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| CLINICAL CHALLENGE |
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| Year : 2012 | Volume
: 4
| Issue : 1 | Page : 44-45 |
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Cerebriform plaque on the scalp
Ambooken Betsy, MP Binitha
Department of Dermatology and Venereology, Medical College, Calicut, Kerala, India
| Date of Web Publication | 12-May-2012 |
Correspondence Address:
M P Binitha
Haritha, P. O. Beypore North, Calicut, Kerala - 673 015
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0974-7753.96091
How to cite this article:
Betsy A, Binitha M P. Cerebriform plaque on the scalp. Int J Trichol 2012;4:44-5 |
A 37-year-old woman presented with numbness of the left hand and edema of both legs, of one- year duration. She also had asymptomatic skin- colored soft papules and plaques with a cerebriform appearance and loss of hair over the occipital region [Figure 1] along with a linear edematous plaque with follicular prominences, anteriorly over the parting line of the scalp [Figure 2]. Soft violaceous papules and plaques were seen on the eyelids, nostrils, lips, and oral mucosa [Figure 3], with purpura over the face and periorbital area [Figure 4]. There was violaceous discoloration of the posterior nail folds with hyperpigmentation of the proximal halves of the nails [Figure 2]. Skin and mucosal biopsy showed eosinophilic, amorphous deposits in the upper dermis and sub- epithelial tissue, respectively, as well as perivascularly [Figure 5]. | Figure 1: Soft papules and plaques with loss of hair over the occipital region
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 | Figure 2: Diffuse infiltration of the skin over the midline of the scalp with hyperpigmentation of the proximal nails
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 | Figure 3: Violaceous papules and plaques on the face and mucosal surfaces
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 | Figure 5: Faintly eosinophilic, amorphous deposits in the upper dermis (H and E ×40)
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 What is your Diagnosis? | |  |
Diagnosis
Primary systemic amyloidosis.
| Discussion | | |
Systemic amyloidosis can be classified as: (1) primary systemic amyloidosis (PSA), not associated with other diseases, paraproteinemia, or plasma-cell dyscrasias, (2) amyloidosis associated with multiple myeloma, or, (3) secondary systemic amyloidosis associated with chronic inflammation or infectious conditions. [1] PSA is a plasma cell dyscrasia characterized by an autonomous proliferation of plasma cells with an overproduction of a monoclonal immunoglobulin protein. PSA can involve any organ system, especially the kidneys, heart, autonomic and sensory nervous systems, the only exception being the brain.
Histopathological examination shows amyloid deposits in the dermis, subcutaneous tissue, blood vessel walls, pilosebaceous units, arrector pili muscles, and sweat glands. Scalp involvement can appear as diffuse or patchy alopecia due to amyloid deposition in the pilosebaceous units. [2] Diffuse infiltration of the scalp skin results in folds resembling cutis verticis gyrata. [3] Our patient had prominent involvement of the scalp and alopecia. Congo red staining of the biopsy specimen was positive [Figure 6] and examination under polarized light revealed green birefringence. She was treated with melphalan and prednisolone, but developed progressive renal failure and nephrotic syndrome, and died of heart failure within a year of diagnosis. | Figure 6: Section stained with Congo red showing brick-red deposits in the subcutaneous tissue with amyloid rings
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| References | | |
| 1. | Nyirady J. Primary systemic amyloidosis. Medscape Reference [serial on the Internet ]. [updated 2011 Aug 1]. Available from: http://emedicine.medscape.com/article/1093258-overview. [Last cited on 2011 Nov 11]. 
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| 2. | Lutz ME, Pittelkow MR. Progressive generalized alopecia due to systemic amyloidosis. J Am Acad Dermatol 2002;46:434-6.
[PUBMED] [FULLTEXT] |
| 3. | Saoji V, Chaudhari S, Gohokar D. Primary systemic amyloidosis: Three different presentations. Indian J Dermatol Venereol Leprol 2009;75:394-7.
[PUBMED]  |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]
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