Letters to the Editor
892
Children usually present with non-specific and insidious symptoms, such as asthenia and irritability, therefore early diagnosis can be challenging. Leukaemic changes in the bone marrow or peripheral blood generally precede skin involvement, however, in rare instances skin involvement is the first sign of leukaemia, preceding the diagnosis of systemic leukaemia by months or even years.2 Cutaneous lesions of leukaemia appear in two forms: leukaemia cutis, wherein the skin is infiltrated by leukemic (blast) cells, and leukaemids, which represent the non-specific cutaneous lesions of leukaemia.3 Leukaemia cutis is observed more frequently in acute myeloid leukaemia, being rare in acute lymphoblastic leukaemia. The clinical appearance of leukaemia cutis is variable. The most common manifestation are erythematous or violaceous plaques, papules or nodules involving the face, trunk and extremities.4 Less common appearances include macules, maculopapules or plaques. Involvement of the scalp is rare. The differential diagnosis of scalp nodules in children includes entities such as cylindromas, dermoid and epidermal cysts, dermatofibrosarcoma protuberans and haemangiomas.5 The correct diagnosis is established by histologic demonstration of true infiltration of the skin with leukemic cells. In summary we describe an unusual presentation of T-cell Acute Lymphoblastic Leukaemia in a child, which appeared clinically as leukaemia cutis. Leukaemia cutis is a rare form of presentation of leukaemia, which is even rarer in T-cell Acute Lymphoblastic Leukaemia. Leukaemic infiltration should be considered on the differential diagnosis of scalp lesions, especially when its presentation includes multiple tumours. lezM. Toro Montecinos,1,* A. Vicente,1 M.A. Gonza ~at,1 A. Catala Temprano,2 M. Sun ~ol3 Ensen Departments of 1Pediatric Dermatology, 2Pediatric Hematology and u, Oncology, 3Pathology, Hospital Universitari Sant Joan de De Barcelona, Spain *Correspondence: M. Toro Montecinos. E-mail: [email protected]
References 1 Yeoh AE, Tan D, Li CK, Hori H, Tse E, Pui CH. Management of adult and paediatric acute lymphoblastic leukaemia in Asia: resource-stratified guidelines from the Asian Oncology Summit 2013. Lancet Oncol 2013; 14: e508–e523. 2 Husak R, Blume-Peytaki U, Orfanos CE. Aleukemic leukemia cutis in an adolescent boy. N Engl J Med 1999; 340: 893–894. 3 Stawiski MA. Skin manifestations of leukemids and lymphomas. Cutis 1978; 21: 814–818. 4 Longacre TA, Smoller BR. Leukemia cutis. Analysis of 50 biopsy-proven cases with an emphasis on occurrence in myelodysplastic syndromes. Am J Clin Pathol 1993; 100: 276–284. 5 Anderson PC, Stotland MA, Dinulos JG, Perry AE. Acute lymphocytic leukemia presenting as an isolated scalp nodule in an infant. Ann Plast Surg 2010; 64: 251–253. DOI: 10.1111/jdv.13055
JEADV 2016, 30, 852–909
Buerger’s disease mimicking cutaneous polyarteritis nodosa Dear Editor A 44-year-old Taiwanese man presented with multiple painful erythematous nodules over his dorsal hands with gradual extension to anterior forearms (Fig. 1a,b) since 3 months ago. He has been a heavy smoker for over two decades. For the present visit, he denied new drug intake, trauma history or any intravenous drug injections. A thorough physical examination revealed no coldness, throbbing pain, Raynaud phenomenon, cyanosis or gangrene changes over distal fingers and toes. No oral ulcers or genital ulcers were noted. Laboratory exam showed negative findings on antinuclear antibodies, anti-dsDNA antibodies, anti-Ro/ La antibodies, anti-ENA antibodies and anti-phospholipid antibodies. Prothrombin time, partial thromboplastin time, protein C and protein S levels were also within normal limit. Biopsy results showed vasculitis of an intermediate vessel with mixed infiltration over entire vessel walls (Fig. 1d). Tissue cultures for bacteria, fungus and tuberculosis were all negative. Tentatively, polyarteritis nodosa (PAN) was suspected due to comparable clinical, laboratory and pathology manifestations. However, a further Orcein staining enhanced the intact internal elastic lamina (Fig. 1e). Angiography study disclosed total occlusion of the superficial palmar arch and common palmar digital arteries at the 2nd to 5th finger with corkscrew-like collateral circulations (Fig. 1c), which is unlike the picture of multiple microaneurysms typically seen in PAN. The laser Doppler imaging test showed increased perfusion of all fingers at first presentation. However, 7 days later, sudden onset of coldness on right 3rd finger with skin lesion progression. Re-evaluation with laser Doppler imaging discovered significant decrease in perfusions over right 3rd finger compared to previous study. With the help of pathological evidence, angiographic picture and the corresponding skin perfusion response, Buerger’s disease was therefore diagnosed. The patient was administrated with prostaglandin E1 infusions 40 lg every 12 h intravenously, combined with oral Pentoxifylline. After 2-week treatment course, the coldness dramatically improved. He was then followed on an out-patient basis with oral Pentoxifylline and total abstinence from cigarettes. No recurrence of symptoms was noted during at least 6 months of follow-up. Buerger’s disease, also known as thromboangitis obliterans, involves medium size arteries and veins with inflammation resulting in intravascular thrombosis. The exact pathogenesis is still unclear, though researches pointed to both inherited and prothrombotic factors.1 Buerger’s disease often presented as peripheral cyanosis, Raynaud phenomenon or even digital ulceration and gangrene, unlike the tender erythematous subcutaneous nodules in our patient. Histopathologically, cutaneous PAN and Buerger’s disease both involve medium size arteries with arterial wall fibrinoid
© 2015 European Academy of Dermatology and Venereology
Letters to the Editor
893
(b)
(a)
(d)
(c)
(e)
Figure 1 Multiple tender erythematous subcutaneous nodules over the patient’s dorsal hand (b) and forearm (a). Also note total occlusion of the superficial palmar arch and disappearance of common palmar digital arteries at the 2nd to 5th finger with corkscrew-like collateral circulations (c) in the angiographic picture. (d) Haematoxylin and eosin staining shows leucocytoclastic vasculitis of an intermediate vessel with mixed infiltration over entire vessel walls (40 9 ), and (e) Orcein staining shows intact internal elastic lamina (40 9 ).
Table 1 Similarities and differences in Buerger’s disease and cutaneous polyarteritis nodosa Similarities in both disease
Buerger’s disease
Cutaneous polyarteritis nodosa
Clinical picture
–
Peripheral cyanosis Raynaud phenomenon Digital ulceration and gangrene
Bilateral, tender erythematous subcutaneous nodules
Pathological finding
Leucocytoclastic vasculitis
Relatively preserved internal elastic lamina
Destruction of internal elastic lamina
Angiographic picture
–
Occlusion of arteries with fine corkscrew collateral vessels
Disseminated segmental ectasis and stenosis as well as microaneurysms
Skin blood flow
–
Decreased
–
degeneration and infiltration by neutrophils and lymphocytes. The main pathological difference lies in the internal elastic lamina, which is destructed in cutaneous PAN and relatively intact in Buerger’s disease.2 In our case, Orcein stain showed intact internal elastic lamina, which favours the diagnosis of Buerger’s disease despite clinically similar to cutaneous PAN (Table 1). Buerger’s disease typically shows occlusions of the distal hand arteries, corkscrew collateral vessels3 and subsequent development of fine collateral network in angiographic studies, which is the case in our patient. On the other hand, disseminated segmental ectasis and stenosis as well as microaneurysms are typical findings in PAN. This implies the importance of angiography in aiding diagnosis of atypical cases in Buerger’s disease (Table 1). Laser Doppler imaging is a convenient and non-invasive tool to evaluate skin perfusion changes. One of the pathophysiological features of Buerger’s disease is vasospasm, thus resulting in decrease in skin blood flow seen in our patient. Our case
JEADV 2016, 30, 852–909
demonstrated the unusual presentation of Buerger’s disease with tender erythematous subcutaneous nodules mimicking cutaneous PAN. We highlighted that a variety of diagnostic strategies, such as pathological staining for internal elastic lamina and angiography may aid in accurate diagnosis of the disease, therefore, the appropriate treatment can be delivered to the patient. Y.-H. Kuo,1 C.-Y. Wu1,2* 1
Department of Dermatology, Kaohsiung Medical University Hospital, 100 Shih-Chuan 1st Road, Kaohsiung, Taiwan, 2College of Medicine, Kaohsiung Medical University, 100 Shih-Chuan 1st Road, Kaohsiung city, 807, Taiwan *Correspondence: C.-Y. Wu. E-mail: [email protected]
References 1 Malecki R, Zdrojowy K, Adamiec R. Thromboangitis obliterans in the 21st century – a new face of disease. Atherosclerosis 2009; 206: 328–334.
© 2015 European Academy of Dermatology and Venereology
Letters to the Editor
894
2 Ishiguro N, Kawashima M. Cutaneous polyarteritis nodosa: a report of 16 cases with clinical and histopathological analysis and a review of the published work. J Dermatol 2010; 37: 85–93. 3 Suzuki S, Yamada I, Himeno Y. Angiographic findings in Buerger disease. Int J Cardiol 1996; 54: S189–S195. DOI: 10.1111/jdv.13056
Fixed sunlight eruption: a case report Editor Fixed drug eruption (FDE) is usually due to drug intake; however, some patients cannot recall an offending drug as their cause of FDE. A 56-year-old man was seen because of persistent hyperpigmented macules. He reported that, for 5 years, every summer, about 6 h after sun exposure, he developed identical lesions that disappeared after 72 h leaving long-lasting residual hyperpigmentation. The lesions always reappeared in the same location on his buttocks and groins; he did not use a swimsuit for his sun exposures. The lesions consisted of round areas of erythema and oedema, with a burning sensation, and recurred every year at the beach. (a)
His medical history was relevant for dyslipidemia on treatment with simvastatin for 4 years. The patients denied having taken any medication associated with any of the reactivation events. On examination, we found 1–3 cm diameter, oval to round, hyperpigmented macules on buttocks and groins (Fig. 1a). Considering a diagnosis of fixed sunlight eruption, we carried out phototesting in a period of inactive lesions. The patient entered the UV cabin (Medisun 2800 innovation) using photoprotective glasses and covering his body except groins and buttocks. Lesions were elicited 5 h after exposure to UVA (1.27 J/cm2, 25% of MED-UVA) in the same sites they had been appearing during the past 5 years (Fig. 1b). A biopsy revealed a spongiosis, apoptotic keratinocytes, a lymphohistiocytic lichenoid dermatitis and extensive deposits of melanin pigment (Fig. 2a,b). When lesions became inactive, the patient was exposed to UVB in identical way as for UVA. A 25% of MED-UVB dose did not induce any reaction, but significant lesions appeared when the dosage was doubled (0.1 J/cm2). A skin biopsy showed identical features to the former one. The patient was recommended strict photoprotection, and new attacks have not occurred. Our patient presented a skin eruption consistent with FDE, but due to the absence of any drug trigger, the temporal relationship between sun exposure and the onset of symptoms, and positive phototesting to UVA and UVB, a
(b)
Figure 1 The physical examination revealed an oval to round, hyperpigmented macules on buttocks (a) and erythematosus plaques 5 h after exposure to UVA (b).
(a)
(b)
Figure 2 The biopsy specimen demonstrates a lymphohistiocytic lichenoid dermatitis (a), apoptotic keratinocytes and extensive deposits of melanin pigment (b).
JEADV 2016, 30, 852–909
© 2015 European Academy of Dermatology and Venereology
892
Children usually present with non-specific and insidious symptoms, such as asthenia and irritability, therefore early diagnosis can be challenging. Leukaemic changes in the bone marrow or peripheral blood generally precede skin involvement, however, in rare instances skin involvement is the first sign of leukaemia, preceding the diagnosis of systemic leukaemia by months or even years.2 Cutaneous lesions of leukaemia appear in two forms: leukaemia cutis, wherein the skin is infiltrated by leukemic (blast) cells, and leukaemids, which represent the non-specific cutaneous lesions of leukaemia.3 Leukaemia cutis is observed more frequently in acute myeloid leukaemia, being rare in acute lymphoblastic leukaemia. The clinical appearance of leukaemia cutis is variable. The most common manifestation are erythematous or violaceous plaques, papules or nodules involving the face, trunk and extremities.4 Less common appearances include macules, maculopapules or plaques. Involvement of the scalp is rare. The differential diagnosis of scalp nodules in children includes entities such as cylindromas, dermoid and epidermal cysts, dermatofibrosarcoma protuberans and haemangiomas.5 The correct diagnosis is established by histologic demonstration of true infiltration of the skin with leukemic cells. In summary we describe an unusual presentation of T-cell Acute Lymphoblastic Leukaemia in a child, which appeared clinically as leukaemia cutis. Leukaemia cutis is a rare form of presentation of leukaemia, which is even rarer in T-cell Acute Lymphoblastic Leukaemia. Leukaemic infiltration should be considered on the differential diagnosis of scalp lesions, especially when its presentation includes multiple tumours. lezM. Toro Montecinos,1,* A. Vicente,1 M.A. Gonza ~at,1 A. Catala Temprano,2 M. Sun ~ol3 Ensen Departments of 1Pediatric Dermatology, 2Pediatric Hematology and u, Oncology, 3Pathology, Hospital Universitari Sant Joan de De Barcelona, Spain *Correspondence: M. Toro Montecinos. E-mail: [email protected]
References 1 Yeoh AE, Tan D, Li CK, Hori H, Tse E, Pui CH. Management of adult and paediatric acute lymphoblastic leukaemia in Asia: resource-stratified guidelines from the Asian Oncology Summit 2013. Lancet Oncol 2013; 14: e508–e523. 2 Husak R, Blume-Peytaki U, Orfanos CE. Aleukemic leukemia cutis in an adolescent boy. N Engl J Med 1999; 340: 893–894. 3 Stawiski MA. Skin manifestations of leukemids and lymphomas. Cutis 1978; 21: 814–818. 4 Longacre TA, Smoller BR. Leukemia cutis. Analysis of 50 biopsy-proven cases with an emphasis on occurrence in myelodysplastic syndromes. Am J Clin Pathol 1993; 100: 276–284. 5 Anderson PC, Stotland MA, Dinulos JG, Perry AE. Acute lymphocytic leukemia presenting as an isolated scalp nodule in an infant. Ann Plast Surg 2010; 64: 251–253. DOI: 10.1111/jdv.13055
JEADV 2016, 30, 852–909
Buerger’s disease mimicking cutaneous polyarteritis nodosa Dear Editor A 44-year-old Taiwanese man presented with multiple painful erythematous nodules over his dorsal hands with gradual extension to anterior forearms (Fig. 1a,b) since 3 months ago. He has been a heavy smoker for over two decades. For the present visit, he denied new drug intake, trauma history or any intravenous drug injections. A thorough physical examination revealed no coldness, throbbing pain, Raynaud phenomenon, cyanosis or gangrene changes over distal fingers and toes. No oral ulcers or genital ulcers were noted. Laboratory exam showed negative findings on antinuclear antibodies, anti-dsDNA antibodies, anti-Ro/ La antibodies, anti-ENA antibodies and anti-phospholipid antibodies. Prothrombin time, partial thromboplastin time, protein C and protein S levels were also within normal limit. Biopsy results showed vasculitis of an intermediate vessel with mixed infiltration over entire vessel walls (Fig. 1d). Tissue cultures for bacteria, fungus and tuberculosis were all negative. Tentatively, polyarteritis nodosa (PAN) was suspected due to comparable clinical, laboratory and pathology manifestations. However, a further Orcein staining enhanced the intact internal elastic lamina (Fig. 1e). Angiography study disclosed total occlusion of the superficial palmar arch and common palmar digital arteries at the 2nd to 5th finger with corkscrew-like collateral circulations (Fig. 1c), which is unlike the picture of multiple microaneurysms typically seen in PAN. The laser Doppler imaging test showed increased perfusion of all fingers at first presentation. However, 7 days later, sudden onset of coldness on right 3rd finger with skin lesion progression. Re-evaluation with laser Doppler imaging discovered significant decrease in perfusions over right 3rd finger compared to previous study. With the help of pathological evidence, angiographic picture and the corresponding skin perfusion response, Buerger’s disease was therefore diagnosed. The patient was administrated with prostaglandin E1 infusions 40 lg every 12 h intravenously, combined with oral Pentoxifylline. After 2-week treatment course, the coldness dramatically improved. He was then followed on an out-patient basis with oral Pentoxifylline and total abstinence from cigarettes. No recurrence of symptoms was noted during at least 6 months of follow-up. Buerger’s disease, also known as thromboangitis obliterans, involves medium size arteries and veins with inflammation resulting in intravascular thrombosis. The exact pathogenesis is still unclear, though researches pointed to both inherited and prothrombotic factors.1 Buerger’s disease often presented as peripheral cyanosis, Raynaud phenomenon or even digital ulceration and gangrene, unlike the tender erythematous subcutaneous nodules in our patient. Histopathologically, cutaneous PAN and Buerger’s disease both involve medium size arteries with arterial wall fibrinoid
© 2015 European Academy of Dermatology and Venereology
Letters to the Editor
893
(b)
(a)
(d)
(c)
(e)
Figure 1 Multiple tender erythematous subcutaneous nodules over the patient’s dorsal hand (b) and forearm (a). Also note total occlusion of the superficial palmar arch and disappearance of common palmar digital arteries at the 2nd to 5th finger with corkscrew-like collateral circulations (c) in the angiographic picture. (d) Haematoxylin and eosin staining shows leucocytoclastic vasculitis of an intermediate vessel with mixed infiltration over entire vessel walls (40 9 ), and (e) Orcein staining shows intact internal elastic lamina (40 9 ).
Table 1 Similarities and differences in Buerger’s disease and cutaneous polyarteritis nodosa Similarities in both disease
Buerger’s disease
Cutaneous polyarteritis nodosa
Clinical picture
–
Peripheral cyanosis Raynaud phenomenon Digital ulceration and gangrene
Bilateral, tender erythematous subcutaneous nodules
Pathological finding
Leucocytoclastic vasculitis
Relatively preserved internal elastic lamina
Destruction of internal elastic lamina
Angiographic picture
–
Occlusion of arteries with fine corkscrew collateral vessels
Disseminated segmental ectasis and stenosis as well as microaneurysms
Skin blood flow
–
Decreased
–
degeneration and infiltration by neutrophils and lymphocytes. The main pathological difference lies in the internal elastic lamina, which is destructed in cutaneous PAN and relatively intact in Buerger’s disease.2 In our case, Orcein stain showed intact internal elastic lamina, which favours the diagnosis of Buerger’s disease despite clinically similar to cutaneous PAN (Table 1). Buerger’s disease typically shows occlusions of the distal hand arteries, corkscrew collateral vessels3 and subsequent development of fine collateral network in angiographic studies, which is the case in our patient. On the other hand, disseminated segmental ectasis and stenosis as well as microaneurysms are typical findings in PAN. This implies the importance of angiography in aiding diagnosis of atypical cases in Buerger’s disease (Table 1). Laser Doppler imaging is a convenient and non-invasive tool to evaluate skin perfusion changes. One of the pathophysiological features of Buerger’s disease is vasospasm, thus resulting in decrease in skin blood flow seen in our patient. Our case
JEADV 2016, 30, 852–909
demonstrated the unusual presentation of Buerger’s disease with tender erythematous subcutaneous nodules mimicking cutaneous PAN. We highlighted that a variety of diagnostic strategies, such as pathological staining for internal elastic lamina and angiography may aid in accurate diagnosis of the disease, therefore, the appropriate treatment can be delivered to the patient. Y.-H. Kuo,1 C.-Y. Wu1,2* 1
Department of Dermatology, Kaohsiung Medical University Hospital, 100 Shih-Chuan 1st Road, Kaohsiung, Taiwan, 2College of Medicine, Kaohsiung Medical University, 100 Shih-Chuan 1st Road, Kaohsiung city, 807, Taiwan *Correspondence: C.-Y. Wu. E-mail: [email protected]
References 1 Malecki R, Zdrojowy K, Adamiec R. Thromboangitis obliterans in the 21st century – a new face of disease. Atherosclerosis 2009; 206: 328–334.
© 2015 European Academy of Dermatology and Venereology
Letters to the Editor
894
2 Ishiguro N, Kawashima M. Cutaneous polyarteritis nodosa: a report of 16 cases with clinical and histopathological analysis and a review of the published work. J Dermatol 2010; 37: 85–93. 3 Suzuki S, Yamada I, Himeno Y. Angiographic findings in Buerger disease. Int J Cardiol 1996; 54: S189–S195. DOI: 10.1111/jdv.13056
Fixed sunlight eruption: a case report Editor Fixed drug eruption (FDE) is usually due to drug intake; however, some patients cannot recall an offending drug as their cause of FDE. A 56-year-old man was seen because of persistent hyperpigmented macules. He reported that, for 5 years, every summer, about 6 h after sun exposure, he developed identical lesions that disappeared after 72 h leaving long-lasting residual hyperpigmentation. The lesions always reappeared in the same location on his buttocks and groins; he did not use a swimsuit for his sun exposures. The lesions consisted of round areas of erythema and oedema, with a burning sensation, and recurred every year at the beach. (a)
His medical history was relevant for dyslipidemia on treatment with simvastatin for 4 years. The patients denied having taken any medication associated with any of the reactivation events. On examination, we found 1–3 cm diameter, oval to round, hyperpigmented macules on buttocks and groins (Fig. 1a). Considering a diagnosis of fixed sunlight eruption, we carried out phototesting in a period of inactive lesions. The patient entered the UV cabin (Medisun 2800 innovation) using photoprotective glasses and covering his body except groins and buttocks. Lesions were elicited 5 h after exposure to UVA (1.27 J/cm2, 25% of MED-UVA) in the same sites they had been appearing during the past 5 years (Fig. 1b). A biopsy revealed a spongiosis, apoptotic keratinocytes, a lymphohistiocytic lichenoid dermatitis and extensive deposits of melanin pigment (Fig. 2a,b). When lesions became inactive, the patient was exposed to UVB in identical way as for UVA. A 25% of MED-UVB dose did not induce any reaction, but significant lesions appeared when the dosage was doubled (0.1 J/cm2). A skin biopsy showed identical features to the former one. The patient was recommended strict photoprotection, and new attacks have not occurred. Our patient presented a skin eruption consistent with FDE, but due to the absence of any drug trigger, the temporal relationship between sun exposure and the onset of symptoms, and positive phototesting to UVA and UVB, a
(b)
Figure 1 The physical examination revealed an oval to round, hyperpigmented macules on buttocks (a) and erythematosus plaques 5 h after exposure to UVA (b).
(a)
(b)
Figure 2 The biopsy specimen demonstrates a lymphohistiocytic lichenoid dermatitis (a), apoptotic keratinocytes and extensive deposits of melanin pigment (b).
JEADV 2016, 30, 852–909
© 2015 European Academy of Dermatology and Venereology
