1/34. Mucha-Habermann disease-like eruptions due to tegafur. The first case of Mucha-Habermann disease-like drug eruptions due to tegafur is reported. A 59-year-old man noticed various skin lesions after he had taken 300 mg of tegafur daily for about 200 days. The patient had papulonecrotic eruptions on his trunk and extremities. The histology from a papular lesion revealed epidermal necrosis surrounded by spongiosis, perivascular inflammatory infiltrations composed of lymphocytes and erythrocytes, and endothelial swelling. The etiology of Mucha-Habermann disease is not known, but an immune mechanism may be supported by our case. ( info) |
| We report three children with clinical features of pityriasis lichenoides (scaly red to brown papules and macules) in whom there were histopathological findings of mycosis fungoides (disproportionate epidermotropism, Pautrier's microabscesses, and wiry and coarse collagen bundles). Immunohistochemical staining revealed a prevalence of T lymphocytes in the infiltrate. T-cell receptor gene rearrangement analysis in lesional skin demonstrated rearrangement of the gamma chain in all cases. Human T-cell lymphotropic virus type 1 serology was negative in the two patients in whom this test was performed. Thus, lesions resembling pityriasis lichenoides can be an unusual and potentially misleading presentation of mycosis fungoides. ( info) |
3/34. Medullary CD30 T cell lymphoma with eosinophilia and hyper-IgE supervening during the relentless course of pityriasis lichenoides. We report a case of extensive pityriasis lichenoides exhibiting a relentless course. puva therapy and oral retinoids cleared temporarily the lesions but did not really halt the course of the disease. eosinophilia and hyper-IgE occurred after 50 years of evolution. An aggressive medullary CD30 T cell lymphoma without skin involvement was then diagnosed when pityriasis lichenoides became more extensive and necrotic. The disease was rapidly fatal. ( info) |
4/34. Reactive hemophagocytic syndrome presenting as a component of multiple organ dysfunction syndrome. OBJECTIVE: To report two cases of severe reactive hemophagocytic syndrome (RHS), to discuss their impact, and to present evidence that RHS may be a constitutive part of multiple organ dysfunction syndrome (MODS). DESIGN: Case-report. SETTING: Pediatric intensive care unit (PICU). patients: Two patients with RHS and MODS. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Case #1: A 3 yr-old boy with Mucha-Haberman syndrome (pityriasis lichenoides) was admitted to the PICU for septic shock, acute respiratory distress syndrome, capillary leak, acute renal failure, liver dysfunction, and RHS (pancytopenia and hemophagocytosis on bone marrow aspirate). The pancytopenia was severe (white blood cell count, 0.9 x 10(9)/L; hemoglobin, 59 g/L; platelets, 36 x 10(9)/L), required many transfusions, and resolved 2 months later. The patient needed mechanical ventilation for 6 wks. length of stay in PICU was 2 months. Case #2: A previously healthy 4 yr-old girl was admitted to the PICU for respiratory failure. She developed acute respiratory distress syndrome, cardiomyopathy with complete atrioventricular block, shock, capillary leak, liver dysfunction, and RHS (pancytopenia and hemophagocytosis on bone marrow aspirate). The pancytopenia was severe (white blood cell count, 1.92 x 10(9)/L; hemoglobin, 65 g/L; platelets, 58 x 10(9)/L) and necessitated transfusional support. serology for respiratory syncytial virus was positive. RHS duration was 20 days; the patient recovered completely. Length of mechanical ventilation was 16 days and length of stay in PICU was 3 wks. CONCLUSIONS: These cases show that RHS may be a significant cause of pancytopenia in the PICU. It needs to be recognized as a clinical entity because it can be reversible and nonneoplastic. RHS and MODS share some pathophysiologic elements and could be related to each other. ( info) |
5/34. Detection of cytomegalovirus infection in a patient with febrile ulceronecrotic Mucha-Habermann's disease. BACKGROUND: Febrile ulceronecrotic Mucha-Habermann's disease (FUMHD) is a severe and very rare variant of pityriasis lichenoides et varilioformis acuta, which is characterized by large coalescing, and ulceronecrotic maculopapules or plaques. Morphological changes of the skin accompanied by persistent high fever and several constitutional symptoms have suggested virus infection in patients with FUMHD. However, the available information of viral origin is limited. In this study we investigated the relationship of cytomegalovirus (CMV), Epstein-Barr virus (EBV), human herpesvirus 8 (HHV8), type I human T-cell lymphotropic virus (HTLV-I), and parvovirus B19 (PVB19) with FUMHD in a Taiwanese patient. methods: The existence of CMV, EBV, HHV8, HTLV-I, and PVB19 was determined by polymerase chain reaction (PCR). The presence of CMV in the endothelial cells was characterized by in situ hybridization (ISH) and immunohistochemistry (IHC). RESULTS: Serologic immunoglobulin to CMV and IHC identification of CMV late gene in the biopsy specimen indicated that the patient was infected with CMV. Detection of CMV was confirmed by PCR and ISH. CONCLUSIONS: These results indicate that FUMHD is associated with dermal CMV manifestation. Nonetheless, the induction mechanism of FUMHD with CMV infection has yet to be determined. ( info) |
| pityriasis lichenoides et varioliformis acuta (PLEVA) is a disorder of unknown aetiology that occurs at any age and can be localized on the skin and on the mucosas. We report the case of a 24-year-old woman who presented PLEVA at her 20th week of gestation. This is, to our knowledge, the second case of PLEVA during pregnancy reported in literature. We report this case for the importance of the diagnosis of PLEVA during pregnancy because of the possible involvement of the mucosal membranes, and in particular of the vagina or cervical os of the uterus, where it may cause premature labour and/or premature rapture of the membranes. ( info) |
| pityriasis lichenoides (PL) is a cutaneous disease of unknown origin. In our 8-year-old female patient with the granulomatous form of common variable immunodeficiency (CVID), PL occurred together with massive splenomegaly and intra-abdominal lymphadenopathy. prednisone was efficient for treatment of her splenomegaly and autoimmune cytopenias. However, PL was resistant to both topical and systemic steroid treatment. Healing of PL was achieved with the use of a super-potent topical steroid, clobetasol propionate. A defect of T-cell function in CVID may contribute to development of PL. In the granulomatous form of CVID, sarcoid-like granulomas are the most commonly reported cutaneous lesions. PL has not been previously reported. ( info) |
| Two patients presented with acrally located papulosquamous lesions that were polymorphic and had an identical morphology to those of pityriasis lichenoides. The lesions remained localized to the feet and ankles for years. Multiple skin biopsies had the histopathological features of pityriasis lichenoides. In one biopsy syringotropic lymphocytes and early syringolymphoid hyperplasia were observed. This rare variant of pityriasis lichenoides may be under-recognized and misdiagnosed as it resembles a variety of papulosquamous disorders particularly psoriasis. ( info) |
9/34. Cytotoxic mycosis fungoides evolving from pityriasis lichenoides chronica in a seventeen-year-old girl. Report of a case. pityriasis lichenoides chronica and its acute form, pityriasis lichenoides et varioliformis acuta, are skin diseases of unknown origin, that probably represent a hypersensitivity reaction to an infective agent. pityriasis lichenoides is often a benign disorder but, because of the presence of a clonal T cell population detected both in the chronic and acute forms, some authors have suggested that it may belong to the group of primary cutaneous T cell lymphomas. Although various studies have clearly documented no significant association between pityriasis lichenoides and malignant lymphomas, cases of long-standing pityriasis lichenoides evolving into mycosis fungoides have been described. Herein we report the case of a girl suffering from pityriasis lichenoides since the age of 11 years, subsequently developing a CD45RO , CD8 , TIA-1 mycosis fungoides. ( info) |
10/34. A case of febrile ulceronecrotic Mucha-Habermann disease requiring debridement of necrotic skin and epidermal autograft. We report a case of febrile ulceronecrotic Mucha-Habermann disease (FUMHD) in a 21-year-old man. This disease is a severe form of pityriasis lichenoides et varioliformis acuta (PLEVA) and is characterized by the sudden onset of diffuse ulcerations associated with high fever and systemic symptoms. It is sometimes lethal especially in elderly patients. In the present case, intense generalized maculopapular erythematous plaques with central necrosis developed progressively in association with a high fever. Initial treatment with systemic betamethasone had been unsuccessful and the skin lesions, which covered about 50% of the body surface, became severely ulcerated. Although the development of new lesions had ceased spontaneously, widespread ulceration of the skin remained. debridement of the necrotic skin and skin grafting using cultured epidermal autografts and meshed allografts of cadaver skin led to prompt reepithelization. ( info) |