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Comparative effectiveness of rituximab, intravenous immunoglobulin with cyclophosphamide, and conventional therapy for the treatment of pemphigus
P2117
P2201
Treatment of an ingrowing nail with cotton ball insertion under the nail Young Sik Kim, Department of Dermatology, College of Medicine, Yeungnam University, Daegu, South Korea; Chan Woo Kim, MD, Department of Dermatology, College of Medicine, Yeungnam University, Daegu, South Korea; Ki-Hong Kim, MD, Department of Dermatology, College of Medicine, Yeungnam University, Daegu, South Korea; Mi Hye Kim, MD, Department of Dermatology, College of Medicine, Yeungnam University, Daegu, South Korea; Woo Jin Kim, MD, Department of Dermatology, College of Medicine, Yeungnam University, Daegu, South Korea An ingrowing nail is a condition where an irregular sharp edge of the lateral nail plate penetrates and injures the soft tissue of the lateral nail fold. It induces so much pain, especially when walking, that a condition feels uncomfortable throughout their daily routines. According to the severity of the condition, there are several treatment options, including conservative treatment, cotton ball insertion, and partial matricectomy. Although the partial matricectomy is a curative treatment, it is invasive and needs postoperative care of the wound for some while. The insertion of cotton balls under the nail lesion relieves the lateral nail fold from the stimulation by the nail plate when walking; it therefore allows the lesion to improve. In addition, the procedure is simple and easy to practice repeatedly. The efficacy of the treatment of an ingrowing nail by insertion of cotton ball was compared with other therapeutic procedures by examining clinical data and records. One hundred and thirty ingrowing nails were treated with one of the following treatments: conservative treatment, cotton ball insertion, or partial matricectomy at the Department of Dermatology in Yeungnam University Hospital between January 2006 and April 2008. On the basis of severity, ingrowing nails were divided into three stages; stage 1 (mild) with erythema and tenderness, stage 2 (moderate) with erythema, tenderness, and pus, and stage 3 (severe) with erythema, tenderness, pus, and granulation of tissue. The cotton ball insertion method was applied mainly in stage 1 and 2 and showed a remission rate of 56.1%. Partial matricectomy showed a remission rate of 57.4% in stages 2 and 3, and the conservative treatment showed a remission rate of 40.7% in stage 1. Although the efficacy of the cotton ball insertion and partial matricectomy are nearly same, the cotton ball insertion is less invasive and patients rarely need follow-up care after a one-time procedure in the outpatient clinic. All these findings suggest the cotton ball insertion method for treating an ingrowing nail is one of best treatment options.
Comparative effectiveness of rituximab, intravenous immunoglobulin with cyclophosphamide, and conventional therapy for the treatment of pemphigus Margarita Lolis, MD, Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, NY, United States; Jean-Claude Bystryn, MD, Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, NY, United States Background: Rituximab has been recently introduced as a treatment for pemphigus. Several studies report a beneficial effect of this agent, but its efficacy remains unclear because no controlled study has been done. In particular, it is not known how the clinical effectiveness of rituximab compares to conventional treatment or to newer therapies such as intravenous immunoglobulin (IVIG) and cyclophosphamide (CP).
Commercial support: None identified.
IMMUNODERMATOLOGY AND BLISTERING DISORDERS
Objective: To compare the clinical effectiveness of rituximab, IVIG 1 cytotoxic drug, and conventional therapy in patients with active pemphigus. Methods: Twenty-three patients with pemphigus were treated with rituximab (n ¼ 7), IVIG 1 CP (n ¼ 6), or prednisone (n ¼ 10). The disease was active in all patients and diagnosis was based on clinical, histologic, and immunofluorescence criteria. The primary clinical endpoints were time to: (1) control disease activity (defined as the cessation of new lesion formation and beginning of healing of established lesions), (2) become lesion-free (no new or healing lesions), and (3) partial remission (absence of lesions for 1 mo on \15 mg/day prednisone). These parameters were measured and the percentages of patients who became lesion free and reached partial remission at three months were calculated, and were compared among the three treatment groups. Results: The median time to control disease activity was 13.5, 18, and 21 days in patients treated with IVIG 1 CP, rituximab, or prednisone alone, respectively (P ¼ .19). Later endpoints of response, such as median time to become lesion-free and induce partial remission, were reached more rapidly by rituximab compared to IVIG 1 CP, though the difference in time to become lesion-free (P ¼.25) or induce partial remission (P ¼ .48) was not statistically significant. Compared to treatment with prednisone alone, time to become lesion-free and reach partial remission was induced six times quicker by rituximab (P ¼.004 and .009) and four times quicker by IVIG 1 CP (P ¼ .04 and .01). By 3 months, 71%, 50%, and 20% of patients treated with rituximab, IVIG 1 CP, and prednisone were lesion-free and 57%, 50%, and 10%, respectively, reached partial remission. Conclusions: Disease activity was controlled most rapidly by IVIG 1 CP compared to rituximab or prednisone alone. By contrast, later endpoints of response to treatment were reached more rapidly in patients treated with rituximab or IVIG 1 CP compared to prednisone alone. There was a trend, which was not significant, for rituximab to reach each of these late endpoints more rapidly than IVIG 1 CP. Commercial support: None identified.
P2202 P2200 Pemphigoid gestationis: Early onset and blister formation but not systemic corticosteroid treatment is associated with adverse pregnancy outcomes Ching-Chi Chi,* Department of Dermatology, Chang Gung Memorial HospitalChiayi, Putz, Chiayi, Taiwan; Fenella Wojnarowska, MD, MBBCh, Department of Dermatology, Churchill Hospital, Oxford, Oxfordshire, United Kingdom; Martin Black, MD, St. John’s Institute of Dermatology, St. Thomas’s Hospital, London, United Kingdom; Shu-Hui Wang, MD, MS,* Department of Dermatology, Buddhist Tzu Chi General Hospital, Taipei Branch, Sindian, Taipei, Taiwan Objective: To evaluate the associations of adverse pregnancy outcomes with clinical features of pemphigoid gestationis (PG) and systemic corticosteroid treatment. Design: Retrospective cohort study. Setting: The PG database at the St. John’s Institute of Dermatology which recruited cases from dermatologists across the United Kingdom, and two tertiary hospitals in the United Kingdom and Taiwan (ie, Churchill Hospital and Chang Gung Memorial Hospital). Participants: Fifty-nine women with 61 pregnancies complicated by PG. Main outcome measures: Gestational age at delivery, preterm birth, birth weight, low birth weight (LBW; ie, birth weight \2500 g), small for gestational age (ie, birth weight below the 10th percentile for gestational age), fetal loss, congenital malformation, and mode of delivery in pregnancies with different trimester of disease onset, extent of involvement, presence or absence of blisters, and with or without systemic corticosteroid treatment. Results: After controlling for maternal age and comorbidity, decreased gestational age at delivery was significantly associated with presence of blisters (P ¼.017) and disease onset in the second trimester (P ¼ .001). Reduced birth weight was significantly associated with disease onset in the first and second trimesters (P ¼ .030 and .018, respectively) as was also LBW (adjusted odds ratio, 13.71 [95% CI, 1.22-154.59] and 10.76 [1.05-110.65], respectively). No significant associations between adverse pregnancy outcomes and systemic corticosteroid treatment were found. Conclusions: Severe PG with blister formation and early onset in the first or second trimester may lead to adverse pregnancy outcomes including decreased gestational age at delivery, preterm birth, and LBW children and should be considered a high risk pregnancy where appropriate obstetric care should be provided. Systemic corticosteroid treatment, in contrast, does not substantially affect pregnancy outcomes, and its use for PG in pregnant women is justified. *Drs Chi and Wang are co-first authors of this work. Commercial support: None identified.
MARCH 2009
Pemphigoid nodularis: Two new cases demonstrating distinguishing clinical clues from prurigo nodularis Jennifer Ray, PhD, Mayo Clinic, Rochester, MN, United States; Michael Camilleri, MD, Mayo Clinic, Rochester, MN, United States Background: Bullous pemphigoid (BP) is the most common autoimmune bullous dermatosis, involving production of autoantibodies against BP230/BPAg1 and BP180/BPAg2. There have been several variants described, including vegetative, vesicular, erythrodermic, urticarial, and nodular. Pemphigoid nodularis, having features of both prurigo nodularis and bullous pemphigoid, is the rare most recent variant of bullous pemphigoid to be described. Because of the fact that prurigo nodularis is not an uncommon disease compared to pemphigoid nodularis, distinguishing clinical features would be invaluable at increasing one’s clinical index of suspicion prompting for further investigations of an underlying autoimmune bullous dermatosis. Objective: Discuss two new cases of pemphigoid nodularis with emphasis on clinical features that help to distinguish it from prurigo nodularis. Methods: Retrospective case analysis with literature review. Results: The primary lesions of classic prurigo nodularis are well circumscribed, acanthotic, and lichenified fibrotic papules and nodules with variable pigmentation, hyperkeratosis, erosion, and ulceration caused by excoriation from intense pruritus. Two patients initially diagnosed with prurigo nodularis were referred for further evaluation and management after several years of refractoriness to treatment, with corticosteroids being the only treatment providing relief. The hyperplastic papules and nodules in both patients were initially clinically found to be more erythematous, with the vast majority demonstrating much larger surface areas affected by central erosion and ulceration compared to that normally seen in prurigo nodularis. There was also less temporal varigation noted in the activity of each papule or nodule. Distinct from prurigo nodularis, there were additionally several large erythematous plaques located on the extremities that had large areas of central erosion and ulceration. Histologic hematoxylineeosin findings from multiple biopsies in both cases demonstrated changes consistent with chronic dermatitis that were initially felt to be consistent with prurigo nodularis. In only a few sections of one specimen, subepidermal clefting was noted, which was initially felt to be artifactual. More than one direct immunofluorescence was required to detect a linear deposition of C3 at the basement membrane zone, with cutaneous indirect immunofluorescence demonstrating very high titers for IgG on monkey esophagus with an epidermal pattern seen on human split skin, consistent with pemphigoid. In addition, indirect enzyme-linked immunosorbent assays detecting antiBP180 and -BP230 IgG from the serum of each patient were found to be positive. Conclusion: Pemphigoid nodularis is a rare form of bullous pemphigoid that often goes misdiagnosed as prurigo nodularis. The following features seen in two new cases of pemphigoid nodularis helped to distinguish the immunobullous disorder from prurigo nodularis: primary lesions including large erythematous plaques,
J AM ACAD DERMATOL
AB103
P2201
Treatment of an ingrowing nail with cotton ball insertion under the nail Young Sik Kim, Department of Dermatology, College of Medicine, Yeungnam University, Daegu, South Korea; Chan Woo Kim, MD, Department of Dermatology, College of Medicine, Yeungnam University, Daegu, South Korea; Ki-Hong Kim, MD, Department of Dermatology, College of Medicine, Yeungnam University, Daegu, South Korea; Mi Hye Kim, MD, Department of Dermatology, College of Medicine, Yeungnam University, Daegu, South Korea; Woo Jin Kim, MD, Department of Dermatology, College of Medicine, Yeungnam University, Daegu, South Korea An ingrowing nail is a condition where an irregular sharp edge of the lateral nail plate penetrates and injures the soft tissue of the lateral nail fold. It induces so much pain, especially when walking, that a condition feels uncomfortable throughout their daily routines. According to the severity of the condition, there are several treatment options, including conservative treatment, cotton ball insertion, and partial matricectomy. Although the partial matricectomy is a curative treatment, it is invasive and needs postoperative care of the wound for some while. The insertion of cotton balls under the nail lesion relieves the lateral nail fold from the stimulation by the nail plate when walking; it therefore allows the lesion to improve. In addition, the procedure is simple and easy to practice repeatedly. The efficacy of the treatment of an ingrowing nail by insertion of cotton ball was compared with other therapeutic procedures by examining clinical data and records. One hundred and thirty ingrowing nails were treated with one of the following treatments: conservative treatment, cotton ball insertion, or partial matricectomy at the Department of Dermatology in Yeungnam University Hospital between January 2006 and April 2008. On the basis of severity, ingrowing nails were divided into three stages; stage 1 (mild) with erythema and tenderness, stage 2 (moderate) with erythema, tenderness, and pus, and stage 3 (severe) with erythema, tenderness, pus, and granulation of tissue. The cotton ball insertion method was applied mainly in stage 1 and 2 and showed a remission rate of 56.1%. Partial matricectomy showed a remission rate of 57.4% in stages 2 and 3, and the conservative treatment showed a remission rate of 40.7% in stage 1. Although the efficacy of the cotton ball insertion and partial matricectomy are nearly same, the cotton ball insertion is less invasive and patients rarely need follow-up care after a one-time procedure in the outpatient clinic. All these findings suggest the cotton ball insertion method for treating an ingrowing nail is one of best treatment options.
Comparative effectiveness of rituximab, intravenous immunoglobulin with cyclophosphamide, and conventional therapy for the treatment of pemphigus Margarita Lolis, MD, Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, NY, United States; Jean-Claude Bystryn, MD, Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, NY, United States Background: Rituximab has been recently introduced as a treatment for pemphigus. Several studies report a beneficial effect of this agent, but its efficacy remains unclear because no controlled study has been done. In particular, it is not known how the clinical effectiveness of rituximab compares to conventional treatment or to newer therapies such as intravenous immunoglobulin (IVIG) and cyclophosphamide (CP).
Commercial support: None identified.
IMMUNODERMATOLOGY AND BLISTERING DISORDERS
Objective: To compare the clinical effectiveness of rituximab, IVIG 1 cytotoxic drug, and conventional therapy in patients with active pemphigus. Methods: Twenty-three patients with pemphigus were treated with rituximab (n ¼ 7), IVIG 1 CP (n ¼ 6), or prednisone (n ¼ 10). The disease was active in all patients and diagnosis was based on clinical, histologic, and immunofluorescence criteria. The primary clinical endpoints were time to: (1) control disease activity (defined as the cessation of new lesion formation and beginning of healing of established lesions), (2) become lesion-free (no new or healing lesions), and (3) partial remission (absence of lesions for 1 mo on \15 mg/day prednisone). These parameters were measured and the percentages of patients who became lesion free and reached partial remission at three months were calculated, and were compared among the three treatment groups. Results: The median time to control disease activity was 13.5, 18, and 21 days in patients treated with IVIG 1 CP, rituximab, or prednisone alone, respectively (P ¼ .19). Later endpoints of response, such as median time to become lesion-free and induce partial remission, were reached more rapidly by rituximab compared to IVIG 1 CP, though the difference in time to become lesion-free (P ¼.25) or induce partial remission (P ¼ .48) was not statistically significant. Compared to treatment with prednisone alone, time to become lesion-free and reach partial remission was induced six times quicker by rituximab (P ¼.004 and .009) and four times quicker by IVIG 1 CP (P ¼ .04 and .01). By 3 months, 71%, 50%, and 20% of patients treated with rituximab, IVIG 1 CP, and prednisone were lesion-free and 57%, 50%, and 10%, respectively, reached partial remission. Conclusions: Disease activity was controlled most rapidly by IVIG 1 CP compared to rituximab or prednisone alone. By contrast, later endpoints of response to treatment were reached more rapidly in patients treated with rituximab or IVIG 1 CP compared to prednisone alone. There was a trend, which was not significant, for rituximab to reach each of these late endpoints more rapidly than IVIG 1 CP. Commercial support: None identified.
P2202 P2200 Pemphigoid gestationis: Early onset and blister formation but not systemic corticosteroid treatment is associated with adverse pregnancy outcomes Ching-Chi Chi,* Department of Dermatology, Chang Gung Memorial HospitalChiayi, Putz, Chiayi, Taiwan; Fenella Wojnarowska, MD, MBBCh, Department of Dermatology, Churchill Hospital, Oxford, Oxfordshire, United Kingdom; Martin Black, MD, St. John’s Institute of Dermatology, St. Thomas’s Hospital, London, United Kingdom; Shu-Hui Wang, MD, MS,* Department of Dermatology, Buddhist Tzu Chi General Hospital, Taipei Branch, Sindian, Taipei, Taiwan Objective: To evaluate the associations of adverse pregnancy outcomes with clinical features of pemphigoid gestationis (PG) and systemic corticosteroid treatment. Design: Retrospective cohort study. Setting: The PG database at the St. John’s Institute of Dermatology which recruited cases from dermatologists across the United Kingdom, and two tertiary hospitals in the United Kingdom and Taiwan (ie, Churchill Hospital and Chang Gung Memorial Hospital). Participants: Fifty-nine women with 61 pregnancies complicated by PG. Main outcome measures: Gestational age at delivery, preterm birth, birth weight, low birth weight (LBW; ie, birth weight \2500 g), small for gestational age (ie, birth weight below the 10th percentile for gestational age), fetal loss, congenital malformation, and mode of delivery in pregnancies with different trimester of disease onset, extent of involvement, presence or absence of blisters, and with or without systemic corticosteroid treatment. Results: After controlling for maternal age and comorbidity, decreased gestational age at delivery was significantly associated with presence of blisters (P ¼.017) and disease onset in the second trimester (P ¼ .001). Reduced birth weight was significantly associated with disease onset in the first and second trimesters (P ¼ .030 and .018, respectively) as was also LBW (adjusted odds ratio, 13.71 [95% CI, 1.22-154.59] and 10.76 [1.05-110.65], respectively). No significant associations between adverse pregnancy outcomes and systemic corticosteroid treatment were found. Conclusions: Severe PG with blister formation and early onset in the first or second trimester may lead to adverse pregnancy outcomes including decreased gestational age at delivery, preterm birth, and LBW children and should be considered a high risk pregnancy where appropriate obstetric care should be provided. Systemic corticosteroid treatment, in contrast, does not substantially affect pregnancy outcomes, and its use for PG in pregnant women is justified. *Drs Chi and Wang are co-first authors of this work. Commercial support: None identified.
MARCH 2009
Pemphigoid nodularis: Two new cases demonstrating distinguishing clinical clues from prurigo nodularis Jennifer Ray, PhD, Mayo Clinic, Rochester, MN, United States; Michael Camilleri, MD, Mayo Clinic, Rochester, MN, United States Background: Bullous pemphigoid (BP) is the most common autoimmune bullous dermatosis, involving production of autoantibodies against BP230/BPAg1 and BP180/BPAg2. There have been several variants described, including vegetative, vesicular, erythrodermic, urticarial, and nodular. Pemphigoid nodularis, having features of both prurigo nodularis and bullous pemphigoid, is the rare most recent variant of bullous pemphigoid to be described. Because of the fact that prurigo nodularis is not an uncommon disease compared to pemphigoid nodularis, distinguishing clinical features would be invaluable at increasing one’s clinical index of suspicion prompting for further investigations of an underlying autoimmune bullous dermatosis. Objective: Discuss two new cases of pemphigoid nodularis with emphasis on clinical features that help to distinguish it from prurigo nodularis. Methods: Retrospective case analysis with literature review. Results: The primary lesions of classic prurigo nodularis are well circumscribed, acanthotic, and lichenified fibrotic papules and nodules with variable pigmentation, hyperkeratosis, erosion, and ulceration caused by excoriation from intense pruritus. Two patients initially diagnosed with prurigo nodularis were referred for further evaluation and management after several years of refractoriness to treatment, with corticosteroids being the only treatment providing relief. The hyperplastic papules and nodules in both patients were initially clinically found to be more erythematous, with the vast majority demonstrating much larger surface areas affected by central erosion and ulceration compared to that normally seen in prurigo nodularis. There was also less temporal varigation noted in the activity of each papule or nodule. Distinct from prurigo nodularis, there were additionally several large erythematous plaques located on the extremities that had large areas of central erosion and ulceration. Histologic hematoxylineeosin findings from multiple biopsies in both cases demonstrated changes consistent with chronic dermatitis that were initially felt to be consistent with prurigo nodularis. In only a few sections of one specimen, subepidermal clefting was noted, which was initially felt to be artifactual. More than one direct immunofluorescence was required to detect a linear deposition of C3 at the basement membrane zone, with cutaneous indirect immunofluorescence demonstrating very high titers for IgG on monkey esophagus with an epidermal pattern seen on human split skin, consistent with pemphigoid. In addition, indirect enzyme-linked immunosorbent assays detecting antiBP180 and -BP230 IgG from the serum of each patient were found to be positive. Conclusion: Pemphigoid nodularis is a rare form of bullous pemphigoid that often goes misdiagnosed as prurigo nodularis. The following features seen in two new cases of pemphigoid nodularis helped to distinguish the immunobullous disorder from prurigo nodularis: primary lesions including large erythematous plaques,
J AM ACAD DERMATOL
AB103