1/8. Late recurrence of inflammatory first-stage lesions in incontinentia pigmenti: an unusual phenomenon and a fascinating pathologic mechanism.BACKGROUND: incontinentia pigmenti (IP) is an X-linked genodermatosis that is manifested by neonatal inflammatory vesicles localized along the lines of Blaschko. These lesions usually clear spontaneously within a few months, leaving hyperpigmentation. Ophthalmologic and neurologic symptoms can be associated with IP. Late recurrences of the first-stage inflammatory lesions after the initial rash are uncommon and have been reported infrequently. The mechanism involved in this phenomenon is unclear. However, the recent identification of NEMO/IKKgamma as the gene responsible for IP sheds new light on its pathophysiologic origins. OBSERVATIONS: We report 5 cases of children who experienced episodes of late reactivation of IP. In all cases, the recurrences occurred on the previously hyperpigmented streaks several months or years after resolution of the initial eruptions. In most cases, the recurrences were preceded by an infectious episode. CONCLUSIONS: These IP recurrences suggest that mutated cells can persist a long time in the epidermis. We theorize that infections trigger the reactivations. The NEMO/IKKgamma gene encodes a protein essential in nuclear factor kappaB activation, which is required for resistance to tumor necrosis factor alpha-induced apoptosis. We discuss the role of a proinflammatory cytokine such as tumor necrosis factor alpha as a triggering factor for the reactivation.- - - - - - - - - - ranking = 1keywords = dermatosis (Clic here for more details about this article) |
2/8. Neutrophilic eccrine hidradenitis in two neutropenic patients.Neutrophilic eccrine hidradenitis is an uncommon, self-limited dermatosis with a variable clinical presentation. It seems to be due to chemotherapeutic drugs in most cases. necrosis of the eccrine gland associated with a neutrophilic infiltrate is the histologic hallmark of this disease. We report two additional cases in neutropenic patients with acute myelogenous leukemia in which there was a striking lack of neutrophil infiltration. A new term, drug-associated eccrine hidradenitis, is suggested.- - - - - - - - - - ranking = 1keywords = dermatosis (Clic here for more details about this article) |
3/8. Neutrophilic eccrine hidradenitis. Evidence implicating bleomycin as a causative agent.A 13-year-old girl receiving multiple agent chemotherapy for osteosarcoma was found to have neutrophilic eccrine hidradenitis (NEH). This dermatosis is marked histopathologically by necrosis of the eccrine structures with a neutrophilic infiltrate. Clinically, the presentation is variable and the differential diagnosis is extensive. Our patient's clinical picture was unique in that she had hyperpigmented plaques instead of purpuric nodules or erythematous plaques as described previously. Currently, NEH is felt to be a complication of chemotherapy. The most likely causative agent in our patient was bleomycin. physicians should be aware of this entity and its variable clinical presentation.- - - - - - - - - - ranking = 1keywords = dermatosis (Clic here for more details about this article) |
4/8. Neutrophilic eccrine hidradenitis--a report of two additional cases.Neutrophilic eccrine hidradenitis is a new neutrophilic dermatosis recently reported in association with acute myelogenous leukaemia and other malignancies, usually occurring during chemotherapy. The authors report two new cases, one of which was a patient with chronic lymphocytic leukaemia who had been treated with chlorambucil for 2 years. The clinical and histopathological features of the published cases are reviewed and the possible pathogenesis of this new syndrome is discussed.- - - - - - - - - - ranking = 1keywords = dermatosis (Clic here for more details about this article) |
5/8. Neutrophilic eccrine hidradenitis: a case report and review of the literature.Neutrophilic eccrine hidradenitis (NEH) is a recently recognized dermatosis occurring in patients receiving chemotherapy for a variety of malignancies. We report the second pediatric case. An 11-year-old boy with non-Hodgkin's lymphoma developed widespread erythematous papulopustules beginning two days after receiving high-dose cytarabine (Ara-C) in preparation for a bone marrow transplant. The lesions spontaneously regressed in two weeks. Histologic examination revealed a neutrophilic infiltrate around and within the eccrine ducts and secretory coils. Bacterial, fungal, and viral cultures were negative. These findings are characteristic of NEH. The condition should be differentiated from infectious dermatoses that may require treatment.- - - - - - - - - - ranking = 1keywords = dermatosis (Clic here for more details about this article) |
6/8. Neutrophilic eccrine hidradenitis in acute myelomonocytic leukemia.Neutrophilic eccrine hidradenitis is a rare but distinct dermatosis that has recently been described in adults with acute myelogenous leukemia. We report the first pediatric case in an 11-year-old girl with acute myelomonocytic leukemia who developed this eruption following her first and second course of VAPA-10 induction chemotherapy. skin biopsy revealed selective neutrophilic infiltration around the eccrine coils, which is characteristic of this dermatosis. The clinical and biopsy findings of our patient are presented and compared with the previous three reported cases.- - - - - - - - - - ranking = 2keywords = dermatosis (Clic here for more details about this article) |
7/8. Neutrophilic eccrine hidradenitis: a distinctive rash associated with cytarabine therapy and acute leukemia.Neutrophilic eccrine hidradenitis (NEH) is a recently described neutrophilic dermatosis associated with acute myelogenous leukemia (AML) and chemotherapy. This disorder is a distinct clinicopathologic entity separate from leukemid reactions and other neutrophilic dermatoses. We describe two cases in which plaques or nodules developed in the second week after initiation of induction chemotherapy for AML. The lesions regressed in 1 week and recurred in one case when induction chemotherapy was given a second time. Histologically, the findings were similar in each case. neutrophils palisaded about and infiltrated the eccrine coil in which necrosis of secretory epithelium was present. Focal mucinous degeneration of the eccrine adipose tissue cuff was the only other significant alteration. No vasculitis was observed. Cultures and histologic preparations for pathogenic organisms were negative. cytarabine was the chemotherapeutic agent used in all three cases. NEH most likely represents either an unusual response caused by cytarabine or a manifestation of AML. Recognition of NEH is important in order to exclude other neutrophilic dermatoses associated with AML, such as sepsis and leukemia cutis, which may appear clinically similar.- - - - - - - - - - ranking = 1keywords = dermatosis (Clic here for more details about this article) |
8/8. Neutrophilic eccrine hidradenitis. A distinctive type of neutrophilic dermatosis associated with myelogenous leukemia and chemotherapy.On two occasions, erythematous edematous plaques developed on the left side of the neck and the left shoulder of a man undergoing induction chemotherapy for acute myelogenous leukemia. The lesions resolved after several days in both instances. Histologically, numerous neutrophils surrounded and focally infiltrated the eccrine secretory coils, in which epithelial necrosis was observed. The "fixed" nature of the plaques and temporal relationship to chemotherapy suggest that the lesions represent an unusual reaction to chemotherapeutic agents. It is possible that this unique clinicopathologic picture represents a neutrophilic dermatosis associated with leukemia.- - - - - - - - - - ranking = 5keywords = dermatosis (Clic here for more details about this article) |