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Behçet disease: A common clinical presentation in a nonendemic area
932
851
Trichotillosis, skin picking and N-acetylcysteine Marisa Taylor, MBBS, MD, Department of Dermatology, Port Talbot, United Kingdom; Krishnan Bhagwandas, MBChB, Department of Dermatology, Port Talbot, United Kingdom We have recently presented a case of trichotillomania that responded well to oral Nacetylcysteine 1, and 1 year after starting she has retained complete hair regrowth. Here, we report its use in another case of trichotillosis/trichotillomania and skin picking. A 20-year-old presented with a 2.5-3 year history of intermittent, bilateral patchy hair loss over his parietal scalp. There is a family history of organ-specific autoimmune disease and OCD. Clinically, his hair loss lacked the findings typical of alopecia areata. His mother volunteered the observation that he ‘‘played with his hair,’’ especially during examination times. Review by a clinical psychologist is pending. Trichotillosis was suspected and he was started on 1200 mg Nacetylcysteine daily which was increased to 2400 mg after 12 weeks. 24 weeks after starting he has had almost complete regrowth. Three patients with clinically subacute prurigo have also been placed on 1200 mg N-acetylcysteine daily. They have experienced varying levels of improvement, not only in the appearance of the skin but also a reduction in the amount of skin picking. We propose therefore that Nacetylcysteine is a useful modality in the treatment of skin disorders that have impulse control as part of their pathogenesis.
An aggressive variant of mucous membrane pemphigoid with an excellent response to the association of intravenous immunoglobulin and rituximab Diana Menis, MD, Hospital Doce de Octubre, Madrid, Spain; Hugo Larrain-Paez, MD, Hospital Doce de Octubre, Madrid, Spain; Lidia Maro~ nas-Jimenez, MD, Hospital Doce de Octubre, Madrid, Spain; Vanessa Gargallo-Moneva, MD, Hospital Doce de Octubre, Madrid, Spain; Carlos Zarco-Olivo, MD, Hospital Doce de Octubre, Madrid, Spain; Rafael Llamas-Martın, MD, Hospital Doce de Octubre, Madrid, Spain; Francisco Vanaclocha-Sebastian, MD, Hospital Doce de Octubre, Madrid, Spain
Commercial support: None identified.
Mucous membrane pemphigoid is a chronic catastrophic mucocutaneous clinical picture that includes several autoimmune blistering disorders, with predominantly mucosal lesions and with a low response to traditional immunosuppressive treatments. It affects any mucous membrane regardless of whether scarring is present or not, with lesser extension of cutaneous lesions. Among the most serious complications that stand out is blindness and airway obstruction. Herein we present a 35-year-old man with a 6-year history of a chronic respiratory failure due to nasal synechiae, that in the last 2 years started with generalized blisters and erosions on trunk, face, scalp, limbs and genitals, multiples oral and upper respiratory tract erosions that led to an intense destruction of the pharyngeal architecture with loss of the tonsillar pillars and uvula, and a chronic conjunctivitis that progressively originate scars, symblepharon and trichiasis. Our histopathologic study confirmed the presence of subepidermal blister and the immunofluorescence direct study (IFD) revealed a linear deposit of IgA along the epithelial membrane zone. This data associated to the clinical predominance of scarring mucosal lesions, led us to the diagnosis of mucous membrane pemphigoid. When he came to our department he had been previously managed with high dose of corticosteroids, dapsone, colchicine and azathioprine without results. Considering the rapid progression of the mucosal involvement (in particular the ocular one) we decided to start a treatment with intravenous immunoglobulin (2 g/kg per cycle administered monthly) and rituximab (375 mg/m2 administered weekly during 8 consecutive weeks). After 2 months of treatment, almost all cutaneous and mucosal lesions have disappeared. This permitted us to progressively tapper the oral corticosteroids. We consider this case of interest for the aggressive clinical picture associated with the young age of the patient, and the remarkable rapid improvement with the association of rituximab and immunoglobulin. Commercial support: None identified.
1964
IMMUNODERMATOLOGY AND BLISTERING DISORDERS
Behc¸et disease: A common clinical presentation in a nonendemic area Roxana Castaneda Yepiz, MD, Centro Dermatol ogico Pascua, Distrito Federal, Mexico; Miguel Cardona, MD, Centro Dermatol ogico Pascua, Distrito Federal, Mexico; Victoria Martınez, MD, Centro Dermatol ogico Pascua, Distrito Federal, Mexico; Laura Fernandez, MD, Centro Dermatol ogico Pascua, Distrito Federal, Mexico; Fermin Jurado, MD, Centro Dermatol ogico Pascua, Distrito Federal, Mexico; Alberto Ramos, Centro Dermatol ogico Pascua, Distrito Federal, Mexico
A 90-year-old woman with a history of severe herpes zoster infection that had previously led to blindness, presented with multiple tender, itchy, and burning vesicles on wrists, palms and thighs with a single tense bulla on her right distal forearm. A skin biopsy obtained from the edge of the bulla on her forearm was sent for hematoxylineeosin staining and showed a subepidermal cleft with eosinophils. Eosinophilic spongiosis and perivascular lymphohistiocytic inflammation with diffuse perivascular and interstitial eosinophils were also seen. A second biopsy from her wrist was sent for direct immunofluorescence (DIF) and revealed 2+ linear deposition of IgG and 3+ linear deposition of C3 along the dermoepidermal junction. These findings were consistent with bullous pemphigoid. Clinicopathologic correlation was most consistent with dyshidrosiform bullous pemphigoid. Dyshidrosiform bullous pemphigoid is known to respond better to topical steroids than other forms of bullous pemphigoid, potentially avoiding the need for systemic steroids in this elderly woman. The patient was subsequently treated with topical clobetasol ointment applied twice daily to affected areas for 4 weeks, leading to clinical resolution of her lesions. This case highlights the importance of considering dyshidrosiform bullous pemphigoid (DBP) in the differential for patients with vesicular lesions, particularly in those with features of dyshidrotic dermatitis of the palms and/or soles and in elderly patients.
Behc¸et disease, classified as a systemic vasculitis, is characterized by relapsing oral aphthae, genital ulcers and hypopyon uveitis. It’s more common from eastern Asia to the Mediterranean, especially in Turkey. Here, we report a case of a male patient in Mexico City with recurrent painful oral ulcers that meet criteria for Behc¸et disease. A 39-year-old male patient was evaluated at Centro Dermatologico Dr Ladislao de la Pascua in Mexico City with numerous painful oral and genital ulcers, with blurred vision. Apparently the patient had similar lesions two years ago, associated with arthralgias in both legs. According to his medical history, the patient presented three episodes of oral ulcers in 12 months. At physical examination, vital signs were normal, he presented multiple aphthae in oral cavity of herpetiform morphology, approximately 1-3 mm and cicatricial lesion in gland of penis. The patient’s Patergy test was positive 48 hrs later, and a biopsy of an oral apthae was reported with unspecific inflammatory process. Ophthalmology test without relevant findings. Laboratory examination reported HLA B51 and B52 positive and HLA B27 negative. With clinical and laboratory findings, the patient meets criteria for Behc¸et disease. Support treatment and mometasone solution for oral cavity lesions were selected, with good response and remission one week later. Patient is evaluated periodically by dermatology, rheumatology and neurology. Behc¸et disease typically affects young adults from 20-40 years of age, but women are affected more commonly in the United States and northern Europe. Nearly all patients present with some form of painful oral mucocutaneous ulcerations in the form of aphthous ulcers or nonscarring oral lesions. Patients may present with erythema nodosum, cutaneous pustular vasculitis, and lesions similar to pyoderma gangrenosum. Inflammatory eye disease can develop early in the disease course and lead to permanent vision loss in 20%. Arthralgia is seen in up to half of patients, and is usually a nonerosive poly or oligoarthritis of the large joints of the lower extremities. The primary mechanism of the damage is an overactive immune system, involving T cells and the disease show a linkage with HLA-B51. This case is relevant because of the area that is not endemic, such as Mexico and also because of the gender of the patient.
Commercial support: None identified.
Commercial support: None identified.
1426 A case of dyshidrosiform bullous pemphigoid Liza A. McClellan, MD, Roger Williams Medical Center, Department of Dermatology, Providence, RI, United States; Ganary Dabiri, MD, PhD, Roger Williams Medical Center, Department of Dermatology, Providence, RI, United States; Doug Heiner, MD, Roger Williams Medical Center, Department of Dermatology, Providence, RI, United States
MAY 2015
J AM ACAD DERMATOL
AB117
851
Trichotillosis, skin picking and N-acetylcysteine Marisa Taylor, MBBS, MD, Department of Dermatology, Port Talbot, United Kingdom; Krishnan Bhagwandas, MBChB, Department of Dermatology, Port Talbot, United Kingdom We have recently presented a case of trichotillomania that responded well to oral Nacetylcysteine 1, and 1 year after starting she has retained complete hair regrowth. Here, we report its use in another case of trichotillosis/trichotillomania and skin picking. A 20-year-old presented with a 2.5-3 year history of intermittent, bilateral patchy hair loss over his parietal scalp. There is a family history of organ-specific autoimmune disease and OCD. Clinically, his hair loss lacked the findings typical of alopecia areata. His mother volunteered the observation that he ‘‘played with his hair,’’ especially during examination times. Review by a clinical psychologist is pending. Trichotillosis was suspected and he was started on 1200 mg Nacetylcysteine daily which was increased to 2400 mg after 12 weeks. 24 weeks after starting he has had almost complete regrowth. Three patients with clinically subacute prurigo have also been placed on 1200 mg N-acetylcysteine daily. They have experienced varying levels of improvement, not only in the appearance of the skin but also a reduction in the amount of skin picking. We propose therefore that Nacetylcysteine is a useful modality in the treatment of skin disorders that have impulse control as part of their pathogenesis.
An aggressive variant of mucous membrane pemphigoid with an excellent response to the association of intravenous immunoglobulin and rituximab Diana Menis, MD, Hospital Doce de Octubre, Madrid, Spain; Hugo Larrain-Paez, MD, Hospital Doce de Octubre, Madrid, Spain; Lidia Maro~ nas-Jimenez, MD, Hospital Doce de Octubre, Madrid, Spain; Vanessa Gargallo-Moneva, MD, Hospital Doce de Octubre, Madrid, Spain; Carlos Zarco-Olivo, MD, Hospital Doce de Octubre, Madrid, Spain; Rafael Llamas-Martın, MD, Hospital Doce de Octubre, Madrid, Spain; Francisco Vanaclocha-Sebastian, MD, Hospital Doce de Octubre, Madrid, Spain
Commercial support: None identified.
Mucous membrane pemphigoid is a chronic catastrophic mucocutaneous clinical picture that includes several autoimmune blistering disorders, with predominantly mucosal lesions and with a low response to traditional immunosuppressive treatments. It affects any mucous membrane regardless of whether scarring is present or not, with lesser extension of cutaneous lesions. Among the most serious complications that stand out is blindness and airway obstruction. Herein we present a 35-year-old man with a 6-year history of a chronic respiratory failure due to nasal synechiae, that in the last 2 years started with generalized blisters and erosions on trunk, face, scalp, limbs and genitals, multiples oral and upper respiratory tract erosions that led to an intense destruction of the pharyngeal architecture with loss of the tonsillar pillars and uvula, and a chronic conjunctivitis that progressively originate scars, symblepharon and trichiasis. Our histopathologic study confirmed the presence of subepidermal blister and the immunofluorescence direct study (IFD) revealed a linear deposit of IgA along the epithelial membrane zone. This data associated to the clinical predominance of scarring mucosal lesions, led us to the diagnosis of mucous membrane pemphigoid. When he came to our department he had been previously managed with high dose of corticosteroids, dapsone, colchicine and azathioprine without results. Considering the rapid progression of the mucosal involvement (in particular the ocular one) we decided to start a treatment with intravenous immunoglobulin (2 g/kg per cycle administered monthly) and rituximab (375 mg/m2 administered weekly during 8 consecutive weeks). After 2 months of treatment, almost all cutaneous and mucosal lesions have disappeared. This permitted us to progressively tapper the oral corticosteroids. We consider this case of interest for the aggressive clinical picture associated with the young age of the patient, and the remarkable rapid improvement with the association of rituximab and immunoglobulin. Commercial support: None identified.
1964
IMMUNODERMATOLOGY AND BLISTERING DISORDERS
Behc¸et disease: A common clinical presentation in a nonendemic area Roxana Castaneda Yepiz, MD, Centro Dermatol ogico Pascua, Distrito Federal, Mexico; Miguel Cardona, MD, Centro Dermatol ogico Pascua, Distrito Federal, Mexico; Victoria Martınez, MD, Centro Dermatol ogico Pascua, Distrito Federal, Mexico; Laura Fernandez, MD, Centro Dermatol ogico Pascua, Distrito Federal, Mexico; Fermin Jurado, MD, Centro Dermatol ogico Pascua, Distrito Federal, Mexico; Alberto Ramos, Centro Dermatol ogico Pascua, Distrito Federal, Mexico
A 90-year-old woman with a history of severe herpes zoster infection that had previously led to blindness, presented with multiple tender, itchy, and burning vesicles on wrists, palms and thighs with a single tense bulla on her right distal forearm. A skin biopsy obtained from the edge of the bulla on her forearm was sent for hematoxylineeosin staining and showed a subepidermal cleft with eosinophils. Eosinophilic spongiosis and perivascular lymphohistiocytic inflammation with diffuse perivascular and interstitial eosinophils were also seen. A second biopsy from her wrist was sent for direct immunofluorescence (DIF) and revealed 2+ linear deposition of IgG and 3+ linear deposition of C3 along the dermoepidermal junction. These findings were consistent with bullous pemphigoid. Clinicopathologic correlation was most consistent with dyshidrosiform bullous pemphigoid. Dyshidrosiform bullous pemphigoid is known to respond better to topical steroids than other forms of bullous pemphigoid, potentially avoiding the need for systemic steroids in this elderly woman. The patient was subsequently treated with topical clobetasol ointment applied twice daily to affected areas for 4 weeks, leading to clinical resolution of her lesions. This case highlights the importance of considering dyshidrosiform bullous pemphigoid (DBP) in the differential for patients with vesicular lesions, particularly in those with features of dyshidrotic dermatitis of the palms and/or soles and in elderly patients.
Behc¸et disease, classified as a systemic vasculitis, is characterized by relapsing oral aphthae, genital ulcers and hypopyon uveitis. It’s more common from eastern Asia to the Mediterranean, especially in Turkey. Here, we report a case of a male patient in Mexico City with recurrent painful oral ulcers that meet criteria for Behc¸et disease. A 39-year-old male patient was evaluated at Centro Dermatologico Dr Ladislao de la Pascua in Mexico City with numerous painful oral and genital ulcers, with blurred vision. Apparently the patient had similar lesions two years ago, associated with arthralgias in both legs. According to his medical history, the patient presented three episodes of oral ulcers in 12 months. At physical examination, vital signs were normal, he presented multiple aphthae in oral cavity of herpetiform morphology, approximately 1-3 mm and cicatricial lesion in gland of penis. The patient’s Patergy test was positive 48 hrs later, and a biopsy of an oral apthae was reported with unspecific inflammatory process. Ophthalmology test without relevant findings. Laboratory examination reported HLA B51 and B52 positive and HLA B27 negative. With clinical and laboratory findings, the patient meets criteria for Behc¸et disease. Support treatment and mometasone solution for oral cavity lesions were selected, with good response and remission one week later. Patient is evaluated periodically by dermatology, rheumatology and neurology. Behc¸et disease typically affects young adults from 20-40 years of age, but women are affected more commonly in the United States and northern Europe. Nearly all patients present with some form of painful oral mucocutaneous ulcerations in the form of aphthous ulcers or nonscarring oral lesions. Patients may present with erythema nodosum, cutaneous pustular vasculitis, and lesions similar to pyoderma gangrenosum. Inflammatory eye disease can develop early in the disease course and lead to permanent vision loss in 20%. Arthralgia is seen in up to half of patients, and is usually a nonerosive poly or oligoarthritis of the large joints of the lower extremities. The primary mechanism of the damage is an overactive immune system, involving T cells and the disease show a linkage with HLA-B51. This case is relevant because of the area that is not endemic, such as Mexico and also because of the gender of the patient.
Commercial support: None identified.
Commercial support: None identified.
1426 A case of dyshidrosiform bullous pemphigoid Liza A. McClellan, MD, Roger Williams Medical Center, Department of Dermatology, Providence, RI, United States; Ganary Dabiri, MD, PhD, Roger Williams Medical Center, Department of Dermatology, Providence, RI, United States; Doug Heiner, MD, Roger Williams Medical Center, Department of Dermatology, Providence, RI, United States
MAY 2015
J AM ACAD DERMATOL
AB117