Multiple Autoimmune Syndrome with Vitiligo, Autoimmune Thrombocytopenia and Autoimmune Dermoepidermal Bullous Dermatosis

Abstract

Multiple auto-immune syndrome is defined by the coexistence of at least three different autoimmune diseases. Immune genetic predisposition and abnormalities of humeral and cell-mediated immunity seems to be implicated in their genesis.In this observation, we describe a combination of three autoimmune diseases in the same patient namely vitiligo evolving since childhood, autoimmune dermo epidermal bullous dermatosis and autoimmune thrombocytopenia. The discovery of such associations may limit the therapeutic arsenal. And temporal sequence indicates that long-term surveillance of these patients is necessary to watch the occurrence of another autoimmune disease.According to the clinical context and the results of immune histology we retained in our patient the combination of three autoimmune diseases: vitiligo, autoimmune thrombocytopenia with autoimmune bullous derma epidermal dermatitis most probably cicatricle pemphigoid. Epidermolysis bullosa acquisita that looks like cicatricle pemphigoid cannot be ruled out given the lack of examination by immune-microscopy.The patient was initially put on oral corticosteroids at a dose of 1mg / kg / day until normalization of platelet levels. Then treatment with daps one at a dose of 2 mg / kg / day associated with mycofenolate mofetil at a rate of 2g / day was initiated with a clinical and laboratory monitoring. The evolution was marked by the stabilization of the disease and a slight improvement in ocular involvement. DisscussionWe report a unique case characterized by the association of vitiligo, an autoimmune thrombocytopenia and autoimmune dermo epidermal bullous dermatosis in a male patient. It is still uncommon IntroductionMultiple autoimmune syndrome is defined by the coexistence of at least three different autoimmune diseases which can occur simultaneously or over several years of evolution. We report an exceptional association of vitiligo, autoimmune thrombocytopenia and autoimmune dermoepidermal bullous dermatosis. ObservationA young patient of 35 years presented since the age of 14 achromic non-painful or pruritic lesions which gradually extended to the level of the body. 4 years ago he developed intermittent oral and nasal erosions associated with red eyes and a decrease of visual acuity impeding his daily and professional activities. He also reported episodic appearance of some liquidiennes skin lesions without signs of infection or systemic disease.Ocular examination revealed bilateral red eyes with Symblepharon, and corneal dystrophy (Figure 1). Cutaneous examination revealed at the left leg the presence of tense bullae with clear contents based on healthy skin, associated with hemorrhagic erosions (Figure 2). The Nikolsky's sign was negative. There were also extensive achromic patches (Figure 3). Laboratory test showed thrombocytopenia at 32000 / mm3. We completed the investigation by sternal puncture which showed a hypercellular marrow with megakaryocytic hyperplasia in support of a peripheral autoimmune origin. Thyroid and anti-nuclear antibody wer...

Multiple Autoimmune Syndrome: A Study of 25 Senegalese Cases

“…MAS were an entity rarely described in the African literature. In North Africa [14]- [20], as in Africa South of the Sahara [12] [13], the entity is reported as an isolated observation or in short series Open Journal of Rheumatology and Autoimmune Diseases was likely to be underestimated in sub-Saharan Africa, as there is a considerable increase in the incidence of autoimmune diseases in those regions [21] [22] [23] [24]. This entity should be considered, because there is evidence that 25 to 30 percent of autoimmune disease are thought to progress to polyautoimmunity [2].…”