An evolving presentation of cutaneous tuberculosis.

Tuberculosis is a fairly common disease in the United States and around the world, newly infecting ten million people throughout the world per year. Despite the pervasiveness of tuberculosis, cutaneous tuberculosis (CTB) rarely manifests worldwide. Tuberculous infections of the skin arise in several distinct variants that can be classified as either multibacillary or paucibacillary; each subtype within these categories presents with its own morphological and histological findings. The diagnosis of CTB can prove clinically challenging as its variants mimic many conditions dermatologist encounter on a daily basis. Additionally, tissue confirmation is difficult. We report a case of CTB which evolved from a lupus vulgaris presentation to the metastatic tuberculous abscess variant.

Lupus Vulgaris Erythematoides: report of a patient initially misdiagnosed as dermatitis.

A small percentage of patients with tuberculosis present with cutaneous findings, which may be difficult to diagnose. We present a patient diagnosed with a rare, non-scarring form of cutaneous tuberculosis (CTB), classically termed as lupus vulgaris erythematoides.

Rapidly metastatic carcinoma in lupo in a patient with lupus vulgaris for more than 50 years.

According to current statistics of the WHO, tuberculosis is the infectious disease that causes the most deaths worldwide. Its most common cutaneous manifestation is lupus vulgaris which is seldom diagnosed today. A 69-year old immunocompetent woman complained of a partly elevated, partly sclerotic plaque on her left thigh which had been present for more than 55 years before slowly becoming ulcerated. After biopsy and subsequent excision of the 13 cm ulcer, the diagnosis of carcinoma in lupo with lymph node metastasis was made. Cutaneous and additional nodal metastases appeared rapidly. Tuberculostatic therapy was initiated. Despite systemic chemotherapy the tumor subsequently progressed and the patient died of metastatic carcinoma in lupo 15 months after the initial diagnosis. Early diagnosis and treatment of lupus vulgaris might have prevented the development of carcinoma in lupo and ensuing metastatic death of the patient.

Squamous cell carcinoma arising from long-term (50-year) Lupus vulgaris: is there a need for a close medical follow-up in such chronic diseases?

Skin manifestations of tuberculous infection (Mycobacterium tuberculosis) are represented by miliary tuberculosis of the skin, tuberculous chancre, scrofuloderma, tuberculosis verrucosa cutis, periorificial tuberculosis, and lupus vulgaris (LV). Among this group, LV is the most common skin condition, diagnosed in 10% of tuberculotic patients. The authors report herein a case of squamous cell carcinoma (SCC) arising from long-standing (50-year) LV and underline the need of an extensive follow-up of tuberculotic lesions.

The color of skin: orange diseases of the skin, nails, and mucosa.

Cutaneous disease can present with lesions of all colors of the visible spectrum. Lesions of the skin, nail, and mucous membranes with an orange color can be due to a variety of etiologies. The conditions may appear as purely orange, yellow-orange, red-orange, tan, or brown with an orange hue. The orange color may also present as a transient phase of a disease process. As with all dermatologic pathology, a key way to distinguish orange-colored lesions is by distribution and morphology. The sclera, palate, lips, gingiva, and nails may also be involved. A literature review using PubMed with keywords, including orange, skin, mucosa, cutaneous, xanthoderma, and granuloma, was conducted to gather all dermatologic conditions that can present with an orange color. The relevant diseases were categorized by etiology and include inflammatory, infectious, neoplastic, and exogenous causes.

Metastasized squamous cell carcinoma developed on lupus vulgaris.

Lupus vulgaris (LV) is the most frequent cutaneous tuberculosis, representing more than 55% of the tuberculoses with this location. Malignization can occur after a long latency (10-30 years), in 1-2% of the cases, and it is mainly in squamous cell carcinoma. The histological exam is highly important in the observation of neoplasic transformations. The authors present a 59-years-old female patient, from the rural environment, working as a farmer, with lupus vulgaris developing since her first childhood years. It started at the age of 2 years, at the right ear lobule, after the empiric perforation for earrings. The evolution was progressive, eccentric, interesting the pinna and the right cheek in the meanwhile. At the first examination, in 2002, a diffuse mass of red-yellowish infiltration was found at the level of the right ear and the right cheek. In the following two years, an ulcero-vegetating tumor developed at the level of the right ear lobule, accompanied by the presence of a right retromandibular adenopathy, of about 1 cm, which was proved by the histopathologic exam to be a squamous cell carcinoma developed from a lupus vulgaris. After scraping out the right retromandibular ganglion, detected by palpation, a histological exam showed ganglion metastasis.

Superficial resection combined with photodynamic therapy for successful treatment of facial lupus vulgaris with squamous cell carcinoma.

Skin squamous cell carcinoma is the second most common non-melanoma skin tumor worldwide. Most skin squamous cell carcinoma patients have underlying diseases. Here, we report a 56 year-old patient diagnosed with skin squamous cell carcinoma and with a 30 year course of neglected lupus vulgaris, which was very rare. In this case, we adopted a treatment strategy involving a small wound: superficial resection combined with photodynamic therapy with a satisfied result.

Antibody induction of lupus-like neuropsychiatric manifestations.

Although systemic lupus erythematosus (SLE) is usually evaluated with regard to autoimmune reactivity toward the kidney, there are multiple psychiatric abnormalities associated with this autoimmune disease. Lupus-prone male NZM88 mice, derived from NZB/NZW F1 mice, develop early neuropsychiatric manifestations without any signs of nephritis. In addition to the usual repertoire of antibody specificities, including autoantibodies to dsDNA and renal antigens, mice of this inbred strain express autoantibodies to numerous brain antigens. Here, we show that autoantibodies to brain antigens, assessed by Western analysis, are as individually varied as are the diverse neuropsychiatric manifestations observed in SLE patients. Additionally, a monoclonal antibody derived from the spleen of an untreated NZM88 male when injected into healthy BALB/cByJ, but not C57BL/6J, mice induced behaviors similar to those of lupus-prone NZM88 mice. This monoclonal antibody, which is specific to dynamin-1, binds preferentially in BALB/cByJ cortex and induces substantial expression of cytokines mainly in the hypothalamus. Thus, an antibody to just one brain antigen can induce multiple behavioral changes, and multiple autoantibodies to different brain antigens exist in lupus-prone mice; however, susceptibility to the induction of neurobehavioral deficits is dependent on host genetics.

Lupus-associated pulmonary hypertension: long-term response to vasoactive therapy.

INTRODUCTION: Pulmonary hypertension (PH) is a serious complication of lupus. The effectiveness of current vasoactive therapy has not been well described. METHODS: Retrospective analysis of 12 patients with lupus-associated PH (age 43+/-10 years, mean+/-SD, all female) treated with pulmonary vasodilators. RESULTS: At baseline, patients had severe PH: median six-minute walk distance (6MWD) 266 m (95% confidence interval [CI], 106 to 362); functional class III (n=7) and IV (n=5); mean pulmonary artery pressure (mPAP) 52 mmHg and cardiac index 2.23 L/min/m(2). Eight patients were started on epoprostenol and 2 each on bosentan or treprostinil. After a mean follow-up of 41+/-25 months, 5 patients were on combination therapy (3 epoprostenol plus bosentan, 1 treprostinil plus bosentan, 1 bosentan plus sildenafil) and 7 were on monotherapy (2 epoprostenol, 4 bosentan, 1 sildenafil); 6MWD increased by 139 m (95% CI, 36 to 259, p=0.007), 8 patients were functional class I or II and 4 were class III; right ventricular systolic pressure (RVSP) decreased by 22 mmHg (95% CI, 6 to 36; p=0.012), mPAP decreased by 18 mmHg (95% CI, 8 to 29; p=0.014), and cardiac index increased by 1.44 L/min/m(2) (95% CI, 0.76 to 2.08; p=0.016). There was no mortality or need for lung transplantation. Therapy was well tolerated. CONCLUSIONS: Vasoactive therapy can achieve sustained clinical and hemodynamic improvement in lupus-associated PH.

Heart involvement in systemic lupus erythematosus, anti-phospholipid syndrome and neonatal lupus.

Cardiac involvement is one of the main complications substantially contributing to the morbidity and mortality of patients suffering from systemic autoimmune diseases. All the anatomical heart structures can be affected, and multiple pathogenic mechanisms have been reported. Non-organ-specific autoantibodies have been implicated in immune complex formation and deposition as the initial triggers for inflammatory processes responsible for Libman-Sacks verrucous endocarditis, myocarditis and pericarditis. Anti-phospholipid antibodies have been associated with thrombotic events in coronary arteries, heart valve involvement and intra-myocardial vasculopathy in the context of primary and secondary anti-phospholipid syndrome. Antibodies-SSA/Ro and anti-SSB/La antigens play a major pathogenic role in affecting the heart conduction tissue leading to the electrocardiographic abnormalities of the neonatal lupus syndrome and have been closely associated with endocardial fibroelastosis.

Lupus-related advanced liver involvement as the initial presentation of systemic lupus erythematosus.

BACKGROUND AND PURPOSE: Systemic lupus erythematosus (SLE), a prototype of systemic autoimmune disease characterized by multiorgan involvement with diverse clinical and serological manifestations, principally affects women in their child-bearing years. Clinically significant hepatic abnormality as the initial presentation of SLE has rarely been reported. METHODS: Eleven patients with lupus with initial presentation of lupus-related hepatitis were included in this retrospective review. Clinical manifestation, immunological profiles, and risk factors for poor prognosis were analyzed. RESULTS: The most commonly associated clinical manifestations were found to be thrombocytopenia, leukopenia, advancing age, and presence of anti-SSA/Ro antibody and anti-thyroid antibodies. The diagnosis of SLE was delayed due to dominant hepatic abnormalities. Age greater than 50 years and marked hepatic decompensation in accordance with Child classification B and C might suggest poor prognosis (p=0.06). However, the p value was not statistically significant because of the small sample size. CONCLUSIONS: Lupus-related hepatitis, particularly in late-onset lupus, is common. In addition, the presence of anti-SSA, anti-thyroglobulin, and anti-microsomal antibodies is indicative of hepatic involvement in patients with SLE.

Rapidly developing giant sized lupus vulgaris on the chest associated with bilateral scrofuloderma on the neck.

Lupus vulgaris and scrofuloderma are the opposite poles of cutaneous tuberculosis. Lupus vulgaris of a giant size and scrofuloderma in the vicinity of this lesion were both present in a 70-year-old female patient. The purified protein derivative of tuberculin (PPD) skin test was strongly positive. In histopathological examination, granulomatous infiltration without caseation necrosis was seen in the dermis. The patient was treated with a four-drug therapy consisting of pyrazinamide (25 mg/kg), isoniazid (5 mg/kg), rifampin (10 mg/kg) and ethambutol (15 mg/kg) daily for 2 months, followed by dual therapy with isoniazid and rifampin for 6 months. Her cutaneous lesions significantly regressed after 4 months, leaving keloid scars.

[Lupus tuberculosis: a cause of medio-facial mutilation not to be ignored]. / Le lupus tuberculeux: une cause de mutilation centrofaciale à ne pas méconnaître.

Medio-facial mutilations are a real problem from a diagnosis and prognosis point of view. Various etiologies may be in question, namely bacterial, parasitic, mycotic, viral infections or yet predisposing factors such as denutrition or a immunodepression. Among these etiologies, the lupus tuberculosis remains a major concern to be taken into account in poor socio-economic background. We report two cases of lupus tuberculosis in teen-agers whose diagnosis difficulty induced sequellae although limited by a further appropriate care management. The clinical spectrum of the cutaneous tuberculosis and the place of this affection featuring ahead of other medio-facial mutilations etiologies remain important in tropical area.

[Acute adrenal insufficiency during unilateral adrenal hemorrhage secondary to the antiphospholipid syndrome]. / Insuffisance surrénale aiguë et hématome surrénalien unilatéral au cours d'un syndrome des antiphospholipides.

INTRODUCTION: Acute adrenal insufficiency during antiphospholipid syndrome is usually due to a bilateral adrenal hemorrhage. For a patient, an unilateral involvement has induced an acute adrenal insufficiency. CASE REPORT: A patient with an antiphospholipid syndrome has developed an acute adrenal insufficiency with an unilateral adrenal hemorrhage. This complication was caused by a concurrent steroid therapy, for an associated lupus, which resulted in a suppression of the contralateral adrenal gland. CONCLUSION: In patients with antiphospholipid syndrome and concurrent steroid therapy, usually for lupus, unilateral adrenal hemorrhage can cause acute adrenal insufficiency. Withdrawal of steroid might be difficult in case of undetected bilateral hemorrhage.

[Cryptococcal infections in non-HIV infected patients. Study of four cases and review of literature]. / Infections à cryptocoque chez des patients non VIH. A propos de quatre cas et revue de la littérature.

BACKGROUND: Cryptococcal infections are frequent in HIV-infected patients and are regularly looked after. This infection may occur in others immunosuppressives situations and, in those cases, diagnosis is often delayed. METHODS: We report four cases of cryptococcal infections in patients whose immunosuppression isn't related with HIV infection but due to chronic lymphocytic leukemia, giant cell temporal arteritis, gastric neoplasm and lupus. Diagnosis, prognostic and treatment are detailed. RESULTS: Four patients aged from 25 to 76 presented a cryptococcal infection (three meningitis). A woman died at the admission. Another died seven years later. The two others are still alive under treatment. When infected, all patients were immunodeficiency. CONCLUSION: Cryptococcal infection may occur in patients non-HIV-infected patients. Early detection is needed to improve prognostic.

Neonatal lupus.

An otherwise healthy 5-week-old infant with erythematous plaques predominantly on the face and scalp presented to our dermatology clinic. The mother had been diagnosed with lupus erythematosus 2 years earlier but her disease was quiescent. Neonatal lupus is a rare condition associated with transplacental transfer of IgG anti-SSA/Ro and anti-SSB/La antibodies from the mother to the fetus. Active connective tissue disease in the mother does not have to be present and in fact is often absent. Although the cutaneous, hematologic and hepatic manifestations are transient, the potential for permanent heart block makes it necessary for this to be carefully ruled out. As in this case, the dermatologist may be the one to make the diagnosis and should be aware of the clinical presentation, work-up, and management of this important disease.