Lichenoid drug reaction to leflunomide

INTRODUCTION: Leflunomide (Arava) is an immunomodulator, recently introduced for systemic treatment of rheumatoid arthritis. We report the first case of lichenoid drug reaction due to this drug. CASE REPORT: A sixty-four year-old woman received leflunomide for rheumatoid arthritis. Two months after initiation of treatment, pruritus and lichenoid papules appeared on her hands and subsequently on her arms and her trunk, with a few bullous lesions. A skin biopsy was evocative for the diagnosis of drug induced lichenoid eruption. The treatment was stopped, and a wash out with colestyramine and topical corticotherapy resulted in dramatic improvement. No relapse was observed. Two months later, patch-tests with leflunomide diluted to 30 p. 100 in white petrolatum were negative. DISCUSSION: Side effects of leflunomide are frequent, generally benign for the cutaneous features. In our case, the delay, clinical and histological aspect and improvement on withdrawal of the drug emphasize the imputability of leflunomide. Few cases have been reported with others immunomodulators.     

Lichen amyloidosus with subepidermal blister formation

We report a 74-year-old man who presented with multiple, itchy keratotic papules or plaques on the trunk and extremities. Erosions and vesicles were also intermingled on keratotic lesions. Histopathologic examination of biopsy specimens taken from three different lesions showed a subepidermal blister with amyloid deposits in the dermal papillae. No systemic disease or involvement of other organs was detected. The clinical and histological findings were compatible with a bullous variant of lichen amyloidosus. Although bullous amyloidosis has been reported in systemic amyloidosis, bullous lesions associated with lichen amyloidosus are very rare.     

Scleroderma-like cutaneous lesions induced by paclitaxel: a case study

Paclitaxel is a recent antineoplastic agent that belongs to the taxane family. Its activity has been demonstrated in advanced and refractory ovarian, breast, lung, and head and neck cancer. Adverse cutaneous reactions to paclitaxel have been reported, namely bullous fixed drug eruption, onycholysis, acral erythema, erythema multiforme, and pustular eruption. We report the first case of scleroderma-like changes after paclitaxel administration. A 63-year-old patient presented with an edematous and infiltrated erythema of the head, neck, axillae, and left hand 10 days after administration of paclitaxel and paraplatin for primitive peritoneal cancer. Cutaneous lesions improved after a change from paclitaxel to cyclophosphamide. Cutaneous lesions recurred 3 months later, after reintroduction of paclitaxel, and progressively evolved to cutaneous sclerosis. Skin biopsy showed a dermal fibrosis. Biologic tests revealed no autoimmunity. Scleroderma-like lesions of this patient were reminiscent of previously reported cases that occurred after administration of docetaxel, which also belongs to the taxan family. Thus, scleroderma-like syndromes seem to represent a unique cutaneous adverse event caused by taxanes.     

The spectrum of cutaneous disease in multiple myeloma

BACKGROUND: Multiple myeloma (MM) is a plasma cell dyscrasia characterized by a clonal proliferation of plasma cells that produces a monoclonal protein. There are dermatologic disorders that have been associated with MM, such as amyloidosis, cryoglobulinemia, POEMS syndrome, normolipemic plane xanthoma, and plasmacytoma. The high volume of patients with MM seen at our institution presents an opportunity to define more extensively the spectrum of cutaneous diseases seen in concert with MM. DESIGN: We reviewed 2357 pathology reports of all patients with a diagnosis of MM to find those who had undergone a skin biopsy. Files were searched for bone-marrow diagnosis, and for type and number of transplants. RESULTS: In all, 284 patients yielded 472 skin biopsy specimens (average 1.7/patient). Skin biopsy specimen diagnoses included neoplastic lesions, (111; 73 malignant, 38 benign), graft-versus-host disease (120), drug-related lesions (46), cutaneous eruption of lymphocyte recovery (3), thrombocytopenia-related lesions (9), normolipemic plane xanthoma (1), amyloidosis (1), Sweet's syndrome (7), panniculitis (1), papulosquamous lesions (18), bullous diseases (17), vasculitis (11), infectious lesions (41), granulomatous dermatitis (6), alopecia cicatrisata (1), nonspecific lesions (77), and unrelated lesions (2). CONCLUSIONS: Skin biopsy specimens from patients with MM less than 60 days from transplant most commonly show sequelae of the transplant such as graft-versus-host disease, Grover's disease (as a result of leukocytopenia and fever, waiting for engraftment), drug eruptions, chemotherapy effect, thrombocytopenic effect, cutaneous eruption of lymphocyte recovery, and Sweet's syndrome (possibly as a result of granulocyte-macrophage colony-stimulating factor). Biopsy specimens taken more than 60 days from transplant most commonly show graft-versus-host disease, drug eruptions, and Sweet's syndrome but also show unrelated conditions such as neoplastic lesions, nevi, papulosquamous lesions, vasculitis, infections, and nonspecific changes.     

Linear IgA bullous dermatosis in a patient with renal cell carcinoma

Linear IgA bullous dermatosis (LABD) is an autoimmune subepidermal bullous disease with heterogeneous clinical manifestations, characterized by linear deposition of IgA along the epidermal basement membrane zone. We report a patient with a metastasized renal cell carcinoma who developed an extensive blistering eruption. The lesions showed immunopathological findings characteristic of LABD. The patient showed a fair response to prednisolone and dapsone. Treatment to control the LABD was no longer required when interferon-alfa was started as palliative therapy for the metastasized renal cell carcinoma. The association of LABD and malignancies has been documented before and is not due to mere chance alone.     

Cutaneous reactions to propranolol (author's transl)]

A 17-year-old male patient with eczematous and psoriasiform eruption that developed during long-term therapy with Propanolol (Avlocardyl) has been studied. This eruption disappeared after removal of the drug; oral challenge was soon followed by a vesiculous and bullous eruption of face and extremities; five months later, sun exposure was followed by a severe eczematous eruption in these areas; nails changes were then observed. Most of side-effects of beta-adrenergic blocking drugs have been reported with Practolol: lichenoid, exanthematous, eczematous, psoriasiform rashes; exfoliative dermatitis; oculo-muco-cutaneous reactions; fibrosing polyseritis and drug induced systemic lupus erythematosus manifestations. Adverse effects of other beta-adrenergic blocking agents are less frequent. The pathogenetic mechanism responsible for these adverse reactions is still obscur: these changes might be caused by blockade of the epidermal cells (and T-lymphocytes) beta-receptors, more than by a direct immunologic, allergic or toxic mechanism.     

IgA multiple myeloma presenting as an acquired bullous disorder

A 63-year-old man presented with an intensely pruritic vesiculo-bullous eruption on the limbs and was subsequently found to have an IgA kappa multiple myeloma. The eruption clinically and histologically was suggestive of linear IgA disease (LAD), dermatitis herpetiformis (DH), epidermolysis bullosa acquisita (EBA), or bullous lupus erythematosus (LE), with the skin biopsy revealing subepidermal bullae and dermal papillary micro-abscesses. However, direct immunofluorescence showed a unique pattern of diffuse dermal IgA staining. Although chemotherapy produced a dramatic resolution of the lesions, which paralleled the fall in serum IgA paraprotein level, the myeloma later became progressive and the resulting paraprotein increase was accompanied by recurrence of the eruption. We propose that this patient's rash was the presenting manifestation of his multiple myeloma, and was a consequence of transudation of IgA paraprotein into the dermis.     

Epidermolysis bullosa acquisita-like reaction associated with penicillamine therapy for sclerodermatous graft-versus-host disease

A case of a bullous eruption occurred in a patient being treated with penicillamine for sclerodermatous graft-versus-host disease following bone marrow transplantation. After 7 days of treatment with 150 mg penicillamine, a painful bullous eruption with accompanying purpuric lesions suddenly developed in previous sclerodermatous infiltrations. A diagnosis of epidermolysis bullosa acquisita-like eruption was made, and the patient was treated with drug withdrawal and administration of cyclosporine and methylprednisolone. Epidermolysis bullosa acquisita-like reaction is an extremely rare cutaneous side effect of penicillamine. The surprisingly early onset of this eruption in lesions of sclerodermatous graft-versus-host disease might have been due to severe immune alteration.     

Adverse Reaction to Prednisone in a Patient with Systemic Lupus Erythematosus

Oral corticosteroids are the main therapeutic choice for systemic lupus erythematosus (SLE). Adverse reactions to systemic corticosteroids rarely occur and the etiology is unclear in most cases. A 14-year-old girl with newly diagnosed SLE developed a pruritic bullous eruption while on prednisone. The patient had been treated successfully in the hospital with intravenous methylprednisolone. In preparation for discharge, the steroid preparation was changed to prednisone to which the patient reacted with a development of new crops of bullous lesions. Skin biopsy specimens of lesional areas showed a bullous eruption consistent with erythema multiforme. The patient underwent immediate and delayed hypersensitivity tests. Intradermal and patch tests to liquid prednisone were positive. The patient was discharged on oral methylprednisolone and has not had recurrence of the skin lesions. In conclusion, a case of prednisone sensitivity in a patient with SLE is presented here. An alternative preparation, methylprednisolone, was used to successfully treat her underlying condition.     

A dilemma: bullous-pemphigoid-like eruption in scabies or scabies-induced bullous pemphigoid

Several diseases may be confused with scabies. Atypical manifestations of scabies have previously been reported, including urticaria, contact dermatitis, and dermatitis herpetiformis. There are also reports of scabies mimicking bullous pemphigoid. Here we report a case of scabies in a patient with manifestation similar to bullous pemphigoid. Direct smear of the bullous lesions was performed and was positive for scabies mites. The skin biopsy specimens submitted for light microscopy and direct immunofluorescence study were considered to be compatible with bullous pemphigoid. The patient treated with single dose oral ivermectin and prednisolone. The pruritus subsided and the eruption improved dramatically in 2 weeks.     

Bullous amyloidosis

Amyloidosis may present with involvement of a variety of organ systems. Cutaneous involvement is a relatively common finding in patients with systemic amyloidosis. The occurrence of bullous skin lesions, however, is rare; only a few such cases have been previously reported. We describe a patient who presented with a subepidermal bullous skin disease initially thought to be bullous pemphigoid based on both clinical and histologic appearances. The patient subsequently developed the nephrotic syndrome. Biopsy specimens of the skin and kidney showed involvement of both organs with amyloid, and amyloid was later found in the spleen, heart, and nervous system. No subsequent evidence of myeloma was found in this patient. The clinical, histopathologic, immunofluorescent, and electron microscopic findings of systemic amyloidosis are discussed.     

Bullous pemphigoid in a chronic renal allograft rejection patient: a case report and review of the literature

Bullous pemphigoid (BP) is an autoimmune blistering disorder of the skin. Various provocative factors have been proposed. However, BP in renal transplantation patients has scarcely been reported. Herein, we report the case of a 27-year-old man who received cadaveric renal transplantation 10 years ago presenting with bullous eruption during chronic renal allograft rejection phase, with the renal allograft being the possible trigger stimulus for BP development in our case. Bullous lesions were soon controlled by oral corticosteroids and did not recur after removal of the graft. The pathogenesis and management of such patients will also be reviewed along with the case report.     

Bullous Systemic Lupus Erythematosus as an Initial Manifestation of SLE

Bullous systemic lupus erythematosus (BSLE) is a rare subset of systemic lupus erythematosus that is often associated with autoimmunity to type VII collagen. We describe a 45-year-old woman with BSLE who presented with vesiculobullous lesions as an initial manifestation of SLE. The patient first noticed a widespread urticarial, erythematous eruption associated with tense blisters, erosions, and crusting. She was diagnosed with bullous pemphigoid and underwent a one-month course of treatment with betamethazone. Because of the appearance of marked proteinuria, a subsequent renal biopsy, and serological tests, the patient was diagnosed with rapidly progressive glomerulonephritis and systemic lupus erythematosus. The patient's IgG circulating antibodies labeled the dermal floor of salt-split skin and recognized type VII collagen in immunoblot studies. Although methylprednisolone pulse therapy for glomerulonephritis did not alleviate the vesicullobullous eruption, treatment with dapsone resulted in dramatic disappearance of the lesions. Cessation of dapsone therapy due to hemolysis with Heinz-body formation did not aggravate the bullous disease. Our case illustrates that a generalized vesiculobullous eruption can be the sole presenting manifestation of SLE. It also emphasizes the close temporal relationship between BSLE and lupus nephritis.     

Cutaneous T-cell lymphoma bullosa: 2 cases

INTRODUCTION: Since the first case described by Kaposi in 1887, bullous forms of cutaneous T-cell lymphomas are extremely. CASE-REPORT: We describe an unusual case of mycosis fungoides bullosa with palmoplantar dyshidrosis-like eruption and, to our knowledge, the first case of pleomorphic cutaneous T-cell lymphoma bullosa. DISCUSSION: Cutaneous T-cell lymphoma bullosa can be very misleading, particularly when the bullous lesions are inaugural. Differential diagnosis have to be excluded (autoimmune blistering diseases or contact dermatitis during topical treatment of lymphoma or bacterial or viral cutaneous infections) by biopsy, direct immunofluorescence and bacteriological examinations. In these cutaneous lymphoma bullosa, the blisters appear to de due to excessive epidermotropism and/or toxicity of the tumoral infiltrate.     

Bullous amyloidosis: the mechanism of blister formation revealed by electron microscopy

BACKGROUND: Few electron microscopic studies of blister lesions in bullous amyloidosis have been reported, and the mechanism of blister formation remains to be elucidated. This study was designed to examine the nature of bullous amyloidosis ultrastructurally, and clarify the pathogenesis of blister formation. METHODS: We examined a 47-year-old woman with IgD-lambda type myeloma, suffering from bullous lesions on her hands and feet caused by trauma or rubbing. Light and electron microscopic studies were performed. RESULT: Ultrastructurally, amyloid deposits aggregated under the lamina densa. Keratinocyte protrusions penetrated the dermis through the gap in the lamina densa and enfolded amyloid deposits. Amyloid globules were found in enlarged intercellular spaces of keratinocytes. Desmosomes were sparsely distributed in some areas of the epidermis. CONCLUSION: These results indicate that keratinocytes enfold the amyloid globules and take them in the intercellular space of epidermis, and that the breakdown of the lamina densa and widening of the intercellular space between keratinocytes induce skin fragility. Trauma or rubbing of her hands and feet appears to act as the localized precipitating factor of blister formation in bullous amyloidosis.     

Linear IgA disease associated with lymphocytic colitis

A 66-year-old woman presented with a bullous skin eruption and chronic diarrhoea. Lesional skin showed subepidermal blistering, and direct immunofluorescence of perilesional skin revealed linear deposits of IgA at the dermoepidermal junction, establishing a diagnosis of linear IgA disease (LAD). Chronic watery diarrhoea complicated by substantial loss of body weight preceded the skin eruption for several months. On endoscopy, the colon appeared macroscopically normal. On histology, the colon mucosa showed increased numbers of intraepithelial lymphocytes and infiltrates of mononuclear cells in the lamina propria, indicative of lymphocytic colitis. Treatment with methylprednisolone and dapsone led to complete clearing of the bullous skin eruption and marked improvement of the patient's diarrhoea. Gastrointestinal disorders such as lymphocytic colitis have rarely been reported in patients with LAD. Whether the simultaneous occurrence of these two diseases is coincidental or due to related pathogenetic mechanisms remains to be seen.     

Pyoderma gangrenosum in a patient with bullous systemic lupus erythematosus

We report a 55-year-old woman with bullous systemic lupus erythematosus, who later developed pyoderma gangrenosum (PG). Dapsone was effective for the eruption of bullous bullous systemic lupus erythematosus but not for pyoderma gangrenosum. Cyclosporine was effective for the skin lesions of pyoderma gangrenosum. This is the first reported case of PG associated with bullous systemic lupus erythematosus.     

Acute febrile neutrophilic dermatosis and malignant hematologic diseases: report of a new bullous case and review of the literature

A new case of Sweet's syndrome (acute febrile neutrophilic dermatosis) associated with a malignant hemopathy is presented. The blood disease was a chronic myelomonocytic dysmyelopoiesis which was discovered during the eruption and resulted in the patient's death within a few months, probably through acutization. The skin lesions were atypical, bullous and ulcerated. On this occasion, the international literature concerning all cases of Sweet's syndrome associated with malignant or premalignant hemopathies is reviewed. Several concepts emerge from this study: the association is frequent (about 20 p. 100 of all published cases of Sweet's syndrome); there is a strong predominance of granulocytic hemopathies over lymphoplasmocytic and monocytic hemopathies; the blood disease is revealed by the skin eruption in some 50 p. 100 of the patients; there are frequent chronological relations between Sweet's syndrome and the events that occur in the course of the hemopathy; finally, the association is usually of poor prognosis. A comparison with Sweet's syndrome unassociated with a blood disease showed only three significant points: the frequency of bullous lesions, of the initial anaemia (the most important element) and of extreme figures in leucocyte counts (leucopenia or major hyperleukocytosis). The atypical character of the skin lesions in the patient presented here incites to discuss the nosological relationship between Sweet's syndrome and bullous pyoderma, an entity closely associated with hemopathies. It has recently been suggested by several authors that this anatomico-clinical kinship should be turned into a wide spectrum of acute neutrophilic dermatoses, with typical Sweet's syndrome at one end and Pyoderma gangrenosum at the other end. The interface between this spectrum and haemopathies seems to be maximum at its intermediate stage: the bullous and superficially ulcerated lesions. The aetiology and pathogenesis of this new nosological entity are uncertain. The presence of chemoattractants or of polymorphonuclear cell abnormalities is still open to discussion. The relationship between the entity and leukocytoclastic vasculitis has recently been questioned.     

Bullous pyoderma as a presentation of acute leukaemia

A patient with an unusual bullous pyoderma preceding the onset of acute leukaemia is reported. The initial lesions were purplish bullae, which later formed superficial ulcers and healed with minimal scarring. Histology showed intra-epidermal bullae with a dense polymorphonuclear infiltrate in the epidermis. This eruption is difficult to classify, but fifteen patients with similar skin lesions have been found in the literature, seven of these patients developed acute leukaemia and one had metastatic adenocarcinoma on presentation. We feel that the recognition of these distinctive skin lesions may be important because of their association with internal malignancy.