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Bilateral ear rash and indurated subcutaneous nodules in a febrile neutropenic patient
5144
4348
Bilateral ear rash and indurated subcutaneous nodules in a febrile neutropenic patient Marta Prieto-Barrios, MD, Hospital Universitario 12 de Octubre. Institute I+12. Universidad Complutense; Virginia Velasco-Tamariz, MD, Hospital Universitario 12 de Octubre. Institute I+12. Universidad Complutense; Sara Burillo-Martinez, MD, Hospital Universitario 12 de Octubre. Institute I+12. Universidad Complutense; Carlos Morales-Raya, MD, Hospital Universitario 12 de Octubre. Institute I+12. Universidad Complutense; Vanessa Gargallo-Moneva, MD, Hospital Universitario 12 de Octubre. Institute I+12. Universidad Complutense; Jose Luis Rodrıguez-Peralto, PhD, Hospital Universitario 12 de Octubre. Institute I+12. Universidad Complutense; Pablo Luis Ortiz-Romero, PhD, Hospital Universitario 12 de Octubre. Institute I+12. Universidad Complutense
Blaschkoid pityriasis rosea Murat Borlu, MD, Erciyes University; K€ ubra Y€ uce Atamulu, MD, Kayseri State Hospital; demet Kartal, MD, Erciyes University; Salih Levent C ¸ inar, MD, Erciyes University Introduction and Objective: Pityriasis rosea (PR) is an acute, self limiting and inflammatory skin disorder which is mostly seen in childhood and young adulthood. Mosaicism is the presence of difference in an individual of two or more cell lines that are karyotypically or genotypically distinct and are derived from a single zygote. Blaschko lines are formed during the migration process in embryogenesis by the borders of normal or mutant skin cells. In this case report we aim to present a case of pityriasis rosea in which the lesions fit the Blaschko lines.
Introduction: Cytarabine, also known as ara-C, is a pyrimidine antagonist used in the treatment of hematologic malignancies. Most frequent cytarabine-induced cutaneous adverse effects are a morbilliform eruption and acral erythema. We present a new case of the so called ara-C ears, a skin adverse-event which has so far been considered a rare subtype of acral erythema. With our report, we describe the actual histopathological findings of ara-C ears for the first time. Case report: A 34-year-old male was diagnosed of acute myeloid leukemia (AML) after 10 days of fever and malaise. Induction chemotherapy with idarubicin and cytarabine was started. Our dermatology service was notified of the appearance of indurated subcutaneous nodules of 1 week evolution. Neutropenic fever persisted and on day 6 he developed swelling and a symmetrical maculopapular rash with vesiculation on both his ears. Both biopsies were consistent with deep and superficial Sweet syndrome respectively. Discussion: Sweet syndrome is associated to hematologic neoplasm in up to 20% of patients. Certain cytokines such as IL-1, IL-6 and GM-CSF could be involved in its development. Krulder et al (1990) described 8 patients had AML with bilateral swelling and erythema of the ears which they associated to cytarabine treatment. Anesi et al (2012) reported a similar case of what they named ara-C ears. They empirically assumed it was an atypical variant of acral erythema, but no biopsy was performed. Conversely, 3 other patients have been reported with AML under treatment with cytarabine and a rash affecting both ears with histologic confirmation of neutrophilic dermatosis. Of the 13 mentioned cases (including ours), all patients suffered from AML and were receiving cytarabine. 12 had bilateral and 1 unilateral expression. 5 presented auricular affection alone, while 8 presented other type of cutaneous lesions. Only 1 received GM-CSF. None recurred. No other case of superficial Sweet syndrome of the ears was found. In our opinion, all cases correspond to a same clinical entity, which is indeed a distinctive manifestation of Sweet syndrome. Despite ara-C has been accused of inducing Sweet syndrome, no compelling evidence exists and, in the same way, we cannot affirm it plays a pathogenetic role in this case. Conclusion: Recognizing this pattern may allow physicians to properly diagnose this cause of febrile neutropenia and to promptly establish appropriate treatment avoiding other aggressive maneuvers.
Case: A 21-year-old male patient applied to our outpatient clinic with a complaint of erythematous rash on his left foot, leg, thigh and buttock ongoing for the last three weeks. He did not have any complaints. He also did not have the history of recent medication, infection or contact exposure. His physical examination was normal. On his dermatologic examination, erythematous, linear and thin-scaled patches drew attention. These lesions matched the left foot, left leg posterior, left thigh posterior and left gluteal Blaschko lines. A skin biopsy was taken with pre-diagnoses of pityriasis rosea and inflammatory linear verrucous epidermal nevus (ILVEN). Upon histopathological examination slight epidermal acanthosis and hyperkeratosis, upper dermal perivascular lymphocytes and melanophages were seen. With the help of clinical and histopathological findings we reached the diagnosis of blaschkoid pityriasis rosea. The patient was given a single dose of betamethasone dipropionate intramuscularly and betamethasone dipropionate ointment for a period of two weeks BID. At the end of two weeks the patient’s lesions disappeared almost completely. Discussion: PR is a self limiting, inflammatory skin disorder. Although its etiology is unknown, infectious agents like HHV-6 and HHV-7 have been charged. Rare forms like vesicular, purpuric, urticarial and erythema multiformeelike pityriasis rosea are presented in the literature. Also there are forms named according to distribution style; such as inverse, unilateral, localized, palmoplantar. Blaschkoid type has been published in the literature only once. Linear psoriasis, linear lichen, lichen striatus, adult blaschitis and ILVEN are the skin disorders that must be differentiated from Blaschkoid pityriasis rosea. Mosaicism is the presence of difference in an individual of two or more cell lines that are karyotypically or genotypically distinct and are derived from a single zygote. Half-cromatid mutations, ionization, post-zygotic mutations, chimerism, chromosomal nondisjunction are the causes of mosaicism. Besides Blaschko lines, mosaicism causes draftboard pattern, phylloid pattern, patchy pattern sparing the midline and lateralization in the skin. After clinical and histopathological examination, the patient was given the diagnosis of Blaschkoid PR. In case of linear, inflammatory skin lesions mosaicism and as a result Blaschkoid PR must be in mind. Commercial support: None identified.
Commercial support: None identified.
4596
5430 Black hairy tongue due to metronidazole Sandy Kuo, MD, Department of Dermatology, University of California, Davis; Nasim Fazel, MD, Department of Dermatology, University of California, Davis Background: Black hairy tongue is a benign condition more commonly seen in older individuals as a result of hyperkeratinization of the filiform papillae of the dorsal tongue. It is caused by a lack of stimulation and abrasion to the dorsal tongue and build-up of keratin. Predisposing factors include smoking, poor oral hygiene, xerostomia, and medications that contain bismuth. Chronic or extensive use of antibiotics has also been noted to be a cause, especially the use of linezolid. However, its occurrence in relation to metronidazole has only been reported once in combination with lansoprazole. Here we report a case of black hairy tongue due to metronidazole exposure alone. Case: A 65-year-old male with a past medical history of diabetes mellitus and end stage renal disease presented to the UC Davis outpatient dermatology clinic with a brown plaque on the dorsal tongue (Figure 1). He stated that it had been present for about two weeks, initially noticed by his primary care physician during routine physical examination. He had not noticed it prior to this because it was asymptomatic. He was given nystatin oral suspension, which he had been using for four days with minimal improvement. He was a former smoker, and had not used cigarettes for the past 20 years. Of note, he recently completed two 14-day courses of metronidazole to treat Clostridium difficile diarrhea, in which his last dose was one month prior to presentation. He was started on a regimen of topical tretinoin 0.1% cream to the tongue nightly and gently brushing the tongue with a hard bristle toothbrush daily with near resolution in a month.
Blindness reversal with oral acitretin therapy in a patient with keratitis-ichthyosis-deafness (KID) syndrome Alexander Davis, BS, Florida State University College of Medicine; Christopher Wolfe, DO, Florida State University College of Medicine, Division of Dermatology; Tarek Shaath, MD, Florida State University College of Medicine, Division of Dermatology; George Cohen, MD, Florida State University College of Medicine, Division of Dermatology Background: Keratitis-ichthyosis-deafness (KID) syndrome is a rare genodermatosis with approximately 100 published cases. It is inherited in an autosomal dominant fashion, however, more than 90% of cases are due to sporadic mutations predominantly GJB2, which encodes connexin 26. The characteristic clinical triad includes congenital bilateral sensorineural hearing loss, vascularizing keratitis, and erythrokeratoderma. Treatment with acitretin has been reported to clear hyperkeratotic ichthyotic lesions with little effect on visual impairment. Case report: A 34-year-old white male diagnosed with KID syndrome confirmed by GJB2 gene analysis presented to our clinic. Medications included loteprednol ophthalmic ointment, triamcinolone cream, urea cream, and oral doxycycline. Physical examination revealed hyperkeratotic plaques on his trunk, neck, face, extremities, and palmoplantar keratoderma. Dystrophic small and absent maxillary teeth were noted. Sensorineural hearing loss was present since childhood. Visual acuity testing demonstrated 20/1200 and 20/800 vision of the right and left eye respectively. Acitretin was initiated at a dose of 25 mg once daily. Visual acuity after 7 months of acitretin therapy was 20/200 in both eyes with correction to 20/40 with gas permeable contact lenses. Punctal plugs were placed by ophthalmology at this time. Near resolution of cutaneous lesions was also noted.
Discussion: Black hairy tongue is an acquired benign condition characterized by hair-like appearance of the dorsal tongue. The use of antibiotics has been linked to this disorder and thought to be due to alteration of the oral flora. Management primarily involves mechanical debridement, oral hygiene, and elimination of potential causative agents. In most cases, elimination of any possible underlying factors and proper oral hygiene measures is adequate to treat this condition.
Discussion: Ocular involvement occurs in 95% of patients with KID syndrome with neovascularization and scarring resulting in progressive decline in visual acuity that can lead to blindness. To date acitretin has demonstrated efficacy in the treatment of cutaneous lesions, whereas other oral retinoids have yielded mixed results. None of the oral retinoids have reported efficacy in treating visual impairment in KID syndrome. We report this case to highlight the significant impact in quality-of-life for our patient with reversal of blindness using oral acitretin and corrective gas permeable contact lenses. The use of punctal plugs likely limited the adverse ophthalmologic side effects associated with systemic retinoid therapy in our patient. Our case is unique in that improvement in visual acuity with the use of acitretin or other systemic retinoids in KID syndrome has not yet been reported in the literature.
Commercial support: None identified.
Commercial support: None identified.
AB56
J AM ACAD DERMATOL
JUNE 2017
4348
Bilateral ear rash and indurated subcutaneous nodules in a febrile neutropenic patient Marta Prieto-Barrios, MD, Hospital Universitario 12 de Octubre. Institute I+12. Universidad Complutense; Virginia Velasco-Tamariz, MD, Hospital Universitario 12 de Octubre. Institute I+12. Universidad Complutense; Sara Burillo-Martinez, MD, Hospital Universitario 12 de Octubre. Institute I+12. Universidad Complutense; Carlos Morales-Raya, MD, Hospital Universitario 12 de Octubre. Institute I+12. Universidad Complutense; Vanessa Gargallo-Moneva, MD, Hospital Universitario 12 de Octubre. Institute I+12. Universidad Complutense; Jose Luis Rodrıguez-Peralto, PhD, Hospital Universitario 12 de Octubre. Institute I+12. Universidad Complutense; Pablo Luis Ortiz-Romero, PhD, Hospital Universitario 12 de Octubre. Institute I+12. Universidad Complutense
Blaschkoid pityriasis rosea Murat Borlu, MD, Erciyes University; K€ ubra Y€ uce Atamulu, MD, Kayseri State Hospital; demet Kartal, MD, Erciyes University; Salih Levent C ¸ inar, MD, Erciyes University Introduction and Objective: Pityriasis rosea (PR) is an acute, self limiting and inflammatory skin disorder which is mostly seen in childhood and young adulthood. Mosaicism is the presence of difference in an individual of two or more cell lines that are karyotypically or genotypically distinct and are derived from a single zygote. Blaschko lines are formed during the migration process in embryogenesis by the borders of normal or mutant skin cells. In this case report we aim to present a case of pityriasis rosea in which the lesions fit the Blaschko lines.
Introduction: Cytarabine, also known as ara-C, is a pyrimidine antagonist used in the treatment of hematologic malignancies. Most frequent cytarabine-induced cutaneous adverse effects are a morbilliform eruption and acral erythema. We present a new case of the so called ara-C ears, a skin adverse-event which has so far been considered a rare subtype of acral erythema. With our report, we describe the actual histopathological findings of ara-C ears for the first time. Case report: A 34-year-old male was diagnosed of acute myeloid leukemia (AML) after 10 days of fever and malaise. Induction chemotherapy with idarubicin and cytarabine was started. Our dermatology service was notified of the appearance of indurated subcutaneous nodules of 1 week evolution. Neutropenic fever persisted and on day 6 he developed swelling and a symmetrical maculopapular rash with vesiculation on both his ears. Both biopsies were consistent with deep and superficial Sweet syndrome respectively. Discussion: Sweet syndrome is associated to hematologic neoplasm in up to 20% of patients. Certain cytokines such as IL-1, IL-6 and GM-CSF could be involved in its development. Krulder et al (1990) described 8 patients had AML with bilateral swelling and erythema of the ears which they associated to cytarabine treatment. Anesi et al (2012) reported a similar case of what they named ara-C ears. They empirically assumed it was an atypical variant of acral erythema, but no biopsy was performed. Conversely, 3 other patients have been reported with AML under treatment with cytarabine and a rash affecting both ears with histologic confirmation of neutrophilic dermatosis. Of the 13 mentioned cases (including ours), all patients suffered from AML and were receiving cytarabine. 12 had bilateral and 1 unilateral expression. 5 presented auricular affection alone, while 8 presented other type of cutaneous lesions. Only 1 received GM-CSF. None recurred. No other case of superficial Sweet syndrome of the ears was found. In our opinion, all cases correspond to a same clinical entity, which is indeed a distinctive manifestation of Sweet syndrome. Despite ara-C has been accused of inducing Sweet syndrome, no compelling evidence exists and, in the same way, we cannot affirm it plays a pathogenetic role in this case. Conclusion: Recognizing this pattern may allow physicians to properly diagnose this cause of febrile neutropenia and to promptly establish appropriate treatment avoiding other aggressive maneuvers.
Case: A 21-year-old male patient applied to our outpatient clinic with a complaint of erythematous rash on his left foot, leg, thigh and buttock ongoing for the last three weeks. He did not have any complaints. He also did not have the history of recent medication, infection or contact exposure. His physical examination was normal. On his dermatologic examination, erythematous, linear and thin-scaled patches drew attention. These lesions matched the left foot, left leg posterior, left thigh posterior and left gluteal Blaschko lines. A skin biopsy was taken with pre-diagnoses of pityriasis rosea and inflammatory linear verrucous epidermal nevus (ILVEN). Upon histopathological examination slight epidermal acanthosis and hyperkeratosis, upper dermal perivascular lymphocytes and melanophages were seen. With the help of clinical and histopathological findings we reached the diagnosis of blaschkoid pityriasis rosea. The patient was given a single dose of betamethasone dipropionate intramuscularly and betamethasone dipropionate ointment for a period of two weeks BID. At the end of two weeks the patient’s lesions disappeared almost completely. Discussion: PR is a self limiting, inflammatory skin disorder. Although its etiology is unknown, infectious agents like HHV-6 and HHV-7 have been charged. Rare forms like vesicular, purpuric, urticarial and erythema multiformeelike pityriasis rosea are presented in the literature. Also there are forms named according to distribution style; such as inverse, unilateral, localized, palmoplantar. Blaschkoid type has been published in the literature only once. Linear psoriasis, linear lichen, lichen striatus, adult blaschitis and ILVEN are the skin disorders that must be differentiated from Blaschkoid pityriasis rosea. Mosaicism is the presence of difference in an individual of two or more cell lines that are karyotypically or genotypically distinct and are derived from a single zygote. Half-cromatid mutations, ionization, post-zygotic mutations, chimerism, chromosomal nondisjunction are the causes of mosaicism. Besides Blaschko lines, mosaicism causes draftboard pattern, phylloid pattern, patchy pattern sparing the midline and lateralization in the skin. After clinical and histopathological examination, the patient was given the diagnosis of Blaschkoid PR. In case of linear, inflammatory skin lesions mosaicism and as a result Blaschkoid PR must be in mind. Commercial support: None identified.
Commercial support: None identified.
4596
5430 Black hairy tongue due to metronidazole Sandy Kuo, MD, Department of Dermatology, University of California, Davis; Nasim Fazel, MD, Department of Dermatology, University of California, Davis Background: Black hairy tongue is a benign condition more commonly seen in older individuals as a result of hyperkeratinization of the filiform papillae of the dorsal tongue. It is caused by a lack of stimulation and abrasion to the dorsal tongue and build-up of keratin. Predisposing factors include smoking, poor oral hygiene, xerostomia, and medications that contain bismuth. Chronic or extensive use of antibiotics has also been noted to be a cause, especially the use of linezolid. However, its occurrence in relation to metronidazole has only been reported once in combination with lansoprazole. Here we report a case of black hairy tongue due to metronidazole exposure alone. Case: A 65-year-old male with a past medical history of diabetes mellitus and end stage renal disease presented to the UC Davis outpatient dermatology clinic with a brown plaque on the dorsal tongue (Figure 1). He stated that it had been present for about two weeks, initially noticed by his primary care physician during routine physical examination. He had not noticed it prior to this because it was asymptomatic. He was given nystatin oral suspension, which he had been using for four days with minimal improvement. He was a former smoker, and had not used cigarettes for the past 20 years. Of note, he recently completed two 14-day courses of metronidazole to treat Clostridium difficile diarrhea, in which his last dose was one month prior to presentation. He was started on a regimen of topical tretinoin 0.1% cream to the tongue nightly and gently brushing the tongue with a hard bristle toothbrush daily with near resolution in a month.
Blindness reversal with oral acitretin therapy in a patient with keratitis-ichthyosis-deafness (KID) syndrome Alexander Davis, BS, Florida State University College of Medicine; Christopher Wolfe, DO, Florida State University College of Medicine, Division of Dermatology; Tarek Shaath, MD, Florida State University College of Medicine, Division of Dermatology; George Cohen, MD, Florida State University College of Medicine, Division of Dermatology Background: Keratitis-ichthyosis-deafness (KID) syndrome is a rare genodermatosis with approximately 100 published cases. It is inherited in an autosomal dominant fashion, however, more than 90% of cases are due to sporadic mutations predominantly GJB2, which encodes connexin 26. The characteristic clinical triad includes congenital bilateral sensorineural hearing loss, vascularizing keratitis, and erythrokeratoderma. Treatment with acitretin has been reported to clear hyperkeratotic ichthyotic lesions with little effect on visual impairment. Case report: A 34-year-old white male diagnosed with KID syndrome confirmed by GJB2 gene analysis presented to our clinic. Medications included loteprednol ophthalmic ointment, triamcinolone cream, urea cream, and oral doxycycline. Physical examination revealed hyperkeratotic plaques on his trunk, neck, face, extremities, and palmoplantar keratoderma. Dystrophic small and absent maxillary teeth were noted. Sensorineural hearing loss was present since childhood. Visual acuity testing demonstrated 20/1200 and 20/800 vision of the right and left eye respectively. Acitretin was initiated at a dose of 25 mg once daily. Visual acuity after 7 months of acitretin therapy was 20/200 in both eyes with correction to 20/40 with gas permeable contact lenses. Punctal plugs were placed by ophthalmology at this time. Near resolution of cutaneous lesions was also noted.
Discussion: Black hairy tongue is an acquired benign condition characterized by hair-like appearance of the dorsal tongue. The use of antibiotics has been linked to this disorder and thought to be due to alteration of the oral flora. Management primarily involves mechanical debridement, oral hygiene, and elimination of potential causative agents. In most cases, elimination of any possible underlying factors and proper oral hygiene measures is adequate to treat this condition.
Discussion: Ocular involvement occurs in 95% of patients with KID syndrome with neovascularization and scarring resulting in progressive decline in visual acuity that can lead to blindness. To date acitretin has demonstrated efficacy in the treatment of cutaneous lesions, whereas other oral retinoids have yielded mixed results. None of the oral retinoids have reported efficacy in treating visual impairment in KID syndrome. We report this case to highlight the significant impact in quality-of-life for our patient with reversal of blindness using oral acitretin and corrective gas permeable contact lenses. The use of punctal plugs likely limited the adverse ophthalmologic side effects associated with systemic retinoid therapy in our patient. Our case is unique in that improvement in visual acuity with the use of acitretin or other systemic retinoids in KID syndrome has not yet been reported in the literature.
Commercial support: None identified.
Commercial support: None identified.
AB56
J AM ACAD DERMATOL
JUNE 2017