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A distinctive variant of pernio. Clinical and histopathologic study of nine cases.

We describe here nine women who had similar distinctive clinical features that consisted of painful red-to-purple macules, papules, and plaques on the digits, predominantly on the toes. All patients reported that exposure to cold had preceded their symptoms. Histologic study demonstrated a lymphocytic vasculitis, characterized by lymphocytes infiltrating the walls of blood vessels in eight (88%) of nine lesions. This pattern of inflammation is different from the perivascular lymphocytic infiltrate that is common to lupus erythematosus, erythema multiforme, lymphocytic infiltration of the skin (Jessner), and drug eruptions. It also serves to distinguish the disorder from other clinical conditions resembling it, such as cellulitis, herpes simplex infection, and disseminated gonococcemia. To the best of our knowledge, this lymphocytic vasculitis has not been previously described as occurring in pernio. We believe the unique disorder that we are reporting is a common, but infrequently recognized, variant of pernio.

Adult↗

[Principal skin manifestations and occupational allergy].

Cutaneous manifestations of occupational allergic diseases are mainly represented by contract dermatitis and less frequently recognized for mucous localizations. Contact erythema multiforme is often a severe disease. Rarely, contact dermatitis mimics a lymphoma. Irritant contact dermatitis usually differs clinically, but the diagnosis may required skin testing and a biopsy. The allergens or the irritants are sometimes transferred by air, and there is an ingrowing literature about airborne dermatoses. Modifications of the eruptions by physical factors such as the sun, or by infections such as dermatophytes may delay the diagnosis and the cure of the eruption. Cancers may occur as occupational diseases, and the event of lymphoma is always discussed.

Air Pollutants, Occupational↗

Cutaneous manifestations of fungal infection.

Fungal infection of the skin and subcutaneous tissue may result from either direct contact or inoculation injury (primary infection) or from hematogenous spread from a primary focus of disease (secondary infection). The parainfectious lesions of erythema nodosum and erythema multiforme are manifestations of the host's immune response to the invading fungus, particularly Histoplasma capsulatum and Coccidiodes immitis. In some patients, skin lesions may be the only sign of a systemic fungal infection, and prompt recognition of these lesions may facilitate early diagnosis and treatment. This article first addresses the pathogenesis, host defenses, and diagnosis of fungal skin infections. The specific cutaneous manifestations of the superficial, cutaneous, subcutaneous, and systemic mycoses are then reviewed.

Antifungal Agents↗

Paraneoplastic pemphigus presenting as a lichen planus pemphigoides-like eruption.

BACKGROUND: Paraneoplastic pemphigus refers to a specific disease entity in which neoplasia is associated with severe mucosal ulceration and polymorphous cutaneous eruptions, sometimes resembling erythema multiforme. Direct immunofluorescence and indirect immunofluorescence on standard substrates are similar to pemphigus vulgaris; however, paraneoplastic pemphigus serum uniquely binds to all epithelia and has distinctive immunoprecipitation characteristics. OBSERVATIONS: A 63-year-old woman with chronic lymphocytic leukemia developed a severe, mucocutaneous, vesiculobullous eruption with the clinical and histopathologic characteristics of lichen planus pemphigoides evolving into a Stevens-Johnson-like presentation and the immunofluorescence findings of pemphigus vulgaris. Evaluation of her serum confirmed the presence of autoantibodies specific for paraneoplastic pemphigus by indirect immunofluorescence and immunoprecipitation criteria. CONCLUSION: The spectrum of paraneoplastic pemphigus must now be expanded to include cases that present clinically and histologically as a lichen planus pemphigoides-like eruption.

Diagnosis, Differential↗

Diclofenac (Voltaren)-induced eosinophilic pneumonitis. Case report and review of the literature.

This report describes dyspnea, cough, erythema multiforme, and a diffuse pulmonary interstitial infiltrate associated with eosinophilic alveolitis in a 67-year-old man taking diclofenac sodium (Voltaren). Onset of these pulmonary abnormalities after beginning diclofenac therapy implicates this drug, which, to our knowledge, has not been described previously to cause eosinophilic pneumonitis. The current report expands the spectrum of nonsteroidal anti-inflammatory drug-induced eosinophilic pneumonitis and reviews available experience with other implicated non-steroidal anti-inflammatory drugs.

Aged↗

Paraneoplastic pemphigus.

Paraneoplastic pemphigus is a newly recognized disease that occurs in some patients with lymphoproliferative neoplasms and occasionally, solid tumors. Patients present with an acute illness of the mucosa and skin that shares clinical and histologic features with erythema multiforme, toxic epidermal necrolysis, and pemphigus vulgaris. These patients have antibodies against a complex of epithelial proteins that are present in desmosomes and hemidesmosomes. The course is usually fatal, except in some patients who undergo total resection of their neoplasm.

Animals↗

[Drug-induced Stevens-Johnson syndrome in a patient with AIDS].

The Stevens-Johnson syndrome is a severe potentially life-threatening form of the erythema multiforme, affecting both skin and mucous membranes. We present a case of a 49-year-old male patient with AIDS who developed a Stevens-Johnson syndrome while being treated with pyrimethamine, sulfadiazine and phenytoin for cerebral toxoplasmosis. Further diagnostic evaluation of this dangerous cutaneous affection may prove difficult for several reasons. In particular, in patients with AIDS who are more susceptible for adverse drug reactions and who are simultaneously receiving a variety of drugs with a considerable potential of cutaneous side effects, therapy cannot be withhold for lack of therapeutic alternatives. Moreover, the low lymphocyte count in this case may have made reliable testing with lymphocyte transformation studies impossible. The evaluation and the differential diagnosis of the drug-induced Stevens-Johnson syndrome are discussed. Especially long- and moderately long-acting sulfonamides belong to the most important agents that can cause a drug-induced Stevens-Johnson syndrome. The pathogenesis and the risk factors for cutaneous hypersensitivity reactions in HIV-infected patients are only poorly understood. These kind of reactions, however, seem to occur more often in patients with a more advanced immunodeficiency.

Acquired Immunodeficiency Syndrome↗

[Stevens-Johnson syndrome after lamotrigine treatment].

We report a 18 years old female with Lennox-Gastaut syndrome under treatment with sodium valproate, carbamazepine and clonazepam. When seizures increased we stopped carbamazepine and introduced lamotrigine slowly. One month later the girl developed haemorrhagic erosions in mucoses and limbs with deterioration of her general state. Skin biopsy confirmed the diagnosis of erythema multiforme, the Stevens-Johnson's form. The immediate withdrawal of lamotrigine and treatment with antihystaminics and steroids was followed of a slowly favourable course with disappearance of symptomatology one month later. It's another case of Stevens-Johnson syndrome related to the introduction of lamotrigine in polytherapy.

Adolescent↗

[Chronic bullous dermatoses of childhood in the differential diagnosis of bullous dermatitis in children].

We report a case of chronic bullous dermatosis of children, a benign dermatologic disease of infancy of unknown etiology, characterized, on IIF, by linear deposits of immunoglobulins in the Basement Membrane Zone of cutis, and clinically by a ring shaped disposition of bullae (rosette-like). This work concerns a ten year old girl admitted to the pediatric ward at E. Agnelli Hospital of Pinerolo (Turin) about one year ago, who started having perioral bullous eruption, subsequently spread all over the body skin, with general symptoms of fever and itching. Round erythematous plaques, crusts, and a ring disposition of sausage-shaped subepidermal bullae were suggestive of CBDC. Diagnosis was confirmed by cutaneous biopsy and DIF. Corticosteroids, given for about six mths., and DDS in the last four mths. of treatment, quickly changed the course of the disease, which has not recurred for about one year. Differential diagnosis with other bullous dermatoses of 2nd and 3rd childhood has been addressed to bacterial and viral diseases as well as to toxico-allergic and autoimmune diseases, the latter rarely seen by Pediatricians. Among those we discuss on clinical and anatomopathological aspects of Dermatitis herpetiformis, Erythema multiforme, Pemphigus and Pemphigoid, with regard to different anatomic sites of bullous lesions and to pathogenesis involved immunoglobulins and skin antigens.

Biopsy↗

[Hypersensitivity syndrome caused by allopurinol. A case of massive hepatic necrosis].

Adverse drug reactions have a wide variety of clinical manifestations mainly related to the affected organ. The skin is one of the most common organs involved due to the visibility of the lesions to the observer. These untoward drug cutaneous responses may mimic any known dermatosis, and may be a clue to a serious systemic disease. We present the case of a 72 year-old woman with erythema multiforme, liver and renal failure due to a unknown dosage of allopurinol self-medication for two weeks. The clinical course was characterized by a Stevens-Johnson syndrome and severe drug-induced liver disease with massive hepatic necrosis corroborated by a transjugular liver biopsy; finally she died of gastrointestinal bleeding. Consent for a postmortem study was not obtained. The clinical course as well as the cutaneous, renal and hepatic drug-related complications corresponds to an allopurinol hypersensitivity syndrome. The relevance of this case was the extension of the liver damage apparently due to the allopurinol, which is an infrequent hepatotoxic complication. In some cases, like the present case, it can be a serious and fatal complication.

Acute Kidney Injury↗

[Clinical and epidemiologic aspects of paraneoplastic dermatoses in hospitalized patients treated at the Clinic for Dermatologic and Venereal Diseases in Novi Sad over a 10-year period (1980-1990)].

Authors report results of a retrospective investigation on frequency of paraneoplastic dermatoses, their clinical characteristics, time of onset and course regarding 10 - year material on hospitalized patients at the Clinic for Infectious and Dermatovenerous Diseases in Novi Sad. Out of 9086 hospitalized patients in 14 patients (0.16%) paraneoplastic dermatisis was diagnosed. Out of 14 patients in 5 patients (35.71%) Herpes zoster was diagnosed; in 4 patients (28.57%) bullous dermatosis; in 2 patients (14.29%) paraneoplastic acrokeratosis; in 1 patient (7.14%) exudative multiform erythema in 1 patient (7.14%) erythema figuratum and in one more patient necrotic Herpes labialis was diagnosed. Concerning malignant neoplasms of internal organs together with paraneoplastic dermatosis in most cases (4 - 28.57%) chronic lymphocyte leucosis was found, and in remaining 10 (71.43%) carcinomas were diagnosed at different internal organs. In 7 cases (50%) malignancy proceeded paraneoplastic dermatosis, in 4 cases (28.57%) the malignancy was diagnosed at the same time as paraneoplastic dermatosis and in 3 cases (28.43%) malignancy was established after the onset of paraneoplastic dermatosis. Authors point to the fact that usual skin changes, characteristic for the dermatologic diseases mentioned, in cases when they are associated with visceral malignomas, are characterized by a more serious clinical picture, a longer course of the disease and resistance concerning usual therapy.

Aged↗

[Systemic diseases: evaluation of important laboratory parameters in 3 cases with unusual skin changes].

A whole spectrum of various clinical and laboratory disorders in patients with skin changes, may present systemic disease manifestations. Serological parameters correlate with the progression of the disease. Authors present cases with unusual skin lesions and abnormal laboratory findings, that were presumed to be manifestations of the systemic disease progression. The first case is a report of a patient age 44, female with dermatomyositis that started suddenly from full health with generalized linear bluish dark erythematous lesion like excoriations, periocular heliotrope violaceous to dusky erythematous rash with edema in a symmetrical distribution involving periorbital skin with no pruritus, diagnosed on admission as the case of acute urticarica. In the second report, a 17-year old female was referred to us because of a spread up linear sclerodermia followed by high immunological disturbances. Our third case was a 21-year old female with a systemic lupus erythematous--Rowell syndrome, with skin lesions of erythema multiforme type with some similarities to dermatitis herpetiformis on the first examination.

Adolescent↗

Toxic epidermal necrolysis.

Toxic epidermal necrolysis (TEN) is a rare but life-threatening disorder of children and adults in which the skin peels off in sheets and mucosal surfaces slough. It is best viewed as a severe from of erythema multiforme, with even more extreme involvement than one sees with the Stevens-Johnson syndrome (SJS). However, the distinction between TEN and SJS is not clear, as there are a number of overlapping definitions. TEN usually represents an idiosyncratic hypersensitivity to a medication, but may result from a variety of infections, or have no clear linkage. Sulfonamide antibiotics, barbiturates, carbamazepine, and allopurinol seem to be the most common offending agents. TEN usually has an acute onset one to three weeks after ingestion of the offending drug. TEN requires distinction from staphylococcal scalded skin syndrome, as treatment is different.

Adult↗

[Cefaclor therapy followed by serum sickness-like symptoms].

Cefaclor is an oral cefalosporin for a wide range of gram-positive and gram-negative infection. A retrospective study of cefaclor toxicoderma cases at the Department of Dermatology of Heim Pál Children's Hospital found 11 cases. After the administration of the drug, serum sickness like syndromes were observed. The characteristic symptoms were: erythema multiforme like urticaria, polyarticular swelling and oedema of joints and fever. Laboratory findings were: arised activity of the liver enzymes. After the acute symptoms urticaria factitia appeared.

Cefaclor↗

[Histopathological diagnosis by skin biopsy].

As post-transfusion graft-vs-host disease (GVHD) has poor prognosis, early diagnosis by skin biopsy is essential. The histopathology of the skin lesion includes a lymphocytic infiltration to the dermo-epidermal junction associated with vacuolization of the basal layer. Dyskeratotic or necrotic epidermal cells are scattered through the epidermis. Some of these necrotic cells are surrounded by a few lymphocytes (satellite cell necrosis). These histopathological findings are referred to as a lichenoid tissue reaction, but is not specific to GVHD, and can be seen in some types of drug eruption (toxic epidermal necrolysis type and erythema multiforme type). Final diagnosis will be confirmed by the clinical symptoms, such as liver dysfunction, diarrhea and pancytopenia.

Biopsy↗

Anticonvulsant hypersensitivity: an unfortunate case of triple exposure to phenytoin.

Anticonvulsant hypersensitivity manifests 2 to 12 weeks after patients start taking phenytoin, phenobarbital, or carbemazepine. The syndrome begins with fever followed by a rash that can progress to erythema multiforme, toxic epidermal necrolysis, and multi-organ system involvement. Other common manifestations include lymphadenopathy, facial edema, eosinophilia, and elevated transaminase levels. Anticonvulsant hypersensitivity can be fatal in 5% to 50% of patients if hepatic involvement or toxic epidermal necrolysis occurs. Most evidence regarding the etiology points to a genetically related defect in the detoxification enzyme. Early recognition is important in order to discontinue drug therapy. Continuity of care assists in prevention of re-exposure. Treatment includes supportive measures and steroids. All patients with anticonvulsant hypersensitivity should wear a medical identification bracelet.

Aged↗

Delayed hypersensitivity reaction to vancomycin.

The use of vancomycin has increased dramatically in recent years with the emergence of methicillin-resistant Staphylococcus aureus and coagulase-negative staphylococci as important hospital pathogens. A patient receiving vancomycin 1.75 g intravenously as a single daily dose developed a syndrome characterized by high fever, erythema multiforme, eosinophilia, and presumed interstitial nephritis. This delayed hypersensitivity reaction resolved with discontinuation of the drug and treatment with methylprednisolone.

Anti-Bacterial Agents↗