RAMB - Maio / Junho 2016

emes

200

ssoc

ras

2016; 62(3):199-201

Pathogenesis is unknown, but the main mechanism

of accumulation of matrix components appears to be re-

lated to abnormal gene expression of extracellular pro-

tein (collagen types I and III and fibronectin) in the skin.

Other possible pathogenic factors would be: hyperinsu-

linemia, vascular damage, lymphatic obstruction, and

streptococcal hypersensitivity.

Systemic manifestations are rare and include: cardi-

ac, ocular, hepatosplenic, musculoskeletal and joint im-

pairment; serositis, myositis and parotiditis; as well as in-

volvement of the tongue, esophagus and pharynx.

On histopathological examination, the epidermis has

normal appearance and the dermis is thickened with

broad collagen bundles and mucopolysaccharide depos-

its, visualized using special staining (Alcian blue at pH

2.5 to 4.0, toluidine blue and colloidal iron). There is no

increase in the number of fibroblasts.

Mucin deposits

can also be evidenced in other organs such as bone mar-

row, nerves, salivary glands, and heart.

Differential diagnosis includes scleroderma and sclero-

myxedema. Scleroderma is distinguished by acral skin in-

volvement, Raynaud’s phenomenon and typical fascies

Histopathological examination may reveal rectified epi-

dermis, dermal sclerosis, and loss of appendages. Sclero-

myxedema, in turn, is characterized by confluent papules

which gives a hardened appearance to the skin, with wide-

spread deposition of mucin in the dermis, and fibrosis

with irregular proliferation of fibroblasts.

5,7

Several treatments have been proposed, such as topical,

systemic and intralesional corticosteroids, immunosuppres-

sives, and PUVA therapy.

1,4

Nevertheless, in most cases, the

disease is self-limiting with spontaneous resolution.

Its im-

portance lies in the possibility of being a systemic marker of

disease, such as difficult-to-control diabetes or paraprotein-

emias. The possibility of systemic involvement makes early

diagnosis critical for proper treatment and better prognosis.

esumo

Escleredema de Buschke associado a

diabetes mellitus

tipo

2 de difícil controle

Escleredema de Buschke (EB) é doença rara do tecido con-

juntivo caracterizada por endurecimento difuso e não de-

pressível da pele, principalmente nas regiões cervical, deltoi-

deanas e dorso. Enquadrado no grupo dasmucinoses cutâneas,

tem etiologia desconhecida e associação com: infecções bac-

terianas ou virais, alterações hematológicas e

diabetes mellitus

O exame histopatológico evidencia derme espessada comfi-

bras colágenas calibrosas separadas por fendas que corre-

FIGURE 3

Material between thickened collagen bundles stained

with Alcian blue at pH 2.5, evidencing mucin deposits.

FIGURE 2

Thickened dermis, forming gaps between collagen

bundles.

influenza, measles, parotiditis, CMV and HIV. It has bet-

ter prognosis and is more prevalent in children and young

adults.

3,4

Type 2 is classically associated with paraproteinemi-

as (monoclonal gammopathy), but there are also reports

of an association with primary hyperparathyroidism, rheu-

matoid arthritis, amyloidosis and Sjogren’s syndrome. It

has a higher probability of chronic progression.

Type 3 SB is associated with

diabetes mellitus

(DM),

both as type 1 DM and type 2 DM. Patients most com-

monly reported in the literature are adult men with long-

standing

diabetes mellitus

with glycemic control that is dif-

ficult to achieve, obesity and high blood pressure.

Consequently, the DM has no tendency to spontaneous

resolution.

4,5