Search

Objectives: We report four cases of eczema induced by alpha interferon in atopic patients treated for chronic hepatitis C., Case Reports: Eczema developed in 4 patients with certain (3 cases) or possible (1 case) atopy treated by subcutaneous injections of alpha interferon for hepatitis C virus infections. Delay to onset was 3 weeks to 6 months. Interferon was highly likely the causal agent: lesions started at site of interferon injection, followed the rhythm of interferon injections (three cases), disappeared at interferon withdrawal. In two patients, the lesions diffused to other sites. Both Introna and Roféron were used. Three patients also took ribavirine. The possible role of a contact factor (antiseptic.) was ruled out. Skin tests (patch tests, prick tests, intradermal reactions) were negative for interferon alpha and for a standard battery., Discussion: The role of interferon in the induction of skin diseases or its influence on the course of certain dermatoses is well known. In atopic patients, interferon might induce eczema via an immunomodulator rather than an allergic mechanism since skin tests (performed in one patient) were negative. This observation is similar to that in psoriasis induced by interferon in predisposed subjects who develop skin lesions at injection sites which sometimes diffuse to distant localizations. The role of other factors (hepatitis C virus infection, ribavirine) remains unknown; they might participate in this mechanism by aggravating skin dryness.

Background: Langerhans cell histiocytosis is an uncommon clonal disorder. Its reactional or genetic nature is debated., Case Report: Three patients aged 71, 75 and 73 years with Langerhans cell histiocytosis also developed myelomoncytic leukemia (AML4, AML5, and chronic myelomonocytic leukemia). In two cases, there was no prior treatment which could potentially induce leukemia. In the third case, AML4 occurred shortly (10 months) after initiation of a vinblastin treatment., Discussion: This pathogenic association suggests a common origin for these two conditions, corresponding to an anomalous pluripotent stem cell line. These cases provide further evidence favoring the hypothesis of a genetic origin rather than a reactional origin for Langerhans cell histiocytosis.

Background: Trichodiscoma is an uncommon benign tumor of the pilosebaceous system., Case Report: An 83-year-old man had a past history of adenomatous polyps of the colon. The subject's brother had died from cancer of the colon. Over the past 4 years, the patient had developed approximately 30 small asymptomatic papulonodular tumors on the trunk. Skin biopsy demonstrated trichodiscomas., Discussion: Together with fibrofolliculomas and perifollicular fibromas, trichodiscomas are benign tumors of the pilosebaceous system. These different tumors may occur simultaneously as in Birt-Hogg-Dubé syndrome which associates trichodiscoma and familial colonic polyposis. This combination has only been reported in Birt-Hogg-Dubé syndrome or with perifollicular fibromas., Conclusion: This case suggests that patients with hamartomas of the pilosebaceous system should undergo explorations in search for digestive tract polyposis.

Coexistence of a systemic vasculitis and an epithelioma is rare. The prevalence of malignancies in patients with vasculitis has been estimated at about 5%, with about two thirds hematological malignancies and one third solid tumors, although the actual figure may be higher. We report three cases of vasculitis in patients with solid tumors. A review of the literature yielded 77 additional cases. A total of 56 cases were fully documented. The sex ratio was 1.26, and mean age was 62.5 years. The most common vasculitides were leukocytoclastic vasculitis (33%), polyarteritis nodosa (16.5%) and Henoch-Schönlein purpura. The most common sites of cancer formation were the lung (23%), the digestive system (17.5%) and the kidney (14%). The diagnosis of vasculitis antedated that of cancer in most patients. Radical cancer treatment was often followed by resolution of the vasculitis and vasculitis recurrences occurred concomitantly with tumor recurrences in some patients, suggesting that the vasculitis was a paraneoplastic syndrome. Patients with unexplained chronic vasculitis with or without a history of cancer should undergo extensive investigations for an occult neoplasm.

We report the first case of cutaneous and articular mucinosis with presence of mucin deposits within the synovium in an HIV-positive patients. Presentation was asymmetric polyarthritis, and subcutaneous nodules developed two months later. Treatment was with hydroxychloroquine. The outcome of both the skin and joint lesions was favourable.

Introduction: We report five cases of pigmented erythroderma occurring during AIDS, noteworthy for its unusual hyperpigmented feature, its advent at the terminal stages of AIDS, and an CD8 cells dermal infiltrate., Patients and Methods: It is a retrospective study of five patients infected with HIV: a woman infected by transfusion and four homosexual men, average 55 years old. No one was intravenous drug user. They were all severely immunocompromised; HTLV I/II serology was negative. Skin biopsies were studied with light microscopy (Hematoxylin-eosin) and immunohistochemical studies were performed on frozen sections., Results: The patients had an erythroderma of particular interest because of the associated hyperpigmentation, the severe repercussion (pruritus, weight loss), and the difficulty in treating (except systemic corticosteroids). The histology demonstrated a mononuclear dermal lymphocytic infiltrate, without epidermotropism and atypical cytonuclear feature. The phenotype of the infiltrate was uniformly of the suppressor-cytotoxic subset (CD8+, CD4-)., Comments: Our cases are like those previously described as "Pseudo-Sezary", mimicking a lymphoma during AIDS. Numerous factors are probably the cause of this hyperpigmented erythroderma: HIV, CD8 cells..., Conclusions: This severe skin disease, complicating AIDS, seems very particular, but not yet clearly defined. In practice, the problem remains the treatment of this severe erythroderma, because only the systemic corticosteroids are effective, but this is debatable during the treatment of AIDS.

Introduction: Paraneoplastic pemphigus is an autoimmune bullous disease described by precise clinical, histological and immunological features presented by Anhalt in 1990. Prognosis is very severe and depends on the associated neoplasia and the gravity of the mucosal damage., Case Report: Paraneoplastic pemphigus was diagnosed in a 62-year-old man with chronic lymphoid leukemia. The course was favorable up to one-year follow-up after general corticosteroid therapy., Discussion: This case illustrates that paraneoplastic pemphigus can be controlled by general corticosteroids and would suggest the severe prognosis may be improved.

Disseminated infection by Fusarium is being increasingly reported in immunocompromised patients. We report the first case of Fusarium oxysporum disseminated infection with well-documented fungal myocarditis. Despite antifungal therapy and hematologic recovery, the patient died in cardiogenic shock, myocardial involvement clearly contributing to the fatal outcome.

Introduction: A case of a cutaneous metastasis as a first sign of a linitis plastica is reported., Case Report: A 57 year-old man presented for a cervical infiltrated skin plaque. Histological examination and immunohistochemical staining gave the diagnosis of metastasis probably of gastrointestinal origin. Gastric endoscopy and biopsy confirmed the diagnosis of a linitis plastica., Discussion: Cutaneous metastases from gastric carcinoma are uncommon and exceptionally the first sign of the disease. Their clinical and histological aspects are reviewed.

Objectives: The aim of this retrospective study of 136 patients was to specify the natural history and the systemic prognosis of chronic cutaneous lupus erythematosus. It has been stated that in most of the cases, the disease only affects the skin., Methods: From 10 October 1980 to 31 December 1990, 136 patients with the following criteria were included in this retrospective study: clinical signs suggestive of chronic cutaneous lupus erythematosus, characteristic histology, insufficient evidence for the diagnosis of systemic lupus erythematosus., Results: The prevalence of systemic clinical involvement in the population under study was nearly the same as in the general population. The following biologic or immunologic abnormalities were quite common: leukopenia, lymphopenia, thrombopenia (significantly more frequent among patients having widespread chronic cutaneous lupus erythematosus), low titers of complement levels, positive antinuclear antibodies (usually at a low titer). Eleven out of the 136 patients developed systemic lupus erythematosus, most often over 5 years after the onset of the cutaneous lesions. Four cases out of these 11 had poor prognosis: renal and/or neurologic involvement., Conclusion: This data suggests that patients with chronic cutaneous lupus erythematosus could benefit from long-term follow up, since the course to systemic disease occurs in only a few. Usually, antimalarials used singly or in combination with topical steroids may lead to the clearing of the lesions. Thalidomide will occasionally be useful whenever the disease is unresponsive to the preceding measures.

Sixteen cases of acquired mid-dermal elastolysis have been reported in the literature. In 1977, Shelley identified this type of elastolysis as a separate entity and described three variants: post-inflammatory elastolysis with cutis laxa predominates in facial areas in African children. The initial inflammatory lesions are replaced by rough wrinkling of the skin; post-inflammatory elastolysis without cutis laxa; non-inflammatory elastolysis without cutis laxa as mid-dermal elastolysis. It is difficult nevertheless to classify certain cases reported in the literature. This would suggest that there is in reality a continuum of acquired elastolysis which could be classed according to a scale of decreasing initial inflammation. The non-inflammatory pole would represent mid-dermal elastolysis. The common features of the different types of elastolysis are dominated by the absence of a causative agent in most cases, unknown pathogenesis and the fact that the empirical treatments which have been used have been ineffective.

Epithelioid hemangioendothelioma is a rare, low-grade vascular malignancy reported for the first time in 1982 by Weiss and Enzinger. It involves one or, more rarely, several organs. We report a case involving the lungs and liver, in which the first manifestation was symptomatic hypertrophic osteoarthropathy. Findings four years after the diagnosis included very slow tumor spread, resolution of symptoms, and stabilization of radiological changes.

Sarcoidosis is rare in children, who usually have only asymptomatic pulmonary involvement. We report granulomatous cheilitis in two children with sarcoidosis. Both children had systemic sarcoidosis with lymph node and pulmonary involvement.

Lupus mastitis is an infrequent dermatologic disease. We describe a case of bilateral lupus mastitis heralding a severe systemic lupus erythematosus with the antiphospholipid syndrome.

Smooth muscle hamartoma is an uncommon cutaneous dysembryoplasia usually diagnosed in infancy. Among the 61 cases published since 1923, 56 were congenital and 3 appeared in young adults. We report a case in which the lesions started at the age of 15 years as a papular plaque in the right mammary region of a young woman. A review of the literature showed that the usual clinical presentation is a frequently pigmented plaque made of often follicular papules and measuring 1 to 10 centimeters on average. Excessive hairiness is the most frequent sign, being observed in more than two-thirds of the cases, and Darier's pseudo-sign is present in about 53 p. 100 of the patients. The disease is electively located on the lumbar region, the back and the root of the limbs. In 3 cases the lesions were generalized and the patients looked like fatty "Michelin-Tire Babies". The course of the disease is always favourable, and associated pathologies remain exceptional: urticaria pigmentosa and psychomotor retardation have been reported in two cases of the generalized form. Histology is characterized by the presence of numerous smooth muscle fibres disseminated in the dermis and diversely oriented, sometimes in contact with hair follicles which retain their normal morphology. The differential clinical diagnosis is with naevocytic naevus, café-au-lait spots, mastocytosis and connective tissue hamartoma. Belatedly revealed forms of the disease must be distinguished from Becker's hamartoma, but it must be known that in certain cases the classification is so difficult that some authors have suggested that smooth muscle hamartoma and Becker's hamartoma are only two poles of a single spectrum of dysembryoplastic lesions involving to varying degrees the epidermic and hair structures. Finally, the distinction between the localized forms of late onset smooth muscle hamartoma and multiple leiomyomas "en plaques" remains difficult both anatomico-clinically and nosologically.

We report a case of ciliated cutaneous cyst located on the dorsal side of the right shoulder in a 22-years old woman. The anatomoclinical features of this rare variety of cyst are described and compared with those of the 17 published cases. The differential diagnosis with other glandular cysts is presented. The mullerian duct origin of ciliated cutaneous cysts is controverted, and the starting point of this lesion remains mysterious.

Traditionally, the prognosis of pemphigus erythematosus is thought to be more favourable than that of pemphigus vulgaris. A retrospective study of the records of 10 patients with pemphigus erythematosus and 13 patients with pemphigus vulgaris was set up to compare the courses of the two diseases. This comparison, carried out in populations with similar age, sex ratio, pretreatment duration of the disease and treatment received, showed that relapses were more frequent in the course of pemphigus erythematosus, whereas remissions, mean duration of the disease and iatrogenic complications were the same in both groups. This study, therefore, throws some doubts on the dogma of relative mildness of pemphigus erythematosus, which goes back to a period long before systemic corticosteroid therapy was known. Mortality studies performed since the event of this treatment have shown that the prognosis had improved and tended to be the same in both diseases. The other data concerning the course of treated pemphigus erythematosus are little known and were never compared with those concerning pemphigus vulgaris. Our study shows that treatments similar to those of pemphigus vulgaris are necessary to obtain remissions in pemphigus erythematosus and that these diseases share the same evolutive profile. However, the question of the best therapeutic strategy to be used has not yet been answered.

Bilateral symmetrical polyarthritis occurred in three patients (2 males and 1 female), with no previous history of inflammatory rheumatologic disease, given alpha-interferon for 1 1/2, 7, and 10 months as treatment of chronic non A-non B hepatitis, myelofibrosis, and thrombocytopenia with myeloproliferative disorder, respectively. Joint manifestations developed 1 1/2, 3, and 10 months after initiation of alpha-interferon in a dosage of 3.10(6) U three times a week, 4.5.10(6) U per day, and 8.10(6) U three times a week. Polyarthritis persisted following withdrawal of alpha-interferon in the two last patients of whom one had rheumatoid nodules and positive rheumatoid serology and the other had scleritis, exanthema, and negative rheumatoid serology. Erosive rheumatoid arthritis was diagnosed after 28 months and 12 months, respectively, in two patients who required systemic corticosteroids with antimalarials (1 case) or azathioprine after failure of methotrexate (one case). Follow-up in the third case (12 months) is too short to allow differentiation of systemic lupus erythematosus (ANA: 1/1500 H with anti-DNA antibodies 58 U/ml) and chronic autoimmune hepatitis. Reports of chronic inflammatory rheumatologic disease during alpha interferon therapy are exceedingly few in number. In the cases reported herein, alpha-interferon may have either triggered or revealed the joint disease. To prevent occurrence of this complication, exclusion from alpha-interferon therapy of patients with autoantibodies or a positive history for clinical evidence of immune dysfunction may be considered.

The occurrence of elastic tissue abnormalities in the digestive tract is not common. There are sometimes observed in genetically transmitted connective tissue diseases in which numerous systemic complications are associated as in Ehlers-Danlos and in Grönblad-Strandberg syndromes. We report 7 cases of an unusual lesion localized in the colon in which abnormally increased amounts of elastic tissue were observed. This elastosis raises many nosological and diagnostic difficulties. We propose the term "colonic elastoma" to describe this lesion. The review of the literature allows to consider several etiopathogenetic possibilities.

The authors report the case of a 26 year old man, in whom recurrent hematuria revealed the diagnosis of urethral condylomata with bladder extension, and HIV serology positive. This case reminds us that it is essential to search for immunodeficiency in every case of extensive or recurrent condylomata. The analysis of such cases in the literature allows discussion of the various clinical courses according to the Human Papilloma Virus types found in condylomatous tissues.

Florid necrotizing lymphadenitis, characterized by segmental infarction and lymphoid hyperplasia, is an uncommon feature of systemic lupus erythematosus (SLE). Kikuchi's disease is a well-defined clinicopathological entity, with a strong preference for the cervical lymph nodes of young women. The etiology of histiocytic necrotizing lymphadenitis (HNL) remains unknown, although viral agents have been proposed. HNL may reflect a self-limited SLE-like autoimmune disease but full-blown SLE associated with this condition has not, to the best of our knowledge, been reported. Thus, ours is the first description of the coexistence of SLE and HNL in 3 patients with immunologically proven parvovirus B19 infections. SLE and HNL were diagnosed simultaneously in 2 patients, but was retrospective in the third, in whom anti-tuberculous therapy was ineffective. Patients 1 and 2 were treated with prednisone (1 mg/kg/d) and responded rapidly. These data suggest that both HNL and SLE flares can be caused by parvovirus B19 infection.

One case of Muir-Torre Syndrome (MTS) is reported. It is characterized by an unusual association of an unique cutaneous sebaceous epithelioma and multiple visceral adenocarcinomas. The patient was a 67 year-old white woman, from a family with inherited cancer syndrome, who developed previously four colic and one endometrial carcinomas. The morphologic spectrum of the cutaneous tumors observed in the MTS is represented by kerato-acanthoma and/or sebaceous tumors as in the original case described by Torre. The cutaneous tumors are usually multiple and follow after numerous visceral cancers such as colic, endometrial, urinary, or gastric carcinomas. The visceral cancers are remarkable because of the familial character, the multiplicity of tumors and the low-grade malignancy. Our case highlights the difficulty to make an accurate diagnosis for rare histological variants of cutaneous tumors in the MTS.