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Unique presentation: Scurvy as a new rash in a patient with hematologic malignancy
3039
2915
Two cases of ophthalmic trigeminal trophic syndrome Ash Mark, MS, East Carolina University, Greenville, NC, United States; Emily Stewart, MD, East Carolina University, Greenville, NC, United States; Charles Phillips, MD, East Carolina University, Greenville, NC, United States Trigeminal trophic syndrome (TTS) is a rare cause of ulcers secondary to skin excoriation due to trigeminal nerve (CN V) anesthesia and paresthesia. TTS typically affects the nasal ala within the maxillary distribution and results from trigeminal ablation and strokes. We report two cases of TTS within the ophthalmic distribution associated with herpes zoster. A 73-year-old male had a herpes zoster outbreak within the left ophthalmic distribution. He was started on acyclovir and also developed a gram negative infection managed with antibiotics. After 10 days, postherpetic neuralgia was noted. He was given 900 mg of gabapentin daily but switched to 150 mg of pregabalin daily at 1 month due to drowsiness. After 7.5 months, a left frontal scalp ulcer developed (negative for HSV1/2 and VZV by DFA). At 8.5 months, a biopsy of the 10-cm ulcer showed lichen simplex chronicus with erosion, ulceration, and mild atypia but no viral cytopathology and no evidence of malignancy. He reported picking the ulcer due to the sensation of a ‘‘hair under it’’ and pain similar to ‘‘hairs being pulled by the root.’’ He was restarted on 900 mg of gabapentin daily. At 14 months, the 9-cm ulcer showed slow healing with silvercel treatment. An 82-year-old male had a herpes zoster outbreak on his left face and scalp, in V1 distribution. He was admitted to the hospital 2-3 weeks later due to malaise, chills, headache, and bilaterally blurred vision. His left eye was swollen by suppurative cellulitis extending from the left orbit to the left occipital scalp, which spread to his right eyelid and scalp. He developed sepsis and respiratory failure. Once recovered, he developed a large scalp ulcer in the V1 distribution. He was started on 600 mg of gabapentin daily. At discharge, the wound was mostly granulated, but after 2 months, a large left scalp ulcer remained and was associated with burning and itching. His left eye visual impairment remained. While gabapentin and other drugs may decrease TTS ulcer inducing paresthesias, management remains challenging. Early detection is critical for patient education and the reduction of paresthesias and self-manipulating behavior. However, the ulcers in the current cases showed slow healing despite gabapentin use. While infrequent, postzoster TTS of ophthalmic CN V can occur and should be included in the differential diagnosis of nonhealing ulcers.
Unilateral, perioral Favre-Racouchot syndrome associated with cigarette smoking: Case and discussion Joseph Dyer, DO, Largo Medical Center, Largo, FL, United States; Mitchell Manway, DO, Manatee Memorial Hospital, Bradenton, FL, United States; Joshua Gapp, MD, Aurora Diagnostics, Aurora, CO, United States; Melinda Greenfield, DO, Albany Dermatology Clinic, Albany, NY, United States Introduction: Favre-Racouchot syndrome (FRS) classically appears as multiple open comedones with solar elastosis in a lateral periorbital distribution. The condition, typically bilateral, is well-described in the literature with a strong association to chronic sun exposure. Descriptions of FRS, however, infrequently recognize another potential risk factor: tobacco use. We highlight the association of FRS and tobacco use through a patient’s peculiar habit of holding a cigarette on one side of her mouth with development of an ipsilateral, perioral comedonal plaque. Case report: A 66-year-old African American female with a 58-pack-year history of cigarette use presented with an asymptomatic eruption beneath the right side of her mouth for 8 months. She denied extensive sun exposure but admitted to a remarkable habit of preferentially placing cigarettes on the right side of her mouth. Inspection of the right lower cutaneous lip revealed small cysts and open comedones in an agminate arrangement. Histopathologic analysis demonstrated several mildly inflamed comedonal cysts embedded in a dermis with extensive nodular solar elastosis. Despite the unusual location and lack of historical actinic damage, a diagnosis of FRS was made. Discussion: FRS affects up to 6% of individuals over age 50, most commonly white males. Its association with severe solar damage is well-established. While cigarette smoke is known to accelerate photoaging through impaired collagen production and increased matrix metalloproteinase activity, few publications have noted tobacco use as an etiologic factor in FRS. One retrospective study found that the likelihood of developing FRS was dose-dependent when comparing heavy versus light smokers. Our case illustrated an exceptional relationship between unilateral cigarette consumption and an ipsilateral FRS-like comedonal plaque. It also led to our hypothesis that chronic thermal damage, as from a lit cigarette, may induce skin changes histologically similar to severe solar elastosis. Commercial support: None identified.
Commercial support: None identified.
2621 Ultrasound-assisted intralesional corticosteroids injection in hidradenitis suppurativa Francisco Javier Garcıa-Martınez, MD, Dermatology Department, Hospital del Sureste, Madrid, Spain; Antonio Martorell-Calatayud, MD, Dermatology Department, Hospital de Manises, Valencia, Spain; Jose Carlos Pascual, MD, Dermatology Department, Hospital General Universitario de Alicante, Alicante, Spain; Eva Vilarrasa, MD, Dermatology Department, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain; Patricia Gonzalez-Mu~ noz, MD, Dermatology Department, University Hospital of Guadalajara, Guadalajara, Spain; Ignacio Hernandez-Arag€ u es, MD, Dermatology Department, Hospital General n, Madrid, Spain; Catiana Silvente, MD, Universitario Gregorio Mara~ no Dermatology Department, Hospital Infanta Leonor, Madrid, Spain; Fernando Alfageme-Roldan, MD, Dermatology Department, Hospital Universitario Puerta de Hierro, Madrid, Spain Background: Hidradenitis suppurativa (HS) is a chronic inflammatory disease of the follicular unit characterized by recurrent, painful, deep-seated skin lesions including inflammatory nodules, abscesses, fistulas and mutilating scarring. Intralesional corticosteroids injection (ICI) for HS references are only mentioned in textbooks or in journal articles. High frequency ultrasound (HFUS) is employed in-depth characterization and staging of HS. Objectives: To evaluate the clinical response of ICI in HS. To identify new applications of HFUS in HS management. Methods: An observational, retrospective, multicenter study of HS patients treated with ICI was conducted in the last year. The data on their management were obtained from their medical records. In this study participated dermatologists specialized in HS and cutaneous HFUS from 8 hospitals in Spain. We considered: complete response (CR) in case of significant clinical impairment or sonographic clearance after 3 months of follow-up, partial response (PR) in case of relapse after clinical impairment within 3 months and null response (NR) if there was worsening or absence of response after the third ICI. Results: We collected 300 HS patients, 49 (16.3%) of which received treatment with ICI. 32 (65%) females and 17 (35%) males. Age range from 12 to 66 y.o. 65 individual lesions were infiltrated, of which 4 (6.2%) were noninflammatory nodules, 24 (36.9%) inflammatory nodules, 28 (43.1%) abscesses and 9 (13.8%) fistulas. The most frequently involved areas were inguinal (32.3%) and axillary (30.8%) regions. Most patients were classified as Hurley stage II (53.8%) and as HS-PGA stage 3 (30.8%). Triamcinolone acetonide (40 mg/ml) was employed in 55 lesions and betamethasone (3 mg/ml) in 10 lesions. Dose most commonly applied range from 0.5 to 1 ml (67.7%). Cs and anesthetics, such as mepivacaine (70.8%) or lidocaine (3.1%) were routinely administered in combination. CR was reached in 46 lesions (70.8%), 16 showed PR (24.6%) and only 3 (4.6%) presented NR. 51 individual lesions underwent HFUS examination before ICI. Association between HFUS and CR showed statistically significant relevance (P \.024).
2982 Unique presentation: Scurvy as a new rash in a patient with hematologic malignancy Valda Kaye, MD, University of Minnesota, Department of Dermatology, Minneapolis, MN, United States; Kelly Aschenbeck, University of Minnesota, Department of Dermatology, Minneapolis, MN, United States; Adam Byrd, MD, University of Minnesota, Department of Dermatology, Minneapolis, MN, United States
Conclusions: Clinical experience supported the use of ICI for individual lesions; however, this therapy should be studied more rigorously. This study shows that ICI is a useful treatment to control all types of lesions in HS and response improves significantly if lesions are previously evaluated with an HFUS.
Vitamin C deficiency, also known as scurvy, is a historically significant disease that continues to be relevant in the current era of medicine. Without corrective treatment, scurvy is invariably fatal. This makes early recognition and supplemental treatment critical, especially in cases with atypical presentations. In this case, a male patient in his 50s with a history of polycythemia vera and recent onset acute myelogenous leukemia developed a new biphasic rash. On his legs, it was a red punctate macular eruption with perifollicular petechiae and keratotic spines. On his abdomen and chest, his rash appeared as light pink macules that occasionally coalesced into patches without overlying scale. No gingival changes were appreciated on exam. Punch skin biopsies and vitamin C levels confirmed a diagnosis of scurvy and oral vitamin C replacements steadily improved the patient’s rash. There is only one other report in the literature of new onset vitamin C deficiency in patients with myelodysplastic disease that has transformed into acute myelogenous leukemia. This case adds to those previously reported and reinforces the need for suspicion of scurvy in modern times.
Commercial support: None identified.
Commercial support: None identified.
AB76
J AM ACAD DERMATOL
MAY 2016
2915
Two cases of ophthalmic trigeminal trophic syndrome Ash Mark, MS, East Carolina University, Greenville, NC, United States; Emily Stewart, MD, East Carolina University, Greenville, NC, United States; Charles Phillips, MD, East Carolina University, Greenville, NC, United States Trigeminal trophic syndrome (TTS) is a rare cause of ulcers secondary to skin excoriation due to trigeminal nerve (CN V) anesthesia and paresthesia. TTS typically affects the nasal ala within the maxillary distribution and results from trigeminal ablation and strokes. We report two cases of TTS within the ophthalmic distribution associated with herpes zoster. A 73-year-old male had a herpes zoster outbreak within the left ophthalmic distribution. He was started on acyclovir and also developed a gram negative infection managed with antibiotics. After 10 days, postherpetic neuralgia was noted. He was given 900 mg of gabapentin daily but switched to 150 mg of pregabalin daily at 1 month due to drowsiness. After 7.5 months, a left frontal scalp ulcer developed (negative for HSV1/2 and VZV by DFA). At 8.5 months, a biopsy of the 10-cm ulcer showed lichen simplex chronicus with erosion, ulceration, and mild atypia but no viral cytopathology and no evidence of malignancy. He reported picking the ulcer due to the sensation of a ‘‘hair under it’’ and pain similar to ‘‘hairs being pulled by the root.’’ He was restarted on 900 mg of gabapentin daily. At 14 months, the 9-cm ulcer showed slow healing with silvercel treatment. An 82-year-old male had a herpes zoster outbreak on his left face and scalp, in V1 distribution. He was admitted to the hospital 2-3 weeks later due to malaise, chills, headache, and bilaterally blurred vision. His left eye was swollen by suppurative cellulitis extending from the left orbit to the left occipital scalp, which spread to his right eyelid and scalp. He developed sepsis and respiratory failure. Once recovered, he developed a large scalp ulcer in the V1 distribution. He was started on 600 mg of gabapentin daily. At discharge, the wound was mostly granulated, but after 2 months, a large left scalp ulcer remained and was associated with burning and itching. His left eye visual impairment remained. While gabapentin and other drugs may decrease TTS ulcer inducing paresthesias, management remains challenging. Early detection is critical for patient education and the reduction of paresthesias and self-manipulating behavior. However, the ulcers in the current cases showed slow healing despite gabapentin use. While infrequent, postzoster TTS of ophthalmic CN V can occur and should be included in the differential diagnosis of nonhealing ulcers.
Unilateral, perioral Favre-Racouchot syndrome associated with cigarette smoking: Case and discussion Joseph Dyer, DO, Largo Medical Center, Largo, FL, United States; Mitchell Manway, DO, Manatee Memorial Hospital, Bradenton, FL, United States; Joshua Gapp, MD, Aurora Diagnostics, Aurora, CO, United States; Melinda Greenfield, DO, Albany Dermatology Clinic, Albany, NY, United States Introduction: Favre-Racouchot syndrome (FRS) classically appears as multiple open comedones with solar elastosis in a lateral periorbital distribution. The condition, typically bilateral, is well-described in the literature with a strong association to chronic sun exposure. Descriptions of FRS, however, infrequently recognize another potential risk factor: tobacco use. We highlight the association of FRS and tobacco use through a patient’s peculiar habit of holding a cigarette on one side of her mouth with development of an ipsilateral, perioral comedonal plaque. Case report: A 66-year-old African American female with a 58-pack-year history of cigarette use presented with an asymptomatic eruption beneath the right side of her mouth for 8 months. She denied extensive sun exposure but admitted to a remarkable habit of preferentially placing cigarettes on the right side of her mouth. Inspection of the right lower cutaneous lip revealed small cysts and open comedones in an agminate arrangement. Histopathologic analysis demonstrated several mildly inflamed comedonal cysts embedded in a dermis with extensive nodular solar elastosis. Despite the unusual location and lack of historical actinic damage, a diagnosis of FRS was made. Discussion: FRS affects up to 6% of individuals over age 50, most commonly white males. Its association with severe solar damage is well-established. While cigarette smoke is known to accelerate photoaging through impaired collagen production and increased matrix metalloproteinase activity, few publications have noted tobacco use as an etiologic factor in FRS. One retrospective study found that the likelihood of developing FRS was dose-dependent when comparing heavy versus light smokers. Our case illustrated an exceptional relationship between unilateral cigarette consumption and an ipsilateral FRS-like comedonal plaque. It also led to our hypothesis that chronic thermal damage, as from a lit cigarette, may induce skin changes histologically similar to severe solar elastosis. Commercial support: None identified.
Commercial support: None identified.
2621 Ultrasound-assisted intralesional corticosteroids injection in hidradenitis suppurativa Francisco Javier Garcıa-Martınez, MD, Dermatology Department, Hospital del Sureste, Madrid, Spain; Antonio Martorell-Calatayud, MD, Dermatology Department, Hospital de Manises, Valencia, Spain; Jose Carlos Pascual, MD, Dermatology Department, Hospital General Universitario de Alicante, Alicante, Spain; Eva Vilarrasa, MD, Dermatology Department, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain; Patricia Gonzalez-Mu~ noz, MD, Dermatology Department, University Hospital of Guadalajara, Guadalajara, Spain; Ignacio Hernandez-Arag€ u es, MD, Dermatology Department, Hospital General n, Madrid, Spain; Catiana Silvente, MD, Universitario Gregorio Mara~ no Dermatology Department, Hospital Infanta Leonor, Madrid, Spain; Fernando Alfageme-Roldan, MD, Dermatology Department, Hospital Universitario Puerta de Hierro, Madrid, Spain Background: Hidradenitis suppurativa (HS) is a chronic inflammatory disease of the follicular unit characterized by recurrent, painful, deep-seated skin lesions including inflammatory nodules, abscesses, fistulas and mutilating scarring. Intralesional corticosteroids injection (ICI) for HS references are only mentioned in textbooks or in journal articles. High frequency ultrasound (HFUS) is employed in-depth characterization and staging of HS. Objectives: To evaluate the clinical response of ICI in HS. To identify new applications of HFUS in HS management. Methods: An observational, retrospective, multicenter study of HS patients treated with ICI was conducted in the last year. The data on their management were obtained from their medical records. In this study participated dermatologists specialized in HS and cutaneous HFUS from 8 hospitals in Spain. We considered: complete response (CR) in case of significant clinical impairment or sonographic clearance after 3 months of follow-up, partial response (PR) in case of relapse after clinical impairment within 3 months and null response (NR) if there was worsening or absence of response after the third ICI. Results: We collected 300 HS patients, 49 (16.3%) of which received treatment with ICI. 32 (65%) females and 17 (35%) males. Age range from 12 to 66 y.o. 65 individual lesions were infiltrated, of which 4 (6.2%) were noninflammatory nodules, 24 (36.9%) inflammatory nodules, 28 (43.1%) abscesses and 9 (13.8%) fistulas. The most frequently involved areas were inguinal (32.3%) and axillary (30.8%) regions. Most patients were classified as Hurley stage II (53.8%) and as HS-PGA stage 3 (30.8%). Triamcinolone acetonide (40 mg/ml) was employed in 55 lesions and betamethasone (3 mg/ml) in 10 lesions. Dose most commonly applied range from 0.5 to 1 ml (67.7%). Cs and anesthetics, such as mepivacaine (70.8%) or lidocaine (3.1%) were routinely administered in combination. CR was reached in 46 lesions (70.8%), 16 showed PR (24.6%) and only 3 (4.6%) presented NR. 51 individual lesions underwent HFUS examination before ICI. Association between HFUS and CR showed statistically significant relevance (P \.024).
2982 Unique presentation: Scurvy as a new rash in a patient with hematologic malignancy Valda Kaye, MD, University of Minnesota, Department of Dermatology, Minneapolis, MN, United States; Kelly Aschenbeck, University of Minnesota, Department of Dermatology, Minneapolis, MN, United States; Adam Byrd, MD, University of Minnesota, Department of Dermatology, Minneapolis, MN, United States
Conclusions: Clinical experience supported the use of ICI for individual lesions; however, this therapy should be studied more rigorously. This study shows that ICI is a useful treatment to control all types of lesions in HS and response improves significantly if lesions are previously evaluated with an HFUS.
Vitamin C deficiency, also known as scurvy, is a historically significant disease that continues to be relevant in the current era of medicine. Without corrective treatment, scurvy is invariably fatal. This makes early recognition and supplemental treatment critical, especially in cases with atypical presentations. In this case, a male patient in his 50s with a history of polycythemia vera and recent onset acute myelogenous leukemia developed a new biphasic rash. On his legs, it was a red punctate macular eruption with perifollicular petechiae and keratotic spines. On his abdomen and chest, his rash appeared as light pink macules that occasionally coalesced into patches without overlying scale. No gingival changes were appreciated on exam. Punch skin biopsies and vitamin C levels confirmed a diagnosis of scurvy and oral vitamin C replacements steadily improved the patient’s rash. There is only one other report in the literature of new onset vitamin C deficiency in patients with myelodysplastic disease that has transformed into acute myelogenous leukemia. This case adds to those previously reported and reinforces the need for suspicion of scurvy in modern times.
Commercial support: None identified.
Commercial support: None identified.
AB76
J AM ACAD DERMATOL
MAY 2016