Cases reported "Leg Dermatoses"

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1/24. Cutaneous polyarteritis nodosa: a case report and literature review.

    Cutaneous polyarteritis nodosa (CPN) is an uncommon form of vasculitis. It exists as a separate entity, though bearing similar name with polyarteritis nodosa (PAN) which is an aggressive systemic vasculitis with multi-organ involvement. CPN runs a chronic but benign course. Its aetiology is unknown and it usually presents with painful nodules on the legs with mild constitutional symptoms, and extracutaneous features of arthralgias, arthritis, neuropathy and myopathy. No mortality has been reported thus far. It is therefore important to distinguish CPN apart from PAN. Symptomatic treatment with judicial use of systemic steroids and anti-inflammatory agents will suffice in most cases.
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ranking = 1
keywords = vasculitis
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2/24. Nodular tuberculid: a report of four patients.

    The tuberculids are hypersensitivity reactions to mycobacterium tuberculosis (MTB) and include papulonecrotic tuberculid (PNT), lichen scrofulosorum, erythema induratum of Bazin (EIB), and phlebitic tuberculid. Papulonecrotic tuberculid displays papulonecrotic lesions mostly on the extensor surfaces of the limbs. Histopathology shows necrosis, granulomatous inflammation (GI), and occasionally vasculitis, usually in the superficial dermis. erythema induratum of Bazin shows nodulo-ulcerative lesions on the posterior aspect of the legs. Histopathology reveals a septolobular panniculitis, necrosis, GI, and vasculitis. The Mantoux test is strongly positive and associated tuberculosis (TB) may be present in both conditions. MTB cannot be demonstrated with a Ziehl-Neelsen (ZN) stain or cultured. The polymerase chain reaction has demonstrated MTB dna in PNT (50%) and EIB (25%). The tuberculids respond to full anti-TB treatment. We document four patients with nodules on the legs in whom the pathologic changes were situated in the deep dermis and adjacent subcutaneous fat. Nodular tuberculid (NT) is regarded as a suitable term for these lesions. All patients were female. Their ages were 19 months, 12 years, 17 years, and 5 years. All patients presented with nodules on the limbs. These nodules were approximately 1 cm in diameter, dull red or bluish-red, and nontender. Ulceration was not present. The number of nodules varied from a few to many. The Mantoux test was strongly positive in all the patients. Associated pulmonary TB was present in two patients. Histopathology showed GI (n = 4), vasculitis (n = 2), and coagulative necrosis (n = 2). A ZN stain was negative in each case. All patients received anti-TB treatment for 6 months [rifampicin (n = 4), isoniazid (n = 4), pyrazinamide (n = 4), and ethambutol (n = 2)]. At 12 months follow-up, skin and pulmonary lesions had resolved in all. Nodular tuberculid should be distinguished from arthropod bites and papular urticaria, dermal erythema multiforme, evolving vasculitis, evolving folliculitis, and erythema nodosum. Histopathologically NT should be distinguished from other causes of granulomatous vasculitis and GI with or without necrosis. In children with nodules on the limbs unresponsive to routine treatment, skin biopsy should be done to exclude NT. Nodular tuberculid represents a hybrid between PNT and EIB with characteristic clinicopathologic features and should be included in the classification of cutaneous TB.
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ranking = 2.5
keywords = vasculitis
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3/24. Perinuclear antineutrophilic cytoplasmic antibody-positive cutaneous polyarteritis nodosa associated with minocycline therapy for acne vulgaris.

    minocycline is an oral antibiotic widely used for the long-term treatment of acne vulgaris. Unusual side effects of this medication include two overlapping autoimmune syndromes: drug-induced lupus and autoimmune hepatitis. In addition, in a few patients livedo reticularis or subcutaneous nodules have developed in association with arthritis and serum perinuclear antineutrophil cytoplasmic antibodies (P-ANCA) during long-term minocycline therapy. We report the cases of two young women receiving long-term minocycline therapy (>3 years) in whom P-ANCA-positive cutaneous polyarteritis nodosa developed. Both patients presented with a violaceous reticulated pattern on the lower extremities. Histologic examination of biopsy specimens from a reticulated area and a subcutaneous nodule showed necrotizing vasculitis of medium-sized arteries in the deep dermis, consistent with the diagnosis of polyarteritis nodosa. The cutaneous lesions rapidly resolved on discontinuation of minocycline and initiation of prednisone therapy. A high index of suspicion and testing for antineutrophil cytoplasmic antibody in addition to the standard antinuclear antibody panel can facilitate diagnosis of minocycline-related autoimmune disorders.
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ranking = 0.5
keywords = vasculitis
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4/24. Erythema elevatum diutinum in a patient with human immunodeficiency virus.

    Erythema elevatum diutinum (EED) is a chronic cutaneous leukocytoclastic vasculitis. This rare disease is characterized by red, brownish-purple, and yellow papules, plaques, and nodules distributed symmetrically about the extremities. There have been recent reports of the disease in association with infection with the human immunodeficiency virus (hiv). We describe the case of a 51-year-old man with hiv who presented with EED, which was successfully treated with dapsone.
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ranking = 0.5
keywords = vasculitis
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5/24. Response of livedoid vasculitis to intravenous immunoglobulin.

    Livedoid vasculitis is a chronic condition characterized by recurrent painful ulceration of the lower limbs, which heals to leave atrophie blanche surrounded by hyperpigmentation and telangiectasia. We report two patients with livedoid vasculitis who, after failure of conventional therapies, responded to intravenous immunoglobulin (IVIg). There was healing of areas of active ulceration and improvement of erythema, swelling and pain. IVIg has been used successfully to treat a variety of vasculitic disorders and appears to be well tolerated. We suggest that this treatment is offered to patients who have livedoid vasculitis that is unresponsive to other therapies.
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ranking = 3.5
keywords = vasculitis
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6/24. erythema induratum of Bazin and Poncet's disease -- successful treatment with antitubercular drugs.

    erythema induratum of Bazin (EIB) is considered a tuberculide reaction and consists of recurrent painful nodules predominantly on the calves. Clinically it has common features with diseases like nodular vasculitis, perniosis, polyarteritis nodosa and erythema nodosum. Poncet's disease is a reactive arthritis that may accompany tuberculosis. We report a case of a young woman in which the simultaneous occurrence of erythema induratum of Bazin and Poncet's disease led to a clinical picture very similar to Lofgren's syndrome. The final diagnosis was obtained by polymerase chain reaction detection of mycobacterial dna in a skin biopsy. A systemic therapy with tuberculostatic drugs led to the disappearance of symptoms. The presented case shows the usefulness of polymerase chain reaction diagnostics in EIB patients without other clinical signs of tuberculosis and a confusing combination of symptoms, and further confirms the presence of mycobacterial dna in EIB lesions.
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ranking = 0.5
keywords = vasculitis
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7/24. minocycline-induced cutaneous polyarteritis nodosa with antineutrophil cytoplasmic antibodies.

    minocycline is an antibiotic widely used in the treatment of acne. Among the induced auto-immune disorders, cutaneous polyarteritis nodosa (PAN) is very rare. A new case is reported below. A 23-year-old female patient treated with minocycline for acne for 24 months developed sub-cutaneous nodules, livedo reticularis and pigmented lesions of the lower limbs. Antineutrophil cytoplasmic antibodies (ANCA) were positive at 1/320. skin biopsy showed vasculitis of a medium-sized artery. The role of minocycline was suspected using the imputability criteria. The diagnosis of minocycline-induced cutaneous PAN with ANCA was sustained. After withdrawal of the treatment, the nodular lesions decreased spontaneously, whereas livedo disappeared and inflammatory parameters were normalized after oral corticosteroid therapy. minocycline is a tetracycline which is efficient for treating acne. Auto-immune disorders are frequently observed. Among them, it is very rare to observe cutaneous PAN associated with positive ANCA. The pathophysiological mechanisms are discussed.
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ranking = 0.5
keywords = vasculitis
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8/24. Paraneoplastic vasculitis associated with multiple myeloma.

    The association between vasculitis and cancer has been widely reviewed in recent decades. The existence of malignancies in patients with vasculitis has been estimated at about 4.5-8%, haematological neoplasms being the most frequently observed. The haematological malignancies most frequently described are lymphoproliferative diseases such as hairy cell leukaemia and lymphomas. On the contrary, the incidence of paraneoplastic vasculitis in patients with myeloma is low; up to now, we have found nine cases reported on this subject. We report the case of a 73-year-old woman who in 1 year showed three outbreaks of acutely painful, purpuric and ulceronecrotic lesions, localized on the lower extremities. Histopathological study demonstrated thrombosis in the arterioles and leucocytoclastic vasculitis. Complementary tests revealed the presence of multiple myeloma.
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ranking = 4
keywords = vasculitis
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9/24. Cutaneous IgA-associated vasculitis induced by alcohol.

    A patient with elevated levels of serum IgA developed purpuric lesions histologically resembling Henoch-Schonlein purpura brought on by consuming alcohol. Alcohol challenge with 5 units of alcohol reproduced the lesions, with a rapid rise of circulating CD4 and CD8 T cells followed by a fall of serum IgA and C3 concentration. The skin lesions and serum abnormalities resolved spontaneously within 6 weeks of the alcohol challenge.
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ranking = 2
keywords = vasculitis
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10/24. Behcet's disease-like presentation of bullous pyoderma gangrenosum associated with Crohn's disease.

    A 47-year-old woman presented with a 2-month history of generalized arthralgia and a 10-day history of oral aphthous ulcers. After hospitalization, papulopustular lesions and perianal ulcerations developed. Pathergy test was positive and ophthalmological examination was normal. The presence of oral aphthous ulcers, genital ulcerations, papulopustular lesions and arthralgia, and the positive pathergy test suggested the diagnosis of Behcet's disease (BD). In a few days, positive pathergy reactions and papulopustular lesions evolved into bullous lesions, which were diagnosed dermatopathologically as pyoderma gangrenosum. Two days after the presentation of papulopustular lesions, the patient experienced diarrhoea accompanied by bloody stools and mucus. Histopathological examination of biopsy specimens showed no vasculitis but revealed findings suggestive of Crohn's disease. The patient responded well to treatment with systemic steroids and 5-aminosalicylic acid. Our case demonstrates that the differential diagnosis of BD and inflammatory bowel disease may be perplexing and that these two diseases may be closely related.
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ranking = 0.5
keywords = vasculitis
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