Scleroderma 098. (885.0Kb)
SCLERODERMA
Blondina Marpaung, Ernita Sinaga
Divisi Rheumatologi Departemen Ilmu Penyakit Dalam
Fakultas Kedokteran Universitas Sumatera Utara
RSUP Haji Adam Malik / RSU.Dr.Pirngadi Medan
ABSTRACT
Scleroderma is derived from the Greek words skleros (hard or indurated) and derma (skin).Hippocrates
first described this condition as thickened skin, Scleroderma. In 1945, Robert H. Goetz first described in
detail the concept of scleroderma as a systemic disease; he introduced the term progressive systemic
sclerosis.This article reports the case of a systemic sclerosis patient. A 33 year old woman with chief
complain stiffnes of the hand joints and skin since 3 year prior to hospital admission. Initially, she had
been experiencing whitish skin lesion that gradually blackened and become tightened. Patient also had
been experiencing tightened of her mouth with the result that she had difficulty to swallow the food
(dysphagia). From physical examination reveals diffuse fibrosis of the skin and internal organ (lung,
esophagus). Laboratory result: ANA test and CRP positive. The patient were treated symptomatically and
supportive and focuses on the organ systems involved.
INTRODUCTION
Scleroderma is derived from the Greek words skleros (hard or indurated) and derma (skin). Hippocrates
first described this condition as thickened skin, Scleroderma. Scleroderma is a rare disease; it usually
presentsbetween the ages of 35 and 55, with an up to 8 - fold female excess.Population prevalence
studies estimate the prevalence of sclerodermato be between 30 and 1130/million — the wide variation is
due to the lack of population studies, as the disease is rare1. In the USA, the prevalence of scleroderma in
the general population has been reported in the range of 50 per 1.000.000. The disease is very rare in
childhood, with peak occurrence in the 30-40 years age group. Women are affected four times more often
than men. Environmental factors seem to play a greater role than genetic factors in the aetiology;
Universitas Sumatera Utara
etiological agents implicated include viruses, silica exposure, vinyl chloride, organic solvents, bleomycin,
and even smoking and alcohol intake. Genetic factors influence the clinical expresion of the disease.2
Genetic influences have long been suspected to impact SSc. In families with a history of SSc, the
incidence of disease can range from 1.5 to 1.7%
3
Systemic sclerosis is a complex and heterogeneous disease with clinical forms ranging from limited skin
involvement (limited cutaneous systemic sclerosis) to forms with diffuse skin sclerosis and severe and
often progressive internal organ involvement (diffuse cutaneous systemic sclerosis), and occasionally a
fulminant course (fulminant systemic sclerosis). Limited cutaneous systemic sclerosis involves areas
distal to the elbows and knees but may involve the face and neck. CREST syndrome (calcinosis, Raynaud
phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias—although not all are needed for
the disorder to be called CREST) is an older term used to describe this subset of limited cutaneous
systemic sclerosis.1,4,5
A systemic sclerosis diagnosis is based on clinical findings, which have substantial heterogeneity and
varying manifestations. The classic clinical presentation is a young or middle-age woman with Raynaud
phenomenon and skin changes accompanied by musculoskeletal discomfort and gastrointestinal
symptoms. Diffuse cutaneous systemic sclerosis refers to skin thickening affecting the trunk and the skin
of the extremities proximal to the elbows and knees besides involvement of the face. Systemic sclerosis
involvement is most obvious in the skin; however, the gastrointestinal tract as well as the respiratory,
renal, cardiovascular, musculoskeletal, endocrine, and genitourinary systems are frequently involved.
have been found with specific autoantibodies such as anti-centromere antibodies and anti-topoisomerase I
(also called Scl-70) antibodies. A number of environmental triggers are thought to be risk factors for the
disease. Exposure to silica dust (stone masons and gold miners) has been linked with the disease but there
is no evidence that silicone implants increase the disease risk. Exposure to organic solvents has been
linked to an increased risk of scleroderma, and there is some evidence from case reports that specific
drugs may be linked with the disease.4,5,7,8
Universitas Sumatera Utara
The American College of Rheumatology (ACR) criteria for the classification of systemic sclerosis require
one major criterion or two minor criteria, as follows6,9,12,13, :
A. Major criterion: Proximal scleroderma is characterized by symmetric thickening (non pitting)
proximal to the MCPs (metatarsophalangeal joints), tightening, and induration of the skin of the
fingers and the skin that is proximal to the metacarpophalangeal or metatarsophalangeal joints.
This symptoms usually bilateral, symetrical, and almost always including sclerodactyly. But the
major criteria is sufficient.
B. Minor criteria : 2 or 3 are needed if no major criterion
o
Sclerodactyly is characterized by thickening, induration, and tightening of the skin,
limited to only the fingers.
o
Digital pitting scars or a loss of substance from the finger pad: As a result of ischemia,
depressed areas of the fingertips or a loss of digital pad tissue occurs.
o
Bibasilar pulmonary fibrosis includes a bilateral reticular pattern of linear or lineonodular
densities most pronounced in basilar portions of the lungs on standard chest
roentgenography. These densities may assume the appearance of diffuse mottling or a
honeycomb lung and are not attributable to primary lung disease
CASE REPORT
Patient is a 33 year old woman came to outpatient ward of internal department with chief complain
stiffnes of the hand joints and skin since 3 year prior to hospital admission. From her history of illness,
initially the patient had been experiencing whitish skin lesion that gradually blackened and become
Universitas Sumatera Utara
tightened. Patient also had been experiencing tightened of her mouth with the result that she had difficulty
to swallow the food. No history of corrosive liquid consumption and vomitus after eating were not found.
During physical examination, the patient was fully alert with normal blood pressure, no sign of fever,
however abnormalities were found in her face, neck, chest and superior extremities. Laboratory results of
complete blood count, liver function test and renal function test were under normal limits, ANA test : 153
(
Blondina Marpaung, Ernita Sinaga
Divisi Rheumatologi Departemen Ilmu Penyakit Dalam
Fakultas Kedokteran Universitas Sumatera Utara
RSUP Haji Adam Malik / RSU.Dr.Pirngadi Medan
ABSTRACT
Scleroderma is derived from the Greek words skleros (hard or indurated) and derma (skin).Hippocrates
first described this condition as thickened skin, Scleroderma. In 1945, Robert H. Goetz first described in
detail the concept of scleroderma as a systemic disease; he introduced the term progressive systemic
sclerosis.This article reports the case of a systemic sclerosis patient. A 33 year old woman with chief
complain stiffnes of the hand joints and skin since 3 year prior to hospital admission. Initially, she had
been experiencing whitish skin lesion that gradually blackened and become tightened. Patient also had
been experiencing tightened of her mouth with the result that she had difficulty to swallow the food
(dysphagia). From physical examination reveals diffuse fibrosis of the skin and internal organ (lung,
esophagus). Laboratory result: ANA test and CRP positive. The patient were treated symptomatically and
supportive and focuses on the organ systems involved.
INTRODUCTION
Scleroderma is derived from the Greek words skleros (hard or indurated) and derma (skin). Hippocrates
first described this condition as thickened skin, Scleroderma. Scleroderma is a rare disease; it usually
presentsbetween the ages of 35 and 55, with an up to 8 - fold female excess.Population prevalence
studies estimate the prevalence of sclerodermato be between 30 and 1130/million — the wide variation is
due to the lack of population studies, as the disease is rare1. In the USA, the prevalence of scleroderma in
the general population has been reported in the range of 50 per 1.000.000. The disease is very rare in
childhood, with peak occurrence in the 30-40 years age group. Women are affected four times more often
than men. Environmental factors seem to play a greater role than genetic factors in the aetiology;
Universitas Sumatera Utara
etiological agents implicated include viruses, silica exposure, vinyl chloride, organic solvents, bleomycin,
and even smoking and alcohol intake. Genetic factors influence the clinical expresion of the disease.2
Genetic influences have long been suspected to impact SSc. In families with a history of SSc, the
incidence of disease can range from 1.5 to 1.7%
3
Systemic sclerosis is a complex and heterogeneous disease with clinical forms ranging from limited skin
involvement (limited cutaneous systemic sclerosis) to forms with diffuse skin sclerosis and severe and
often progressive internal organ involvement (diffuse cutaneous systemic sclerosis), and occasionally a
fulminant course (fulminant systemic sclerosis). Limited cutaneous systemic sclerosis involves areas
distal to the elbows and knees but may involve the face and neck. CREST syndrome (calcinosis, Raynaud
phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias—although not all are needed for
the disorder to be called CREST) is an older term used to describe this subset of limited cutaneous
systemic sclerosis.1,4,5
A systemic sclerosis diagnosis is based on clinical findings, which have substantial heterogeneity and
varying manifestations. The classic clinical presentation is a young or middle-age woman with Raynaud
phenomenon and skin changes accompanied by musculoskeletal discomfort and gastrointestinal
symptoms. Diffuse cutaneous systemic sclerosis refers to skin thickening affecting the trunk and the skin
of the extremities proximal to the elbows and knees besides involvement of the face. Systemic sclerosis
involvement is most obvious in the skin; however, the gastrointestinal tract as well as the respiratory,
renal, cardiovascular, musculoskeletal, endocrine, and genitourinary systems are frequently involved.
have been found with specific autoantibodies such as anti-centromere antibodies and anti-topoisomerase I
(also called Scl-70) antibodies. A number of environmental triggers are thought to be risk factors for the
disease. Exposure to silica dust (stone masons and gold miners) has been linked with the disease but there
is no evidence that silicone implants increase the disease risk. Exposure to organic solvents has been
linked to an increased risk of scleroderma, and there is some evidence from case reports that specific
drugs may be linked with the disease.4,5,7,8
Universitas Sumatera Utara
The American College of Rheumatology (ACR) criteria for the classification of systemic sclerosis require
one major criterion or two minor criteria, as follows6,9,12,13, :
A. Major criterion: Proximal scleroderma is characterized by symmetric thickening (non pitting)
proximal to the MCPs (metatarsophalangeal joints), tightening, and induration of the skin of the
fingers and the skin that is proximal to the metacarpophalangeal or metatarsophalangeal joints.
This symptoms usually bilateral, symetrical, and almost always including sclerodactyly. But the
major criteria is sufficient.
B. Minor criteria : 2 or 3 are needed if no major criterion
o
Sclerodactyly is characterized by thickening, induration, and tightening of the skin,
limited to only the fingers.
o
Digital pitting scars or a loss of substance from the finger pad: As a result of ischemia,
depressed areas of the fingertips or a loss of digital pad tissue occurs.
o
Bibasilar pulmonary fibrosis includes a bilateral reticular pattern of linear or lineonodular
densities most pronounced in basilar portions of the lungs on standard chest
roentgenography. These densities may assume the appearance of diffuse mottling or a
honeycomb lung and are not attributable to primary lung disease
CASE REPORT
Patient is a 33 year old woman came to outpatient ward of internal department with chief complain
stiffnes of the hand joints and skin since 3 year prior to hospital admission. From her history of illness,
initially the patient had been experiencing whitish skin lesion that gradually blackened and become
Universitas Sumatera Utara
tightened. Patient also had been experiencing tightened of her mouth with the result that she had difficulty
to swallow the food. No history of corrosive liquid consumption and vomitus after eating were not found.
During physical examination, the patient was fully alert with normal blood pressure, no sign of fever,
however abnormalities were found in her face, neck, chest and superior extremities. Laboratory results of
complete blood count, liver function test and renal function test were under normal limits, ANA test : 153
(