Letters to the Editor
889
Macular amyloidosis: a case report with scanning electron microscopy Editor Macular Amyloidosis (MA) is characterized by the deposition of an amorphous hyaline protein, restricted to the papillary dermis, normally without extracutaneous manifestations. Clinically, MA presents as hyperpigmented macular lesions on the back, involving the interscapular area and legs. Light microscopy using special stainings confirms the diagnosis. Transmission electron microscopy (TEM) is another technique that allows the visualization of long non-branching fibrils in the papillary dermis, that are isolated or in bundles.1,4 We report a case of extensive MA with an innovative approach using scanning electron microscopy (SEM). A 35-year-old female patient, skin phototype IV, presented extensive areas of hyperpigmented macules, with rippled pattern, slightly pruritic, which began approximately 8 years ago, involving the legs and dorsal region (Fig. 1a,b). The following tests were performed and the results were normal: CBC, liver and kidney function, protein electrophoresis and immunoelectrophoresis. Bence-Jones protein in urine was absent. The tests for HbsAg, VDRL HCV, HIV I and II were negative. Light microscopy showed amyloid deposits, with rippled pattern, in the papillary dermis and in some areas in direct contact with basal cells, either by haematoxylin–eosin and more easily viewed by crystal violet staining (Fig. 1c,d). Using SEM to examine the lateral surface of the punch biopsy, it was possible to observe in the papillary dermis, with low
magnification (91800), irregularly arranged collagen fibrils, intermingled with deposits (Fig. 2a). Higher magnification showed that these deposits had variable morphology, predominantly globular, sometimes flattened, stone or gravel-like, among other forms. Very high magnifications (96000–8000) allowed a better view of the irregular, stony or gravel-like deposits, which measured from 2 to 5 microns (Fig. 2c,d). In normal skin, the collagen fibers are well arranged and without deposits (92400) (Fig. 2b). This case is a primary extensive macular amyloidosis, as there was no evidence of systemic involvement or association with other diseases, papules, nodules, purpuric or infiltrative lesions or macroglossia were not found. MA is commonly diagnosed by the presence of eosinophilic, hyaline, homogeneous and fissured deposits in the upper dermis. Sparse perivascular lymphohistiocytic infiltrate and incontinentia pigmenti may also be present. Other criteria include the identification of amyloid deposit using Congo red staining with birefringence seen through a polarized light and bright aspect with ultraviolet fluorescent microscopy. Crystal Violet, which was used in this case report, also allows the visualization of amyloid accumulation. Two other clinical forms of primary skin amyloidosis are also recognized: Lichen Amyloidosis (LA) and Nodular Amyloidosis (NA), which are characterized by papules and nodules respectively. Histologically, the deposits extend beyond the papillary dermis towards the depth.2,3 The exact cause of macular amyloidosis is still unknown, but the most accepted theory is that the epidermal cells are discharged into the dermis and converted into amyloid deposits. Transmission electron microscopy has been used for many years to detect different forms of amyloidosis, including
(a)
(b)
(c)
(d)
Figure 1 (a) Hyperpigmented macules on the back, (b) extensive lace-like macules on the legs (c) normal epidermis with wide dermal papillae due to the accumulation of hyaline droplets. Rare lymphocytes in perivascular of the superficial dermis. HE 4009. (d) Crystal Violet staining allows a clearer visualization of the deposits in the dermal papillae. Crystal Violet 4009.
JEADV 2016, 30, 852–909
© 2015 European Academy of Dermatology and Venereology
Letters to the Editor
890
(a)
(b)
(c)
(d)
extracutaneous.5 Filament deposits, non-branching and nonanastomosing, with a width varying between 7 and 10 nm, can be observed.2,3 With the use of SEM in the present study, some unexplored characteristics of the deposit morphology for example ‘stoneshape’, were visualized. It is not only an innovative but also an important method because it documents a three-dimensional image of the dermal deposits. Although this method is not frequently used in daily clinical practice, it could be indicated in cases where there is suspicion of the disease if light microscopy failed to show the amyloid deposits. Finally, it is suggested that SEM should be an alternative diagnostic method for MA. Further studies are required to validate its use with other forms of amyloidosis. R.R. da Cunha Filho,1,* H.L. de Almeida Jr,2 E.P. Brum,1 M.K. Lima,1 R. Marques e Silva3 1
Department of Dermatology, Western University of Santa Catarina, Joacßaba, 2Department of Dermatology, Federal University of Pelotas and Catholic University of Pelotas, 3Laboratory of Electron Microscopy, Federal University of Pelotas, Pelotas, Brazil *Correspondence: R.R. da Cunha Filho. E-mail: robertodermatologista@ yahoo.com.br
References 1 Sarkany RPE, Breathnach SM, Seymour CA, Weismann K, Burns DA. Metabolic and nutritional disorders. In Burns T, Breanthnach S, Cox N, Griffiths C, eds. Rook’s Textbook of Dermatology, 7th edn. Blackwell Science Ltd, Oxford; 2004: 5336–5371. 2 Groves RW, Black MM. Amylodosis. In: Bolognia JL, Jorizzo JL, Schaffer JV, eds. Dermatology. Elsevier Saunders, New Haven, CT, 2012: 699–708.
JEADV 2016, 30, 852–909
Figure 2 Scanning electron microscopy of affected and normal skin Scanning electron microscopy. (a) Irregularly arranged collagen fibrils, intermingled with deposits that varies from spheres, flattened spheres, sometimes resembling stones in the papillary dermis in affected skin (91800). (b) collagen fibres well arranged and without deposits in normal skin (92400). (c, d). Deposits measuring from 2 to 5.5 microns in affected skin (96000–8000).
3 Maize JC, Maize JC Jr, Metcalf J. Doencßas metab olicas da pele. In Elder DE, Elenesitas R, Johnson BL Jr, Murphy GF, Xu X, eds. Lever Histopatologia da Pele. Guanabara Koogan. Philadelphia, PA: Lippincontt Williams & Wilkins; 2009: 403–406. 4 Clement CG, Truong LD. An evaluation of Congo red fluorescence for the diagnosis of amyloidosis. Hum Pathol 2014; 45: 1766–1772. 5 Electron Microscopy in the Diagnosis of Amyloidosis. In: Amyloidosis – Mechanisms and Prospects for Therapy. [WWW document] 2011. URL http://www. http://cdn.intechopen.com/pdfs-wm/20526.pdf (last accessed: 4 November 2014). DOI: 10.1111/jdv.13054
T-cell Acute Lymphoblastic Leukaemia presenting as multiple scalp tumours in a child Editor Acute leukaemia is the most common childhood malignancy. In rare instances, childhood leukaemia may present directly on the skin as the first manifestation of the disease, a condition known as leukaemia cutis. We report skin involvement in the scalp of a child as the first manifestation of acute lymphoblastic leukaemia. A previously healthy 7-year-old girl presented with a 1-month history of asymptomatic scalp lesions. She was referred to our paediatric dermatology department with the diagnosis of epidermoid cysts. Dermatological examination revealed large, well-circumscribed, red to tan tumours on the frontal area, temple and scalp
© 2015 European Academy of Dermatology and Venereology
889
Macular amyloidosis: a case report with scanning electron microscopy Editor Macular Amyloidosis (MA) is characterized by the deposition of an amorphous hyaline protein, restricted to the papillary dermis, normally without extracutaneous manifestations. Clinically, MA presents as hyperpigmented macular lesions on the back, involving the interscapular area and legs. Light microscopy using special stainings confirms the diagnosis. Transmission electron microscopy (TEM) is another technique that allows the visualization of long non-branching fibrils in the papillary dermis, that are isolated or in bundles.1,4 We report a case of extensive MA with an innovative approach using scanning electron microscopy (SEM). A 35-year-old female patient, skin phototype IV, presented extensive areas of hyperpigmented macules, with rippled pattern, slightly pruritic, which began approximately 8 years ago, involving the legs and dorsal region (Fig. 1a,b). The following tests were performed and the results were normal: CBC, liver and kidney function, protein electrophoresis and immunoelectrophoresis. Bence-Jones protein in urine was absent. The tests for HbsAg, VDRL HCV, HIV I and II were negative. Light microscopy showed amyloid deposits, with rippled pattern, in the papillary dermis and in some areas in direct contact with basal cells, either by haematoxylin–eosin and more easily viewed by crystal violet staining (Fig. 1c,d). Using SEM to examine the lateral surface of the punch biopsy, it was possible to observe in the papillary dermis, with low
magnification (91800), irregularly arranged collagen fibrils, intermingled with deposits (Fig. 2a). Higher magnification showed that these deposits had variable morphology, predominantly globular, sometimes flattened, stone or gravel-like, among other forms. Very high magnifications (96000–8000) allowed a better view of the irregular, stony or gravel-like deposits, which measured from 2 to 5 microns (Fig. 2c,d). In normal skin, the collagen fibers are well arranged and without deposits (92400) (Fig. 2b). This case is a primary extensive macular amyloidosis, as there was no evidence of systemic involvement or association with other diseases, papules, nodules, purpuric or infiltrative lesions or macroglossia were not found. MA is commonly diagnosed by the presence of eosinophilic, hyaline, homogeneous and fissured deposits in the upper dermis. Sparse perivascular lymphohistiocytic infiltrate and incontinentia pigmenti may also be present. Other criteria include the identification of amyloid deposit using Congo red staining with birefringence seen through a polarized light and bright aspect with ultraviolet fluorescent microscopy. Crystal Violet, which was used in this case report, also allows the visualization of amyloid accumulation. Two other clinical forms of primary skin amyloidosis are also recognized: Lichen Amyloidosis (LA) and Nodular Amyloidosis (NA), which are characterized by papules and nodules respectively. Histologically, the deposits extend beyond the papillary dermis towards the depth.2,3 The exact cause of macular amyloidosis is still unknown, but the most accepted theory is that the epidermal cells are discharged into the dermis and converted into amyloid deposits. Transmission electron microscopy has been used for many years to detect different forms of amyloidosis, including
(a)
(b)
(c)
(d)
Figure 1 (a) Hyperpigmented macules on the back, (b) extensive lace-like macules on the legs (c) normal epidermis with wide dermal papillae due to the accumulation of hyaline droplets. Rare lymphocytes in perivascular of the superficial dermis. HE 4009. (d) Crystal Violet staining allows a clearer visualization of the deposits in the dermal papillae. Crystal Violet 4009.
JEADV 2016, 30, 852–909
© 2015 European Academy of Dermatology and Venereology
Letters to the Editor
890
(a)
(b)
(c)
(d)
extracutaneous.5 Filament deposits, non-branching and nonanastomosing, with a width varying between 7 and 10 nm, can be observed.2,3 With the use of SEM in the present study, some unexplored characteristics of the deposit morphology for example ‘stoneshape’, were visualized. It is not only an innovative but also an important method because it documents a three-dimensional image of the dermal deposits. Although this method is not frequently used in daily clinical practice, it could be indicated in cases where there is suspicion of the disease if light microscopy failed to show the amyloid deposits. Finally, it is suggested that SEM should be an alternative diagnostic method for MA. Further studies are required to validate its use with other forms of amyloidosis. R.R. da Cunha Filho,1,* H.L. de Almeida Jr,2 E.P. Brum,1 M.K. Lima,1 R. Marques e Silva3 1
Department of Dermatology, Western University of Santa Catarina, Joacßaba, 2Department of Dermatology, Federal University of Pelotas and Catholic University of Pelotas, 3Laboratory of Electron Microscopy, Federal University of Pelotas, Pelotas, Brazil *Correspondence: R.R. da Cunha Filho. E-mail: robertodermatologista@ yahoo.com.br
References 1 Sarkany RPE, Breathnach SM, Seymour CA, Weismann K, Burns DA. Metabolic and nutritional disorders. In Burns T, Breanthnach S, Cox N, Griffiths C, eds. Rook’s Textbook of Dermatology, 7th edn. Blackwell Science Ltd, Oxford; 2004: 5336–5371. 2 Groves RW, Black MM. Amylodosis. In: Bolognia JL, Jorizzo JL, Schaffer JV, eds. Dermatology. Elsevier Saunders, New Haven, CT, 2012: 699–708.
JEADV 2016, 30, 852–909
Figure 2 Scanning electron microscopy of affected and normal skin Scanning electron microscopy. (a) Irregularly arranged collagen fibrils, intermingled with deposits that varies from spheres, flattened spheres, sometimes resembling stones in the papillary dermis in affected skin (91800). (b) collagen fibres well arranged and without deposits in normal skin (92400). (c, d). Deposits measuring from 2 to 5.5 microns in affected skin (96000–8000).
3 Maize JC, Maize JC Jr, Metcalf J. Doencßas metab olicas da pele. In Elder DE, Elenesitas R, Johnson BL Jr, Murphy GF, Xu X, eds. Lever Histopatologia da Pele. Guanabara Koogan. Philadelphia, PA: Lippincontt Williams & Wilkins; 2009: 403–406. 4 Clement CG, Truong LD. An evaluation of Congo red fluorescence for the diagnosis of amyloidosis. Hum Pathol 2014; 45: 1766–1772. 5 Electron Microscopy in the Diagnosis of Amyloidosis. In: Amyloidosis – Mechanisms and Prospects for Therapy. [WWW document] 2011. URL http://www. http://cdn.intechopen.com/pdfs-wm/20526.pdf (last accessed: 4 November 2014). DOI: 10.1111/jdv.13054
T-cell Acute Lymphoblastic Leukaemia presenting as multiple scalp tumours in a child Editor Acute leukaemia is the most common childhood malignancy. In rare instances, childhood leukaemia may present directly on the skin as the first manifestation of the disease, a condition known as leukaemia cutis. We report skin involvement in the scalp of a child as the first manifestation of acute lymphoblastic leukaemia. A previously healthy 7-year-old girl presented with a 1-month history of asymptomatic scalp lesions. She was referred to our paediatric dermatology department with the diagnosis of epidermoid cysts. Dermatological examination revealed large, well-circumscribed, red to tan tumours on the frontal area, temple and scalp
© 2015 European Academy of Dermatology and Venereology
