Scleroderma in Guinea’s African: Epidemiological, Clinical and Therapeutic Aspects

Introduction: Scleroderma is a generalized condition of the connective tissue, arterioles and micro-vessels characterized by tissue fibrosis, vascular ob-literation and abnormal humoral and cellular immune responses. The aim of this study was to describe the epidemiological, clinical, therapeutic and evolutionary aspects of scleroderma at the Department of Dermatolo-gy-Venerology at Donka National Hospital. Methods: This was a descriptive cross-sectional study with a retrospective collection of data for a period of 11 years, from January 1, 2000, to December 31, 2010, covering all inpatient records of Scleroderma in the service. Results: We collected 17 cases of scleroderma out of 3289, a frequency of 0.04%. The average age of patients was 33.33 years with extremes of 10 and 60 years. A female predominance was noted with a sex ratio M/F of 0.13. Cutaneous induration was the main symptom associated with the phenomenon of Raynaud, and disorders of pigmentation. The extra-skin manifestations were mainly dominated by gastrointestinal involvement (32%), pulmonary involvement (24%), cardiac (20%), articular (20%) and renal (12%) involvement. The systemic form was the most common (99.45%). Corticotherapy was the most prescribed treatment (24.59%). Clinical improvement was noted in (93.2%) of the cases. Conclusion: Scleroderma, although rare, deserves special attention at all levels for early diagnosis.


Introduction
Scleroderma (Sc) is a rare autoimmune disease of unknown etiology, characterized by the presence of sclerotic lesions affecting the dermis and/or certain internal organs, associated with vascular changes and immunological abnormalities [1]. Two major forms are described: localized scleroderma and systemic scleroderma whose prognosis is more reserved [2].
Diagnosis can occur in the presence of epidemiological factors' characteristic dermatological lesions and histological data. The cutaneous manifestations have a major interest for the diagnosis, making it possible to evoke it at the first glance. Scleroderma justifies multidisciplinary management combining mainly symptomatic treatments and, in case of visceral involvement, immuno-modulatory treatment [3].
Its exact prevalence is still poorly known with significant disparity between regions and countries. The prevalence is estimated in the United States of America and Australia between 200 and 260 cases/million inhabitants, in Asia between 20 and 50 cases/million inhabitants and in Europe between 100 and 200 cases/million inhabitants [4] [5].
In Africa, data on this condition are patchy. Thus, Tunisia El Fekih and colleagues [6] reported 92 cases of localized scleroderma from 1991 to 2004. Benin Yedomon and his team [7] reported 20 cases of scleroderma between 1980 and 1989. In Senegal 117 cases were reported within 16 years, or 7.3 cases per year [8], while in Burkinas Faso, 14 cases were reported from 1 January 2004 to 31 December 2009 [9].
In Guinea, there are no published data on this condition. The objectives of this study were to determine the hospital prevalence of scleroderma, to describe the socio-demographic and clinical characteristics of patients with Scleroderma, and to assess the modalities of therapeutic and progressive management of patients with scleroderma.

Methods
This was a descriptive cross-sectional study with a retrospective collection of data from an 11-year period (from January 1, 2000 to December 31, 2010). The study reviewed all the files of patients hospitalized in the Department of Dermatology-Venerology of Donka National Hospital for Scleroderma during the study period.
We included in this study the charts of patients seen for scleroderma diagnosed according to the criteria of the ACR [10], of any age and of any sex, whatever the clinical form, having received a medical treatment and whatever their origin.
We conducted a comprehensive recruitment of all patient records followed for scleroderma. Our data were collected on pre-established survey cards, manually processed, seized; we expressed the quantitative variables on average, and the qualitative variables in percentage. The variables of the study were grouped into: -Sociodemographic variables: Sex, age, occupation and residence.
For confidentiality measures we have assigned an identifier to each patient record to preserve anonymity.
The immunological assessment with the antinuclear antibody test was not carried out due to inavailability of resources. So the diagnosis was based solely on clinical evaluation.
The data collected were entered and analyzed in Epi info software version 6.

Results
Over a period of 11 years (from January 1, 2000 to December 31, 2010), we collected data on 17 cases of scleroderma out of a total of 3289 inpatients. This gives a disease prevalence of 0.04%. The average age of patients was 33.33 years with extremes of 10 and 59 years. The age group 30 -39 was the most common (47%), followed by the age group 50 -59 (24%), the age groups 20 -29 and 40 -49 represented 12% each and age 10 -19 years was 5%. A female predominance was noted, 15 women against 2 men, a sex ratio of 0.13.
According to socio-occupational categories, housewives were the most frequent (65%), followed by shopkeepers (12%) and pharmacists, students, farmers and mechanics, who each accounted for 5.75%. Sixty-seven percent (67%) of patients resided in Conakry and 33% came from within the country.
According to the topography of the lesion, the trunk was the most affected (34%), followed by the face (19%), limbs (16%) and neck (13%). The lesions were generalized in 19% of cases. The systemic form of scleroderma was the most common (76%) and the localized form involved 24% of patients.
All of the patients were put on steroids and hygiene and dietary measures.
NSAIDs were prescribed in 87% of cases and antihypertensive treatment in 40%.
One patient (1.6%) was put on immunosuppressive therapy. Clinical improvement was noted in 94.12% and death in 5.88% of cases.

Discussion
We The demographic characteristics of our patients are comparable to those found in the literature. These are young adults (mean age = 33.33 years) and, for the majority, female (88.24%); as reported by El Fekih N et al. [6] in Tunisia  [7] in Benin where the average age was 33 years and all patients were female.
This female predominance reinforces the hypothesis of fetal-maternal micro-chemistry in the pathogenesis of scleroderma. Indeed, Lee et al. [11] showed that women with systemic scleroderma had a significantly higher level of Y chromosome-derived deoxyribonucleic acid (DNA) in their mononuclear blood cells than women who were free of the disease. In addition, these scleroderma women most often had an HLA type II phenotype allowing better tolerance of fetal cells and greater microchemistry. The team of Artlett N et al. confirmed these results and also found this significant increase at the cutaneous level compared to the control group [12].
However, this theory, although attractive, does not explain the occurrence of systemic scleroderma in children, men or nulliparous women, except, possibly, in case of a history of blood transfusion, organ transplantation, miscarriage or maternofetal passage of maternal cells (maternal anti-fetal reaction), situations that may lead to cellular micro-chemistry.
The clinical manifestations of scleroderma are polymorphic. The sensation of cold found in 7 (14.29%) of our patients was reported by Hachulla E et al. [13].
Depending on the type of scleroderma, the predominance of the systemic form (11/15 cases) was noted in our series, while in Benin, Yedomon HG et al. [7] reported a predominance of localized forms or 16 cases of localized scleroderma for 4 cases of systemic scleroderma. It is the same for El Fekih et al. [6] in Tunisia, which in 14 years have reported 92 cases of localized scleroderma.
The cutaneous signs noted in our study have been described by other African series, notably that of Yedomon HG et al. in Benin [8]. Indeed, all these studies agree on the frequency of diffuse cutaneous involvement with induration type, speckled achromia (vitiligo spots), the relative rarity and the discretion of Raynaud's phenomenon. These semiological characteristics have already been noted in African black [14]; but in the absence of true epidemiological studies, the individualization of an African form of Scleroderma cannot yet be considered [8].
In Western series, however, the phenomenon of Raynaud is widely described in but that of the esophagus is the most common and best studied; it is found in 50 to 90% of cases according to the diagnostic method used. The pulmonary involvement is also frequent during scleroderma, it is more severe in diffuse cutaneous forms; pulmonary fibrosis is the most common anatomical expression. It has also been reported by other African series including that of Yedomon HG et al. [8] (1cas). As for cardiac involvement, it is often the consequence of renal or pulmonary complications, however there is a primitive form of extremely common myocardial damage when evaluated by sensitive techniques which, when it leads to clinical signs is very pejorative for the prognosis. In the end kidney damage is the main cause of death specifically scleroderma, and the renal crisis which is manifested by malignant hypertension and acute renal failure, is the main manifestation.
Therapeutically, corticosteroids and lifestyle and dietary measures were used in all patients. Immunosuppressants were used in one patient. In the series of A.
Diop et al. [8] In Senegal, corticosteroids were associated with D-penicillamine, hydroxychloroquine or cyclophosphamide in all cases. The inaccessibility of our patients to these treatments of references mentioned above would be explained by their high cost, out of reach of the patients often deprived and living in an extreme precariousness.
On the evolutionary level, in our study, the majority of patients (94.12%) evolved favorably. However, we recorded one death case. However, this result should not obscure the severity of scleroderma, especially in its systemic form.

Conclusion
Scleroderma, although rare, can be localized or systemic and deserves special attention at all levels to obtain early diagnosis and multidisciplinary care.