First case of cutaneous reconstitution inflammatory syndrome associated with HIV infection and leishmaniasis.
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Amphotericin B↗
Biomedical subjects
M D Vignon-Pennamen
Publications and source records attributed to M D Vignon-Pennamen.
Idiopathic systemic capillary leak syndrome: cutaneous involvement can be misleading.
BACKGROUND: Systemic capillary leak syndrome (SCLS) is a severe disorder characterized by unexplained rapid transfer of considerable volumes of plasma from the intravascular to the extravascular compartment. For some cases of SCLS, no aetiology is evident and these cases are reported as idiopathic (ISCLS). OBJECTIVES: To describe the cutaneous findings in 3 patients with ISCLS. RESULTS: Cutaneous involvement consisted in sclerosis, livedo, purpura and photodistributed maculopapular erythematous rash. Dermal mucinosis was proven by biopsy in 1 patient. No underlying disease was diagnosed during follow-up. CONCLUSION: The above-mentioned cutaneous findings can be present during acute attacks of ISCLS. They seem specifically related to the ISCLS and not indicative of an underlying disease.
Aged↗
Bullous and non-bullous ichthyosiform erythroderma associated with generalized pustular psoriasis of von Zumbusch type.
Bullous ichthyosiform erythroderma (BIE) and non-bullous ichthyosiform erythroderma (NBIE) are rare congenital ichthyoses. Generalized pustular psoriasis (GPP) of von Zumbusch type is a rare and severe form of psoriasis marked by desquamative and pustular erythroderma associated with fever and altered general conditions. We report two adults with an ichthyosis typical of BIE in the first case and NBIE in the second, without any previous history of psoriasis, who presented with a severe and relapsing GPP of von Zumbusch type. Using current knowledge of the genetic relationship between psoriasis and congenital ichthyoses, we discuss the possibility of a common physiopathological link between congenital ichthyoses and GPP, and examine the possible therapeutic problems resulting from this pathological association, especially in BIE.
Adult↗
Reactive angioendotheliomatosis secondary to dermal amyloid angiopathy.
Reactive angioendotheliomatosis (RAE) is a rare benign cutaneous vascular proliferation characterized by intravascular hyperplasia of endothelial cells and tuft-like proliferation of vessels. A 75-year-old man had erythematous and violaceous macules, some stellate and others arranged in a livedoid pattern, evolving toward necrosis with central areas having an "atrophie blanche" appearance spread on the trunk, inguinal folds, and right thigh. He was on hemodialysis and had a benign monoclonal gammopathy. Cutaneous biopsy revealed RAE characterized by the proliferation of epithelioid and spindle-shaped cells in superficial and middermis lining vascular channels, arranged in clusters, and sometimes displaying an intravascular growth pattern. These cells stained for CD31, CD34, and actin. Interestingly, prominent amyloid deposits were found in the wall of some vessels in deep dermis, often causing obstruction of their lumina. The cause of RAE is unknown, but it can be associated with infections, antiphospholipid syndrome, dysglobulinemia, cryoproteinemia, and lower extremities arteritis, and it may occur near arteriovenous fistulas. In this patient, we believe that RAE was caused by obliteration of dermal vessels by amyloid deposits. Indeed, it is thought that RAE could be caused by ischemia secondary to vascular obstruction. This is the first reported patient with RAE associated with amyloid deposits.
Aged↗
Successful treatment in two cases of steroid-dependent cutaneous polyarteritis nodosa with low-dose methotrexate.
To the best of our knowledge, only 3 cases of cutaneous polyarteritis nodosa (PAN) treated successfully with methotrexate (MTX) have been reported in the medical literature. We report 2 further cases of steroid-dependent cutaneous PAN treated successfully with low-dose weekly MTX therapy. The clinical and biological tolerance of MTX was excellent. The cutaneous lesions started to regress within 3 weeks. One of the patients reported full recovery which lasted 2 years after stopping the therapy. So, MTX seems to be an interesting therapy in the treatment of PAN because of its relatively low toxicity, its simple use, its quick action and prolonged results after MTX has been stopped.
Adrenal Cortex Hormones↗
No evidence for a role of human herpesvirus type 8 in sarcoidosis: molecular and serological analysis.
The aim of this study was to analyse the association between human herpesvirus type 8 (HHV8) and sarcoidosis. Using nested polymerase chain reaction (PCR), we tested the presence of HHV8 DNA sequences in 13 skin specimens and peripheral blood mononuclear cells from eight patients suffering from sarcoidosis. We also looked for the presence of HHV8 antibodies in the sera of 28 patients with sarcoidosis using three techniques: two indirect immunofluorescence assays and an enzyme-linked immunosorbent assay with recombinant capsid protein fragment encoded by open-reading frame 65. HHV8 PCR analysis was negative while HHV8 serological studies showed an overall prevalence of 18% among patients suffering from sarcoidosis: 43% in patients from sub-Saharan Africa, 17% in patients from Northern Africa, 12.5% in patients from the French West Indies and 0% in French patients. In conclusion, our results do not indicate an association between HHV8 and sarcoidosis but reflect the seroepidemiology of this virus in different geographical regions.
Adult↗
[Eosinophilic pustulosis in an infant accompanied by immune deficit].
BACKGROUND: Eosinophilic is a skin eruption which occurs in the first years of life, progressing by pruriginous flare-ups with amicrobial papulopustulae on a hairless scalp. Eosinophil infiltration of the skin is variable (follicular or perifollicular dermal infiltration). In adults, eosinophilic pustulosis is often associated with immune deficiency, but this association has not been reported in children. We report two cases. CASE REPORTS: Two boys had a pruriginous papulopustular eruption involving the scalp and the trunk which had progressed with periods of exacerbation since birth. Search for bacteriological or mycological involvement was negative. Histology showed folliculitis with major polynuclear eosinophil infiltration. Both children had a past history of repeated skin and extracutaneous infections strongly suggesting an immune deficit. Buckly syndrome was suspected in the second case. DISCUSSION: Juvenile eosinophilic pustulosis belongs to the spectrum of childhood eosinophilic dermatoses. The presence of eosinophil infiltration in the skin demonstrates localized or systemic immune dysfunction. A hematology and immunology work-up is needed in case of associated skin or deep infections.
Adult↗
[Sporadic superficial pemphigus in the child: 2 cases].
INTRODUCTION: Excepting the endemic foliaceus form, childhood pemphigus is uncommon. We report two cases of pemphigus foliaceus in children with typical clinical manifestations. CASE REPORTS: Case n(o) 1. A 5-year-old girl was seen for a vesiculobullous crusted dermatosis involving the trunk and the face which had developed over the last 5 months, predominantly in periorificial and fold localizations. Histology showed intragranulous acatholysis. Direct skin immunofluorescence was positive for anti-intercellular substance IgG and C3. Indirect immunofluorescence was positive for anti-intercellular substance antibodies at 1/500. The diagnosis of superficial pemphigus was retained and the child was given dapsone associated with systemic prednisone (1.5 then 2.5 mg/kg/d). Dapsone was stopped on day 15 due to poor hematological tolerance. Outcome was favorable allowing withdrawal of prednisone at 18 months. Case n(o) 2. A 6-year-old had developed since the age of 18 months a generalized and polycyclic pruriginous erythemato-squamous dermatosis with oozing discharge which started and predominated on the face (periorificial zones). Trace element (copper, selenium, zinc) and vitamin (A, E and B1) assays were within the normal range. Glucagon was normal. Histological examinations of several biopsies were non-contributive. Diagnosis of pemphigus foliaceus was finally obtained after repeated direct immunofluorescence tests which revealed anti-intercellular substance IgG. Indirect immunofluorescence was negative. The child was given prednisone (2 mg/kg/d). DISCUSSION: In children, pemphigus foliaceus has an exceptional frequency and diagnosis is often made quite late (mean 8 months). The diagnosis should always be entertained in children who develop chronic extensive erythemato-squamous and crusted dermatosis, even if formation is absent. Direct skin immunofluorescence confirms the diagnosis and should be repeated if negative in cases with highly suggestive clinical presentations. It would be reasonable to attempt "minor" treatments as the first line approach. Systemic corticosteroids are however the treatment of choice despite the risk of classical side effects. Childhood pemphigus foliaceus is not an attenuated clinical form of adult pemphigus. Mortality is not negligible and is close to that in adults.
Adult↗
[Neonatal monoblastic leukemia revealed by transitory specific skin lesions].
BACKGROUND: Specific cutaneous involvement is frequently observed in congenital leukemia and may be the inaugural sign. Lesions may be non-specific and even regress spontaneously, misleading diagnosis and delaying care. CASE REPORT: A infant in good health had diffuse ecchymotic maculae at birth which totally regressed within 10 days. On day 20, a macular rash and three violet nodules developed. The physical examination found enlarged nodes, liver enlargement and poor general status. Blood cell counts and the myelogram led to the diagnosis of type 5 acute myeloblastic leukemia (AML 5). Search for extension revealed cutaneous leukemia and meningeal and renal involvement. DISCUSSION: The predominance of myelocyte forms is specific for neonatal leukemia (AML 5 and 4). Specific skin lesions are frequent (25 to 30 p. 100 of cases) and sometimes precede (7 p. 100) anomalies in peripheral and medullary smears. Skin biopsy with direct smear can provide rapid diagnosis. Classically subcutaneous nodules or bluish macropapulae give the blueberry muffin baby aspect. The polymorphous features and the remarkably fluctuating skin lesions in our case are unusual, especially since the infant did not have spontaneously reversible neonatal leukemia, a rare unpredictable phenomenon described in newborns with a normal phenotype which can mislead therapeutic management.
Age Factors↗
[Eruptive epithelioid hemangioendothelioma with spindle cells. Nosological place in the spectrum of epithelioid vascular tumors].
The epithelioid vascular tumors include epithelioid hemangioma, epithelioid hemangioendothelioma and epithelioid angiosarcoma. We report the case of a difficult to define vascular epithelioid tumor. The tumor localized in the head developed in an eruptive way in multiple bone and skin locations in a 22-year-old man. The lesions had the same histological features as those of epithelioid hemangioma and epithelioid hemangioendothelioma with a spindle-cell component. This tumor was called eruptive epithelioid hemangioendothelioma with spindle cells. In our case, its development with recurrences and destructive features has some similarities to tumors of intermediate malignancy.
Adult↗
Lichen planus pemphigoides is a heterogeneous disease: a report of five cases studied by immunoelectron microscopy.
Lichen planus pemphigoides (LPP) is a rare and controversial disease. It is characterized by bullae arising on lichen planus papules and on uninvolved skin, subepidermal bullae in histology, and linear deposits of IgG and C3 along the basal membrane zone on immunofluorescence of peribullous skin. Our goal was to identify the localization of the target antigen in cases of LPP. Five patients diagnosed with LPP on clinical, histological and immunofluorescence criteria were explored by immunoelectron microscopy and immunoblot. Our results show that the target antigen in LPP is not unique. The localization of the immune deposits was consistent with a diagnosis of bullous pemphigoid in two cases, of cicatricial pemphigoid in two cases and of epidermolysis bullosa acquisita in one case. Our study supports the view that LPP is a heterogeneous condition in which lichen planus may induce different subepidermal acquired bullous dermatoses.
Adolescent↗
[A tumor can hide another one].
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Antigens, CD34↗
[Palisading granuloma].
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Diagnosis, Differential↗
[Lichen planus in children: 12 cases].
OBJECTIVE: Lichen planus is in children uncommon and poorly understood. The classical description is comparable to lichen planus in adults. We conducted a retrospective analysis of 12 cases in children. PATIENTS AND METHODS: Twelve children with lichen planus consulted the Saint-Louis or Robert-Debré hospitals between February 1994 and March 1996. Data collected included: age, sex, ethnic origin, drug use, anti-hepatitis vaccination status, disease history, physical examination, skin histology, liver tests, hepatitis B and C serology, treatment and outcome. Histological proof was obtained in all cases but one (a child with isolated ungueal involvement whose sister had histologically proven ungueal lichen planus). RESULTS AND DISCUSSION: The clinical features classically described in adults were atypical in all our childhood cases. A rapidly extensive eruption was the main sign in 6 cases. The localizations were unusual with lesions involving all four limbs and the trunk as well as the face in 5 cases and the scalp in 1. Mucosal involvement, observed in 65 p. 100 of adult cases was only found in one of our children. Unguel involvement also appears to be uncommon in children. The etiological pattern was also unusual since we did not observe a single case related to drugs or hepatitis B or C infection. Three children developed a lichen eruption after anti-hepatitis B infection. Four other cases of lichen planus after anti-hepatitis B vaccination have been reported in the literature. Mean delay between the booster vaccination and onset of eruption is reported to be 40 days. The increased incidence of childhood lichen planus in tropical zones suggests ethnic, genetic and climatic factors may be involved. Prognosis is poorly defined in the literature. Certain authors emphasize the long duration of the disease and resistance to treatment in cases of childhood lichen planus. Currently, there is no consensus on treatment. Dermocorticoids in combination with antihistaminics are usually prescribed. General corticosteroid therapy would appear to be warranted in extensive progressive forms with important functional and esthetic impact (scalp involvement with cicatricial alopecia, pigmentation sequellae). The role of other drugs, particularly retinoids, remains to be defined. This retrospective series was not statistically significant. Data in the literature are rather discordant, emphasizing the need for a prospective analysis to acquire a better understanding of the real incidence of childhood lichen planus and better define the therapeutic strategy.
Adolescent↗
[Adult T-cell lymphoma associated with HTLV-1: a familial form].
BACKGROUND: Adult T-cell leukemia-lymphoma (ATL) can occur in siblings infected with HTLV-1. CASE REPORTS: Two Caribbean siblings developed ATL a few years apart. One case has been reported previously. Both individuals had peripheral lymph node T-cell lymphoma and a few atypical lymphocytes on blood smear. Lymphocytosis, bone marrow biopsy, abdominal computed tomographic scanning, and chest radiography were normal. Clonal rearrangement of T-cell receptor was present in skin lesions for both patients and in the blood for one. HTLV-1 serology was positive. Clonal integration of HTLV-1 provirus was demonstrated in skin lesions in one patient and in blood lymphocytes in the other. Chemotherapy, then interferon alpha, were unsuccessful in the first patient. Topical metchloretamine was partially effective for the second patient. DISCUSSION: ATL in siblings is explained by mother-to-child transmission of HTLV-1 infection during breastfeeding.
Adult↗
[Cutaneous and subcutaneous sarcomas].
Cutaneous sarcomas are uncommon tumors presenting many histological types. The diagnosis is based on pathological, immunohistochemical and sometimes ultrastructural studies. The development of cytogenetic and molecular analysis may constitute an additional aid to the diagnosis and classification. Prognosis and therapeutic strategies are established on the basis of various criteria using different types of staging and grading, but these classifications have not yet been standardized. The rarity of cutaneous sarcoma, and the diversity of clinical presentations account for the difficulties of management, which requires a multidisciplinary approach.
Adolescent↗
Schönlein-Henoch purpura in adult patients. Predictive factors for IgA glomerulonephritis in a retrospective study of 57 cases.
OBJECTIVES: To evaluate the incidence of extracutaneous manifestations and to identify predictive factors for renal involvement in adult patients with Schönlein-Henoch purpura. DESIGN: Retrospective study with a comparative analysis of patients with and without renal involvement. SETTING: Patients who were attending the dermatologic department of an academic medical center. PATIENTS: In patients with purpura of the lower limbs and cutaneous vascular IgA deposits for which cases were recorded from 1985 to 1993, the following selection criteria were used: age older than 15 years and absence of thrombocytopenia, of IgA deposits in the basement membrane zone, and of a known hematologic or connective tissue disorder. MAIN OUTCOME MEASURES: Clinical and biological data, results of histological studies, and findings from direct immunofluorescence studies of skin biopsy specimens were compared in patients with and without renal involvement. RESULTS: Fifty-seven patients were included: 23% had an IgA glomerulonephritis confirmed by results of a renal biopsy, and a further 26% showed abnormalities on urine microscopy. Joint and gastrointestinal involvement was noted in, respectively, 33% and 19% of the patients. A comparative analysis of patients with and without renal involvement failed to reveal significant differences with regard to age, sex, the presence of bullous or necrotic cutaneous lesions, gastrointestinal or joint involvement, histological features, and findings from direct immunofluorescence studies. An IgA glomerulonephritis was significantly associated with purpura above the waist (P = .03), a recent infectious history (P = .02), pyrexia (P = .01), and biological markers of inflammation (P = .006). CONCLUSIONS: Despite a lower incidence of systemic involvement compared with that in other published series, the incidence of renal involvement remained high (ie, between 23% and 49%). A recent infectious history, pyrexia, the spread of purpura to the trunk, and biological markers of inflammation were predictive factors for renal involvement.
Adult↗
Atypical Lyme borreliosis in an HIV-infected man.
We report the fourth case of Lyme borreliosis in a man infected with human immunodeficiency virus (HIV). The erythema chronicum migrans was persistent, overlapping with meningoradiculitis. Repeated immunofluorescence tests for Borrelia burgdorferi sensu lato remained negative in both sera and cerebrospinal fluid (CSF), the enzyme-linked immunosorbent assay was weakly positive in serum and CSF and a Western blot was positive. The skin infiltrate was composed mostly of T lymphocytes with a CD4/CD8 ratio of 0.5. The course of the disease was favourable after treatment with intravenous ceftriaxone. Further studies are necessary to evaluate whether HIV infection influences, as does syphilis, the course and response to treatment of Lyme borreliosis. Serological tests are insufficiently sensitive and the Western blot assay is necessary to confirm Lyme disease in HIV-positive patients.
Blotting, Western↗