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AN EVOLVING DIAGNOSIS OF A DIFFUSE ERYTHRODERMIC RASH
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Abstracts: Medically Challenging Cases / Ann Allergy Asthma Immunol 121 (2018) S63−S134
discontinued all products containing these substances without improvement. She continued to develop new lesions. There were no new medications except cetirizine and then levocetirizine, which she continued for the management of pruritus. She was treated with multiple courses of systemic and topical corticosteroids, and hydroxychloroquine, without resolution. The patient was advised to discontinue levocetirizine, avoid cetirizine, and initiate fexofenadine. At follow up at two weeks and one year later, she reported complete resolution of the rash after discontinuation of levocetirizine. Discussion: Piperazine derivative antihistamines, such as hydroxyzine, cetirizine, and levocetirizine, share a similar structure with ethylenediamine, which is a common etiology of contact dermatitis. Hypersensitivity to H1 antihistamines is rare. Continued use of H1antihistamines, despite lack of improvement or worsening of symptoms, may be incorrectly interpreted as a failure of response to treatment. Development of generalized dermatitis induced by cetirizine or levocetirizine is rarely reported. This case illustrates the importance of re-evaluation in patients who fail to respond to treatment with consideration of piperazine derivative H1 antihistamines as a possible trigger.
M507 IMPROVEMENT OF SYSTEMIC SCLEROSISLIKE SYMPTOMS AFTER THERAPY FOR MULTIPLE MYELOMA S. Davuluri*,1, A. Tieng2, G. Franchin2, M. Niazi2, 1. Sunnyvale, CA; 2. Bronx, NY Introduction: Systemic sclerosis is known to carry an increased risk of malignancy. In this report we describe a rare case of systemic sclerosis as the presenting sign of amyloidosis associated with multiple myeloma. Case Description: A 59-year-old woman presented to the clinic with a 6-month history of bilateral hand pain and puffiness. Physical examination revealed melasma, multiple enlarged right supraclavicular lymph nodes, and sclerodactyly. Laboratory tests were positive for antinuclear antibodies (1:160) in a nucleolar pattern and nephrotic range proteinuria (>8g/day). The initial impression was systemic sclerosis associated arthritis and patient was treated with hydroxychloroquine, resulting in slight improvement in her range of motion. Computed tomography of the neck showed mildly enlarged right supraclavicular lymph nodes measuring up to 1.6 centimeters in diameter. Biopsy of a cervical lymph node revealed malignant lymphoma. Bone marrow biopsy showed plasma cell myeloma (>80%); serum immunoelectrophoresis revealed elevated levels of free kappa lambda light chains (8447 mg per liter; reference range, 3.3-19.4). Initial management included cyclophosphamide, bortezomib, and dexamethasone, resulting in significant improvement in joint pain. Furthermore, she developed multiple periorbital papules. A biopsy of her left upper eyelid lesion demonstrated apple-green birefringence after Congo red staining, compatible with a diagnosis of amyloidosis. Discussion: This patient initially presenting with a picture suggestive of systemic sclerosis was subsequently diagnosed with multiple myeloma and amyloidosis. She developed significant improvement in the range of motion of her finger joints as well as the skin tautness after initiating chemotherapy.
M508 AN EVOLVING DIAGNOSIS OF A DIFFUSE ERYTHRODERMIC RASH M. Kuder, F. Hsieh, Cleveland, OH Introduction: Lymphocytic variant hypereosinophilic syndrome (LHES) is a rare condition that can be challenging to diagnose. In patients presenting with an erythrodermic skin rash and eosinophilia, it is imperative to review the L-HES diagnostic criteria and
periodically reassess for alternative etiologies, particularly with disease progression. Case Description: A 39-year-old female presented with a 4-year history of an erythematous, confluent plaque-like rash. Initially, her skin biopsy demonstrated granulomatous dermatitis with necrobiosis but no eosinophils. She had a peripheral absolute eosinophil count of 1100/uL. Her bone marrow biopsy demonstrated a CD4+, CD3-T-cell population with small, non-necrotizing granulomas and no clonal eosinophilia. She was diagnosed with L-HES and started on high-dose glucocorticoids with initial skin improvement; however, skin symptoms subsequently progressed, covering 90% of her body surface area. She was then managed as cutaneous sarcoidosis, but treatment with methotrexate and leflunomide did not improve symptoms. Given her persistent symptoms, she underwent repeat skin biopsy, which demonstrated cutaneous T-cell lymphoma. Symptoms improved with romidepsin. Discussion: This patient had initial findings suggestive of L-HES but did not meet L-HES diagnostic criteria. She never had an absolute eosinophil count >1500/uL, nor tissue biopsy demonstrating eosinophil-mediated organ damage. Her disease progression led to re-evaluation and a malignancy diagnosis. Although patients with L-HES can progress to cutaneous T-cell lymphoma, clonal CD4+, CD3- T cells can be found in primary cutaneous T-cell lymphoma without prior HES diagnosis. This case illustrates the importance of continued diagnostic inquiry when the diagnosis is unclear and symptoms progress despite therapy.
M509 HAND DERMATITIS IN A SURGEON: A DIFFICULT PROBLEM AND A THREEPRONGED SOLUTION S. Mahapatra, L. Caraballo, V. Dimov*, F. Eidelman, Weston, FL Introduction: Hand dermatitis is a common problem affecting up to 25% of health workers, it can be caused by contact dermatitis (CD). CD is categorized as irritant in 80% of patients and allergic in 20%. Case Description: A 42-year old male surgeon with a history of recurrent eczematous lesions on hands and trunk presented with a recurrence of pruritic rash. The rash had persisted despite using OTC hydrocortisone, triamcinolone and OTC moisturizers. Hand dermatitis interfered with his work as a surgeon. Patch testing for allergic contact dermatitis was positive for 1, 3-diphenylguanidine, ammonium persulfate, carba mix, cinnamic aldehyde, cocamidopropyl betaine, coconut diethanolamide (cocamide DEA), colophony, formaldehyde, fragrance mix, hydrocortisone-17-butyrate, Iodopropynyl butylcarbamate, lidocaine-HCl and propylene glycol. The glove materials (1, 3diphenylguanidine, carba mix), fragrances, preservatives (propylene glycol) and topical steroids were correlated with the patient's symptoms. After contact dermatitis was confirmed with patch testing, a targeted avoidance was recommended. Treatment modalities were adjusted. Triamcinolone and hydrocortisone were eliminated based on patch test results (triamcinolone 0.1% cream contained propylene glycol). Symptoms improved with: 1. topical calcineurin inhibitor (tacrolimus); 2. avoidance of irritants and allergens based on skin patch test; 3. use of barrier protection such as cotton undergloves. Discussion: Hand dermatitis can have a potentially devastating effect on a surgeon’s career based on the limitations it imposes on the ability to practice. The three-pronged approach described above can offer a clinical solution to a difficult problem.
M510 A MYSTERIOUS RASH AROUND SURGICAL WOUNDS W. Jithpratuck*,1, D. Kays2, P. Sriaroon3, 1. St petersburg, FL; 2. ST Petersburg, FL; 3. St Petersburg, FL Introduction: Contact dermatitis (CD) that occurs shortly after a surgery is often caused by a direct irritant effect of medical products or,
Abstracts: Medically Challenging Cases / Ann Allergy Asthma Immunol 121 (2018) S63−S134
discontinued all products containing these substances without improvement. She continued to develop new lesions. There were no new medications except cetirizine and then levocetirizine, which she continued for the management of pruritus. She was treated with multiple courses of systemic and topical corticosteroids, and hydroxychloroquine, without resolution. The patient was advised to discontinue levocetirizine, avoid cetirizine, and initiate fexofenadine. At follow up at two weeks and one year later, she reported complete resolution of the rash after discontinuation of levocetirizine. Discussion: Piperazine derivative antihistamines, such as hydroxyzine, cetirizine, and levocetirizine, share a similar structure with ethylenediamine, which is a common etiology of contact dermatitis. Hypersensitivity to H1 antihistamines is rare. Continued use of H1antihistamines, despite lack of improvement or worsening of symptoms, may be incorrectly interpreted as a failure of response to treatment. Development of generalized dermatitis induced by cetirizine or levocetirizine is rarely reported. This case illustrates the importance of re-evaluation in patients who fail to respond to treatment with consideration of piperazine derivative H1 antihistamines as a possible trigger.
M507 IMPROVEMENT OF SYSTEMIC SCLEROSISLIKE SYMPTOMS AFTER THERAPY FOR MULTIPLE MYELOMA S. Davuluri*,1, A. Tieng2, G. Franchin2, M. Niazi2, 1. Sunnyvale, CA; 2. Bronx, NY Introduction: Systemic sclerosis is known to carry an increased risk of malignancy. In this report we describe a rare case of systemic sclerosis as the presenting sign of amyloidosis associated with multiple myeloma. Case Description: A 59-year-old woman presented to the clinic with a 6-month history of bilateral hand pain and puffiness. Physical examination revealed melasma, multiple enlarged right supraclavicular lymph nodes, and sclerodactyly. Laboratory tests were positive for antinuclear antibodies (1:160) in a nucleolar pattern and nephrotic range proteinuria (>8g/day). The initial impression was systemic sclerosis associated arthritis and patient was treated with hydroxychloroquine, resulting in slight improvement in her range of motion. Computed tomography of the neck showed mildly enlarged right supraclavicular lymph nodes measuring up to 1.6 centimeters in diameter. Biopsy of a cervical lymph node revealed malignant lymphoma. Bone marrow biopsy showed plasma cell myeloma (>80%); serum immunoelectrophoresis revealed elevated levels of free kappa lambda light chains (8447 mg per liter; reference range, 3.3-19.4). Initial management included cyclophosphamide, bortezomib, and dexamethasone, resulting in significant improvement in joint pain. Furthermore, she developed multiple periorbital papules. A biopsy of her left upper eyelid lesion demonstrated apple-green birefringence after Congo red staining, compatible with a diagnosis of amyloidosis. Discussion: This patient initially presenting with a picture suggestive of systemic sclerosis was subsequently diagnosed with multiple myeloma and amyloidosis. She developed significant improvement in the range of motion of her finger joints as well as the skin tautness after initiating chemotherapy.
M508 AN EVOLVING DIAGNOSIS OF A DIFFUSE ERYTHRODERMIC RASH M. Kuder, F. Hsieh, Cleveland, OH Introduction: Lymphocytic variant hypereosinophilic syndrome (LHES) is a rare condition that can be challenging to diagnose. In patients presenting with an erythrodermic skin rash and eosinophilia, it is imperative to review the L-HES diagnostic criteria and
periodically reassess for alternative etiologies, particularly with disease progression. Case Description: A 39-year-old female presented with a 4-year history of an erythematous, confluent plaque-like rash. Initially, her skin biopsy demonstrated granulomatous dermatitis with necrobiosis but no eosinophils. She had a peripheral absolute eosinophil count of 1100/uL. Her bone marrow biopsy demonstrated a CD4+, CD3-T-cell population with small, non-necrotizing granulomas and no clonal eosinophilia. She was diagnosed with L-HES and started on high-dose glucocorticoids with initial skin improvement; however, skin symptoms subsequently progressed, covering 90% of her body surface area. She was then managed as cutaneous sarcoidosis, but treatment with methotrexate and leflunomide did not improve symptoms. Given her persistent symptoms, she underwent repeat skin biopsy, which demonstrated cutaneous T-cell lymphoma. Symptoms improved with romidepsin. Discussion: This patient had initial findings suggestive of L-HES but did not meet L-HES diagnostic criteria. She never had an absolute eosinophil count >1500/uL, nor tissue biopsy demonstrating eosinophil-mediated organ damage. Her disease progression led to re-evaluation and a malignancy diagnosis. Although patients with L-HES can progress to cutaneous T-cell lymphoma, clonal CD4+, CD3- T cells can be found in primary cutaneous T-cell lymphoma without prior HES diagnosis. This case illustrates the importance of continued diagnostic inquiry when the diagnosis is unclear and symptoms progress despite therapy.
M509 HAND DERMATITIS IN A SURGEON: A DIFFICULT PROBLEM AND A THREEPRONGED SOLUTION S. Mahapatra, L. Caraballo, V. Dimov*, F. Eidelman, Weston, FL Introduction: Hand dermatitis is a common problem affecting up to 25% of health workers, it can be caused by contact dermatitis (CD). CD is categorized as irritant in 80% of patients and allergic in 20%. Case Description: A 42-year old male surgeon with a history of recurrent eczematous lesions on hands and trunk presented with a recurrence of pruritic rash. The rash had persisted despite using OTC hydrocortisone, triamcinolone and OTC moisturizers. Hand dermatitis interfered with his work as a surgeon. Patch testing for allergic contact dermatitis was positive for 1, 3-diphenylguanidine, ammonium persulfate, carba mix, cinnamic aldehyde, cocamidopropyl betaine, coconut diethanolamide (cocamide DEA), colophony, formaldehyde, fragrance mix, hydrocortisone-17-butyrate, Iodopropynyl butylcarbamate, lidocaine-HCl and propylene glycol. The glove materials (1, 3diphenylguanidine, carba mix), fragrances, preservatives (propylene glycol) and topical steroids were correlated with the patient's symptoms. After contact dermatitis was confirmed with patch testing, a targeted avoidance was recommended. Treatment modalities were adjusted. Triamcinolone and hydrocortisone were eliminated based on patch test results (triamcinolone 0.1% cream contained propylene glycol). Symptoms improved with: 1. topical calcineurin inhibitor (tacrolimus); 2. avoidance of irritants and allergens based on skin patch test; 3. use of barrier protection such as cotton undergloves. Discussion: Hand dermatitis can have a potentially devastating effect on a surgeon’s career based on the limitations it imposes on the ability to practice. The three-pronged approach described above can offer a clinical solution to a difficult problem.
M510 A MYSTERIOUS RASH AROUND SURGICAL WOUNDS W. Jithpratuck*,1, D. Kays2, P. Sriaroon3, 1. St petersburg, FL; 2. ST Petersburg, FL; 3. St Petersburg, FL Introduction: Contact dermatitis (CD) that occurs shortly after a surgery is often caused by a direct irritant effect of medical products or,