Letters to the Editor
147
5 Fan W, Zhang Q. Risk factors for male patients with gonorrhoea complicated by inflammation of the paraurethral glands around the external urethral orifice. Int J STD AIDS 2012; 23: 400–402. 6 Fan W, Zhang Q, Song L. Acquired microcyst in the paraurethral glands of a male patient. Int J STD AIDS 2013; 24: 159–160. 7 Fan W, Zhang Q. Bilateral inflammation of the paraurethral glands around the external urethral orifice due to Chlamydia trachomatis in a male. Int J Dermatol 2013; 52: 1567–1568. 8 Fan W, Zhang Q, Jiang T. Pathogen profile in men with inflammation of paraurethral glands. Sex Transm Infect 2014; 90: 52–54. 9 Bruhns C. Rieckes Lehrbuch der Haut und Geschlechts-Krankheiten. Gustav Fisher, Jena, 1931: 710–713. 10 Buchanan GN, Halligan S, Bartram CI et al. Clinical examination, endosonography, and MR imaging in preoperative assessment of fistula in ano: comparison with outcome-based reference standard. Radiology 2004; 233: 674–681. DOI: 10.1111/jdv.12638
Figure 2 High-frequency ultrasound showed tubular hypoechoic areas with well-defined borders and smooth margins. One end was blind and the other open to the atmosphere. The ducts lay parallel to the urethra. The lumen diameter was 0.7 mm, and length 12.0 mm.
considered if first-line treatment fails. As the lesions are located in the glans, and the paraurethral duct is too tiny to be measured accurately, it has previously been difficult for surgeons to judge the depth of incision and amount of tissue to excise. Highfrequency ultrasound examination, however, overcomes this difficulty and provides valuable information to guide more invasive surgical treatments when they are needed. Anechoic areas containing liquid were seen in tubular hypoechoic areas in five of the patients, which may be attributed to a small amount of residual pus in the paraurethral ducts after physical examination and specimen collection. W. Fan,1,* Q. Zhang,2 X. Ye3 1
Department of Dermatology, First People’s Hospital of Changshu City, Changshu Hospital, Affiliated to Soochow University, 2Department of Dermatology, Traditional Chinese Medical Hospital of Changshu City, 3 Department of Ultrasound, First People’s Hospital of Changshu City, Changshu Hospital, Affiliated to Soochow University, Changshu, Jiangsu Province, China *Correspondence: W. Fan. E-mail: [email protected]
References 1 King A, Nicol C. Venereal diseases, 2nd edn. Davis, Philadelphia, 1969: 144–161. 2 Gilhooly P, Hensle TW. Parameatal ducts of glans penis. Structure, symptoms, and treatment of uncommon focus of infection. Urology 1984; 24: 375–378. 3 Fan W. Gonococcal inflammation of paraurethral glands around external urethral orifice in males: a commonly encountered disease? Int J STD AIDS 2010; 21: 225–226. 4 Fan W, Zhang Q, Fan Z. Sequential therapy for gonococci inflammation of paraurethral glands in males. J Dermatolog Treat 2012; 23: 184–188.
JEADV 2016, 30, 124–200
Acquired lymphangioma circumscriptum of the vulva in a twin pregnancy Editor Lymphangiectasia is also called acquired lymphangioma circumscriptum (LC) or secondary lymphangiomas. The clinical appearance of LC may vary greatly, ranging from clear fluidfilled blisters to smooth flesh-coloured papules or nodules.1 The condition appears to be uncommon and to the best of our knowledge only a single case of lymphangiectasia of the vulva occurring in pregnancy has been reported. We report a case of acquired LC to emphasize the importance of recognizing this condition in pregnancy and differentiating it from other vulvar dermatoses which can have a similar clinical presentation, but may require a different approach to management. A 28-year-old primigravida presented with complaints of multiple raised pruritic lesions over her genitalia since one month. The patient was in the second trimester of her twin pregnancy, while rest of her obstetric history was not significant. There was no history of sexually transmitted diseases, tuberculosis, filariasis, long-term medication, Crohn’s disease, lower limb swelling or similar episodes in the past. Additionally, there was no history suggestive of abdominal tumours, surgical or radiation treatment to the pelvis in the recent past. Examination of the labia minora and majora revealed multiple non-tender translucent papules and vesicles arranged in groups giving an apparent verrucous appearance (Fig. 1) .A majority of the lesions were located over the labia minora, which itself appeared slightly oedematous (Fig. 2). There was no inguinal lymphadenopathy, varicose veins and oedema of the lower limbs or haemorrhoids. One of the translucent papules was punctured with an 18-gauge needle to
© 2014 European Academy of Dermatology and Venereology
148
Figure 1 The vulva showing numerous translucent papules and vesicles arranged in groups.
Figure 2 A closer view showing the verrucous surface of oedematous labia minora.
reveal flow of clear yellow fluid. A clinical diagnosis of lymphangiectasia of the vulva was made. It was decided not to subject the patient to a biopsy due to her underlying pregnancy. No therapeutic intervention was performed and the patient was asked to
JEADV 2016, 30, 124–200
Letters to the Editor
Figure 3 Complete resolution of the acquired lymphangiectasias in the post-partum period.
return in the post-partum period. Two months after delivery, the patient returned with a complete resolution of the condition (Fig. 3). Much confusion has arisen in dermatological literature regarding the nomenclature of the lymphangiectasias. The suffix ‘-ectasia’ means dilation or distension of a tubular structure, as in lymphangiectasia. The other terms of lymphangioma, acquired lymphangiomas and acquired lymphangioma circumscriptum are not valid descriptions, as the suffix ‘-oma’ denotes a tumour and lymphangiectasias are clearly not tumours.2 Verma in his article proposed that the term ‘superficial lymph-vessel ectasia’ is the correct term to describe this condition.3 Genital skin is particularly prone to lymphangiectasia. Lymph blisters of the penis or scrotum (and less commonly vulva), leaking lymph or chyle, may be seen in primary lymphoedema. Lymphangiectasia/acquired lymphangioma of the vulva or scrotum has been described following cancer treatment, tuberculous inguinal lymphadenitis and genital involvement with Crohn’s disease.2 Lymphangiectases arise following damage to previously normal, deep lymphatic vessels. The mechanism by which they form is identical to congenitally determined lymphatic malformations (lymphangiomas), that is obstruction to drainage leads to backpressure and dermal backflow, with subsequent dilatation of upper dermal lymphatics.4Only one case of pregnancy-induced lymphangiectasias of the vulva has been reported by Verma.5 Lymphangiectasia is difficult to diagnose, especially when it presents in the genital region. This is a particular problem in developing countries where specialists from various fields of medicine
© 2014 European Academy of Dermatology and Venereology
Letters to the Editor
149
(surgeons, gynaecologists, dermatologists and general practitioners) may see the condition, and where there is often a tendency to avoid invasive and expensive diagnostic procedures.3 Unlike the classic, frog spawn appearance of lymphangioma circumscriptum, lymphangiectasias of the vulva present as large clusters of vesicles and papules or oedematous polypoid nodules mimicking genital warts.6 Hence, vulvar LC needs to be differentiated from various dermatological disorders for which treatment methods are different, such as genital warts, herpes zoster, molluscum contagiosum and leiomyoma.7 Histologically, numerous dilated lymphatics in the superficial and papillary dermis are seen. There is clear fluid and, less frequently, red blood cells in their lumina. In the overlying epidermis there is some degree of acanthosis and hyperkeratosis. The surrounding stroma shows scattered lymphocytes.8 Recognition and appropriate treatment of vulval lymphangiectasia is important primarily because the lesions may act as portals of entry for infection. In addition, persistent leakage of lymphatic fluid may be mistaken for urinary incontinence.3 There is no standard therapy for the management of LC. The most common procedures are abrasive therapy, sclerotherapy, electrocoagulation and surgical resection. Even with the best treatment option, recurrence is common.9 In our case, the obstruction of the lymphatics by an overtly distended gravid uterus appears to be the most plausible explanation for the vulvar lymphangiectasia occurring in the setting of a twin pregnancy, resolving spontaneously in the postpartum period with the physiological involution of the uterus. U. Khanna, P. D’Souza* Department of Dermatology and Venereology, ESIPGIMSR, Basaidarapur, New Delhi 110015, India *Correspondence: Paschal D Souza. E-mail: paschaldsouza@yahoo. com We report a case of lymphangiectasia of the vulva occurring in the setting of a twin pregnancy to emphasize the importance of recognising this condition in pregnancy and differentiating it from other vulvar dermatoses which can present similarly, but may require a different approach to management
References 1 Harwood CA, Mortimer PS. Acquired vulval lymphangiomata mimicking genital warts. Br J Dermatol 1993; 129: 334–336. 2 Mortimer PS. Disorders of lymphatic vessels. In Burns T, Breathnach T, Cox N, Griffiths C, eds. Rook’s Textbook of Dermatology, Vol. 3, 8th edn. Blackwell Science Ltd, Oxford, 2010: 48.25–48.28. 3 Verma SB. Lymphangiectasias of the skin: victims of confusing nomenclature. Clin Exp Dermatol 2009; 34(5): 566–569. 4 Mallett RB, Curley RK, Mortimer PS. Acquired lymphangiomata: report of four cases and a discussion of the pathogenesis. Br J Dermatol 1992; 126: 380–382. 5 Verma S. Pregnancy-induced lymphangiectasias of the vulva. Int J STD AIDS 2008; 19(3): 211–212. 6 Peachey RDG, Lim CC, Whimster IW. Lymphangioma of the skin. Br J Dermatol 1970; 83: 519. 7 Vlastos AT, Malpica A, Follen M. Lymphangioma circumscriptum of the vulva: a review of the literature. Obstet Gynecol 2003; 101: 946–954.
JEADV 2016, 30, 124–200
8 Mu XC, Tran TA, Dupree M, Carlson JA. Acquired vulvar lymphangioma mimicking genital warts. A case report and review of the literature. J Cutan Pathol 1999; 26(3): 150–154. 9 Roy KK, Agarwal R, Agarwal S, Kumar S, Malhotra N, Gopendru N. Recurrent vulvar lymphangioma circumscriptum - a case report and literature review. Int J Gynecol Cancer 2006; 16: 930–934. DOI: 10.1111/jdv.12640
Colour Doppler ultrasound findings in the nail in cystic fibrosis Editor Cystic fibrosis (CF) is an autosomal recessive disease resulting from mutations in the CF transmembrane conductance regulator gene. The main clinical manifestations are related to lung disease, pancreatic insufficiency and infertility.1 CF can also present with skin changes, however, such cutaneous manifestations seem to be underestimated.2 A common, but not specific sign of CF is digital clubbing. Nevertheless, the pathophysiology of clubbing still remains unclear. Possible causes of clubbing have included several factors such as hypoxia, platelet activation, releasing of platelet-derived growth factor and vascular endothelial growth factor.3,4 Prostaglandins, ferritin, bradykinin and oestrogen have also been implicated in the genesis of this morphological alteration.5 The ‘spongy’ feel of the nail have been postulated to be caused by a fibrovascular hyperplasia of the underlying connective tissue of the nail bed.6 A high presence of electrolytes such as sodium and chloride has been reported in the nail bed of the CF patients.7 Ultrasound has been proven as a useful imaging technique for studying the nails non- invasively. Thus, a wide range of inflammatory and tumoural conditions have been well described on sonography.8,9 To date, there are no reports on the imaging characteristics of the nail bed in CF. We report the case of a 25-year-old male CF patient who was diagnosed by the age of 7. The physical examination revealed clubbing in both hands (Fig. 1). To rule out underlying nail pathology an ultrasound examination of the ungual region was requested. The sonographic examination demonstrated a diffuse increase of the thickness and decreased echogenicity of the nail bed in the fingernails of both hands which was more important on both thumbs. This alteration involved the matrix region in all nails and was accompanied by an upward displacement of the ungual plates. The proximal part of the ungual plates in both thumbs was irregular being the latter finding more prominent in the left thumb. In some of the fingers loss of the proximal part of the
© 2014 European Academy of Dermatology and Venereology
147
5 Fan W, Zhang Q. Risk factors for male patients with gonorrhoea complicated by inflammation of the paraurethral glands around the external urethral orifice. Int J STD AIDS 2012; 23: 400–402. 6 Fan W, Zhang Q, Song L. Acquired microcyst in the paraurethral glands of a male patient. Int J STD AIDS 2013; 24: 159–160. 7 Fan W, Zhang Q. Bilateral inflammation of the paraurethral glands around the external urethral orifice due to Chlamydia trachomatis in a male. Int J Dermatol 2013; 52: 1567–1568. 8 Fan W, Zhang Q, Jiang T. Pathogen profile in men with inflammation of paraurethral glands. Sex Transm Infect 2014; 90: 52–54. 9 Bruhns C. Rieckes Lehrbuch der Haut und Geschlechts-Krankheiten. Gustav Fisher, Jena, 1931: 710–713. 10 Buchanan GN, Halligan S, Bartram CI et al. Clinical examination, endosonography, and MR imaging in preoperative assessment of fistula in ano: comparison with outcome-based reference standard. Radiology 2004; 233: 674–681. DOI: 10.1111/jdv.12638
Figure 2 High-frequency ultrasound showed tubular hypoechoic areas with well-defined borders and smooth margins. One end was blind and the other open to the atmosphere. The ducts lay parallel to the urethra. The lumen diameter was 0.7 mm, and length 12.0 mm.
considered if first-line treatment fails. As the lesions are located in the glans, and the paraurethral duct is too tiny to be measured accurately, it has previously been difficult for surgeons to judge the depth of incision and amount of tissue to excise. Highfrequency ultrasound examination, however, overcomes this difficulty and provides valuable information to guide more invasive surgical treatments when they are needed. Anechoic areas containing liquid were seen in tubular hypoechoic areas in five of the patients, which may be attributed to a small amount of residual pus in the paraurethral ducts after physical examination and specimen collection. W. Fan,1,* Q. Zhang,2 X. Ye3 1
Department of Dermatology, First People’s Hospital of Changshu City, Changshu Hospital, Affiliated to Soochow University, 2Department of Dermatology, Traditional Chinese Medical Hospital of Changshu City, 3 Department of Ultrasound, First People’s Hospital of Changshu City, Changshu Hospital, Affiliated to Soochow University, Changshu, Jiangsu Province, China *Correspondence: W. Fan. E-mail: [email protected]
References 1 King A, Nicol C. Venereal diseases, 2nd edn. Davis, Philadelphia, 1969: 144–161. 2 Gilhooly P, Hensle TW. Parameatal ducts of glans penis. Structure, symptoms, and treatment of uncommon focus of infection. Urology 1984; 24: 375–378. 3 Fan W. Gonococcal inflammation of paraurethral glands around external urethral orifice in males: a commonly encountered disease? Int J STD AIDS 2010; 21: 225–226. 4 Fan W, Zhang Q, Fan Z. Sequential therapy for gonococci inflammation of paraurethral glands in males. J Dermatolog Treat 2012; 23: 184–188.
JEADV 2016, 30, 124–200
Acquired lymphangioma circumscriptum of the vulva in a twin pregnancy Editor Lymphangiectasia is also called acquired lymphangioma circumscriptum (LC) or secondary lymphangiomas. The clinical appearance of LC may vary greatly, ranging from clear fluidfilled blisters to smooth flesh-coloured papules or nodules.1 The condition appears to be uncommon and to the best of our knowledge only a single case of lymphangiectasia of the vulva occurring in pregnancy has been reported. We report a case of acquired LC to emphasize the importance of recognizing this condition in pregnancy and differentiating it from other vulvar dermatoses which can have a similar clinical presentation, but may require a different approach to management. A 28-year-old primigravida presented with complaints of multiple raised pruritic lesions over her genitalia since one month. The patient was in the second trimester of her twin pregnancy, while rest of her obstetric history was not significant. There was no history of sexually transmitted diseases, tuberculosis, filariasis, long-term medication, Crohn’s disease, lower limb swelling or similar episodes in the past. Additionally, there was no history suggestive of abdominal tumours, surgical or radiation treatment to the pelvis in the recent past. Examination of the labia minora and majora revealed multiple non-tender translucent papules and vesicles arranged in groups giving an apparent verrucous appearance (Fig. 1) .A majority of the lesions were located over the labia minora, which itself appeared slightly oedematous (Fig. 2). There was no inguinal lymphadenopathy, varicose veins and oedema of the lower limbs or haemorrhoids. One of the translucent papules was punctured with an 18-gauge needle to
© 2014 European Academy of Dermatology and Venereology
148
Figure 1 The vulva showing numerous translucent papules and vesicles arranged in groups.
Figure 2 A closer view showing the verrucous surface of oedematous labia minora.
reveal flow of clear yellow fluid. A clinical diagnosis of lymphangiectasia of the vulva was made. It was decided not to subject the patient to a biopsy due to her underlying pregnancy. No therapeutic intervention was performed and the patient was asked to
JEADV 2016, 30, 124–200
Letters to the Editor
Figure 3 Complete resolution of the acquired lymphangiectasias in the post-partum period.
return in the post-partum period. Two months after delivery, the patient returned with a complete resolution of the condition (Fig. 3). Much confusion has arisen in dermatological literature regarding the nomenclature of the lymphangiectasias. The suffix ‘-ectasia’ means dilation or distension of a tubular structure, as in lymphangiectasia. The other terms of lymphangioma, acquired lymphangiomas and acquired lymphangioma circumscriptum are not valid descriptions, as the suffix ‘-oma’ denotes a tumour and lymphangiectasias are clearly not tumours.2 Verma in his article proposed that the term ‘superficial lymph-vessel ectasia’ is the correct term to describe this condition.3 Genital skin is particularly prone to lymphangiectasia. Lymph blisters of the penis or scrotum (and less commonly vulva), leaking lymph or chyle, may be seen in primary lymphoedema. Lymphangiectasia/acquired lymphangioma of the vulva or scrotum has been described following cancer treatment, tuberculous inguinal lymphadenitis and genital involvement with Crohn’s disease.2 Lymphangiectases arise following damage to previously normal, deep lymphatic vessels. The mechanism by which they form is identical to congenitally determined lymphatic malformations (lymphangiomas), that is obstruction to drainage leads to backpressure and dermal backflow, with subsequent dilatation of upper dermal lymphatics.4Only one case of pregnancy-induced lymphangiectasias of the vulva has been reported by Verma.5 Lymphangiectasia is difficult to diagnose, especially when it presents in the genital region. This is a particular problem in developing countries where specialists from various fields of medicine
© 2014 European Academy of Dermatology and Venereology
Letters to the Editor
149
(surgeons, gynaecologists, dermatologists and general practitioners) may see the condition, and where there is often a tendency to avoid invasive and expensive diagnostic procedures.3 Unlike the classic, frog spawn appearance of lymphangioma circumscriptum, lymphangiectasias of the vulva present as large clusters of vesicles and papules or oedematous polypoid nodules mimicking genital warts.6 Hence, vulvar LC needs to be differentiated from various dermatological disorders for which treatment methods are different, such as genital warts, herpes zoster, molluscum contagiosum and leiomyoma.7 Histologically, numerous dilated lymphatics in the superficial and papillary dermis are seen. There is clear fluid and, less frequently, red blood cells in their lumina. In the overlying epidermis there is some degree of acanthosis and hyperkeratosis. The surrounding stroma shows scattered lymphocytes.8 Recognition and appropriate treatment of vulval lymphangiectasia is important primarily because the lesions may act as portals of entry for infection. In addition, persistent leakage of lymphatic fluid may be mistaken for urinary incontinence.3 There is no standard therapy for the management of LC. The most common procedures are abrasive therapy, sclerotherapy, electrocoagulation and surgical resection. Even with the best treatment option, recurrence is common.9 In our case, the obstruction of the lymphatics by an overtly distended gravid uterus appears to be the most plausible explanation for the vulvar lymphangiectasia occurring in the setting of a twin pregnancy, resolving spontaneously in the postpartum period with the physiological involution of the uterus. U. Khanna, P. D’Souza* Department of Dermatology and Venereology, ESIPGIMSR, Basaidarapur, New Delhi 110015, India *Correspondence: Paschal D Souza. E-mail: paschaldsouza@yahoo. com We report a case of lymphangiectasia of the vulva occurring in the setting of a twin pregnancy to emphasize the importance of recognising this condition in pregnancy and differentiating it from other vulvar dermatoses which can present similarly, but may require a different approach to management
References 1 Harwood CA, Mortimer PS. Acquired vulval lymphangiomata mimicking genital warts. Br J Dermatol 1993; 129: 334–336. 2 Mortimer PS. Disorders of lymphatic vessels. In Burns T, Breathnach T, Cox N, Griffiths C, eds. Rook’s Textbook of Dermatology, Vol. 3, 8th edn. Blackwell Science Ltd, Oxford, 2010: 48.25–48.28. 3 Verma SB. Lymphangiectasias of the skin: victims of confusing nomenclature. Clin Exp Dermatol 2009; 34(5): 566–569. 4 Mallett RB, Curley RK, Mortimer PS. Acquired lymphangiomata: report of four cases and a discussion of the pathogenesis. Br J Dermatol 1992; 126: 380–382. 5 Verma S. Pregnancy-induced lymphangiectasias of the vulva. Int J STD AIDS 2008; 19(3): 211–212. 6 Peachey RDG, Lim CC, Whimster IW. Lymphangioma of the skin. Br J Dermatol 1970; 83: 519. 7 Vlastos AT, Malpica A, Follen M. Lymphangioma circumscriptum of the vulva: a review of the literature. Obstet Gynecol 2003; 101: 946–954.
JEADV 2016, 30, 124–200
8 Mu XC, Tran TA, Dupree M, Carlson JA. Acquired vulvar lymphangioma mimicking genital warts. A case report and review of the literature. J Cutan Pathol 1999; 26(3): 150–154. 9 Roy KK, Agarwal R, Agarwal S, Kumar S, Malhotra N, Gopendru N. Recurrent vulvar lymphangioma circumscriptum - a case report and literature review. Int J Gynecol Cancer 2006; 16: 930–934. DOI: 10.1111/jdv.12640
Colour Doppler ultrasound findings in the nail in cystic fibrosis Editor Cystic fibrosis (CF) is an autosomal recessive disease resulting from mutations in the CF transmembrane conductance regulator gene. The main clinical manifestations are related to lung disease, pancreatic insufficiency and infertility.1 CF can also present with skin changes, however, such cutaneous manifestations seem to be underestimated.2 A common, but not specific sign of CF is digital clubbing. Nevertheless, the pathophysiology of clubbing still remains unclear. Possible causes of clubbing have included several factors such as hypoxia, platelet activation, releasing of platelet-derived growth factor and vascular endothelial growth factor.3,4 Prostaglandins, ferritin, bradykinin and oestrogen have also been implicated in the genesis of this morphological alteration.5 The ‘spongy’ feel of the nail have been postulated to be caused by a fibrovascular hyperplasia of the underlying connective tissue of the nail bed.6 A high presence of electrolytes such as sodium and chloride has been reported in the nail bed of the CF patients.7 Ultrasound has been proven as a useful imaging technique for studying the nails non- invasively. Thus, a wide range of inflammatory and tumoural conditions have been well described on sonography.8,9 To date, there are no reports on the imaging characteristics of the nail bed in CF. We report the case of a 25-year-old male CF patient who was diagnosed by the age of 7. The physical examination revealed clubbing in both hands (Fig. 1). To rule out underlying nail pathology an ultrasound examination of the ungual region was requested. The sonographic examination demonstrated a diffuse increase of the thickness and decreased echogenicity of the nail bed in the fingernails of both hands which was more important on both thumbs. This alteration involved the matrix region in all nails and was accompanied by an upward displacement of the ungual plates. The proximal part of the ungual plates in both thumbs was irregular being the latter finding more prominent in the left thumb. In some of the fingers loss of the proximal part of the
© 2014 European Academy of Dermatology and Venereology
