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Necrotizing eosinophilic folliculitis: A new manifestation of the atopic diathesis?
3210
2665
Necrotizing eosinophilic folliculitis: A new manifestation of the atopic diathesis? Fiona Cunningham, MBBCh, MD, Western Infirmary Dermatology Department, Glasgow, United Kingdom; G. Pallavi, MBChB, MD, Western Infirmary Dermatology Department, Glasgow, United Kingdom; Gabrielle Becher, MBBCh, MD, Western Infirmary Dermatology Department, Glasgow, United Kingdom; Cynthia Magro, MBBCh, MD, The New York Hospital Cornell Medical Centre, New York, NY, United States A 49-year-old man with longstanding severe atopic eczema presented with a 6-year history of recurrent painful nodules affecting his face and limbs associated with fatigue and malaise and resolving spontaneously. He had an extensive travel history with recent travel to Africa. Clinical examination revealed widespread chronic atopic eczema and also tender subcutaneous 3 cm nodules on his forehead and upper limbs. A previous biopsy from a thigh nodule showed marked neutrophilia with abscess formation and eosinophilia mainly in the subcutaneous tissue. This was regarded as neutrophilic panniculitis. Given this inflammatory pattern, he was thoroughly investigated for any underlying infectious etiology; however, none was found. Subsequently he presented with an acute flare of his symptoms and had further biopsies taken from the left cheek and forehead. Both biopsies demonstrated a striking pattern of necrotizing folliculitis with pyodermatous alteration of the dermis. A mixed perifollicular infiltrate was noted with small numbers of eosinophils. Gram-positive cocci were also identified but were thought to represent commensal bacteria. On this basis, a diagnosis of necrotizing eosinophilic folliculitis was proposed. Eosinophilic folliculitis (EF) is frequently seen in HIV-positive patients; however, Magro et al proposed a new pustular variant of EF termed ‘necrotizing eosinophilic folliculitis’ (NEF) reported in a case series of 10 HIVnegative atopic patients and diagnosed based on clinical and histopathologic findings. In EF, antibody formation and creation of immune complexes are believed to mediate clinical manifestations. These antibodies are directed to the intercellular substance of the lower epidermis and outer root sheath of the hair follicle. An abnormal Th2-type immune response to a follicular antigen (ie, Demodex species) may be responsible for HIV-associated EF. However, in NEF, it has been suggested that severe atopy creates Th1/2 dysregulation. The interplay between an innocent trigger (ie, commensal bacteria), and T cell dysregulation in atopic dermatitis is thought to lead to the destructive folliculocentric process which is pathognomonic of this condition. This is an unusual and important diagnosis to consider in patients with atopic dermatitis presenting with these symptoms.
Peristomal intestinal metaplasia: A case report Jessica Davey, MD, Gosford Hospital, Gosford, NSW, Australia; Debra Day, RN, Gosford Hospital, Gosford, NSW, Australia; Vicki Howard, MBBS, Douglas Hanly Moir Pathology, Macquarie Park, NSW, Australia; Saxon Smith, MBChB, The Dermatology and Skin Cancer Centre, Gosford, NSW, Australia Peristomal intestinal metaplasia (PIM) of ileostomies has been rarely reported. We present the third case known to the authors. An 88-year-old Asian-white female with a history of ulcerative colitis and 55-year ileostomy presented with a 5-year history of peristomal skin irritation worsening over 2 years after the development of a peristomal hernia. It was associated with appliance leakage and a well-developed peristomal skin ulcer. It was initially seen in a specialized stoma clinic and treated with Kenacomb and Aquacel dressings and a new convex ostomy bag. It did not resolve and she was referred to a dermatologist. On presentation a 5 cm x 4 cm eroded, granulomatous papulonodular friable plaque was noted from 2 o’clock to 7 o’clock of the ileostomy mucosa-cutaneous junction extending inferomedially onto the abdomen. Two shave biopsies showed mild papillomatosis, hyperkeratosis, and acute on chronic inflammation with focal erosions. There were epithelial islands resembling colonic epithelium, mixed with squamous epithelium indicating cutaneous PIM. The glandular epithelium showed mild reactive, nondysplastic, nonmalignant changes. Initial treatment with silver nitrate was unsuccessful. Subsequently, electrocautery under local anesthetic was performed in the dermatologist’s rooms after an initial test patch. Three treatments using low setting on a 40-W high frequency desiccator were performed. The eroded plaque completely reepithelialized. However, 4 months later some small recurrences were noted and treated with higher setting electrocautery. The range of reported peristomal dermatoses is wide, the most common precipitant being effluent leakage and mechanical stripping of the peristomal skin secondary to ill-fitting appliances. Strauch et al, J Am Coll Surg 2007, found in an in vitro study that bile salt exposure increases intestinal epithelial cell migration after wounding, hypothesizing this as a cause of PIM. Another hypothesis of Ono et al, BJD 2012, asserts metaplastic tubular gland secretions destroy the epidermis, and their destruction using electrocoagulation eliminates these, allowing reepithelialization. Finally, peristomal over granulation with intestinal metaplasia has been known to undergo malignant transformation. We could find no reports reflecting such of PIM but given the few known cases the possibility should not be discounted. We therefore recommend patients with PIM be monitored for recurrence and suspicious lesions biopsied. Commercial support: None identified.
Commercial support: None identified.
2519 Plasma cell reactive benign proliferations (Plasmacytosis). Report of three typical cases and review of the literature Isabel Bernad, MD, Clınica Universidad de Navarra, Pamplona, Spain; Raquel Santesteban, MD, Complejo Hospitalario de Navarra, Pamplona, Spain; Juan Ignacio Yanguas, MD, Complejo Hospitalario de Navarra, Pamplona, Spain; Rosario Vives, MD, Complejo Hospitalario de Navarra, Pamplona, Spain; Alicia C ordoba, MD, Complejo Hospitalario de Navarra, Pamplona, Spain; Agustın Espa~ na, MD, Clınica Universidad de Navarra, Pamplona, Spain
3008 Papular epidermal nevus with ‘‘skyline’’ basal cell layer Alison Spiker, MD, Geisinger Medical Center, Danville, PA, United States; Howard Pride, MD, Geisinger Medical Center, Danville, PA, United States; Tammie Ferringer, MD, Geisinger Medical Center, Danville, PA, United States Background: Papular epidermal nevus with ‘‘skyline’’ basal cell layer (PENS) is a recently described variant of epidermal nevus that is often present at birth and can potentially be associated with neurologic disorders. Case report: An otherwise healthy 9-month-old infant presented for evaluation of an asymptomatic, congenital skin lesion. Clinically, the lesion was a linear 1.5-cm fleshcolored, thin, wrinkled plaque on his center chest. The patient’s brother had a similar lesion on his right lateral chest that followed the lines of Blaschko. No medical issues, including no neurologic disorders, were initially identified in the patient. Biopsy of the lesion revealed orthokeratotic hyperkeratosis, acanthosis with broad rete ridges, and a prominent palisaded, ‘‘skyline’’ basal cell layer consistent with PENS. Subsequently, the patient was diagnosed with strabismus at 27 months and found to have a developmental language disorder and hearing sensitivity at 34 months. Discussion: Papular epidermal nevus with ‘‘skyline’’ basal cell layer (PENS) was first described by Torrelo et al in 2011 with few subsequent case reports. Inheritance is both sporadic and familial paradominant. Lesions typically are asymptomatic and can follow the lines of Blaschko. Tadini et al reported several patients with PENS in association with neurologic disorders for which he labeled PENS syndrome. Neurologic findings in PENS syndrome include speech and language impairment, hyperactivity and attention deficit, epilepsy and psychomotor retardation of various degrees. Neurologic symptom onset is within the first 1-2 years of life and patients with PENS syndrome have a good prognosis. Conclusion: Identification of PENS is important given the potential for neurologic disorders. A complete medical history is indicated and close follow-up with pediatrics is advised. Commercial support: None identified.
AB100
J AM ACAD DERMATOL
Introduction: Plasmacytosis is characterized by benign reactive proliferations of plasma cells secondary to a known or unknown exogenous agent and was first described by Zoon in 1952 in gland mucosa. Since then, several authors have described similar lesions with different presentations appearing in both mucous membranes or the skin. The nomenclature of the disorder has been not well defined to date but recently a new classification has been proposed by Senol et al. We present 3 typical cases of plasmacytosis located in periorificial areas. Case reports: The first patient was a 76-year-old liver transplanted man who presented with erythematous overgrowth tissue and fissures in both angles of the mouth which had appeared 1 year previously. The second patient, a 52-year-old man had a red, papular lesion at the entrance of the right nostril which had appeared 2 months previously with no known triggering factor. The third patient, a 46-year-old man, had a red papular lesion with a little central crust on the tip of his nose which had appeared 2 months previously. Histopathologically all of the lesions presented a dense infiltrate of plasma cells, CD38+. Inmunohistochemical staining for kappa and lambda chains revealed polyclonality in all cases. A biopsy from the first patient also showed acanthosis and was thus classified as plasmoacanthoma. The other patients were classified as mucocutaneous and cutaneous plasmacytosis respectively because of the location of the lesions. A serologic test for syphilis and immunohistochemical analysis for spirochetes were negative in all patients. Discussion: We present 3 typical cases of plasmacytosis located in periorificial areas and describe the associated clinical features and histopathologic findings. We categorize the cases using the new classification. We also perform a review of the literature describing how the heterogeneous presentations of this disease have been progressively described in the literature, and how it has been concluded that, given the common histopathologic findings, it represents one single entity. Conclusions: At present, the nomenclature of this multiform, reactive and inflammatory disorder is not well defined and as a result we believe that it is an underdiagnosed disease. It is important to clarify that it represents a single entity with various forms of presentation. The new classification appears to be a good option for the rigorous classification of this heterogeneous entity. Commercial support: None identified.
MAY 2016
2665
Necrotizing eosinophilic folliculitis: A new manifestation of the atopic diathesis? Fiona Cunningham, MBBCh, MD, Western Infirmary Dermatology Department, Glasgow, United Kingdom; G. Pallavi, MBChB, MD, Western Infirmary Dermatology Department, Glasgow, United Kingdom; Gabrielle Becher, MBBCh, MD, Western Infirmary Dermatology Department, Glasgow, United Kingdom; Cynthia Magro, MBBCh, MD, The New York Hospital Cornell Medical Centre, New York, NY, United States A 49-year-old man with longstanding severe atopic eczema presented with a 6-year history of recurrent painful nodules affecting his face and limbs associated with fatigue and malaise and resolving spontaneously. He had an extensive travel history with recent travel to Africa. Clinical examination revealed widespread chronic atopic eczema and also tender subcutaneous 3 cm nodules on his forehead and upper limbs. A previous biopsy from a thigh nodule showed marked neutrophilia with abscess formation and eosinophilia mainly in the subcutaneous tissue. This was regarded as neutrophilic panniculitis. Given this inflammatory pattern, he was thoroughly investigated for any underlying infectious etiology; however, none was found. Subsequently he presented with an acute flare of his symptoms and had further biopsies taken from the left cheek and forehead. Both biopsies demonstrated a striking pattern of necrotizing folliculitis with pyodermatous alteration of the dermis. A mixed perifollicular infiltrate was noted with small numbers of eosinophils. Gram-positive cocci were also identified but were thought to represent commensal bacteria. On this basis, a diagnosis of necrotizing eosinophilic folliculitis was proposed. Eosinophilic folliculitis (EF) is frequently seen in HIV-positive patients; however, Magro et al proposed a new pustular variant of EF termed ‘necrotizing eosinophilic folliculitis’ (NEF) reported in a case series of 10 HIVnegative atopic patients and diagnosed based on clinical and histopathologic findings. In EF, antibody formation and creation of immune complexes are believed to mediate clinical manifestations. These antibodies are directed to the intercellular substance of the lower epidermis and outer root sheath of the hair follicle. An abnormal Th2-type immune response to a follicular antigen (ie, Demodex species) may be responsible for HIV-associated EF. However, in NEF, it has been suggested that severe atopy creates Th1/2 dysregulation. The interplay between an innocent trigger (ie, commensal bacteria), and T cell dysregulation in atopic dermatitis is thought to lead to the destructive folliculocentric process which is pathognomonic of this condition. This is an unusual and important diagnosis to consider in patients with atopic dermatitis presenting with these symptoms.
Peristomal intestinal metaplasia: A case report Jessica Davey, MD, Gosford Hospital, Gosford, NSW, Australia; Debra Day, RN, Gosford Hospital, Gosford, NSW, Australia; Vicki Howard, MBBS, Douglas Hanly Moir Pathology, Macquarie Park, NSW, Australia; Saxon Smith, MBChB, The Dermatology and Skin Cancer Centre, Gosford, NSW, Australia Peristomal intestinal metaplasia (PIM) of ileostomies has been rarely reported. We present the third case known to the authors. An 88-year-old Asian-white female with a history of ulcerative colitis and 55-year ileostomy presented with a 5-year history of peristomal skin irritation worsening over 2 years after the development of a peristomal hernia. It was associated with appliance leakage and a well-developed peristomal skin ulcer. It was initially seen in a specialized stoma clinic and treated with Kenacomb and Aquacel dressings and a new convex ostomy bag. It did not resolve and she was referred to a dermatologist. On presentation a 5 cm x 4 cm eroded, granulomatous papulonodular friable plaque was noted from 2 o’clock to 7 o’clock of the ileostomy mucosa-cutaneous junction extending inferomedially onto the abdomen. Two shave biopsies showed mild papillomatosis, hyperkeratosis, and acute on chronic inflammation with focal erosions. There were epithelial islands resembling colonic epithelium, mixed with squamous epithelium indicating cutaneous PIM. The glandular epithelium showed mild reactive, nondysplastic, nonmalignant changes. Initial treatment with silver nitrate was unsuccessful. Subsequently, electrocautery under local anesthetic was performed in the dermatologist’s rooms after an initial test patch. Three treatments using low setting on a 40-W high frequency desiccator were performed. The eroded plaque completely reepithelialized. However, 4 months later some small recurrences were noted and treated with higher setting electrocautery. The range of reported peristomal dermatoses is wide, the most common precipitant being effluent leakage and mechanical stripping of the peristomal skin secondary to ill-fitting appliances. Strauch et al, J Am Coll Surg 2007, found in an in vitro study that bile salt exposure increases intestinal epithelial cell migration after wounding, hypothesizing this as a cause of PIM. Another hypothesis of Ono et al, BJD 2012, asserts metaplastic tubular gland secretions destroy the epidermis, and their destruction using electrocoagulation eliminates these, allowing reepithelialization. Finally, peristomal over granulation with intestinal metaplasia has been known to undergo malignant transformation. We could find no reports reflecting such of PIM but given the few known cases the possibility should not be discounted. We therefore recommend patients with PIM be monitored for recurrence and suspicious lesions biopsied. Commercial support: None identified.
Commercial support: None identified.
2519 Plasma cell reactive benign proliferations (Plasmacytosis). Report of three typical cases and review of the literature Isabel Bernad, MD, Clınica Universidad de Navarra, Pamplona, Spain; Raquel Santesteban, MD, Complejo Hospitalario de Navarra, Pamplona, Spain; Juan Ignacio Yanguas, MD, Complejo Hospitalario de Navarra, Pamplona, Spain; Rosario Vives, MD, Complejo Hospitalario de Navarra, Pamplona, Spain; Alicia C ordoba, MD, Complejo Hospitalario de Navarra, Pamplona, Spain; Agustın Espa~ na, MD, Clınica Universidad de Navarra, Pamplona, Spain
3008 Papular epidermal nevus with ‘‘skyline’’ basal cell layer Alison Spiker, MD, Geisinger Medical Center, Danville, PA, United States; Howard Pride, MD, Geisinger Medical Center, Danville, PA, United States; Tammie Ferringer, MD, Geisinger Medical Center, Danville, PA, United States Background: Papular epidermal nevus with ‘‘skyline’’ basal cell layer (PENS) is a recently described variant of epidermal nevus that is often present at birth and can potentially be associated with neurologic disorders. Case report: An otherwise healthy 9-month-old infant presented for evaluation of an asymptomatic, congenital skin lesion. Clinically, the lesion was a linear 1.5-cm fleshcolored, thin, wrinkled plaque on his center chest. The patient’s brother had a similar lesion on his right lateral chest that followed the lines of Blaschko. No medical issues, including no neurologic disorders, were initially identified in the patient. Biopsy of the lesion revealed orthokeratotic hyperkeratosis, acanthosis with broad rete ridges, and a prominent palisaded, ‘‘skyline’’ basal cell layer consistent with PENS. Subsequently, the patient was diagnosed with strabismus at 27 months and found to have a developmental language disorder and hearing sensitivity at 34 months. Discussion: Papular epidermal nevus with ‘‘skyline’’ basal cell layer (PENS) was first described by Torrelo et al in 2011 with few subsequent case reports. Inheritance is both sporadic and familial paradominant. Lesions typically are asymptomatic and can follow the lines of Blaschko. Tadini et al reported several patients with PENS in association with neurologic disorders for which he labeled PENS syndrome. Neurologic findings in PENS syndrome include speech and language impairment, hyperactivity and attention deficit, epilepsy and psychomotor retardation of various degrees. Neurologic symptom onset is within the first 1-2 years of life and patients with PENS syndrome have a good prognosis. Conclusion: Identification of PENS is important given the potential for neurologic disorders. A complete medical history is indicated and close follow-up with pediatrics is advised. Commercial support: None identified.
AB100
J AM ACAD DERMATOL
Introduction: Plasmacytosis is characterized by benign reactive proliferations of plasma cells secondary to a known or unknown exogenous agent and was first described by Zoon in 1952 in gland mucosa. Since then, several authors have described similar lesions with different presentations appearing in both mucous membranes or the skin. The nomenclature of the disorder has been not well defined to date but recently a new classification has been proposed by Senol et al. We present 3 typical cases of plasmacytosis located in periorificial areas. Case reports: The first patient was a 76-year-old liver transplanted man who presented with erythematous overgrowth tissue and fissures in both angles of the mouth which had appeared 1 year previously. The second patient, a 52-year-old man had a red, papular lesion at the entrance of the right nostril which had appeared 2 months previously with no known triggering factor. The third patient, a 46-year-old man, had a red papular lesion with a little central crust on the tip of his nose which had appeared 2 months previously. Histopathologically all of the lesions presented a dense infiltrate of plasma cells, CD38+. Inmunohistochemical staining for kappa and lambda chains revealed polyclonality in all cases. A biopsy from the first patient also showed acanthosis and was thus classified as plasmoacanthoma. The other patients were classified as mucocutaneous and cutaneous plasmacytosis respectively because of the location of the lesions. A serologic test for syphilis and immunohistochemical analysis for spirochetes were negative in all patients. Discussion: We present 3 typical cases of plasmacytosis located in periorificial areas and describe the associated clinical features and histopathologic findings. We categorize the cases using the new classification. We also perform a review of the literature describing how the heterogeneous presentations of this disease have been progressively described in the literature, and how it has been concluded that, given the common histopathologic findings, it represents one single entity. Conclusions: At present, the nomenclature of this multiform, reactive and inflammatory disorder is not well defined and as a result we believe that it is an underdiagnosed disease. It is important to clarify that it represents a single entity with various forms of presentation. The new classification appears to be a good option for the rigorous classification of this heterogeneous entity. Commercial support: None identified.
MAY 2016