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[Lichen planus and thymoma. A case].

INTRODUCTION: Lichen planus is a chronic inflammatory disease of the skin, rarely associated with a thymoma. CASE REPORT: A 72-year-old woman with erosive buccal lichen, hypertrophic lichen planus of the lower limbs, severe myasthenia and acquired hypogammaglobulinaemia associated with thymoma. CD8 lymphocyte count was increased. In the months following surgical ablation of the thymoma, the clinical examination and laboratory findings progressively returned to normal. Two years later, the patient was in good health. DISCUSSION: To our knowledge, this is the first report of favourable outcome after surgical treatment despite the aggressive symptomatology. The particular outcome allow confirmation that the thymoma plays a causal role in this syndrome and emphasizes the effects a thymoma can have on the immune system.

Agammaglobulinemia↗

[Cutaneous larva migrans, autochthonous in France. Apropos of a case].

INTRODUCTION: Cutaneous larva migrans is rarely contracted in temperate countries. CASE REPORT: When his house became flooded, he had to stand for a long period of time with mud up to the thigh. Some days later, he developed multiple erythematous, serpiginous pruritic tracts moving 1-2 cm per day over preexisting lesions of the right leg. Local and systemic treatment with thiabendazole led to rapid and definitive cure. DISCUSSION: Cutaneous larva migrans results from the migration of hookworm larvae in the dead-end human host. It is mainly an imported disease and native cases in Europe as reported here are rare. This case demonstrates that the conditions leading to the development of cutaneous larva migrans are rarely found simultaneously in temperate zones.

Adult↗

[Cutaneous and lymphatic cryptococcosis after transient lymphopenia in an immunocompetent patient].

INTRODUCTION: Cryptococcosis is an opportunistic infection, that rarely occurs in immunocompetent patients. CASE REPORT: We report a case of a 82 year-old woman who suffered from a bacterial pneumonia associated with lymphopenia. During the hospitalisation, she developed a cutaneous and ganglionnary cryptococcosis. No other cause of immunosuppression was found. The lymphopenia disappeared with the healing of the pneumonia treated with systemic antibiotics. Treatment with fluconazole was effective in 2 months on the cryptococcosis. COMMENTS: To our knowledge, this is the first case of cryptococcosis after a transitory lymphopenia, occurring in a immunocompetent patient.

Aged↗

Leucocytoclastic vasculitis induced by prolonged exercise.

Many people develop skin symptoms after long-distance walks, but little is known about the aetiology of these. In this study we took 11 biopsies from 10 long-distance walkers who walked 80 km. All biopsies originated from purpuric lesions on the lower legs, which had appeared during walking. In all 11 specimens, signs of a leucocytoclastic vasculitis were present with leucocytoclasis, exocytosis of erythrocytes and a granulocyte/mononuclear perivascular infiltrate. Immunofluorescence investigations showed deposition of C3c in many specimens and immunoglobulin M in some. The occurrence of a leucocytoclastic vasculitis after prolonged exercise may be explained by the existence of an exercise altered cutaneous microcirculation, complement activation and an altered immune function.

Complement C3c↗

Hypergammaglobulinaemic purpura of Waldenström and Ro/SSA autoantibodies.

Seven out of nine patients (78%), seen over a 13-year period with hypergammaglobulinaemic purpura of Waldenström were found to have antibodies to Ro/SSA. Over this period we saw 175 patients who had antibodies to Ro/SSA. In six of the seven patients, associated diseases were recognized. Five had Sjögren's syndrome, and one had systemic lupus erythematosus with Sjögren's syndrome and thyroiditis (and died 19 years after initial presentation from cerebral infarction). Screening for antibodies to Ro/SSA is important in the diagnosis of patients with hypergamma-globulinaemic purpura, and is helpful in predicting prognosis.

Adult↗

[Perforating verruciform collagenoma, an exogenous inclusion skin disease? Apropos of a case induced by calcium chloride].

INTRODUCTION: Perforating verruciform collagenoma, first described in 1963, designates papular and keratotic post-trauma lesions which show an aspect of epidermal perforation microscopically with large fragments of collagen issuing through fistulous chimneys, more or less pycnotic polynuclears and squamating or necrotic epidermal cells. Three publications have presented this diagnosis. CASE REPORT: We observed a patient with multiple lesions with macroscopic and microscopic presentations suggestive of perforating verruciform collagenoma. The lesions appeared after scratches and inoculation with calcium chloride. DISCUSSION: This case is similar to those observed after intradermal inoculation of calcium salts. In the 4 previous cases, perforating verruciform collagenoma was associated with trauma allowing the intradermal penetration of a particular foreign material (glass wool, vegetable debris, drugs or intravenous injections, wound caused by a metallic garbage bin). The exceptional nature of perforating verruciform collagenoma in highly frequent skin wounds would suggest that a very particular post-trauma process is occurring caused by the introduction of an exogenous substance within the derma: calcium chloride in our case, a non-specific material in the four previous cases.

Adult↗

[Conjugal mycosis fungoides].

INTRODUCTION: The pathophysiology of mycosis fungoides remains uncertain but HTLV I or a similar virus could be involved. We observed a couple who developed mycosis fungoides suggesting the infectious hypothesis might indeed be valid. CASE REPORT: A 70-year-old man who had often travelled in foreign countries developed parapsoriasis en plaques, lymphomatoid papulosis and mycosis fungoides successively over a thirty year period. Several years after the first manifestation of mycosis fungoides, his wife also developed a single plaque of mycosis fungoides. The diagnosis was confirmed on pathology slides and immunohistochemistry tests as well as on the basis of T-receptor gene rearrangement in both patients. Search for HTLV I was negative using serology tests and PCR on circulating lymphocytes. COMMENTS: The epidemiological situation in our observation (several trips in foreign countries and the delayed development of mycosis fungoides in the wife) favours the hypothesis of an infectious mechanism. Search for HTLV I was unsuccessful with classical virology methods. Certain recent work suggests a virus similar but different from the HTLV I virus could be involved in the pathogenesis of mycosis fungoides.

Aged↗

[Elastosis perforans serpiginosa with vitamin A deficiency in a child with trisomy 21].

INTRODUCTION: Elastosis perforans serpiginosa frequently occurs in trisomy 21. Usually no cause is found. We report a case in which vitamin deficiency may have contributed to the development of skin lesions. CASE REPORT: A 11-year-old girl with trisomy 21 developed papulokeratosic eruptions with a linear serpiginous distribution, predominantly involving the lower limbs. On ultrastructure examination, numerous elastic fibers penetrated the epiderma and the baseline membrane had disappeared. The patient also had a totally patent atrioventricular canal with hypoplasia of the left ventricle and cardiac liver. Serum vitamin A level was low (0.56 mumol/l, normal > 1.55). Acitretine was prescribed at the dose of 0.5 mg/kg/day but had to be stopped 2 months later due to elevated liver enzyme levels despite a clear clinical improvement. DISCUSSION: This association between elastosis perforans serpiginosa and vitamin A deficiency, observed here in a child with trisomy 21, has never been reported by others. Vitamin A deficiency might aggravate the skin lesions. In our case, there was probably a relationship between the vitamin A deficiency and the cardiogenic liver disease. The keratoregulatory effect of vitamin A on elastic tissue is less well known. Treatment with retinoids provided clinical improvement but had to be stopped due to hepatotoxicity. Parenteral vitamin A would be an interesting alternative but the risk of side effects would theoretically be greater than with oral retinoids.

Acitretin↗

[Pseudomonas aeruginosa folliculitis after depilation].

INTRODUCTION: Pseudomonas aeruginosa is usually the responsible agent for epidemics of folliculitis acquired in swimming pools and whirlpools. Sporadic cases of Pseudomonas folliculitis after epilation of the legs are well known by dermatologists, but unfrequently reported in the literature. CASE REPORT: We report the case of a 18-year-old female affected of folliculitis of the legs caused by Pseudomonas aeruginosa, after depilation. A meticulous investigation of contaminant sources was performed, and the responsible was the sponge used to wash the legs after depilation. DISCUSSION: Pseudomonas aeruginosa folliculitis after depilation is more frequent than usually reported. A meticulous study of the concomitant source of contamination is essential to avoid recurrent episodes.

Administration, Topical↗

A reticulate vascular abnormality in patients with lymphoedema: observations in eight patients.

We describe eight patients with lymphoedema who had prominent compressible ridges of tissue in a reticulate pattern, situated predominantly on the upper part of the lower leg. In five patients the lymphoedema was primary, two patients had circumferential venous ulceration, and one had marked venous disease with a small ulcer. One patient had a squamous cell carcinoma of the medial thigh and dysplastic keratoses in the distribution of the reticulate ridges. In three of the four cases in whom histological examination of the ridges was performed, the skin at these areas was demonstrated to contain grossly dilated angular vessels in the mid-dermis, many with valves visible. The vessel walls had a single layer of endothelial cells (anti-factor VIII-related antigen positive) and a basement membrane containing type IV collagen. Abnormal elastic tissue in these biopsies was similar to that in erythema ab igne. Indirect lymphography in one case did not demonstrate dilated lymphatic vessels. The body site distribution and clinical pattern of the abnormality appeared to be similar to erythema ab igne but associated with an underlying abnormality of lymphatic rather than blood vasculature. We propose that our cases may represent 'lymphoedema ag igne'.

Aged↗

Acute panniculitis: a clinical and histopathologic study of 34 cases.

Thirty-four patients had histopathologic findings of acute panniculitis. 15 had clinical findings of erythema nodosum; 6 had infectious lesions; and 5 had Weber-Christian-like conditions with recurrent febrile suppurative or nonsuppurative nodular eruptions; 3 of the 5 had amylase and lipase enzyme abnormalities with or without pancreatic disease. Five additional patients had acute panniculitis that would be termed "deythema induratum" or "nodular vasculitis" by most clinicians, and the subsequent pathologic findings and courses were consistent with this view. Two patients had limited, traumatic panniculitis, forming a distinctive group, but trauma was a factor in lesion formation in all groups, and one patient had vasculitis. Whereas acute panniculitis, with or without fat necrosis, may be associated with many inflammatory syndromes of the skin and subcutaneous tissue and may rarely occur in all, the most common relationship is with erythema nodosum in its most acute forms.

Acute Disease↗

Erythema nodosum caused by omeprazole.

We report a case of erythema nodosum caused by omeprazole. This side effect of omeprazole has not been previously reported. A 35-year-old white woman developed multiple tender erythematous nodules over the anterior aspect of her upper and lower extremities, fever, malaise, and edema. Her symptoms resolved with withdrawal of all preexisting medications and conservative treatment. Her symptoms flared soon after a rechallenge with omeprazole. A thorough history, physical examination, laboratory evaluation, and roentgenogram failed to reveal another cause for erythema nodosum. It is clear from the rechallenge that this patient's erythema nodosum was caused by omeprazole. We consider that omeprazole should be added to the list of medications known to cause erythema nodosum.

Adult↗

Concomitant Graves' disease and Hashimoto's thyroiditis, presenting as primary hypothyroidism.

Hypothyroidism in patients with Graves' disease is usually the result of ablative treatment. We describe a 58 year old man with Graves' ophthalmopathy and pre-tibial myxoedema, who presented with spontaneous primary hypothyroidism. Circulating TSH receptor antibody activity was increased, while thyroid microsomal antibody was detectable in titres greater than one in one hundred thousand. It is likely that the TSH receptor antibody of Graves' disease was ineffective in stimulating hyperthyroidism because of concomitant thyroid destruction due to Hashimoto's disease. Alternatively, primary hypothyroidism could have resulted from the effects of a circulating TSH receptor blocking antibody.

Graves Disease↗

IgG, IgA and C3 deposits in the extra-thyroidal manifestations of autoimmune Graves' disease: their in vitro solubilization by intravenous immunoglobulin.

OBJECTIVE: To study the involvement of antibodies in the extrathyroidal manifestations of autoimmune Graves' disease, we determined the presence of IgG, IgA and IgM antibodies and C3c in connective tissue samples from patients with Graves' disease and pretibial myxedema (PTM) or thyroid associated ophthalmopathy (TAO). METHODS: Connective orbital tissue samples were obtained from 12 patients undergoing orbital decompression for TAO, and skin samples from lesions on the pretibial area were obtained in 7 patients with PTM. Sections from each tissue sample were stained with fluorescin-isothiocianate conjugated anti-human IgG, IgA, IgM and C3c and were examined by a fluorescence optical instrument. Other serial sections from each sample were incubated with human IgG solutions (concentration 6 mg/ml or 20 mg/ml), human albumin (40 mg/ml), PBS, myoglobin (40 mg/ml), or IgA (20 mg/ml), and were then processed by a standard direct immunofluorescence staining procedure. RESULTS: Among the samples from TAO patients 8/12 (67%) were positive for IgG deposition, 4/9 (44%) were positive for IgA, 1/9 (11%) was positive for IgM and 4/9 (44%) were positive for C3c deposition. Orbital connective samples from 3 non-TAO patients were all negative. Among samples from PTM patients 4/7 (57%) were positive for IgG deposition, 3/ 4 (75%) were positive for IgA, 0/4 was positive for IgM and 3/7 (43%) were positive for C3c deposition. Skin samples from 5 control patients undergoing skin biopsy for non-autoimmune diseases were all negative. Incubation with human IgG (20 mg/ml) resulted in the complete disappearance of IgG and C3c deposition in all positive patients. No significant variation in IgG fluorescent staining after incubation with either 6 mg/ml of IgG solution, human albumin, PBS, myoglobin or IgA was observed. CONCLUSION: The results of our study suggest that different classes of antibodies, mainly IgG and IgA, may be implicated in the disease process in autoimmune TAO and PTM. Activation of the complement cascade, via the classic or the alternative pathway, could take place in about 40% of these patients. IVIG in vitro may solubilize, by a specific mechanism, IgG and complement immune complex deposition in the extrathyroidal manifestations of autoimmune Grave's disease.

Complement C3↗

Significance of tissue specific and tissue non specific autoimmune reactions of Graves' disease.

The relationship between the many immunologic abnormalities demonstrated in the peripheral blood of patients with Graves' disease (GD) and the broad spectrum of clinical features with which patients may present has not yet been addressed in detail. In this review we examine the evidence to support the notion that GD could be considered a multi-system autoimmune disorder in which tissue damage is restricted to the thyroid gland, connective tissue of the skin and orbit, extra-ocular and other skeletal muscles and, possibly, the lacrimal glands. Apart from the well recognized reactions of autoantibodies and sensitized T lymphocytes with epitopes on the thyroid specific TSH receptor, thyroid peroxidase and thyroglobulin, in patients with hyperthyroidism, there is also good evidence for autoantibody and, to a lesser extent, T lymphocyte reactivity with several eye muscle, other skeletal muscle and connective tissue, antigens in patients with ophthalmopathy, systemic myopathy, dermopathy and acropachy. There is also some evidence for immunoreactivity against lacrimal gland antigens in patients with ophthalmopathy associated with other features of GD. There are, in addition, a variety of organ non-specific reactions in GD; antinuclear antibodies are detected in serum from about one-third of patients with hyperthyroidism and ophthalmopathy, while from 5% to 10% have antibodies reactive with several other ubiquitous tissue proteins. Cloned proteins which are autoantigenic in some patients with hyperthyroidism or Hashimoto's thyroiditis and ophthalmopathy include collagen XIII, nebulin, the calcium binding protein calmitine, and the Mac-II antigen. All antibodies reactive with eye muscle antigens, except the 64 kDa protein which is also expressed in the thyroid, cross react with the same, or a related, protein in other skeletal muscle. Future research should focus on the underlying mechanisms for this broad loss of tolerance to self antigens and the effect of environmental factors such as stress, radioiodine and viral infection of the thyroid gland and other target tissues, in precipitating disease.

Animals↗