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What is the world of dermatology talking about? Text analysis of abstracts from the 23rd World Congress of Dermatology
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5685
What is the world of dermatology talking about? Text analysis of abstracts from the 23rd World Congress of Dermatology Diana Diao, MD, Department of Dermatology and Skin Science, University of British Columbia; David McLean, MD, Department of Dermatology and Skin Science, University of British Columbia; Harvey Lui, MD, Department of Dermatology and Skin Science, University of British Columbia
Which drug is it? Utility of lymphocyte transformation testing in the diagnosis of drug hypersensitivity in a university teaching hospital Jennifer Boggs, MBBCh, Connolly Hospital Blanchardstown; Marina O’Kane, MBBCh, Connolly Hospital Blanchardstown Introduction: Adverse cutaneous reactions to drugs are common, affecting 2 to 3% of hospitalized patients, while an estimated 1 in 1000 hospitalized patients develop a serious cutaneous drug reaction. However diagnosis of drug hypersensitivity is difficult, particularly type IV reactions as they are delayed in onset sometimes by days to weeks following exposure to the culprit drug and the reaction can take many different forms, varying from inconvenient to life threatening. Type IV reactions may be evaluated with various diagnostic tests including lymphocyte transformation testing (LTT), which aims to detect circulating drug specific memory T cells, which proliferate upon drug stimulation. Objectives: We aimed to evaluate whether LTT helped to define the incriminating drug in our patients with drug reactions.
Background: The quadrennial World Congress of Dermatology (WCD) of the International League of Dermatologic Societies (ILDS) showcases topics from across the globe. The spectrum of abstracts submitted and presented at the WCD provides insights into disease priorities, research developments, and therapeutic trends according to different world regions. Objective: To identify and compare prevailing diseases of interest and trends in therapeutics from world regions by text-based abstract analysis. Methods: Text from the abstract bodies of all proffered and accepted oral and poster presentations was categorized according to five geographic ILDS regions [1. Asia Pacific (Asia-Pac); 2. Europe (Eur); 3. Latin America (Lat-Amer); 4. South Asia, Middle East, and Africa (SAsia-MEast-Africa); and 5. USA and Canada (US-Can)] based on the location of the submitting author. Abstracts from each region were analyzed for word frequency, and the top 10 most frequent words or closely related word clusters denoting either a diagnosis or treatment in each region were tabulated by relative and absolute frequencies. Results: A total of 4,505 abstracts containing 970,070 words were systematically analyzed from 104 countries (counts by ILDS region: Asia-Pac, 924; Eur, 1275; LatAmer, 442; SAsia-MEast-Afr, 766; US-Can, 1074). The most frequent disease entities across all 5 regions were psoriasis, acne, and dermatitis. Melanoma and squamous cell carcinoma ranked in the top 10 in all regions except SAsia-MEast-Afr. Basal cell carcinoma ranked in the top 10 in Eur and US-Can only. Words related to infectious diseases such as ‘leprosy’, ‘tuberculosis’, ‘wart’, and ‘fungal’ were absent from the top 10 lists in Europe and the US-Can. For therapeutics, ‘topical’, ‘corticosteroid’, and words related to light-based modalities (eg, UVB, UVA, phototherapy, PDT, fractional) appeared most frequently in all 5 regions. ‘Excision’ was the most common surgical word in 4 regions with ‘Mohs’ being the most common for the USCan. Methotrexate appeared in the top 10 list for all regions except the Asia-Pac. Biologics were most prevalent in the US-Can, representing 3 of the top 10 list, but were absent from the top 10 lists in Lat-Amer and SAsia-MEast-Afr. Conclusions: There are regional similarities and differences in dermatologic disease entities and therapeutic trends of interest. Textual analysis can assist in quantifying the relative priorities ascribed by dermatologists to topics of interest. Commercial support: None identified.
Methods: We conducted a retrospective study of all patients from our department who had a LTT performed between 01/03/2009- 01/03/2016. Patients were identified from a patient database (n ¼ 34) and a chart review was performed. Results: Of the 34 patients, 21 were female and 13 were male, with a mean age of 52.8 years (range 18-85 years). 97% presented with a skin eruption, whilst 20% had systemic symptoms including fever, nausea, and malaise at presentation. The predominant morphology was maculopapular, 29%. 88% patients required admission to hospital. The mean duration between drug exposure and onset of reaction was 10.9 days. Of the 56% that underwent skin biopsy, 79% had histology consistent with a drug eruption. The average number of drugs requested for LTT per patient was 3 (range 1-9 drugs). 76% patients had a positive LTT to one or more drugs. 69% (18/26) had positive LTT reactions to an antibiotic, of which the majority were Beta-lactams, comprising 61% (11/18). 20% had a Severe Cutaneous Adverse Reaction (SCAR), of this group 100% had a positive LTT. The causative drugs were withdrawn once the diagnosis of drug reactions was made in 82%, followed by significant improvement in the majority of patients (82%). There were 2 fatalities secondary to multiorgan failure and 1 patient developed interstitial nephritis requiring hemodialysis. Two known accidental re-challenges occurred, both with severer recurrences of the skin eruption, with one patient developing DRESS. Conclusion: We found that a positive LTT was a valuable contribution in the diagnosis of drug allergy and in identifying the drug involved in the reaction. However as the sensitivity of LTT is limited, a negative LTT cannot exclude a drug hypersensitivity. False negatives may also occur with mistiming of the test and in patients on high dose systemic corticosteroids. As reflected in our study there is a significant morbidity and mortality associated with drug hypersensitivity reactions and LTT is a useful test in the armamentarium of the dermatologist when faced with this challenging clinical situation. Commercial support: None identified.
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5551 When a skin tag is not just a skin tag: Birt-Hogg-Dub e syndrome Charlotte Gollins, MBChB, St George Hospital; Steven Kossard, MBBS, Kossard Dermatopathologists; Dedee Murrell, MBBCh, University of New South Wales History: A 50-year-old gentleman of Macedonian origin was admitted to hospital with a left sided spontaneous pneumothorax, for which he underwent thoracoscopy and a wedge resection. Whilst in hospital it was noted that he had multiple facial papules and axillary skin tags. The patient reported that these had been present for decades. He had a history of acne as a teenager and had previously had a right sided pneumothorax 8 years earlier. His mother and grandmother had also had similar lesions on their faces but there was no family history of cancers or pulmonary or renal problems.
Widespread biphasic amyloidosis related to ipilimumab treatment for metastatic melanoma Virginia Velasco-Tamariz, MD, Department of Dermatology, Hospital 12 de Octubre; Marta Prieto-Barrios, MD, Department of Dermatology, Hospital 12 de Octubre; Fatima Tous-Romero, MD, Department of Dermatology, Hospital 12 de Octubre; Sara Burillo-Martınez, MD, Department of Dermatology, Hospital 12 de Octubre; Carlos Morales-Raya, MD, Department of Dermatology, Hospital 12 de Octubre; Raquel Arag on-Miguel, MD, Department of Dermatology, Hospital 12 de Octubre; Pablo Ortiz-Romero, PhD, Department of Dermatology, Hospital 12 de Octubre Introduction: Ipilimumab is a fully human monoclonal antibody approved for treatment of metastatic or unresectable melanoma. Due to its immunomodulatory mechanism of action, it has been associated with multiple immune-related adverse events (irAEs). We report the first case of a patient under treatment with Ipilimumab who developed classic cutaneous and histopathological findings of lichenoid and macular amyloidosis (biphasic amyloidosis).
Discussion: These histologic findings, along with the history of bilateral spontaneous pneumothoraces, strongly indicate a diagnosis of Birt-Hogg-Dube syndrome. This rare autosomal dominant genetic disorder is caused by a mutation in the folliculin (FLCN) gene and leads to bilateral lung cysts, skin lesions (most commonly fibrofolliculomas, trichodischomas, and achrocordons) and renal tumors. It is vital to consider this diagnosis in patients with multiple abnormal papules and skin tags if they appear with other suggestive signs. Once a diagnosis is made the patient can be closely monitored, which allows for earlier treatment of any complications that occur.
Case report: A 60-year-old male with an acral melanoma with a Breslow of 9.67 mm, underwent amputation and sentinel node biopsy which resulted negative. Two years later, image studies revealed lymph node and pulmonary metastases. As BRAF V600E mutation was negative, he was started on a course of ipilimumab. From the start of treatment he suffered from moderate pruritus. After the fourth dose, he abruptly developed extensive macular brown symmetrical pigmentation on his upper chest and back, and also papules on his arms, back, buttocks and legs. Histologic examination revealed eosinophilic material in upper dermis. Inmunohistochemmical staining was positive for cytokeratins using monoclonal antibody CK-903, confirming the diagnosis of biphasic amyloidosis. The lesions showed mild improvement with topical corticosteroids. Image studies revealed tumoral progression, so treatment was switched to pembrolizumab. However, pruritus persisted and the eruption worsened. Discussion. Even though ipilimumab has been associated with the highest rates of pruritus compared with other targeted therapies, prurigo or lichen simplex chronicus like-lesions have not been reported. Although the etiology of lichen amyloidosis (LA) is not yet fully understood, deposition of the amyloid in papillary dermis is thought to be consequence of necrosis of keratinocytes due to chronic rubbing of skin. Nevertheless, some authors have suggested that underlying immune-mediated factors may be implicated, because of the simultaneous occurrence with various autoimmune/immune disorders. In our case, it is surprising that the lesions developed so fast, taking into account that this dermatosis is considered a manifestation of chronic scratching. We cannot elucidate if the development of cutaneous amyloidosis is only consequence of pruritus, a known immune-related adverse effect of immunotherapy and subsequent scratching, or maybe our case-patient can reinforce the immune-related pathogenesis theory of amyloid deposition.
Commercial support: None identified.
Commercial support: None identified.
Physical Signs: On examination he had multiple skin colored papules on his face, neck, back and arms. He also had brown axillary lesions bilaterally that appeared to be achrocordons. He did not have any ash leaf macules, nail changes or shagreen patches. Investigations: Four biopsies were taken of lesions on his face and in the supraclavicular area. The results showed one trichodischoma and three fibrofolliculomas, which interestingly included one lesion that had looked macroscopically like an acrochordon.
JUNE 2017
J AM ACAD DERMATOL
AB283
5685
What is the world of dermatology talking about? Text analysis of abstracts from the 23rd World Congress of Dermatology Diana Diao, MD, Department of Dermatology and Skin Science, University of British Columbia; David McLean, MD, Department of Dermatology and Skin Science, University of British Columbia; Harvey Lui, MD, Department of Dermatology and Skin Science, University of British Columbia
Which drug is it? Utility of lymphocyte transformation testing in the diagnosis of drug hypersensitivity in a university teaching hospital Jennifer Boggs, MBBCh, Connolly Hospital Blanchardstown; Marina O’Kane, MBBCh, Connolly Hospital Blanchardstown Introduction: Adverse cutaneous reactions to drugs are common, affecting 2 to 3% of hospitalized patients, while an estimated 1 in 1000 hospitalized patients develop a serious cutaneous drug reaction. However diagnosis of drug hypersensitivity is difficult, particularly type IV reactions as they are delayed in onset sometimes by days to weeks following exposure to the culprit drug and the reaction can take many different forms, varying from inconvenient to life threatening. Type IV reactions may be evaluated with various diagnostic tests including lymphocyte transformation testing (LTT), which aims to detect circulating drug specific memory T cells, which proliferate upon drug stimulation. Objectives: We aimed to evaluate whether LTT helped to define the incriminating drug in our patients with drug reactions.
Background: The quadrennial World Congress of Dermatology (WCD) of the International League of Dermatologic Societies (ILDS) showcases topics from across the globe. The spectrum of abstracts submitted and presented at the WCD provides insights into disease priorities, research developments, and therapeutic trends according to different world regions. Objective: To identify and compare prevailing diseases of interest and trends in therapeutics from world regions by text-based abstract analysis. Methods: Text from the abstract bodies of all proffered and accepted oral and poster presentations was categorized according to five geographic ILDS regions [1. Asia Pacific (Asia-Pac); 2. Europe (Eur); 3. Latin America (Lat-Amer); 4. South Asia, Middle East, and Africa (SAsia-MEast-Africa); and 5. USA and Canada (US-Can)] based on the location of the submitting author. Abstracts from each region were analyzed for word frequency, and the top 10 most frequent words or closely related word clusters denoting either a diagnosis or treatment in each region were tabulated by relative and absolute frequencies. Results: A total of 4,505 abstracts containing 970,070 words were systematically analyzed from 104 countries (counts by ILDS region: Asia-Pac, 924; Eur, 1275; LatAmer, 442; SAsia-MEast-Afr, 766; US-Can, 1074). The most frequent disease entities across all 5 regions were psoriasis, acne, and dermatitis. Melanoma and squamous cell carcinoma ranked in the top 10 in all regions except SAsia-MEast-Afr. Basal cell carcinoma ranked in the top 10 in Eur and US-Can only. Words related to infectious diseases such as ‘leprosy’, ‘tuberculosis’, ‘wart’, and ‘fungal’ were absent from the top 10 lists in Europe and the US-Can. For therapeutics, ‘topical’, ‘corticosteroid’, and words related to light-based modalities (eg, UVB, UVA, phototherapy, PDT, fractional) appeared most frequently in all 5 regions. ‘Excision’ was the most common surgical word in 4 regions with ‘Mohs’ being the most common for the USCan. Methotrexate appeared in the top 10 list for all regions except the Asia-Pac. Biologics were most prevalent in the US-Can, representing 3 of the top 10 list, but were absent from the top 10 lists in Lat-Amer and SAsia-MEast-Afr. Conclusions: There are regional similarities and differences in dermatologic disease entities and therapeutic trends of interest. Textual analysis can assist in quantifying the relative priorities ascribed by dermatologists to topics of interest. Commercial support: None identified.
Methods: We conducted a retrospective study of all patients from our department who had a LTT performed between 01/03/2009- 01/03/2016. Patients were identified from a patient database (n ¼ 34) and a chart review was performed. Results: Of the 34 patients, 21 were female and 13 were male, with a mean age of 52.8 years (range 18-85 years). 97% presented with a skin eruption, whilst 20% had systemic symptoms including fever, nausea, and malaise at presentation. The predominant morphology was maculopapular, 29%. 88% patients required admission to hospital. The mean duration between drug exposure and onset of reaction was 10.9 days. Of the 56% that underwent skin biopsy, 79% had histology consistent with a drug eruption. The average number of drugs requested for LTT per patient was 3 (range 1-9 drugs). 76% patients had a positive LTT to one or more drugs. 69% (18/26) had positive LTT reactions to an antibiotic, of which the majority were Beta-lactams, comprising 61% (11/18). 20% had a Severe Cutaneous Adverse Reaction (SCAR), of this group 100% had a positive LTT. The causative drugs were withdrawn once the diagnosis of drug reactions was made in 82%, followed by significant improvement in the majority of patients (82%). There were 2 fatalities secondary to multiorgan failure and 1 patient developed interstitial nephritis requiring hemodialysis. Two known accidental re-challenges occurred, both with severer recurrences of the skin eruption, with one patient developing DRESS. Conclusion: We found that a positive LTT was a valuable contribution in the diagnosis of drug allergy and in identifying the drug involved in the reaction. However as the sensitivity of LTT is limited, a negative LTT cannot exclude a drug hypersensitivity. False negatives may also occur with mistiming of the test and in patients on high dose systemic corticosteroids. As reflected in our study there is a significant morbidity and mortality associated with drug hypersensitivity reactions and LTT is a useful test in the armamentarium of the dermatologist when faced with this challenging clinical situation. Commercial support: None identified.
4708
5551 When a skin tag is not just a skin tag: Birt-Hogg-Dub e syndrome Charlotte Gollins, MBChB, St George Hospital; Steven Kossard, MBBS, Kossard Dermatopathologists; Dedee Murrell, MBBCh, University of New South Wales History: A 50-year-old gentleman of Macedonian origin was admitted to hospital with a left sided spontaneous pneumothorax, for which he underwent thoracoscopy and a wedge resection. Whilst in hospital it was noted that he had multiple facial papules and axillary skin tags. The patient reported that these had been present for decades. He had a history of acne as a teenager and had previously had a right sided pneumothorax 8 years earlier. His mother and grandmother had also had similar lesions on their faces but there was no family history of cancers or pulmonary or renal problems.
Widespread biphasic amyloidosis related to ipilimumab treatment for metastatic melanoma Virginia Velasco-Tamariz, MD, Department of Dermatology, Hospital 12 de Octubre; Marta Prieto-Barrios, MD, Department of Dermatology, Hospital 12 de Octubre; Fatima Tous-Romero, MD, Department of Dermatology, Hospital 12 de Octubre; Sara Burillo-Martınez, MD, Department of Dermatology, Hospital 12 de Octubre; Carlos Morales-Raya, MD, Department of Dermatology, Hospital 12 de Octubre; Raquel Arag on-Miguel, MD, Department of Dermatology, Hospital 12 de Octubre; Pablo Ortiz-Romero, PhD, Department of Dermatology, Hospital 12 de Octubre Introduction: Ipilimumab is a fully human monoclonal antibody approved for treatment of metastatic or unresectable melanoma. Due to its immunomodulatory mechanism of action, it has been associated with multiple immune-related adverse events (irAEs). We report the first case of a patient under treatment with Ipilimumab who developed classic cutaneous and histopathological findings of lichenoid and macular amyloidosis (biphasic amyloidosis).
Discussion: These histologic findings, along with the history of bilateral spontaneous pneumothoraces, strongly indicate a diagnosis of Birt-Hogg-Dube syndrome. This rare autosomal dominant genetic disorder is caused by a mutation in the folliculin (FLCN) gene and leads to bilateral lung cysts, skin lesions (most commonly fibrofolliculomas, trichodischomas, and achrocordons) and renal tumors. It is vital to consider this diagnosis in patients with multiple abnormal papules and skin tags if they appear with other suggestive signs. Once a diagnosis is made the patient can be closely monitored, which allows for earlier treatment of any complications that occur.
Case report: A 60-year-old male with an acral melanoma with a Breslow of 9.67 mm, underwent amputation and sentinel node biopsy which resulted negative. Two years later, image studies revealed lymph node and pulmonary metastases. As BRAF V600E mutation was negative, he was started on a course of ipilimumab. From the start of treatment he suffered from moderate pruritus. After the fourth dose, he abruptly developed extensive macular brown symmetrical pigmentation on his upper chest and back, and also papules on his arms, back, buttocks and legs. Histologic examination revealed eosinophilic material in upper dermis. Inmunohistochemmical staining was positive for cytokeratins using monoclonal antibody CK-903, confirming the diagnosis of biphasic amyloidosis. The lesions showed mild improvement with topical corticosteroids. Image studies revealed tumoral progression, so treatment was switched to pembrolizumab. However, pruritus persisted and the eruption worsened. Discussion. Even though ipilimumab has been associated with the highest rates of pruritus compared with other targeted therapies, prurigo or lichen simplex chronicus like-lesions have not been reported. Although the etiology of lichen amyloidosis (LA) is not yet fully understood, deposition of the amyloid in papillary dermis is thought to be consequence of necrosis of keratinocytes due to chronic rubbing of skin. Nevertheless, some authors have suggested that underlying immune-mediated factors may be implicated, because of the simultaneous occurrence with various autoimmune/immune disorders. In our case, it is surprising that the lesions developed so fast, taking into account that this dermatosis is considered a manifestation of chronic scratching. We cannot elucidate if the development of cutaneous amyloidosis is only consequence of pruritus, a known immune-related adverse effect of immunotherapy and subsequent scratching, or maybe our case-patient can reinforce the immune-related pathogenesis theory of amyloid deposition.
Commercial support: None identified.
Commercial support: None identified.
Physical Signs: On examination he had multiple skin colored papules on his face, neck, back and arms. He also had brown axillary lesions bilaterally that appeared to be achrocordons. He did not have any ash leaf macules, nail changes or shagreen patches. Investigations: Four biopsies were taken of lesions on his face and in the supraclavicular area. The results showed one trichodischoma and three fibrofolliculomas, which interestingly included one lesion that had looked macroscopically like an acrochordon.
JUNE 2017
J AM ACAD DERMATOL
AB283