Multiple myeloma with primary systemic amyloidosis affecting cutaneous, gastro intestinal and cardio-vascular system

Amyloidosis refers to a group of disorders characterized by extracellular deposition of protein fibrils. Primary systemic amyloidosis is commonly due to an underlying plasma cell dyscrasia. Infiltrative amyloid cardiomyopathy is a rare cause of predominantly diastolic myocardial disease. Restrictive cardiomyopathy is the main finding in cardiac amyloidosis and results from the replacement of normal myocardial contractile elements by infiltration and interstitial deposits of amyloid, leading to alterations in cellular metabolism, calcium transport, receptor regulation, and cellular edema. Injury can also occur from circulating light chains in the absence of amyloid fibril formation. Cardiac amyloidosis should be considered in any patient presenting with congestive heart failure, preserved systolic function, and a discrepancy between a low QRS voltage on electrocardiography and an apparent left ventricular hypertrophy on sonogram. The pattern of left ventricular diastolic dysfunction changes during the course of amyloidosis and the classically described restrictive physiology occurs only in advanced stages of the disease. This is a case report where an unusual extensive cutaneous and cardiac involvement provided the sign of widespread systemic deposition of amyloid protein which eventually led to the diagnosis of multiple myeloma.

Dermoscopic features of primary cutaneous amyloidosis / 中华皮肤科杂志

Objective To describe common dermoscopic features of primary cutaneous amyloidosis (PCA),and to explore the application value of dermoscopy in assisting the diagnosis of PCA.Methods Seventeen patients with PCA were collected from Department of Dermatology of Peking Union Medical College Hospital between April 2014 and December 2016.A total of 45 skin lesions were subjected to dermoscopy,and their dermoscopic features were analyzed.Results The common dermoscopic features of PCA included central hubs,which could be white,brown or scar-like areas,various pigment structures and shiny white streaks.Dermoscopy showed that central hubs could be observed in all the lesions (100％),while white center hubs alone were observed in 14 (31％) lichenoid lesions,both white center hubs and scar -like areas in 5 (11％) lichenoid lesions,brown center hubs alone in 8 (18％) macular lesions,both white and brown center hubs in 6 (13％) lichenoid lesions and 17 (38％) macular lesions.All the lesions showed various pigment structures.Shiny white streaks were observed in 4 (9％) lichenoid lesions.Conclusion Dermoscopy has good application value in assisting the diagnosis of PCA.

Pathological Significance of CK5 Expression in Primary Cutaneous Amyloidosis / 华中科技大学学报(医学版)

Objective To analyze the pathological significance of CK5 expression in primary cutaneous amyloidosis(PCA).Methods The expression of CK5 in superficial dermis of PCA group and the control group[lichen planus(LP),lupus erythematosus(LE)]were detected by CK5 monoclonal antibody.The infiltration densities of CD3-positive T lymphocytes and CD68-positive macrophages in superficial dermis of PCA and control group were measured respectively by immunohistochemical staining using anti-CD3 antibodies and anti-CD68 antibodies.The skin lesions of PCA and control groups were analyzed by immunofluorescence to detect whether CK5 was phagocytosed by macrophages in superficial dermis.Results Totally,39 cases of PCA all were CK5-positive.Some control cases were positive.The number of CD3-positive T lymphocytes and CD68-positive macrophages in 8 cases of PCA group was lower than that of control group.The result of immunofluorescence colocalization of monoclonal anti-CK5 antibodies and anti-CD68 antibodies in 5 cases of PCA lesions was negative;that in 2 cases of LE lesions were both positive,and that in 2 cases of LP lesions were both negative.Conclusion Amyloid protein may be derived from the basal keratinocytes after interface damage.The amyloid protein deposits may be related to the number decrease or the functional defect of macrophages.

Amiloidosis cutánea primaria localizada nodular. Presentación de un caso y revisión de la literatura / Primary localized cutaneous nodular amyloidosis. Case report and literature review

La amiloidosis cutánea primaria localizada designa a aquellas amiloidosis con afectación exclusivamente cutánea, sin afectacióna otros niveles. La amiloidosis nodular es una forma rara de amiloidosis cutánea primaria. Se presenta como nódulos de color rojo-amarillentos de superficie brillante, generalmente asintomáticos. Presentamos un paciente de 62 años de edad, obeso mórbido, con antecedente de hipertensión, EPOC, cardiopatía isquémica y dislipemia. Presentaba múltiples placas eritemato-amarronadas, localizadas en tórax y abdomen de 12 años de evolución. Lahistopatología confirmó el diagnóstico de amiloidosis nodular. No se halló evidencia de enfermedad sistémica.

Primary localized cutaneous amyloidosis refers to those amyloidosis with only skin involvement without affecting other levels.Nodular amyloidosis is a rare variant of primary cutaneous amyloidosis presenting asymptomatic red-yellowish nodules withbright surface. We report a morbidly obese 62-year-old male with a history of hypertension, COPD, ischemic heart diseaseand dyslipidemia presenting multiple erythematous brownish plaques, located in thorax and abdomen of 12 years of evolution.Histopathologic examination confirmed nodular amyloidosis diagnosis. Evidence of systemic disease was not found.

Primary cutaneous amyloidosis: A clinico-pathological study with emphasis on polarized microscopy.

Background: Primary localized cutaneous amyloidosis (PCA) is a relatively rare condition characterized by amyloid deposition in dermis without systemic involvement. Although, histopathological examination of the lesion reveals amorphous eosinophilic deposits in papillary dermis examination of congo red stained slides under polarized light will give definitive diagnosis Aims: To study the clinicopathological features of cutaneous amyloidosis emphasizing the utility of polarized light in diagnosis. Materials and Methods : A clinicopathological study of primary cutaneous amyloidosis over a period of 8 years was undertaken. All the cases, clinically diagnosed and histopathologically proven as cutaneous amyloidosis were stained with congo red and studied under polarized light. Results and Conclusions: Of the 45 cases of clinically suspected amyloidosis, 32 cases were proven histopathologically as primary cutaneous amyloidosis and confirmed by congo red stain under polarized light which showed apple green birefringence. Among the two types of PCA, lichen amyloidosis was the most common variant accounting to 65.63% with pure cases of macular amyloidosis accounting for only 15.63%. Biphasic amyloidosis was seen in 18.75%. Knee was the commonest site of involvement with pruritis being the most common symptom. Histopathologically, the most common findings were hyperkeratosis, irregular acanthosis and expansion of dermal papillae by amyloid deposits showing apple green birefringence under polarized microscope with congo red staining. Although, H and E stain gives a clue for the diagnosis of amyloid nevertheless congo red staining under polarized light forms a very sensitive and definitive method for confirmation.

Lichen amyloidosis in a 57 year old female: A case report

We describe a case of a 57 year old female who presented with multiple papules on bilateral extremities with no other systemic findings. Skin lesions consisted of multiple hyperpigmented scaly papules in a rippled pattern some coalescing into plaques. Histologic examination showed deposits of amorphous eosinophilic materials in the papillary dermis. The diagnosis of lichen amyloidosis was made. Treatment with high potency topical steroid in combination with salicylic acid ointment, emollients, and systemic antihistamine which afforded improvement after 2 weeks. There was 90 percent clearance of lesion within 5 months of therapy. The origin and clinical features of papular primary localized cutaneous amyloidosis (PLCA) are reviewed.

A Case of Familial Primary Localized Cutaneous Amyloidosis / 대한피부과학회지

Primary localized cutaneous amyloidosis comprises macular, papular and rare nodular amyloidosis. Macular and papular amyloidosis are considered as different manifestations of the same disease process and can occur in the same patient, which is known as biphasic amyloidosis. Although most cases of primary cutaneous amyloidosis occur sporadically, some cases have been reported to have familial incidence with papular, biphasic or rarely macular amyloidosis. We report a case of a family in which a mother and her son have brown hyperkeratotic pruritic papules on both upper and lower extremities. The skin lesions initially began as macules on the shins in the second or third decade, but gradually spread with increasing papules and pigmentation. They had no kind of manifestation suggesting systemic amyloidosis. A skin biopsy specimen disclosed amyloid deposition in papillary dermis.

A Case of Familial Primary Localized Cutaneous Amyloidosis / 대한피부과학회지

Primary localized cutaneous amyloidosis comprises macular, papular and rare nodular amyloidosis. Macular and papular amyloidosis are considered as different manifestations of the same disease process and can occur in the same patient, which is known as biphasic amyloidosis. Although most cases of primary cutaneous amyloidosis occur sporadically, some cases have been reported to have familial incidence with papular, biphasic or rarely macular amyloidosis. We report a case of a family in which a mother and her son have brown hyperkeratotic pruritic papules on both upper and lower extremities. The skin lesions initially began as macules on the shins in the second or third decade, but gradually spread with increasing papules and pigmentation. They had no kind of manifestation suggesting systemic amyloidosis. A skin biopsy specimen disclosed amyloid deposition in papillary dermis.

A Case of Diffuse Biphasic Cutaneous Amyloidosis

We described a case of diffuse biphasic cutaneous amyloidosis, a unique form of localized cutaneous amyloidosis. A 41-year-old man has gradually developed a lichenoid papular and a grouped spotted pigmented macular eruption on the trunk and upper extremities over the past 15 years. Histopathologic examination revealed that amyloid deposits were present in the papillary dermis. It was confirmed by Congo red staining, immunohistochemistry and electron microscopy. There was no evidence of systemic amyloidosis.

A case of anosacral cutaneous amyloidosis / 대한피부과학회지

We report a case of anosacral cutaneous amyloidosis in a 74 year-old-man. The skin finding is a patch of the size of a childs palm on the sacral area, consisting of brownish macular lines that radiate frorn the anus. Histopathology revealed defiosits of homogenous eosinophilic mateial s in the papillary dermis. Congo red staining and dylon staining was positive. Immuohistochemical staining using anti SAP antibodies and anti-keratin antibodies showed positive reactions.

Immunohistochemical Staining of Amyloid Deposit with Monoclonal Anti - keratin Antibodies in Primary Localized Cutaneous Amyloidosis / 대한피부과학회지

Nine cases of primary localized cutaneous amyloidosis were studied by immunoperoxidase technique (ABC method) employing anti-keratin antibodies. All specimens were examined using consecutive paraffin sections to confirm the correspondence between amyloid existing area and reactive sites. Anti-keratin antibody 34pE which recognize 68, 58, 56.5, 56kd keratin peptides reacted with amyloid deposits in both lichen amyloidosus and macular amyloidosis. However, anti-keratin antibodies 34pB4 and 35pH did not react with amyloids. In general, Dylon staining positive material, keratin reacted with 34pE and amyloid P showed similar distribution in serial sections, but did not show the same one. Several keratin bodies reacted with 34pE, which were not stained with Dylon staining or antiamyloid P were found in the dermis of one specimen. These results suggest that immunohistochemical staining with antikeratin antibody 34pE using formalin-fixed, paraffin-embedded sections appeared to be a useful method in studying the histogenesis of primary localized cutaneous arnyloidosis.

Two Cases of Lacalized Cutaneous Amyloidosis Associated with Neurodermatitis / 대한피부과학회지

We report herein two cases of localized curaneous amyloidosis (LCA) associated with neurodermatitis. Both patients suffered from persistent pruritus during several years and had small brown discrete lichenoid papules or neurodermatitis like lesion. Biopsy specimen taken from lichehoid papules revealed amyloid deposition in the papillary dermis. In histologic examination of the neurodermatitis like lesion, hematoxylin eosin stain revealed only chronic eczematous changes but special stain and election microscopic examination revealed amyloid deposition in the papillary dermis. Although neurodermatitis and LCA may be able to develop concomitantly in one patient, it was suggested that neurodermatitis may transfrom into LCA.