Letters to the Editor
3. 4. 5. 6. 7. 8. 9. 10. 11.
12. 13. 14. 15. 16.
17.
melanoma and blue naevi. Nature 2009;457:599-602. Patterson JW. Nevus cell aggregates in lymph nodes. Am J Clin Pathol 2004;121:13-5. Hendricks WM. Eruptive blue nevi. J Am Acad Dermatol 1981;4:50-3. Yonei N, Kimura A, Furukawa F. Common blue nevus with satellite lesions needs a differential diagnosis from malignant melanoma. Case Rep Dermatol 2013;5:244-7. Diehl JW, Berk DR, Ney A, Bayliss SJ. Diffuse dermal melanoytosis: Follow-up 30 years later with novel findings of eruptive blue nevi. Arch Dermatol 2011;147:1339-40. Chen T, Kurwa HA, Trotter MJ, Haber RM. Agminated blue nevi in a patient with dermatomyositis. J Am Acad Dermatol 2013;68:e52-3. deGiorgi V, Massi D, Brunasso G, Salvini C, Mastrolorenzo A, Zuccati G, et al. Eruptive multiple blue nevi of the penis: A clinical dermoscopic pathologic case study. J Cutan Pathol 2004;31:185-8. Shenfield HT, Maize JC. Multiple and agminated blue nevi. J Dermatol Surg Oncol 1980;6:725-8. Suchniak JM, Griego RD, Rudolph AH, Waidhofer W. Acquired multiple blue nevi on extremity. J Am Acad Dermatol 1995;33:1051-2. Krause MH, Bonnekoh B, Weisshaar E, Gollnick H. Coincidence of multiple, disseminated, tardive-eruptive blue nevi with cutis marmorata teleangiectatica congenital. Dermatology 2000;200:134-8. Knopp E, Diette K, Ko C, Lazova R. Multiple blue macules and papules on the scalp. Arch Dermatol 2009;145:1183-8. Blicker JA, Rootman J, White VA. Cellular blue nevus of the conjunctiva. Ophthalmology 1992;99:1714-7. Nardini P, De Giorgi V, Massi D, Carli P. Eruptive disseminated blue naevi of the scalp. Br J Dermatol 1999;140:178-80. Madan V, Gangodpadhyay M, Dawn G. A collection of blue papules. Clin Exp Derm 2009;34:121-2. Carney JA, Ferreiro JA. The epithelioid blue nevus: a multicentric familial tumor with important associations, including cardiac myxoma and psammomatous melanotic schwannoma. Am J Surg Pathol 1996;20:259-72. Granter SR, McKee PH, Calonje E, Mihm MC Jr. Busam K. Melanoma associated with blue nevus and melanoma mimicking cellular blue nevus: A clinicopathologic study of 10 cases on the spectrum of so-called ‘malignant blue nevus’. Am J Surg Pathol 2001;25:316-23. Access this article online Quick Response Code:
Website: www.ijdvl.com DOI: 10.4103/0378-6323.152302 PMID: *****
Churg–Strauss syndrome with asymptomatic pulmonary emboli Sir, A 73-year-old man presented with recurrent painful purpura and hemorrhagic bullae on both legs for a month [Figure 1]. In addition, he had a six month history of bilateral pedal edema, anorexia and weight loss of approximately 6 kgs. He appeared cachectic
[body mass index (BMI) 14.4] with prominent muscle wasting and numbness of all four extremities. He had also been asthmatic for the last 5 years.Laboratory tests showed leukocytosis (12,000/μl) with 46% segments; a platelet count of 285,000/μl and a markedly elevated C-reactive protein (CRP) of 100 mg/l. There was a striking increase in eosinophils (42% on differential leucocyte count) with an absolute eosinophil count of 3159/μl. IgE was elevated at 834 IU/ml. Other biochemical tests were within normal limits. He denied using any medications. No parasitic infestations were found on stool examination. Tests for human immunodeficiency virus 1 and 2 antibodies and rapid plasma reagin were negative. C3 and C4 were normal and antinuclear antibody was negative. Rheumatoid factor was slightly elevated (53.8 IU/ml). Perinuclear and cytoplasmic anti-neutrophil cytoplasmic antibodies (P-ANCA and C-ANCA) were both positive. A whole body computer tomography (CT) for malignancy screening revealed paranasal sinusitis, right upper lung granuloma, and the unexpected finding of a filling defect in the left pulmonary artery, suggestive of pulmonary emboli [Figure 2]. The patient was clinically asymptomatic with no signs of dyspnea. Echocardiogram was normal and venous ultrasonography of the lower extremities did not show any thrombosis. Coagulopathy work-up including antiphospholipid antibody, anti-cardiolipin antibody, protein C, protein S, anti-thrombin III, and Factor V were all within normal limits. The cryoglobulin test was positive and serum protein electrophoresis showed polyclonal elevation, suggesting a reactive process. A biopsy of the skin lesion showed necrotizing vasculitis with prominent eosinophilic infiltrates [Figure 3]. Direct immunofluorescence detected vascular deposits of granular C3 and C1q alone. Nerve conduction studies revealed severe axonal motor–sensory polyneuropathy with symmetrical acral distribution. The diagnosis of Churg–Strausss syndrome (CSS) was established based on the 1990 criteria of the American College of Rheumatology.[1] We treated the patient with methylprednisolone, initially intravenously, 1.5 mg/ kg/day for a week, and subsequently, slowly tapered it to a maintenance dose of 0.5 mg/kg/day orally. With this treatment, skin lesions improved remarkably. Enoxaparin 1 mg/kg/q12 h was administered for the incidentally found pulmonary embolism; later on, it was replaced by oral warfarin 5 mg/day with a target prothrombin time-international normalized ratio (PT/ INR) of 2–3. The patient was followed up regularly and no relapse occurred during 1 year of follow-up.
Indian Journal of Dermatology, Venereology, and Leprology | March-April 2015 | Vol 81 | Issue 2
201
Letters to the Editor
Figure 1: The patient presented with multiple painful, and non-blanchable purpura with hemorrhagic bullae on both legs
Figure 2: Thoracic CT showing emboli in the left pulmonary vessel (arrow)
a
b
Figure 3: (a) Detached necrotic epidermis with hemorrhagic exudate and neutrophils H and E, ×40. (b) Fibrinoid necrosis of thrombosed medium-sized vessels with heavy infiltrates of eosinophils and neutrophils, nuclear dust, and hemorrhage H and E, ×200
Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis, is a rare disease characterized by systemic inflammation, asthma, vasculitis, and eosinophilia.[1] This syndrome is well known for its association with ANCA, predominantly P-ANCA directed against myeloperoxidase (MPO).[2] The complexity and multi-organ involvement of the disorder point towards a multifactorial etiology which incriminates genetic factors, allergic reactions, and external triggers.[3] The most widely accepted diagnostic criteria for Churg-Strauss syndrome were laid down 202
in 1990 by the American College of Rheumatology.[1] They include asthma, eosinophilia >10%, mono- or poly- neuropathy, non-fixed pulmonary infiltrates, paranasal sinus abnormality and extravascular eosinophils. The presence of four out of six criteria confirms the diagnosis of Churg-Strauss syndrome with a sensitivity and specificity of 85% and 99.7%, respectively.[1] Our patient’s clinical presentation with late-onset asthma, eosinophilia, and palpable purpura bilaterally on the lower legs led us to suspect the possibility of Churg-Strauss syndrome. Although not included in the diagnostic criteria, cutaneous manifestations are frequently observed in Churg-Strauss syndrome, with a frequency ranging from 40 to 81%.[4] As the skin is the most easily accessible site for histological samples, greater awareness of the cutaneous features would help in earlier diagnosis. The most common skin lesions are papules and nodules (58.8%), followed by purpura (36.7%) and vesiculobullous lesions (10.2%).[4] The main histopathological features of Churg-Straus syndrome have been reported to be vasculitis (67%), eosinophil infiltration (58%), and extravascular granuloma (16%).[4] The differential diagnosis of cutaneous small vessel vasculitis with eosinophil-predominant infiltrates includes primary eosinophilic vasculitis, parasitic infestation, hypereosinophilic syndrome, Churg-Strauss syndrome, and connective tissue diseases including Sjogren’s syndrome and rheumatoid arthritis. Thromboembolism is a rare complication of Churg-Strauss syndrome and may occur in larger vessels unaffected by vasculitis. However, asymptomatic pulmonary embolism in Churg-Strauss syndrome is exceedingly rare; we were able to find only one previously reported case in the English literature.[5] Although asymptomatic in our patient, pulmonary embolism is a potentially life-threatening event and its symptoms can mimic other respiratory problems that occur more commonly in Churg-Strauss syndrome. This raises the question of routine screening for venous thromboembolism in these patients. Studies have suggested that hypereosinophilia is the culprit that predisposes to the pro-thrombotic state. Eosinophilic cationic proteins, hypothiocyanous acid, and Charcot–Leyden crystals, components produced by eosinophils, are thought to induce the thrombotic diathesis in these patients. There is currently no recommendation regarding prophylactic or therapeutic anticoagulant treatment in these patients. Physicians should be aware of this rare but life-threatening complication.
Indian Journal of Dermatology, Venereology, and Leprology | March-April 2015 | Vol 81 | Issue 2
Letters to the Editor
Chau Yee Ng, Rosaline Chung-Yee Hui, Tseng-tong Kuo1, Chun-Yu Cheng, Jennifer Wu Departments of Dermatology and 1Pathology, Chang Gung Memorial Hospital, Taipei, Taiwan Address for correspondence: Rosaline Chung-Yee Hui, Department of Dermatology, Chang Gung Memorial Hospital, Taipei, 199, Dunhua North Road, Taipei 105, Taiwan. E-mail: [email protected]
REFERENCES 1.
2. 3. 4. 5.
Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strausss syndrome. Arthritis Rheum 1990;33:1094-100. Abril A. Churg-Strauss syndrome: An update. Curr Rheumatol Rep 2011;13:489-95. Wardle EN. Churg-Strauss syndrome. Lancet 2003;361:1746. Davis MD, Daoud MS, McEvoy MT, Su WP. Cutaneous manifestations of Churg-Strausss syndrome: A clinicopathologic correlation. J Am Acad Dermatol 1997;37:199-203. Maria A, Guilpain P, Forestier A, Delhom E, Schiffman A, Rivière S, et al. Asymptomatic bilateral pulmonary embolism in Churg-Strauss syndrome. Eur Respir Rev 2012;21:75-7. Access this article online Quick Response Code:
Website: www.ijdvl.com DOI: 10.4103/0378-6323.152303 PMID: *****
Sweet’s syndrome associated with chronic neutrophilic leukemia Sir, Sweet’s syndrome is characterized by fever, leucocytosis, tender erythematous plaques and nodules, and a dense dermal neutrophilic infiltrate.[1] It has been reported in association with both hematologic malignancies and solid organ tumors.[2] We report a patient of chronic neutrophilic leukemia (CNL), presenting with Sweet’s syndrome. We were able to find only one previous report of this association.[3] A 33-year-old male presented with a 3-week history of skin lesions over face, neck, and upper limbs accompanied by intense burning sensation, fever, anorexia, and myalgia. His past medical history was unremarkable. Examination revealed multiple, well defined, violaceous, edematous, papules and plaques, over the entire face, front and back of
neck, retroauricular area, extensor aspect of arms, dorsal aspect of both hands, and fingers. The surface showed pseudovesiculation and marked edema [Figures 1 and 2]. Similar lesions developed over dorsum of both feet and lower legs over the next few days. There was conjunctival congestion, hepatomegaly and marked splenomegaly, but no lymphadenopathy or oral erosions. Investigations revealed leucocytosis of 71.8 × 109/L (reference range 4–10 × 109/L) with a differential count of 81% neutrophils, 7% lymphocytes, 1% monocytes, 3% eosinophils, 5% myelocytes, and 3% metamyelocytes. The immature myeloid precursors were less than 10%, without blasts, and basophils or toxic granules were not seen. Hemoglobin was 10.2 g/dL, and platelet count was 9 × 109/L (reference range 150–410 × 109/L). Erythrocyte sedimentation rate (ESR) was 115 mm/h (reference range 0–20 mm/h), C-reactive protein was 171.2 mg/L (reference range 0–6 mg/L), and serum lactate dehydrogenase was 463 U/L (135–225 U/L). Leukocyte alkaline phosphatase (LAP) score was 120 (reference range: 20-120). Serum vitamin B 12 levels were 2000 pg/ml (reference value 191–663 pg/ml). Liver and renal function tests, urine analysis, thyroid profile were normal; screening for antinuclear antibodies and serology for Hepatitis B, C and human immunodeficiency virus was negative. Blood and urine cultures were sterile and test for stool occult blood was negative. Chest X-ray was normal while computed tomography (CT) scan of abdomen revealed marked splenomegaly with peripheral hypo-dense, non-enhancing areas suggestive of infarcts. Histopathology of skin lesions showed a diffuse neutrophilic infiltrate within the upper dermis, dermal edema, and prominent leucocytoclasia [Figure 3]. No immature or atypical cells, granuloma formation or microorganisms were detected. Based on these features, the diagnosis of Sweet’s syndrome was established [Table 1]. Peripheral blood findings were suggestive of a leukemoid reaction or a chronic myeloproliferative neoplasm. Bone marrow aspiration smears showed a hypercellular marrow exhibiting myeloid hyperplasia with myeloid: erythroid ratio of 8:1. The myeloid series showed an orderly cell maturation having no prominence of blast cells, eosinophils or their precursors. Erythroid series showed normoblastic maturation and megakaryocytes were normal in number and morphology. There was no prominence of plasma cells or dysplasia in any lineage [Figure 4a]. Bone marrow biopsy showed a hypercellular marrow exhibiting marked myeloid hyperplasia. Both megakaryocytes and erythroid precursors were identified. No fibrosis was noted [Figure 4b]. BCR/ABL gene translocation assay
Indian Journal of Dermatology, Venereology, and Leprology | March-April 2015 | Vol 81 | Issue 2
203
Copyright of Indian Journal of Dermatology, Venereology & Leprology is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
3. 4. 5. 6. 7. 8. 9. 10. 11.
12. 13. 14. 15. 16.
17.
melanoma and blue naevi. Nature 2009;457:599-602. Patterson JW. Nevus cell aggregates in lymph nodes. Am J Clin Pathol 2004;121:13-5. Hendricks WM. Eruptive blue nevi. J Am Acad Dermatol 1981;4:50-3. Yonei N, Kimura A, Furukawa F. Common blue nevus with satellite lesions needs a differential diagnosis from malignant melanoma. Case Rep Dermatol 2013;5:244-7. Diehl JW, Berk DR, Ney A, Bayliss SJ. Diffuse dermal melanoytosis: Follow-up 30 years later with novel findings of eruptive blue nevi. Arch Dermatol 2011;147:1339-40. Chen T, Kurwa HA, Trotter MJ, Haber RM. Agminated blue nevi in a patient with dermatomyositis. J Am Acad Dermatol 2013;68:e52-3. deGiorgi V, Massi D, Brunasso G, Salvini C, Mastrolorenzo A, Zuccati G, et al. Eruptive multiple blue nevi of the penis: A clinical dermoscopic pathologic case study. J Cutan Pathol 2004;31:185-8. Shenfield HT, Maize JC. Multiple and agminated blue nevi. J Dermatol Surg Oncol 1980;6:725-8. Suchniak JM, Griego RD, Rudolph AH, Waidhofer W. Acquired multiple blue nevi on extremity. J Am Acad Dermatol 1995;33:1051-2. Krause MH, Bonnekoh B, Weisshaar E, Gollnick H. Coincidence of multiple, disseminated, tardive-eruptive blue nevi with cutis marmorata teleangiectatica congenital. Dermatology 2000;200:134-8. Knopp E, Diette K, Ko C, Lazova R. Multiple blue macules and papules on the scalp. Arch Dermatol 2009;145:1183-8. Blicker JA, Rootman J, White VA. Cellular blue nevus of the conjunctiva. Ophthalmology 1992;99:1714-7. Nardini P, De Giorgi V, Massi D, Carli P. Eruptive disseminated blue naevi of the scalp. Br J Dermatol 1999;140:178-80. Madan V, Gangodpadhyay M, Dawn G. A collection of blue papules. Clin Exp Derm 2009;34:121-2. Carney JA, Ferreiro JA. The epithelioid blue nevus: a multicentric familial tumor with important associations, including cardiac myxoma and psammomatous melanotic schwannoma. Am J Surg Pathol 1996;20:259-72. Granter SR, McKee PH, Calonje E, Mihm MC Jr. Busam K. Melanoma associated with blue nevus and melanoma mimicking cellular blue nevus: A clinicopathologic study of 10 cases on the spectrum of so-called ‘malignant blue nevus’. Am J Surg Pathol 2001;25:316-23. Access this article online Quick Response Code:
Website: www.ijdvl.com DOI: 10.4103/0378-6323.152302 PMID: *****
Churg–Strauss syndrome with asymptomatic pulmonary emboli Sir, A 73-year-old man presented with recurrent painful purpura and hemorrhagic bullae on both legs for a month [Figure 1]. In addition, he had a six month history of bilateral pedal edema, anorexia and weight loss of approximately 6 kgs. He appeared cachectic
[body mass index (BMI) 14.4] with prominent muscle wasting and numbness of all four extremities. He had also been asthmatic for the last 5 years.Laboratory tests showed leukocytosis (12,000/μl) with 46% segments; a platelet count of 285,000/μl and a markedly elevated C-reactive protein (CRP) of 100 mg/l. There was a striking increase in eosinophils (42% on differential leucocyte count) with an absolute eosinophil count of 3159/μl. IgE was elevated at 834 IU/ml. Other biochemical tests were within normal limits. He denied using any medications. No parasitic infestations were found on stool examination. Tests for human immunodeficiency virus 1 and 2 antibodies and rapid plasma reagin were negative. C3 and C4 were normal and antinuclear antibody was negative. Rheumatoid factor was slightly elevated (53.8 IU/ml). Perinuclear and cytoplasmic anti-neutrophil cytoplasmic antibodies (P-ANCA and C-ANCA) were both positive. A whole body computer tomography (CT) for malignancy screening revealed paranasal sinusitis, right upper lung granuloma, and the unexpected finding of a filling defect in the left pulmonary artery, suggestive of pulmonary emboli [Figure 2]. The patient was clinically asymptomatic with no signs of dyspnea. Echocardiogram was normal and venous ultrasonography of the lower extremities did not show any thrombosis. Coagulopathy work-up including antiphospholipid antibody, anti-cardiolipin antibody, protein C, protein S, anti-thrombin III, and Factor V were all within normal limits. The cryoglobulin test was positive and serum protein electrophoresis showed polyclonal elevation, suggesting a reactive process. A biopsy of the skin lesion showed necrotizing vasculitis with prominent eosinophilic infiltrates [Figure 3]. Direct immunofluorescence detected vascular deposits of granular C3 and C1q alone. Nerve conduction studies revealed severe axonal motor–sensory polyneuropathy with symmetrical acral distribution. The diagnosis of Churg–Strausss syndrome (CSS) was established based on the 1990 criteria of the American College of Rheumatology.[1] We treated the patient with methylprednisolone, initially intravenously, 1.5 mg/ kg/day for a week, and subsequently, slowly tapered it to a maintenance dose of 0.5 mg/kg/day orally. With this treatment, skin lesions improved remarkably. Enoxaparin 1 mg/kg/q12 h was administered for the incidentally found pulmonary embolism; later on, it was replaced by oral warfarin 5 mg/day with a target prothrombin time-international normalized ratio (PT/ INR) of 2–3. The patient was followed up regularly and no relapse occurred during 1 year of follow-up.
Indian Journal of Dermatology, Venereology, and Leprology | March-April 2015 | Vol 81 | Issue 2
201
Letters to the Editor
Figure 1: The patient presented with multiple painful, and non-blanchable purpura with hemorrhagic bullae on both legs
Figure 2: Thoracic CT showing emboli in the left pulmonary vessel (arrow)
a
b
Figure 3: (a) Detached necrotic epidermis with hemorrhagic exudate and neutrophils H and E, ×40. (b) Fibrinoid necrosis of thrombosed medium-sized vessels with heavy infiltrates of eosinophils and neutrophils, nuclear dust, and hemorrhage H and E, ×200
Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis, is a rare disease characterized by systemic inflammation, asthma, vasculitis, and eosinophilia.[1] This syndrome is well known for its association with ANCA, predominantly P-ANCA directed against myeloperoxidase (MPO).[2] The complexity and multi-organ involvement of the disorder point towards a multifactorial etiology which incriminates genetic factors, allergic reactions, and external triggers.[3] The most widely accepted diagnostic criteria for Churg-Strauss syndrome were laid down 202
in 1990 by the American College of Rheumatology.[1] They include asthma, eosinophilia >10%, mono- or poly- neuropathy, non-fixed pulmonary infiltrates, paranasal sinus abnormality and extravascular eosinophils. The presence of four out of six criteria confirms the diagnosis of Churg-Strauss syndrome with a sensitivity and specificity of 85% and 99.7%, respectively.[1] Our patient’s clinical presentation with late-onset asthma, eosinophilia, and palpable purpura bilaterally on the lower legs led us to suspect the possibility of Churg-Strauss syndrome. Although not included in the diagnostic criteria, cutaneous manifestations are frequently observed in Churg-Strauss syndrome, with a frequency ranging from 40 to 81%.[4] As the skin is the most easily accessible site for histological samples, greater awareness of the cutaneous features would help in earlier diagnosis. The most common skin lesions are papules and nodules (58.8%), followed by purpura (36.7%) and vesiculobullous lesions (10.2%).[4] The main histopathological features of Churg-Straus syndrome have been reported to be vasculitis (67%), eosinophil infiltration (58%), and extravascular granuloma (16%).[4] The differential diagnosis of cutaneous small vessel vasculitis with eosinophil-predominant infiltrates includes primary eosinophilic vasculitis, parasitic infestation, hypereosinophilic syndrome, Churg-Strauss syndrome, and connective tissue diseases including Sjogren’s syndrome and rheumatoid arthritis. Thromboembolism is a rare complication of Churg-Strauss syndrome and may occur in larger vessels unaffected by vasculitis. However, asymptomatic pulmonary embolism in Churg-Strauss syndrome is exceedingly rare; we were able to find only one previously reported case in the English literature.[5] Although asymptomatic in our patient, pulmonary embolism is a potentially life-threatening event and its symptoms can mimic other respiratory problems that occur more commonly in Churg-Strauss syndrome. This raises the question of routine screening for venous thromboembolism in these patients. Studies have suggested that hypereosinophilia is the culprit that predisposes to the pro-thrombotic state. Eosinophilic cationic proteins, hypothiocyanous acid, and Charcot–Leyden crystals, components produced by eosinophils, are thought to induce the thrombotic diathesis in these patients. There is currently no recommendation regarding prophylactic or therapeutic anticoagulant treatment in these patients. Physicians should be aware of this rare but life-threatening complication.
Indian Journal of Dermatology, Venereology, and Leprology | March-April 2015 | Vol 81 | Issue 2
Letters to the Editor
Chau Yee Ng, Rosaline Chung-Yee Hui, Tseng-tong Kuo1, Chun-Yu Cheng, Jennifer Wu Departments of Dermatology and 1Pathology, Chang Gung Memorial Hospital, Taipei, Taiwan Address for correspondence: Rosaline Chung-Yee Hui, Department of Dermatology, Chang Gung Memorial Hospital, Taipei, 199, Dunhua North Road, Taipei 105, Taiwan. E-mail: [email protected]
REFERENCES 1.
2. 3. 4. 5.
Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strausss syndrome. Arthritis Rheum 1990;33:1094-100. Abril A. Churg-Strauss syndrome: An update. Curr Rheumatol Rep 2011;13:489-95. Wardle EN. Churg-Strauss syndrome. Lancet 2003;361:1746. Davis MD, Daoud MS, McEvoy MT, Su WP. Cutaneous manifestations of Churg-Strausss syndrome: A clinicopathologic correlation. J Am Acad Dermatol 1997;37:199-203. Maria A, Guilpain P, Forestier A, Delhom E, Schiffman A, Rivière S, et al. Asymptomatic bilateral pulmonary embolism in Churg-Strauss syndrome. Eur Respir Rev 2012;21:75-7. Access this article online Quick Response Code:
Website: www.ijdvl.com DOI: 10.4103/0378-6323.152303 PMID: *****
Sweet’s syndrome associated with chronic neutrophilic leukemia Sir, Sweet’s syndrome is characterized by fever, leucocytosis, tender erythematous plaques and nodules, and a dense dermal neutrophilic infiltrate.[1] It has been reported in association with both hematologic malignancies and solid organ tumors.[2] We report a patient of chronic neutrophilic leukemia (CNL), presenting with Sweet’s syndrome. We were able to find only one previous report of this association.[3] A 33-year-old male presented with a 3-week history of skin lesions over face, neck, and upper limbs accompanied by intense burning sensation, fever, anorexia, and myalgia. His past medical history was unremarkable. Examination revealed multiple, well defined, violaceous, edematous, papules and plaques, over the entire face, front and back of
neck, retroauricular area, extensor aspect of arms, dorsal aspect of both hands, and fingers. The surface showed pseudovesiculation and marked edema [Figures 1 and 2]. Similar lesions developed over dorsum of both feet and lower legs over the next few days. There was conjunctival congestion, hepatomegaly and marked splenomegaly, but no lymphadenopathy or oral erosions. Investigations revealed leucocytosis of 71.8 × 109/L (reference range 4–10 × 109/L) with a differential count of 81% neutrophils, 7% lymphocytes, 1% monocytes, 3% eosinophils, 5% myelocytes, and 3% metamyelocytes. The immature myeloid precursors were less than 10%, without blasts, and basophils or toxic granules were not seen. Hemoglobin was 10.2 g/dL, and platelet count was 9 × 109/L (reference range 150–410 × 109/L). Erythrocyte sedimentation rate (ESR) was 115 mm/h (reference range 0–20 mm/h), C-reactive protein was 171.2 mg/L (reference range 0–6 mg/L), and serum lactate dehydrogenase was 463 U/L (135–225 U/L). Leukocyte alkaline phosphatase (LAP) score was 120 (reference range: 20-120). Serum vitamin B 12 levels were 2000 pg/ml (reference value 191–663 pg/ml). Liver and renal function tests, urine analysis, thyroid profile were normal; screening for antinuclear antibodies and serology for Hepatitis B, C and human immunodeficiency virus was negative. Blood and urine cultures were sterile and test for stool occult blood was negative. Chest X-ray was normal while computed tomography (CT) scan of abdomen revealed marked splenomegaly with peripheral hypo-dense, non-enhancing areas suggestive of infarcts. Histopathology of skin lesions showed a diffuse neutrophilic infiltrate within the upper dermis, dermal edema, and prominent leucocytoclasia [Figure 3]. No immature or atypical cells, granuloma formation or microorganisms were detected. Based on these features, the diagnosis of Sweet’s syndrome was established [Table 1]. Peripheral blood findings were suggestive of a leukemoid reaction or a chronic myeloproliferative neoplasm. Bone marrow aspiration smears showed a hypercellular marrow exhibiting myeloid hyperplasia with myeloid: erythroid ratio of 8:1. The myeloid series showed an orderly cell maturation having no prominence of blast cells, eosinophils or their precursors. Erythroid series showed normoblastic maturation and megakaryocytes were normal in number and morphology. There was no prominence of plasma cells or dysplasia in any lineage [Figure 4a]. Bone marrow biopsy showed a hypercellular marrow exhibiting marked myeloid hyperplasia. Both megakaryocytes and erythroid precursors were identified. No fibrosis was noted [Figure 4b]. BCR/ABL gene translocation assay
Indian Journal of Dermatology, Venereology, and Leprology | March-April 2015 | Vol 81 | Issue 2
203
Copyright of Indian Journal of Dermatology, Venereology & Leprology is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
