Difficulties in diagnosis and treatment of severe secondary Raynaud's phenomenon in a Cameroonian woman: a case report.

BACKGROUND: Raynaud's phenomenon is a microvascular disorder that results in exaggerated vasoconstriction over vasodilatation secondary to an alteration in autonomic control. Though benign, it can result in severe ulceration and ultimately gangrene associated with disfiguration and permanent deformity. We present a case of severe secondary Raynaud's phenomenon in a black-African patient from a resource-limited setting, with focus on the difficulties encountered in the diagnosis and treatment. CASE PRESENTATION: A 43-year-old female Cameroonian farmer with a 7-year history of episodic paresthesia in her fingers and toes (when exposed to cold) presented to our emergency department with severe pain, ulceration, and "darkening" of her fingertips over a period of 2 days. An examination revealed bilateral ulceration and dry gangrene of her fingers and toes, based on which a diagnosis of secondary Raynaud's phenomenon due to a connective tissue disease was proposed. Results of paraclinical investigations were normal. Lifestyle modification along with a calcium channel blocker and phosphodiesterase type 5 inhibitor provided significant relief. CONCLUSIONS: An early diagnosis and knowledge on appropriate treatment of Raynaud's phenomenon is of vital importance to prevent permanent tissue damage and disability. Relying on biphasic color change for the diagnosis of Raynaud's phenomenon in black Africans can be potentially misleading.

Coexistence of osteopoikilosis with seronegative spondyloarthritis and Raynaud's phenomenon: first case report with evaluation of the nailfold capillary bed and literature review.

Osteopoikilosis (OPK) is a rare autosomal dominant bone disorder characterized by numerous hyperostotic areas that tend to localize in periarticular osseous regions. It is usually asymptomatic and is often diagnosed incidentally during X-rays. OPK may be an isolated finding or associated with other pathologies, e.g. skin manifestations, rheumatic and/or skeletal disorders. We report a literature review and, for the first time, the coexistence of OPK with seronegative spondyloarthritis and Raynaud's phenomenon in a 48-year old female. To the best of our knowledge, this is the first case of OPK studied by videocapillaroscopy, demonstrating the absence of specific microvascular abnormalities of nailfold capillaries.

Síndrome adyuvante por implante de protesis mamarias de silicón (siliconosis): ¿una condición emergente? / Adjuvant syndrome due to silicone breast prosthesis implant (siliconosis): an emergent condition?

Los implantes mamarios de silicona se han asociado con una variedad de condiciones médicas que aparecen en coincidencia con su implantación; ello constituye la emergencia de una nueva y poco conocida patología de la modernidad. Más del 87% de los enfermos sintomáticos, presentan neuropatía desmielinizante y axonal, comprobada en la biopsia de nervio y músculo, mientras que aproximadamente el 22%-25% tienen evidencia de enfermedad tiroidea autoinmune. Un pequeño porcentaje del 10%-12%, presentan enfermedad desmielinizante primaria del sistema nervioso: esclerosis múltiple diagnósticada mediante resonancia magnética y estudios de líquido cefalorraquideo. Otros presentan condiciones inmunológicas diversas como síndrome de fibromialgia, tiroiditis de Hashimoto, polimiositis, dermatomiositis, lupus eritematoso sistémico, artritis reumatoide, esclerodermia y presencia de autoanticuerpos. Para estos pacientes sintomáticos se propone como diagnóstico unitario un síndrome adyuvante por implante de prótesis mamarias de silicon. Se presentan los casos de dos pacientes ilustrativos.

Silicone breast prosthesis has been associated with a variety of medical conditions or autoimmune diseases, which has coincidental relation with the implants insertion; it's loomed as a new and unknown pathology of the modern times. More than 87% of symptomatic patients developed demyelination axonal neuropathy demonstrated by nerve and muscle biopsy; 22% to 25% have evidence of autoimmune thyroid disease. An a small group of patients (10%-12%) have primary central nervous system demyelination disease as. multiple selerosis. The diagnosis of multiple selerosis was corroborated by magnetic resonance imaging and cerebrospinal fluid analysis. Also, an other wide spectrum of immunological diseases have been observed, such as fibromyalgia. Hashimoto's, polymyositis, dermatomyositis, lupus erythematosus, rheumatoid arthritis, scleroderma, and the presence of autoantibodies. Finally, for symptomatic patients, an adjuvant syndrome of silicone breast prosthesis or implant is proposed as a unitary diagnosis. The authors presented two patients whom illustrated this entity.

Peripheral blood perfusion correlates with microvascular abnormalities in systemic sclerosis: a laser-Doppler and nailfold videocapillaroscopy study.

OBJECTIVE: To investigate possible correlations between fingertip blood perfusion (FBP) status, assessed by laser Doppler flowmetry (LDF), and morphological microvascular abnormalities, detected by nailfold videocapillaroscopy (NVC), in patients with systemic sclerosis (SSc). The effects on FBP of intravenous (IV) treatment with the prostacyclin analog iloprost were also investigated. METHODS: Thirty-four consecutive patients with SSc and 16 healthy subjects were evaluated. LDF was performed by analyzing blood perfusion at the fingertips in both hands. Patients with SSc were distributed into the appropriate NVC pattern of microangiopathy (early, active, and late). Iloprost was administered to inpatients with SSc by 24-hour IV infusion for 7 consecutive days (4 microg/h). RESULTS: FBP was significantly lower in patients with SSc (p < 0.05) compared to controls. Heating of the LDF probe at 36 degrees C induced a significant increase of FBP in all subjects (p < 0.001), but the slope of variation was significantly lower in patients with SSc compared to controls (p < 0.05). Patients with SSc showing the late NVC pattern of microangiopathy had significantly lower FBP than patients with the active and early NVC patterns (p < 0.05). A negative correlation was observed between FBP and NVC rating of the microvascular damage (p < 0.05). After iloprost treatment, a significant increase of FBP was observed in patients with SSc (p < 0.05). CONCLUSION: Patients with SSc show a decreased FBP partially reversible by local skin heating. The FBP correlated negatively with the extent of nailfold microvascular damage, and IV iloprost treatment increased the FBP.

Raynaud's disease.

We report five children who presented with Raynaud's disease in whom we could find no clinical, haematological, or immunological evidence of a collagen disorder. Oral phenoxybenzamine proved useful for maintenance treatment in most, with infusions of prostacyclin, nitroprusside, and ketanserin during acute attacks.