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Verrucous epidermal nevus, efficacy and safety of treatment with CO2 laser
4569
4951
Vasculitis-like erythema infectiosum in children Daniela Subiabre, MD, General Universitary Hospital of Valencia; Lorena Martinez, MD, General Universitary Hospital of Valencia; Jose Maria Ortiz, MD, General Universitary Hospital of Valencia; Altea Esteve, MD, General Universitary Hospital of Valencia Erythema infectiosum, also known as fifth disease, is usually a benign childhood condition characterized by mild prodromal symptoms followed by a classic slappedcheek appearance and an erythematous maculopapular rash that fades into a reticular pattern as confluent areas clear. It results from infection with human parvovirus (PV) B19, an erythrovirus. We present the cases of two children, a boy and a girl of 6 and 9 years old with no previous diseases that came into the emergency room with palpable purpuric plaques. The boy had purpuric papules that confluent in plaques in both popliteal fossas, the back of the right thigh and both arms of 3 days evolution. He had no fever, just a self-limited abdominal pain. He was admitted with the diagnosis of vasculitis for study and a biopsy and blood tests were performed. In the biopsy there were no signs of vasculitis and the DIF was negative. The girl came with a history of purpuric plaques in both legs and petechiae in the abdomen of 2 days evolution associate with fever that resolved with symptomatic treatment. In both cases the serology showed positivity of the PV IgM with negative IgG and was confirmed with real time PCR. The Vasculitis-like PV primoinfection diagnose was made in both cases and the symptoms resolved spontaneously. Erythema infectiosum usually progresses through a three stages skin disease. The exanthema begins with the classic slapped-cheek appearance, which typically fades over a few days, 1-4 days later, appears an erythematous maculopapular rash that fades into a classic lacelike reticular pattern as confluent areas clear, and finally, there are frequent clearing and recurrences for weeks or months. There are some vasculitis-like descriptions in PV primoinfection in the literature especially in adults and less common in children. The diagnosis is based on clinical presentation, and a workup for patients with the classic presentation is not necessary. For patients with other signs or symptoms confirmation of infection may be helpful and can be accomplished with IgM assays, ELISA or PCR assay as in our cases. Erythema infectiosum most often is a benign, self-limited disease, so reassuring the parents and symptomatic treatment are often the only interventions necessary. We present two cases of vasculitis-like PV primoinfection in children, and we think that this clinical presentation must be considered into the differential diagnosis to make a correct evaluation and management.
Verrucous epidermal nevus, efficacy and safety of treatment with CO2 laser Gabriela Moreno Agraz, Hospital HMG Coyoacan; Marysol Macedo Perez, Facultad de Medicina, UNAM; Juan Antonio Suarez Cuenca, Centro Medico Nacional 20 de Noviembre. Divisi on de Investigaci on Clınica Background: Verrucous epidermal nevus (VEN) is a benign hamartomatous growth of the skin. It appears as a linear patch following the Blaschko lines, with a distribution located or disseminated, with prevalence from 0.1 to 0.5%. Frequently this lesion is resistant to multiple modalities of treatment; sometimes the results in cosmetic aspects could be unfavorable by its large size and visible location. Among several therapeutic alternatives,CO2 laser elicits the treatment of widespread areas in one session, with minimal cosmetic consequences. Objective: Determine effectiveness and safety of CO2 laser in patients with VEN.
Commercial support: None identified.
Methods: Descriptive, prospective study. Diagnosis of VEN was confirmed by biopsy. Patients with allergy to local analgesics or diagnosis of epidermal nevus syndrome were excluded. CO2 laser therapy was applied in ultrapulsed mode-300 mJ. Early response to therapy was defined as 80% to 100% of clinical improvement of the lesion in the 2-4 weeks. Patients were followed up and occurrences of hypertrophic scars, hyperpigmentation or hypopigmentation of the lesion were registered during the next 2, 4 and 12 weeks after treatment. Results: Twenty-five patients were included, aged 15.5 6 7.6 years old, being 15 (64%) males and 10 (36%) females. VEN showed a located (12 [88%]) or disseminated distribution (3 [12%]), with a predominance of facial location (22 [34%]). An early response to treatment was achieved in up to 80% of the cases. Complications were observed in 40% of the cases; consisted in recurrence 2 (8%), hypertrophic scar 2 (8%), hyperpigmentation 5 (20%) and hypopigmentation 3 (12%). According to multivariable analysis, the morphology of the VEN was associated to hyperpigmentation (Morphology vs Hyperpigmentation (Exp [B]) 11.2 (95% CI: 1.10-114.4 P ¼ .04); while significant interaction was observed with extension (morphology*extension vs Hyperpigmentation (Exp [B]) 13.5 (95% CI: 1.3-135.9 P ¼ .27). Conclusion: CO2 laser therapy for VEN provides an acceptable clinical response in approximately 80% of the cases, with a relatively low number of complications which seemed to be associated with the basal characteristics of morphology and extension of the lesion. Commercial support: None identified.
5194 5625 Verrucous carcinoma arising within hypertrophic lichen planus Thy Huynh, MD, Northwestern University Dept. of Dermatology; Lauren Graham, MD, Northwestern University Dept. of Dermatology; Joan Guitart, MD, Northwestern University Dept. of Dermatology; Sarah Lyon, BS, Northwestern University Dept. of Dermatology; Joaquin Brieva, MD, Northwestern University Dept. of Dermatology A 16 -year-old Southeast Asian male with lichen planus since age five presented with an evolving tumor on the left pretibia for several months. The lesion responded minimally to potent steroids. Exam revealed a 4 cm lichenified plaque with eroded, hyperkeratotic center and raised borders. Lesional biopsy showed a verrucous process with prominent hyperkeratosis, parakeratosis, papillomatosis, and an infiltrating process resembling pseudoepitheliomatous hyperplasia without significant nuclear atypia. A lichenoid infiltrate was noted. Several bacterial colonies and focal koilocytotic changes were noted. Gram stain showed numerous cocci within the cornified layer. HPV and DPAS were negative. CBC, BMP, LFTs, serum lipids, and Quantiferon gold were normal. A diagnosis of hypertrophic lichen planus with evolving verrucous carcinoma was made. Mohs micrographic surgery was performed in 3 stages, and patient was started on low-dose prednisone daily. One month later, acitretin and hydroxychloroquine were added with significant improvement. After one year he developed steroid-induced acne, so prednisone was replaced with intralesional injections of triamcinolone with continued improvement. He stopped acitretin and hydroxychloroquine afterwards and returns for intralesional triamcinolone injections as needed if symptomatic. Lichen planus is generally a benign cell-mediated skin disorder of unclear etiology with predilection for the pretibial region. Less than 100 reported cases of malignant transformation are found in the literature. Clinical suspicion for malignancy should be noted if lesions ulcerate, evolve into tumors, or become unresponsive to therapy. We present this case due to clinical interest given uncommon sequela of the malignant transformation occurring with hypertrophic lichen planus. Commercial support: None identified.
AB280
J AM ACAD DERMATOL
Violaceous plaques on the buttocks Benjamin Bigbie, MD, University of Oklahoma; Thomas Stasko, MD, University of Oklahoma Case Report: A 52-year-old white male presented to the dermatology clinic in 2011 with a 6-year history of progressive violaceous plaques on the bilateral buttocks. The lesions were pruritic and mildly painful upon sitting. He had been diagnosed with fungal infections and psoriasis in the past, but failed antifungal and topical corticosteroid therapy. He had no family history of similar lesions and was otherwise healthy. Physical exam revealed confluent pink to violaceous flat plaques with peripheral scale present throughout the bilateral gluteal crease and sacral area. Initial biopsy was nondiagnostic but subsequent biopsies revealed a column of parakeratosis which was consistent with a cornoid lamella. Discussion: Porokeratosis ptychotropica is a rare variant of porokeratosis in which violaceous, flat, scaly plaques are present on the bilateral buttocks with reported cases of extension on to the upper thighs and genital area. The lesions tend to follow a chronic course and as with other forms of porokeratosis, treatment is difficult. The entity was first described in 1995 by Lucker et al and name is derived from the Greek words ptyche (fold) and trope (a turning) to describe the plaques which involve the body folds such as the gluteal crease. Histologically the lesions feature multiple cornoid lamellae which differentiate the entity from other subtypes of porokeratosis. There are less than 25 case reports to date and these reports have detailed the difficulty in treating this condition including failure with topical or intralesional steroids, imiquimod, cryotherapy, retinoids, and CO2 laser. However, there are also reports of success with excision and partial thickness removal with a dermatome. Our patient experienced numerous, failed treatments including topical and intralesional steroids, cantharidin, and topical retionoids. He had partial improvement, primarily in thickness and pruritus, with imiquimod, 5-fluorouracil, ingenol mebutate, shave excision, and oral retinoids. Fractionated CO2 laser resulted in initial improvement, but the lesions recurred in a few months. Our patient is currently awaiting removal of lesions with a dermatome. Our patient’s presentation and difficulty with treatment mirror the descriptions of porokeratosis ptychotropia in the literature. Commercial support: None identified.
JUNE 2017
4951
Vasculitis-like erythema infectiosum in children Daniela Subiabre, MD, General Universitary Hospital of Valencia; Lorena Martinez, MD, General Universitary Hospital of Valencia; Jose Maria Ortiz, MD, General Universitary Hospital of Valencia; Altea Esteve, MD, General Universitary Hospital of Valencia Erythema infectiosum, also known as fifth disease, is usually a benign childhood condition characterized by mild prodromal symptoms followed by a classic slappedcheek appearance and an erythematous maculopapular rash that fades into a reticular pattern as confluent areas clear. It results from infection with human parvovirus (PV) B19, an erythrovirus. We present the cases of two children, a boy and a girl of 6 and 9 years old with no previous diseases that came into the emergency room with palpable purpuric plaques. The boy had purpuric papules that confluent in plaques in both popliteal fossas, the back of the right thigh and both arms of 3 days evolution. He had no fever, just a self-limited abdominal pain. He was admitted with the diagnosis of vasculitis for study and a biopsy and blood tests were performed. In the biopsy there were no signs of vasculitis and the DIF was negative. The girl came with a history of purpuric plaques in both legs and petechiae in the abdomen of 2 days evolution associate with fever that resolved with symptomatic treatment. In both cases the serology showed positivity of the PV IgM with negative IgG and was confirmed with real time PCR. The Vasculitis-like PV primoinfection diagnose was made in both cases and the symptoms resolved spontaneously. Erythema infectiosum usually progresses through a three stages skin disease. The exanthema begins with the classic slapped-cheek appearance, which typically fades over a few days, 1-4 days later, appears an erythematous maculopapular rash that fades into a classic lacelike reticular pattern as confluent areas clear, and finally, there are frequent clearing and recurrences for weeks or months. There are some vasculitis-like descriptions in PV primoinfection in the literature especially in adults and less common in children. The diagnosis is based on clinical presentation, and a workup for patients with the classic presentation is not necessary. For patients with other signs or symptoms confirmation of infection may be helpful and can be accomplished with IgM assays, ELISA or PCR assay as in our cases. Erythema infectiosum most often is a benign, self-limited disease, so reassuring the parents and symptomatic treatment are often the only interventions necessary. We present two cases of vasculitis-like PV primoinfection in children, and we think that this clinical presentation must be considered into the differential diagnosis to make a correct evaluation and management.
Verrucous epidermal nevus, efficacy and safety of treatment with CO2 laser Gabriela Moreno Agraz, Hospital HMG Coyoacan; Marysol Macedo Perez, Facultad de Medicina, UNAM; Juan Antonio Suarez Cuenca, Centro Medico Nacional 20 de Noviembre. Divisi on de Investigaci on Clınica Background: Verrucous epidermal nevus (VEN) is a benign hamartomatous growth of the skin. It appears as a linear patch following the Blaschko lines, with a distribution located or disseminated, with prevalence from 0.1 to 0.5%. Frequently this lesion is resistant to multiple modalities of treatment; sometimes the results in cosmetic aspects could be unfavorable by its large size and visible location. Among several therapeutic alternatives,CO2 laser elicits the treatment of widespread areas in one session, with minimal cosmetic consequences. Objective: Determine effectiveness and safety of CO2 laser in patients with VEN.
Commercial support: None identified.
Methods: Descriptive, prospective study. Diagnosis of VEN was confirmed by biopsy. Patients with allergy to local analgesics or diagnosis of epidermal nevus syndrome were excluded. CO2 laser therapy was applied in ultrapulsed mode-300 mJ. Early response to therapy was defined as 80% to 100% of clinical improvement of the lesion in the 2-4 weeks. Patients were followed up and occurrences of hypertrophic scars, hyperpigmentation or hypopigmentation of the lesion were registered during the next 2, 4 and 12 weeks after treatment. Results: Twenty-five patients were included, aged 15.5 6 7.6 years old, being 15 (64%) males and 10 (36%) females. VEN showed a located (12 [88%]) or disseminated distribution (3 [12%]), with a predominance of facial location (22 [34%]). An early response to treatment was achieved in up to 80% of the cases. Complications were observed in 40% of the cases; consisted in recurrence 2 (8%), hypertrophic scar 2 (8%), hyperpigmentation 5 (20%) and hypopigmentation 3 (12%). According to multivariable analysis, the morphology of the VEN was associated to hyperpigmentation (Morphology vs Hyperpigmentation (Exp [B]) 11.2 (95% CI: 1.10-114.4 P ¼ .04); while significant interaction was observed with extension (morphology*extension vs Hyperpigmentation (Exp [B]) 13.5 (95% CI: 1.3-135.9 P ¼ .27). Conclusion: CO2 laser therapy for VEN provides an acceptable clinical response in approximately 80% of the cases, with a relatively low number of complications which seemed to be associated with the basal characteristics of morphology and extension of the lesion. Commercial support: None identified.
5194 5625 Verrucous carcinoma arising within hypertrophic lichen planus Thy Huynh, MD, Northwestern University Dept. of Dermatology; Lauren Graham, MD, Northwestern University Dept. of Dermatology; Joan Guitart, MD, Northwestern University Dept. of Dermatology; Sarah Lyon, BS, Northwestern University Dept. of Dermatology; Joaquin Brieva, MD, Northwestern University Dept. of Dermatology A 16 -year-old Southeast Asian male with lichen planus since age five presented with an evolving tumor on the left pretibia for several months. The lesion responded minimally to potent steroids. Exam revealed a 4 cm lichenified plaque with eroded, hyperkeratotic center and raised borders. Lesional biopsy showed a verrucous process with prominent hyperkeratosis, parakeratosis, papillomatosis, and an infiltrating process resembling pseudoepitheliomatous hyperplasia without significant nuclear atypia. A lichenoid infiltrate was noted. Several bacterial colonies and focal koilocytotic changes were noted. Gram stain showed numerous cocci within the cornified layer. HPV and DPAS were negative. CBC, BMP, LFTs, serum lipids, and Quantiferon gold were normal. A diagnosis of hypertrophic lichen planus with evolving verrucous carcinoma was made. Mohs micrographic surgery was performed in 3 stages, and patient was started on low-dose prednisone daily. One month later, acitretin and hydroxychloroquine were added with significant improvement. After one year he developed steroid-induced acne, so prednisone was replaced with intralesional injections of triamcinolone with continued improvement. He stopped acitretin and hydroxychloroquine afterwards and returns for intralesional triamcinolone injections as needed if symptomatic. Lichen planus is generally a benign cell-mediated skin disorder of unclear etiology with predilection for the pretibial region. Less than 100 reported cases of malignant transformation are found in the literature. Clinical suspicion for malignancy should be noted if lesions ulcerate, evolve into tumors, or become unresponsive to therapy. We present this case due to clinical interest given uncommon sequela of the malignant transformation occurring with hypertrophic lichen planus. Commercial support: None identified.
AB280
J AM ACAD DERMATOL
Violaceous plaques on the buttocks Benjamin Bigbie, MD, University of Oklahoma; Thomas Stasko, MD, University of Oklahoma Case Report: A 52-year-old white male presented to the dermatology clinic in 2011 with a 6-year history of progressive violaceous plaques on the bilateral buttocks. The lesions were pruritic and mildly painful upon sitting. He had been diagnosed with fungal infections and psoriasis in the past, but failed antifungal and topical corticosteroid therapy. He had no family history of similar lesions and was otherwise healthy. Physical exam revealed confluent pink to violaceous flat plaques with peripheral scale present throughout the bilateral gluteal crease and sacral area. Initial biopsy was nondiagnostic but subsequent biopsies revealed a column of parakeratosis which was consistent with a cornoid lamella. Discussion: Porokeratosis ptychotropica is a rare variant of porokeratosis in which violaceous, flat, scaly plaques are present on the bilateral buttocks with reported cases of extension on to the upper thighs and genital area. The lesions tend to follow a chronic course and as with other forms of porokeratosis, treatment is difficult. The entity was first described in 1995 by Lucker et al and name is derived from the Greek words ptyche (fold) and trope (a turning) to describe the plaques which involve the body folds such as the gluteal crease. Histologically the lesions feature multiple cornoid lamellae which differentiate the entity from other subtypes of porokeratosis. There are less than 25 case reports to date and these reports have detailed the difficulty in treating this condition including failure with topical or intralesional steroids, imiquimod, cryotherapy, retinoids, and CO2 laser. However, there are also reports of success with excision and partial thickness removal with a dermatome. Our patient experienced numerous, failed treatments including topical and intralesional steroids, cantharidin, and topical retionoids. He had partial improvement, primarily in thickness and pruritus, with imiquimod, 5-fluorouracil, ingenol mebutate, shave excision, and oral retinoids. Fractionated CO2 laser resulted in initial improvement, but the lesions recurred in a few months. Our patient is currently awaiting removal of lesions with a dermatome. Our patient’s presentation and difficulty with treatment mirror the descriptions of porokeratosis ptychotropia in the literature. Commercial support: None identified.
JUNE 2017