Relapsing bullous amyloidosis of the oral mucosa and acquired cutis laxa in a patient with multiple myeloma: a rare triple association

It is well known that primary systemic amyloidosis [light chain (AL) amyloidosis] is associated with hidden dyscrasia or multiple myeloma. Acquired cutis laxa (cutis laxa acquisita; CLA) has also been described in patients with plasma cell dyscrasias, including multiple myeloma. We report a case in which haemorrhagic oral bullae were the first sign of an undiagnosed primary systemic amyloidosis related to multiple myeloma IgG‐λ and previously diagnosed CLA. There is only one report in literature of this rare triple association; however, in that case the patient did not have oral mucosal involvement or bullous amyloidosis.     

Lichen amyloidosis,ankylosing spondylitis and autoimmune thyroiditis: coincidence or association?

Primary localized cutaneous amyloidosis (PLCA) is characterized by the deposition of amyloid in a previously apparently normal skin with the absence of other systemic or cutaneous disorder. Although ankylosing spondylitis may be associated with secondary systemic amyloidosis, no reports have been found showing the association of this disease with PLCA. In addition, the association of PLCA with autoimmune thyroiditis has not been previously reported. We report a concomitant occurrence of lichen amyloidosis, ankylosing spondylitis and autoimmune thyroiditis in a caucasian woman.     

Nodular amyloidosis: Case report and literature review

BACKGROUND: Amyloidosis refers to a group of depositional diseases that are classified into two main types: systemic and localized. Large nodules of localized cutaneous amyloidosis of the nasal ala and surrounding skin are rare and the treatment is often unsatisfactory. OBJECTIVE: We report a case of rapidly enlarging, localized, nodular cutaneous amyloidosis of the nose and the surrounding skin with a brief review of the current literature regarding treatment of this rare disease. CONCLUSION: Nodular amyloidosis can be treated successfully with cold steel excision in combination with carbon dioxide laser. Close followup of these patients is warranted, as nodular amyloidosis may be the precursor to systemic amyloidosis.     

Systemic amyloidosis secondary to chronic leg ulcers.

Chronic leg ulcers due to any cause are almost invariably associated with an inflammatory process. As with any long-standing inflammation, leg ulcers may be complicated by systemic amyloidosis, which occurs in rheumatoid arthritis, cystic fibrosis, tuberculosis, and other disorders. There are, however, very few reports on the association between these two conditions. We report a patient with severe leg ulcers of twenty years' duration in whom reactive systemic amyloidosis presented as a nephrotic syndrome.     

Unusual clinical presentation of amyloidosis: bilateral stenosis of the external auditory canal, hoarseness and a rapid course of cutaneous lesions

We report a patient with multiple myeloma associated with primary systemic amyloidosis who had a rapid evolution and a very unusual form of presentation. The association of amyloidosis in patients with multiple myeloma is 15%, and clinically evident mucocutaneous involvement occurs in up to 40% of patients.     

Amiloidosis cutánea primaria localizada nodular con patrón diseminado

Amyloid is a proteinaceous material that is deposited in the tissues in a large variety of clinical contexts; in the skin it can be found with or without concomitant systemic disease. Primary localized cutaneous amyloidosis encompasses those amyloidoses restricted to the skin without involvement of other systems. The most common forms within this group are macular and lichen amyloidosis. Nodular amyloidosis is extremely rare, and there are notable differences in clinical presentation, prognosis, histology, and pathogenesis between this entity and the macular and lichenoid variants.We report a new case of nodular primary localized cutaneous amyloidosis with disseminated plaques and nodules in which no systemic disease developed in the 3 years following the appearance of the lesions.     

An unusual presentation of systemic amyloidosis

We report a case of systemic amyloidosis presenting as intracutaneous haemorrhage following cardiac arrest.     

Nodular primary localized cutaneous amyloidosis: immunohistochemical evaluation and treatment with the carbon dioxide laser

Nodular primary localized cutaneous amyloidosis is an uncommon disorder for which there is no consistently satisfactory treatment. The amyloid fibrils are thought to have an immunoglobulin light chain derivation and systemic involvement must be excluded in all cases. We report a patient with a large scalp lesion of nodular primary localized cutaneous amyloidosis whose immunohistochemical evaluation revealed lambda light chain deposits and who thus far has no apparent systemic involvement. The lesion was treated by the carbon dioxide (CO2) laser with excellent cosmetic results and minimal morbidity.     

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.     

Secondary amyloidosis complicating arthropathic psoriasis

We report a patient with secondary systemic amyloidosis complicating arthropathic psoriasis, At autopsy amyloidosis was observed in the spleen, kidneys, liver, gastrointestinal tract, lung, heart, pancreas, adrenal glands, thyroid and gall-bladder in addition to the skin by historical, histochemical and ultrastructural studies. The amyloid deposits in these organs were of amyloid A protein type.     

Mucocutaneous bullous amyloidosis with an unusual mixed protein composition of amyloid deposits

We describe a case of fatal systemic amyloidosis presenting with mucocutaneous bullous lesions in a patient with IgA kappa monoclonal gammopathy. The amyloid plaques were composed of an unusual mixture of immunoglobulin kappa light chain and amyloid A proteins. Whereas oesophageal and oropharyngeal blisters are known to occur in several types of bullous dermatoses, to our knowledge this is the first report of oesophagopharyngeal blisters complicating bullous amyloidosis.     

Nail abnormalities and scleroderma-like lesions on the face associated with systemic amyloidosis

INTRODUCTION: Mucocutaneous involvement in systemic amyloidosis occurs in 29 to 40 p. 100 of cases. Nail abnormalities are infrequent in AL amyloidosis. We report an original case of AL amyloidosis associated with cutaneous and integument alterations and scleroderma-like infiltration of the face. CASE REPORT: A 73 year-old woman was hospitalized because of weight loss and asthenia. She had been treated 4 years earlier with chemotherapy for a IgG-type multiple myeloma with complete resolution of the underlying monoclonal gammapathy. Cutaneous examination showed nail dystrophy of all fingernails associated with scleroderma-like skin changes on the chin and lips. Histopathologic study of a chin biopsy confirmed the presence of amyloid deposits in the dermis. Laboratory data were normal, without signs of recurrence of multiple myeloma. DISCUSSION: We report an original case of a patient who developed two unusual cutaneous manifestations associated with AL amyloidosis. Moreover, there was no correlation between the severity of the cutaneous lesions and the extent of the underlying hematological disease.     

Bullous poikilodermatitic amyloidosis of the skin with junctional bulla development in IgG light chain plasmacytoma of the lambda type. Histology, immunohistology and electron microscopy]

A 75-year-old patient presented with bullae, poilokilodermatic skin and nail dystrophy as signs of systemic amyloidosis 1 year before an IgG myeloma of the lambda type was diagnosed. The skin lesions appeared at mechanically irritated locations on the trunk and at the tensor sites of the extremities. Histology showed a subepidermal blister and necrotic keratinocytes in the epidermis. There were amyloid deposits in the papillary dermis perivascular, and in the deep dermis around eccrine glands and in arrectores pilorum muscles. Polyclonal antibodies allowed classification of the deposits as amyloid composed of lambda light-chain immunoglobulins. Electron microscopy revealed globoid deposits of non-branching filaments typical of amyloid. The blister was formed at the level of the lamina lucida, with the lamina densa at the base of the bottom of the bulla. So far, junctional blister formation in bullous amyloidosis of the skin has been described only once. Our case is the second report of this blister type, and to our knowledge the first published report of a junctional blister in myeloma-associated systemic amyloidosis.     

Primary cutaneous amyloidosis of the external ear: a clinicopathological and immunohistochemical study of 17 cases

Primary cutaneous amyloidosis includes several forms of localized amyloidosis characterized by superficial amyloid deposits occurring at or near the dermal–epidermal junction in the absence of systemic involvement. Primary cutaneous amyloidosis of the auricular concha and external ear represents a rarely described variant. There have been 27 cases reported in the English language literature, and herein we report 17 additional cases. This article demonstrates that the amyloid observed in this context is generally positive for Congo red, crystal violet and thioflavin T. It also expresses cytokeratin 34ßE12 via immunohistochemistry. Our immunohistochemical results and review of the literature suggest that the amyloid in amyloidosis of the external ear is the result of basal keratinocyte degeneration and does not signify deposition from a systemic or generalized process. Wenson SF, Jessup CJ, Johnson MM, Cohen LM, Mahmoodi M. Primary cutaneous amyloidosis of the external ear: a clinicopathological and immunohistochemical study of 17 cases.     

Amyloidosis complicating psoriatic arthritis

BACKGROUND: Secondary AA amyloidosis is a classical complication of rheumatismal or chronic infectious diseases. Psoriasis is a rare cause of secondary amyloidosis with only around thirty cases reported in the literature. CASE REPORT: A 62 year-old man exhibited cutaneous lesions of psoriasis for six years complicated by articular involvement over the past year. The occurrence of an isolated proteinuria revealed renal and hepatic AA amyloidosis. Treatment with methotrexate (Méthrotrexate), enalapril (Renitec) and colchicine (Colchicine) was initiated and led to a stabilization of the proteinuria for two years. DISCUSSION: In psoriatic patients, secondary amyloidosis mainly complicates the arthritic diseases of prolonged progression. Our case report is original in the rapid onset of amyloidosis after the first articular signs. The clinical manifestations of secondary amyloidosis are related to renal or gastrointestinal involvement. Prognosis is usually poor. Treatment of secondary amyloidosis is difficult and relies on systemic treatment. Colchicine may be helpful.     

Extended survival of patients with primary systemic amyloidosis.

Systemic amyloidosis can produce a wide variety of clinical manifestations, including characteristic cutaneous findings. Large series of patients with primary systemic amyloidosis have shown that systemic amyloidosis, with or without associated myeloma, has a median survival of no more than twenty-four months. We present a case of systemic amyloidosis that has been present in a woman for eighteen years, as manifested by periorbital purpura and an immunoglobulin G kappa light chain paraproteinemia. She was otherwise healthy; results of bone marrow examination showed no overt myeloma. We speculate that kappa light chain paraproteinemia could prove to be a marker for a more benign type of systemic amyloidosis.     

Primary systemic amyloidosis presenting as extensive cutaneous ulceration

BACKGROUND: We report a case of primary systemic amyloidosis in a 78-year-old Caucasian woman presented as a nonhealing ulcer on the right thigh for 3 months. Histopathology of the skin revealed widely thickened walls of middermal and subcutaneous vessels from deposition of amorphous eosinophilic material that stained positively with Congo red and crystal violet. OBJECTIVE: This case represents a very unusual presentation of primary systemic amyloidosis, one in which the cutaneous manifestations provided the first signs of a devastatingly widespread multiorgan infiltration of amyloid protein. CONCLUSION: This presentation of the disease may signify an advanced stage with a grave prognosis as our patient passed away 3 months after development of the cutaneous ulceration.     

Unusual skin manifestation of cutaneous amyloidosis

A 76-year-old man with a 20-year history of extensive cutaneous amyloidosis is reported. He had asymptomatic symmetric brownish reticulated pigmented patches with well-demarcated borders on his thighs, lower legs, dorsal feet and both arms. The trunk and popliteal fossae were not affected. A skin biopsy specimen showed abundant amyloid deposits in the papillary dermis and reticular dermis. Despite the extensive cutaneous involvement and large amount of amyloid in the deep dermis, no evidence of systemic amyloidosis could be found. Various manifestations of cutaneous amyloidosis are reviewed. We report this case to remind dermatologists of the protean presentations of cutaneous amyloidosis.     

Systemic Amyloidosis with Initial Polyneuropathy

We report a case of systemic amyloidosis with the initial sign of polyneuropathy. The patient eventually developed heart failure, macroglossia, and ecchymoses 6 years later. Biopsies from the sural nerve and normal-looking skin both proved the existence of amyloid.     

Amyloid in localized cutaneous amyloidosis: immunofluorescence studies with anti-keratin antiserum especially concerning the difference between systemic and localized cutaneous amyloidosis

Amyloid of localized cutaneous amyloidosis and systemic amyloidosis were subjected to study with an indirect immunofluorescence technique using anti-keratin antiserum. Anti-keratin antiserum was prepared ad modum Sun & Green. Amyloid of localized cutaneous amyloidosis was positively stained for the antiserum, whereas amyloid of systemic amyloidosis (primary and multiple myeloma-associated) was negative. There was no difference between primary localized cutaneous amyloidosis (lichen amyloidosus and macular amyloidosis) and secondary localized cutaneous amyloidosis (amyloidosis associated with skin tumor). These results indicate that amyloid of localized cutaneous amyloidosis contains components derived from epidermal fibrous protein, probably tonofilaments of keratinocytes.