Multiple Sclerotic Fibromas in an HIV‐Positive Patient Without Evidence of Cowden''s Disease

Dermatomyositis is an inflammatory condition of the skin and muscles, and an underlying malignancy is noted in 10% or more of cases. Clinical features of dermatomyositis include increasing general fatigue and proximal (thighs and shoulders) muscle weakness accompanied by erythematous lesions of the skin. There have been several distinct types of dermatomyositis described. Here we describe a case of vesiculo‐bullous dermatomyositis, which is a rare variant of dermatomyositis. A 49‐year‐old woman was admitted to our hospital with a painful erythematous vesicular eruption of the face, trunk and extremities. In addition, edema of the face and fever were observed. Clinically, dermatomyositis was considered because of typical skin rashes (Gottron's papules, periorbital heliotrope rash and poikiloderma) and serum creatine phosphokinase level of 1,031 IU/L. A skin biopsy was performed. Microscopically, subepidermal vesiculation with marked edema was present. Lymphoplasmacytic infiltration was also observed in the upper dermis. So far only a few case reports of vesiculo‐bullous dermatomyositis have been reported in the literature. It should be kept in mind that dermatomyositis may present subepidermal vesiculation in order to avoid a misdiagnosis and unnecessary delayed treatment. Furthermore, an internal malignancy should be considered in such a variant of dermatomyositis.     

Dermatomyositis,Carcinoma of Colon and Meningioma in the Same Patient

Dermatomyositis and carcinoma of colon were diagnosed in a 66-year-old woman. Meticulous physical examination excluded further systemic or cutaneous involvement. The musculocutaneous disorders responded well to daily oral corticosteroid, and the malignant tumor was totally removed surgically. After a seven-year follow-up of actual dermatomyositis controlled by maintenance doses of prednisone ranging from 5 to 15 mg daily, the patient developed a meningioma. Current concepts and data regarding various aspects of the combination between dermatomyositis and tumors are discussed. To our knowledge, this is the first reported case of meningioma associated with dermatomyositis.     

Possible paraneoplastic syndrome case of bullous pemphigoid with immunoglobulin G anti‐BP180 C‐terminal domain antibodies associated with psoriasis and primary macroglobulinemia

A 61‐year‐old Japanese man developed bullous skin lesions during topical therapy for psoriasis vulgaris. Physical examination demonstrated numerous tense bullae and scaly erythemas on the trunk and extremities. Histopathology of the skin biopsy demonstrated subepidermal bullae and lymphocytic infiltration with eosinophils in the dermis. Direct immunofluorescence revealed linear deposits of immunoglobulin (Ig)G, IgA and C3 along the basement membrane zone. Indirect immunofluorescence of 1 mol/L NaCl‐split skin showed IgG reactivity with both epidermal and the dermal sides. IgM reactivity with both the epidermal and dermal sides was also detected. Enzyme‐linked immunosorbent assays showed negative results for both BP180 and BP230. Immunoelectrophoresis of serum and bone marrow aspiration revealed underlying primary macroglobulinemia with M‐proteinemia of IgM‐κ type. Immunoblot analysis revealed IgG, but not IgM, antibodies to recombinant protein of BP180 C‐terminal domain. We diagnosed the present case as bullous pemphigoid with IgG anti‐BP180 C‐terminal domain autoantibodies associated with primary macroglobulinemia and psoriasis vulgaris. Systemic administration of prednisolone 30 mg/day resulted in dramatic improvement of both bullous and psoriatic skin lesions. When the bullous and psoriatic lesions relapsed, DRC chemotherapy (dexamethasone, rituximab and cyclophosphamide) for macroglobulinemia was performed. Then, the psoriatic lesions improved and the bullous lesions disappeared. We suggested that the present case may be paraneoplastic syndrome of bullous pemphigoid associated with primary macroglobulinemia and psoriasis vulgaris.     

Dermatomyositis and Nasopharyngeal Carcinoma

A nasopharyngeal carcinoma was diagnosed in a 37-year-old man with a two year history of dermatomyositis. The physical and laboratory examinations excluded further medical problems. To our knowledge, this is the first case of dermatomyositis associated with nasopharyngeal carcinoma in a white Israeli Jewish patient.     

Case of severe bullous erythema including intertrigo‐like eruptions with angioedema induced by pegylated liposomal doxorubicin

Pegylated liposomal doxorubicin (PLD) is an anthracycline anticancer agent used in ovarian cancer and a form of doxorubicin enclosed in pegylated liposomes. There are only a few reports on intertrigo‐like eruptions caused by PLD. We describe the first case of severe bullous erythema, including intertrigo‐like eruptions with angioedema, induced by PLD in Japan. We present the case of a 53‐year‐old woman who was diagnosed with stage IIIC ovarian cancer. After receiving three cycles of PLD, the patient developed swelling of the upper lip and painful erythema with blisters and erosions on the axilla, upper back, flank and wrists. The patient was diagnosed with angioedema and severe skin lesions, including intertrigo‐like eruptions induced by PLD. Although treatment with oral prednisolone and topical steroids was effective against these eruptions, the administration of PLD was discontinued because of its ineffectiveness against the primary disease. Several risk factors, such as obesity, perspiration and racial differences, may contribute toward a severe manifestation such as that seen in our patient. Moreover, our case was the first accompanied by angioedema. The mechanism of coexistence of intertrigo‐like eruptions and angioedema is not clear; further studies are required to clarify the pathological mechanism of intertrigo‐like eruptions.     

Bullous Pemphigoid in a Renal Transplant Recipient

Bullous pemphigoid (BP) is an autoimmune disease with chronic, recurrent bullous eruptions. BP has been reported to be associated with drugs, physical stimuli, malignancies, and immune abnormalities. Its association with renal transplant is rare and only four cases have been reported. We present a case of BP in a 52-year-old man with chronic hepatitis B and C infection who underwent a cadaveric renal transplant 13 years earlier. His graft was still functioning well when BP appeared. The occurrence of BP in our patient might be a result of drugs (furosemide or tacrolimus), viruses, or renal allograft. As the patient was receiving regular T-cell immunosuppressant therapy, his BP lesions were recalcitrant to corticosteroid treatment. We discuss the pathogenesis and treatment of such patients.     

Bullous eruption associated with scabies: evidence for scabetic induction of true bullous pemphigoid

Some patients with scabies develop bullae concomitantly with, or subsequently after, the occurrence of scabetic lesions. Although several immunofluorescence studies have demonstrated immunoglobulin deposition in the basement membrane zone of bullous lesions, it remained unclear whether these antibodies are directed to bullous pemphigoid antigens. We clearly show that two scabetic patients with bullous eruptions had circulating antibodies against BP180 and/or BP230 as determined by Western blotting analysis. This is the first report to demonstrate that at least some of the bullous eruptions occurring in scabetics are true bullous pemphigoid.     

皮肌炎并发乳腺癌及乳房Paget病

报告1例并发乳腺癌和乳房Paget病的皮肌炎.患者女,51岁,右乳房出现肿物20余年,红斑、糜烂4年,面部及四肢红斑、丘疹伴肌痛2个月余,根据临床表现结合组织病理检查结果确诊为皮肌炎并发乳腺癌及乳房Paget病.     

Dermatomyositis with a pityriasis rubra pilaris-like eruption: an uncommon cutaneous manifestation in dermatomyositis

A pityriasis rubra pilaris-like eruption has been described in patients with dermatomyositis. We describe an 11-year-old girl with dermatomyositis who had additional clinical findings of pityriasis rubra pilaris. Over a year, she developed muscle weakness, increasing fatigue, and a markedly elevated creatinine kinase level in addition to her cutaneous eruption and was seen in our clinic for these complaints. A year earlier, when a generalized, scaly erythematous eruption had appeared, she had been diagnosed as pityriasis rubra pilaris clinically and histopathologically. Dermatologic examination found scaling erythematous plaques involving the trunk and upper and lower extremities. Islands of unaffected skin were intermingled with erythematous plaques that were characteristic of pityriasis rubra pilaris. A skin biopsy specimen showed the findings of dermatomyositis and that diagnosis was made. The laboratory findings, electromyographic pattern, and muscle biopsy were also consistent with dermatomyositis. Her presentation is interesting, as she had been diagnosed as pityriasis rubra pilaris both clinically and histopathologically 1 year earlier and, although the cutaneous lesions had not changed, a diagnosis of dermatomyositis was made a year later.     

Sarcoidosis in a patient with linear IgA disease

We report a patient with four conditions in association with linear IgA disease (LAD), only three of which have been reported previously; these latter are ulcerative colitis, autoimmune thyroid disease and carcinoma of the colon, although the carcinoma may have been caused by the ulcerative colitis in this case. Recently, our patient also presented with respiratory symptoms and was found to have sarcoidosis as well, a previously unreported association of this autoimmune bullous disorder. The aetiology of this development may be related to the patient's HLA status or possibly to his treatment with the immunosuppressive agent cyclophosphamide; it is also possible that it is coincidental.     

Bullous pemphigoid associated with internal malignancies: identification of 180-kDa antigen by Western immunoblotting

Summary We report three cases of bullous pemphigoid associated with internal malignancies. Two cases were associated with gastric cancer and one with rectal cancer. Immunoblot analysis, using epidermal extract of normal human skin, revealed that these patients' sera reacted only with bullous pemphigoid antigen with a molecular weight of 180 kDa (BP180). The relationship of BP180 to malignant tumours is discussed.     

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.     

A rare presentation of congenital syphilis: Pemphigus syphiliticus in a newborn infant with extensive desquamation of the extremities

Congenital syphilis is an infection transmitted from mother to fetus and can present with early but variable cutaneous manifestations. In rare situations, a bullous eruption known as pemphigus syphiliticus may develop. We present an unusual case of broad desquamation of the extremities in a newborn infant who was found to have congenital syphilis. Pemphigus syphiliticus should be considered in the differential diagnosis of neonatal bullous eruptions and erosions.     

Bullous pemphigoid associated with silicosis

Bullous pemphigoid (BP) has never before been reported to associate with silicosis, although there are numerous reports of silicosis accompanied by different autoimmune diseases, such as systemic sclerosis, systemic lupus erythematosus, dermatomyositis or rheumatoid arthritis. We report on a 63-year-old Japanese patient with silicosis who developed tensed bullae, erosions and macular pigmentation on the trunk and extremities. Indirect immunofluorescence revealed anti-basement-membrane-zone antibodies; immunoblotting analysis demonstrated that the patient's serum reacted with the 230-kD BP antigen in the epidermal extracts, as well as a recombinant protein of the NC16a domain of 180-kD BP antigen. Clinical symptoms improved after treatment with systemic steroids. To the best of our knowledge, this is the first reported case of BP associated with silicosis.     

Amyopathic dermatomyositis in Hong Kong – association with nasopharyngeal carcinoma

Background Amyopathic dermatomyositis (ADM) is a rare variant of dermatomyositis (DM) in which there is no apparent muscle involvement at initial presentation, but clinical and laboratory evidence of myopathy may appear later. Like DM, it has a known association with malignancy. Objective To record the occurrence of ADM in Hong Kong during the period from January 1988 to June 1996, and to establish the types of carcinoma with which it is associated. Methods The records of all patients diagnosed with ADM in three major dermatology clinics in Hong Kong were retrospectively studied to confirm the diagnosis and to look for any associated malignancy at any stage of the disease. Results Between 1988 and 1996, six patients in Hong Kong (five men and one woman; mean age, 50 years) fulfilled the criteria of ADM. During a follow-up period ranging from 20 to 90 months (average, 35.6 months), none developed any evidence of myositis. Associated malignancy was found in the five male patients. There were three cases of nasopharyngeal carcinoma (NPC), one case of non-small cell carcinoma of the lung, and one case of metastatic carcinoma of the cervical lymph node of unknown primary. No carcinoma had developed in the female patient during a follow-up period of 90 months. Conclusions There is a strong association between ADM and NPC in Hong Kong.     

Resolution of bullous pemphigoid and improvement of vitiligo after successful treatment of squamous cell carcinoma of the skin

The significance of the association of malignant diseases with bullous pemphigoid is still unknown. We report a case of squamous cell carcinoma of the skin associated with both bullous pemphigoid and vitiligo. It is possible that there is a common underlying pathogenic mechanism involved in the co-existence of these three skin diseases as successful treatment of the carcinoma was accompanied by resolution of the bullous pemphigoid and improvement of the vitiligo.     

Linear morphea with overlying lichen sclerosus and calcinosis cutis associated with juvenile dermatomyositis

Juvenile dermatomyositis (JDM) is associated with many distinguishing features including cutaneous calcinosis, vasculitis, and ulcerated lesions. In this case, we describe an unusual presentation in a 12-year-old girl who had muscle weakness along with linear morphea over the right upper and lower extremities with overlying lichen sclerosus and calcinosis cutis. Of interest, these initial cutaneous manifestations occurred years before onset of myositis.     

A Case of Bullous Pemphigoid in a Patient on Hemodialysis

We describe a case of bullous pemphigoid (BP) in a patient with chronic renal failure maintained on hemodialysis. We diagnosed BP by histopathological and immunofluorescence studies. The relationship between BP and chronic renal failure and/or hemodialysis is not clear, but we believe that immune disarrangement due to chronic renal failure and/or hemodialysis may have influenced the pathogenesis of BP in our case.     

A case of mixed bullous disease of epidermolysis bullosa acquisita and linear IgA bullous dermatosis

A 75-year-old Japanese male visited us with bullous eruptions on the extremities. Physical examination revealed large bullae on the hands, lower legs and feet. The oral mucosa was also involved. Histology disclosed subepidermal blister with inflammatory cell infiltrates in the dermis. Direct immunofluorescence showed deposits of IgG and IgA at the cutaneous basement membrane zone. Indirect immunofluorescence on 1 M NaCl-split human skin sections demonstrated that the patient's IgG antibodies reacted with the dermal side of the split, while IgA antibodies reacted with the epidermal side. Immunoblotting showed that the patient's serum reacted with the NC1 domain of type VII collagen (290-kDa epidermolysis bullosa acquisita antigen) as well as the 120-kDa linear IgA bullous dermatosis antigen, LAD-1. Systemic prednisolone resulted in a favorable response. From the clinicopathological findings, the present case is not consistent with either epidermolysis bullosa acquisita or IgA bullous dermatosis. Therefore, we regarded the case as mixed bullous disease of epidermolysis bullosa acquisita and linear IgA bullous dermatosis. Such a case has not been previously reported.     

以皮肌炎为首发症状的胸腺瘤一例

61岁男性患者,面部红斑6个月、加重泛发四肢伴四肢肌肉酸痛3个月。双上肢肌力Ⅳ级,双下肢肌力Ⅳ级。皮肤科情况:双眼睑水肿性紫红斑,全身泛发水肿性紫红色鳞屑性斑片,指关节伸侧可见紫红色扁平Gottron疹。胸部计算机断层增强扫描示:前纵隔肿瘤。纵膈肿物切除术后行组织病理检查,结果示前纵隔胸腺瘤(B2型)。诊断:①皮肌炎,②胸腺瘤。