenocarcinoma, coinciding with the histopathologic diagnosis of the resected gastric cancer. Further systemic evaluation revealed a lung metastasis and left supraclavicular lymph node metastasis. The patient was treated with chemotherapeutic reagents. However, she died of the disease 9 months after the diagnosis of skin metastasis. We reviewed the cases of 50 Japanese patients with skin metastasis from gastric cancer and analyzed the clinicopathologic features.     

Rhabdomyolysis,Acute Renal Failure,and Multiple Focal Neuropathies After Drinking Alcohol Soaked with Centipede

Many Chinese like to drink alcohol soaked with creatures for promoting health. This study reports a 49‐year‐old male who presented with multiple focal neuropathies of the upper limbs, coagulopathy, erythematous swelling of the bilateral upper extremities and trunk with bullous skin lesions, and rhabdomyolysis associated with acute renal failure after drinking alcohol soaked with centipede. Soaking a centipede, Scolopendra subspinipes mutilans, in 53% alcohol, produced the wine. Supportive treatment was administered, and the skin lesions and renal failure improved with subsequent neurologic deficit during the week following initial presentation. Alcohol binge or immobilization was the likely cause of neuropathy, bullous skin lesions and rhabdomyolysis in the patient. However, there is a possibility that centipede venom also contributed to the illness in this patient.     

Dramatic resolution of bullous pemphigoid after surgery for gastric cancer: A case report

INTRODUCTION

An association between bullous pemphigoid (BP) and internal malignancy has been suggested. However, no reports have documented a dramatic improvement in BP after surgery for gastric cancer.

PRESENTATION OF CASE

An 82-year-old Japanese woman was admitted to a local hospital for severe fatigue. On examination, she was diagnosed with BP and gastric cancer. Her BP was resistant to steroid treatment; however, it improved dramatically after surgery for gastric cancer.

DISCUSSION

In this case, a strong relationship appeared to exist between BP and gastric cancer.

CONCLUSION

This is the first report of a dramatic improvement in BP after surgery for gastric cancer.Abbreviations: BP, bullous pemphigoid; CRP, C-reactive protein     

Genitourinary complications of epidermolysis bullosa

Epidermolysis bullosa is a rare inherited bullous disorder of the skin that occasionally involves the urinary tract. We describe a child with a history of epidermolysis bullosa who presented with hematuria, urgency, and dysuria. On radiographic investigation, bladder wall lesions were found. On cystoscopy, these were bullous lesions on the urinary bladder that at biopsy were shown to have inflammatory changes without evidence of malignancy. This is consistent with rare involvement of the bladder in epidermolysis bullosa. Symptomatic treatment resolved this patient's urologic complaints.     

Localized Bullous Pemphigoid Following Orthopedic Surgery

Bullous pemphigoid is an autoimmune condition whereby the immune system forms antibodies that target the skin, resulting in the formation of blisters in a generalized and symmetric pattern. Localized bullous pemphigoid can occur in special circumstances. Here, we describe two patients that developed localized bullous pemphigoid in one lower extremity following orthopedic surgery. We propose that underlying edema following orthopedic surgery is a potential trigger for localized bullous pemphigoid.     

Scalp metastasis as an initial presentation of lung adenocarcinoma : A case report and literature review

IntroductionCutaneous metastasis from primary visceral malignancy is a relatively uncommon clinical entity, with a reported incidence of 0.22%–10% among various series. However, the presence of cutaneous metastasis as the first sign of a clinically silent visceral cancer is exceedingly rare.Presentation of caseWe describe here a case of an asymptomatic male patient who presented with a solitary scalp metastasis as the initial manifestation of an underlying lung cancer. Diagnostic evaluation revealed advanced disease.DiscussionThe report emphasizes that physicians should be aware of this rare clinical entity, and appropriate investigation should be arranged for early diagnosis and initiation of the appropriate treatment. The occurrence of skin lesions in lung cancer announces an ominous prognosis.ConclusionWe conclude that the possibility of metastatic skin disease should always be considered in the differential diagnosis in patients with a history of smoking or lung cancer presenting with cutaneous nodules.     

Ureteral cancer associated with dermatomyositis

Dermatomyositis is a rare inflammatory myopathy that has characteristic cutaneous lesions. Although many malignancies are associated with dermatomyositis, urogenital malignancies have rarely been reported to be associated with dermatomyositis. We report here on the first case of ureteral cancer associated with dermatomyositis. A 42-year-old man presented to us with a skin rash. A clinical diagnosis of dermatomyositis was made due to the skin lesions, muscle weakness, arthralgia, the increased erythrocyte sedimentation rate and the increased creatine kinase level. The patient revealed microscopic hematuria and abnormal urine cytology during the investigation for the underlying malignancy. Retrograde pyelography demonstrated a suspicious lesion in the right mid-ureter, and the ureteroscopic biopsy revealed the urothelial carcinoma. Although an operation was recommended, the patient died of pneumonia associated with his interstitial lung disease, which is one of the poor prognostic indicators of dermatomyositis.     

Mohs Micrographic Surgery-Induced Pemphigus

BACKGROUND: Pemphigus is an autoimmune blistering disease that presents with flaccid intraepidermal blisters, erosions of the skin and mucous membranes, acantholysis, and in vivo bound and circulating autoantibodies against keratinocyte antigen. Currently a handful of reports incriminate surgical trauma as an initiating factor in this disease. OBJECTIVE: To document pemphigus evolving in a wound after Mohs micrographic surgery. METHODS: Case report. RESULTS: We present a case of pemphigus that started in a Mohs surgical wound after the excision of a squamous cell carcinoma (SCC) from a 49-year-old woman. Biopsy of the preoperative lesion did not reveal pemphigus. Biopsy of the postoperative lesion revealed pemphigus with no residual SCC. CONCLUSION: We suggest that Mohs surgery, like any other skin surgery, may nonspecifically activate pemphigus. This change must be differentiated from postoperative wound infection and other causes of poor wound healing.     

Lepromatous leprosy in a kidney transplant recipient: a case report

Leprosy is a chronic granulomatous disease of the skin and peripheral nerves caused by Mycobacterium leprae. Among mycobacterial infections, leprosy is rare in renal transplant recipients. Here, we report the manifestations of lepromatous leprosy in a 41-year-old renal transplant recipient. Before the renal transplant, the patient had recurrent bullous lesions on his extremities with no systemic complaints. He was on an immunosuppressive regimen that included prednisolone (1 mg/kg/d), cyclosporine (6 mg/kg), and mycophenolate mofetil (2000 mg/d), and had 2 serologically confirmed acute episodes of cytomegalovirus infection that responded favorably to intravenous ganciclovir. The density of his bullous skin lesions decreased after renal transplant. During his regular posttransplant visits, we noticed a decrease in his eyebrow hairs on their lateral margins bilaterally. Later, he developed generalized, symmetric, erythematous papules. With a positive acid-fast bacilli with Fite staining, the results of a skin biopsy showed diffuse foamy histiocyte infiltration in the dermis. These findings are compatible with lepromatous leprosy. After antileprosy therapy, no deterioration of renal allograft function or lepra reactions was noted in a 4-month follow-up. Clinicians should consider leprosy in the differential diagnosis of skin lesions in immunocompromised hosts, and in particular, solid organ transplant recipients in endemic areas.     

Pyloric atresia associated with epidermolysis bullosa: report of two cases and review of the literature

The coexistence of pyloric atresia (PA) and epidermolysis bullosa (EB) is a rare but well-known surgical emergency in neonates. PA/EB is described by the association of atresia of the pylorus and bullous lesions on the skin. Ninety one cases have been reported in the literature to date. We present two new cases and evaluate the association of PA/ EB, its etiopathogenesis and the clinical properties. Case 1: A three-day-old female presented with nonbilious vomiting and bullous lesions 2-3 cm in diameter on the extremities. Abdominal X-ray showed a single air-fluid level in the left upper quadrant. At laparotomy, we found PA and performed a pyloro-pylorostomy. The patient died due to sepsis complication of EB two months after surgery. Case 2: A two-day-old male presented with severe dermal bullous lesions on the trunk, neck and extremities. His stomach was dilated and there was no gas distally. We found PA and performed gastroduodenostomy. Initially, he tolerated the feeding well, but he died due to severe sepsis on the postoperative 23rd day. Almost all neonates born with the PA/EB result in a fatal outcome in the first few years. The complications related to EB are usually the cause of death. Even after successful repair of PA, skin lesions lead to death due to infection.     

Cutaneous metastasis revealing a relapse of gastric linitis: Another case

INTRODUCTIONCutaneous metastasis from gastric cancer is a rare occurrence. The linitis gastric carcinoma accounts only 8.7% of all gastric cancers.PRESENTATION OF CASEWe report a case of female patient who was followed for linits cancer with peritoneal metastasis treated by six cycles of chemotherapy. After seventeen months of control, the relapse of the disease revealed by occurrence of cutaneous metastatsis.DISCUSSIONCutaneous metastasis from linit gastric is rare and the prognostic remains poor. The treatment is palliative.CONCLUSIONThis rare presentation should encourage the practitioners to biopsy any suspicion skin lesion.     

Low-level laser therapy on the treatment of oral and cutaneous pemphigus vulgaris: case report

Pemphigus vulgaris is a chronic autoimmune mucocutaneous disease that initially is manifested by painful intraoral erosions and ulcers which spread to other mucosa and the skin, generally more than 5 months after oral lesion manifestation. The treatment consists of prednisone alone or in combination with an immunosuppressive agent, and the clinical response is perceived within 2 to 4 weeks. Low-level laser therapy has been effective in accelerating the healing of injured tissue, thus inducing cell proliferation and increasing ATP, nucleic acid, and collagen synthesis. We reported two cases of pemphigus vulgaris that received systemic treatment associated with low-level laser therapy for oral and cutaneous lesions. We observed prompt analgesic effect in oral lesions and accelerated healing of oral and cutaneous wounds. Therefore, the present report suggests LLLT as a noninvasive technique that should be considered as an adjuvant therapy in oral and skin disorders in patients with PV.