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Year : 1992  |  Volume : 58  |  Issue : 4  |  Page : 285-286

Nodular amyloidosis associated with scleredema of buschke

Correspondence Address:
R R Mittal

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A 56-year old man had scleredema for 2˝ years. He also suffered from chest infection and late latent syphilis. In addition, he had one firm oval yellowish-red plaque on the back which on histopathology was diagnosed as nodular amyloidosis.

Keywords: Nodular amyloidosis, Scleredema of Buschke

How to cite this article:
Mittal R R, Gill S S, Singla A. Nodular amyloidosis associated with scleredema of buschke. Indian J Dermatol Venereol Leprol 1992;58:285-6

How to cite this URL:
Mittal R R, Gill S S, Singla A. Nodular amyloidosis associated with scleredema of buschke. Indian J Dermatol Venereol Leprol [serial online] 1992 [cited 2020 May 28];58:285-6. Available from: http://www.ijdvl.com/text.asp?1992/58/4/285/3821

  Introduction Top

Scleredema is a chronic, rare disorder characterized by non-pitting, board-like induration starting from neck and spreading to other parts of the body. [1] Scleredema is of 3 types, [2] one with abrupt onset after respiratory tract infection, [3] second with insidious onset, and the third preceded by diabetes. In many chronic cases monoclonal gammopathy is associated. [4] Histopatho­logically the dermis is about 3 times thicker than normal. The collagen bundles are thickened and separated by clear spaces due to deposition of non-sulphated hyaluronic acid. Sweat glands surrounded by fat are located in upper or mid dermis rather than in lower dermis as fat is replaced by collagen. [5]

  Case Report Top

A 56-year-old man presented with generalized progressive induration and tightly bound down skin except that of hands, feet and genitalia for 2 1/2 years. Stiffness and restriction of movements started from posterolateral aspect of neck and slowly progressed to other parts. There were repeated chest infections and late latent syphilis preceding the induration. One 6x4 cms, oval erythematous plaque with 1 cm yellowish, atrophic, papery centre where palpating finger could pass upto 4-5 mm was present on the upper back for last 3-4 months [Figure - 1].

Investigations were normal except honey-combing of chest and positive V.D.R.L. test in 1:32 dilutions. Biopsy from indurated skin showed marked increase and thickening of collagen bundles with fenestrations and widening of spaces between them. Skin adnexa were in mid dermis [Figure - 2]. Histopathology of the plaque showed focal acanthosis, atrophy, suprabasilar vacuolisation to partial necrosis of epidermal cells. The dermis showed proliferating and thickened vessels with intense perivascular and periadnexal lymphohistiocytic infiltrate intermingled with plasma cells and eosinophils. Congo red stain was positive for amyloidosis.

  Comments Top

Preceding infections are known to initiate scleredema of Buschke. In our case chronic chest infection and latent syphilis could have triggered it. Plaque could be either due to tertiary syphilis or nodular variety of primary systemic amyloidosis as monoclonal paraproteinaemias is known to occur with scleredema [4] We could not confirm about monoclonal para-proteinaemia due to lack of facilities.

  References Top

1.Curtis AC, Shulak BM . Scleredema adultorum. Arch Dermatol 1965; 92: 526-41.  Back to cited text no. 1  [PUBMED]  
2.Venecie P Y, Powell F C, SU WPD. et al..Scleredema, a review of thirty three cases.J Am Acad Dermatol 1984; 11: 128-34.  Back to cited text no. 2    
3.O'Leary P, Waisman M, Harrison N W Scleredema Adultorum. Amer J Med Sc 1990; 199: 459.  Back to cited text no. 3    
4.Kovary P M, Vakilzaden F, Macher E, et al. Monoclonal gammopathy in scleredema. Arch Dermatol 1981, 117: 536-9.  Back to cited text no. 4    
5.Lever W F, Schaumburg-Lever G . Metabolic Diseases. In : Histopathology of the Skin, 7th edn. Philadelphia : JB Lippincott, 1990; 475.  Back to cited text no. 5    


[Figure - 1], [Figure - 2]


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