Correspondence
A 36-year-old man presented with a 5-day history of an itchy facial rash. His medical history was unremarkable apart from acne vulgaris as a teenager. On physical examination, striking tumid erythematous papules, plaques and nodules were seen predominantly on the lower cheeks, and scaly erythematous plaques over the central forehead, upper cheeks, chin and neck (Fig 1a). The eyebrows, beard and scalp appeared normal with no obvious hair loss, and the cutaneous findings were limited to the head and neck. The differential diagnoses considered included granulomatous rosacea, sarcoidosis and lupus erythematosus. Pending histological confirmation, twicedaily topical 0.1% tacrolimus ointment was commenced. On histological examination of an incisional biopsy taken from the left pre-auricular area, a prominent perifollicular inflammatory cell infiltrate was seen, comprised mainly of lymphocytes, with mucinous degeneration of the follicular epithelium (Fig. 2a,b). There was no significant lymphocyte atypia, and the lymphoid immunoprofile was normal. Follicular destruction and granulomatous inflammation were absent. Fungal, bacterial and mycobacterial cultures were negative, as was direct immunofluorescence. Alcian blue staining confirmed the presence of intrafollicular mucin deposits (Fig 2c). Findings were consistent with a diagnosis of benign FM. On review 10 days later, the patient’s facial eruption had dramatically improved (Fig 1b). Full blood count, erythrocyte sedimentation rate, lactate dehydrogenase, human T cell lymphotropic virus (HTLV)1 and HTLV2 serology, and lymphocyte immunophenotyping were all normal or negative. T-cell gene rearrangement studies confirmed a polyclonal population, and a final diagnosis of primary idiopathic FM was made. Treatment with topical 0.1% tacrolimus ointment was tapered over the following 4 weeks, and remission has been maintained for > 1 year. A single case of recalcitrant FM successfully treated with 1% pimecrolimus cream has previously been reported.5 The response to tacrolimus ointment in our case was unusually rapid, and spontaneous resolution of lesions cannot be excluded. The risk of activating what may be considered a premalignant condition remains a concern, and further studies evaluating the efficacy, safety and tolerability of topical tacrolimus ointment in FM are required before it can be recommended. J. Kluk,1 N. Krassilnik,2 and S. R. McBride1 Departments of 1Dermatology and 2Pathology, Royal Free London NHS Foundation Trust, Pond street, London, NW3 2QG, UK E-mail: [email protected] Conflict of interest: none declared. Accepted for publication 15 August 2013
References 1 Kim KR, Lee JY, Kim MK et al. Successful treatment of recalcitrant primary follicular mucinosis with
228
Clinical and Experimental Dermatology (2014) 39, pp216–234
2
3
4
5
indomethacin and low-dose intralesional interferon alpha. Ann Dermatol 2009; 21: 285–7. Cerroni L, Fink-Puches R, B€ ack B et al. Follicular mucinosis: a critical reappraisal of clinicopathologic features and association with mycosis fungoides and Sezary syndrome. Arch Dermatol 2002; 138: 182–9. Rongioletti F, De LD, Meyes D et al. Follicular mucinosis: a clinicopathologic, histochemical, immunohistochemical and molecular study comparing the primary benign form and the mycosis fungoides-associated follicular mucinosis. J Cutan Pathol 2010; 37: 15–19. Schneider SW, Metze D, Bonsmann G. Treatment of so-called idiopathic follicular mucinosis with hydroxychloroquine. Br J Dermatol 2010; 163: 420–3. Gorpelioglu C, Sarifakioglu E, Bayrak R. A case of follicular mucinosis treated successfully with pimecrolimus. Clin Exp Dermatol 2008; 34: 86–7.
Borderline lepromatous leprosy with type 1 (downgrading) reaction doi: 10.1111/ced.12247 In their interesting paper, describing a patient with fever, nodulobullous eruption and swollen ears, Golberg et al.1 made the diagnosis of borderline lepromatous leprosy with type 1 (reversal) reaction. However, I suggest that the diagnosis is more likely to be borderline lepromatous leprosy with type 1 (downgrading) reaction because the reaction had occurred before the appropriate therapy was started, and there were multiple acid-fast bacilli visible in the histopathological figure. Clinically, both reversal and downgrading reactions are indistinguishable without history-taking and histopathology results.2 A. Gohar Gohar’s Skin and Sexual Health Clinic, Doctors’ Building (7B), First Central Corridor, 6 October City, 12451, Greater Cairo, Egypt E-mail: [email protected] Conflict of interest: none declared. Accepted for publication 30 September 2013
References 1 Golberg O, Burd R, Sahota A, Wiselka M. Fever, nodulobullous eruption and swollen ears. Clin Exp Dermatol 2013; 38: 811–13. 2 Bryceson ADM, Pfaltzgraff RE. Leprosy, 3rd edn. Edinburgh: Churchill Livingstone, 1990; 115–26.
Cutaneous collagenous vasculopathy: report of a case doi: 10.1111/ced.12258 Cutaneous collagenous vasculopathy (CCV) is a rare syndrome described as an ‘idiopathic microangiopathy
ª 2013 British Association of Dermatologists
Correspondence
(a)
(b)
(c)
(d)
(e)
Figure 1 Cutaneous collagenous vasculopathy. Telangectasia of (a) the face, neck, presternal region, breasts, abdomen and arms, and
(c) the back, hips and buttocks. (b) Dermoscopic appearance of trunk telangectasia: straight and serpentine vessels following a branched pattern. (d) Reddish superficial telangiectatic vessels and dilated bluish deeper vessels on the legs. (e) Dermoscopic appearance of telangectasia on the legs: short, darker, more dilated serpentine vessels arranged in a less branching pattern. Dermoscopic images for each lesion were obtained with a DermLite DL3 dermoscope (3Gen LLC, Dana Point, CA, USA). Digital images were captured in JPEG format using a digital camera (Canon G12; Canon Inc. Tokyo, Japan).
involving the cutaneous blood vessels with vascular fibrosis of indeterminate etiology’.1 We report a new case of CCV with particularly extensive involvement. A 57-year-old white woman presented with a 9-year history of generalized and progressive telangiectases. The lesions had initially appeared on her legs and progressively spread over her body. On physical examination, symmetrical, widespread, reticulated and reddish lesions, which blanched with pressure, were seen on the legs, abdomen, buttocks, hips, presternal region, breasts, arms, malar region and proximal part of the fingers (Fig. 1a,c). The lesions were characterized dermoscopically by straight and serpentine vessels, following a branched pattern (Fig. 1b,e). Venular ectasias showing dilated bluish vessels were also seen on the legs (Fig. 1d). The oral mucosa, conjunctiva and nail beds were spared, and nail-fold capillaroscopy was normal. The patient was asymptomatic, denied photosensitivity, a family history of teleangectases and a personal or family history of a bleeding diathesis, but reported worsening of the lesions with heat and emotional stress, and a family history positive for venous insufficiency. Her medical history included uveitis, allergic diathesis and hypertension under treatment with losartan and hydrochlorothiazide.
ª 2014 British Association of Dermatologists
Darier sign was negative. Routine and rheumatological blood examinations were normal, and Doppler ultrasound of the legs showed no alterations. On histological examination of a skin biopsy taken from the thigh, and stained with haematoxylin and eosin, the epidermis was seen to be thin, and there was multiple dilatation of the small dermal blood vessels with thickened walls in the upper dermis, Giemsa staining showed a normal number of mast cells. Staining with periodicacid–Schiff showed that in the vessel walls, there were perivascular deposits of hyaline eosinophilic material (Fig. 2a,b), and immunohistochemical staining with collagen type IV antibody showed duplication of the basal membrane, suggesting a diagnosis of CCV. CCV is an uncommon primary cause of symmetrical generalized cutaneous telangiectasia, involving post-capillary venules of the horizontal dermal plexus and presenting with distinct histopathological features.1 It usually affects middle-aged and elderly people, but children may also be affected.2 CCV is often seen in patients with diabetes and hypertension,3 and has also been seen after pregnancy, radiotherapy4 and use of intense pulsed light.5 Telangiectasias in CCV have been described as proceeding from the distal limbs to the trunk, but facial involvement is rare, and in our opinions it should represent an advanced stage of the disease not strictly related to its
Clinical and Experimental Dermatology (2014) 39, pp216–234
229
Correspondence
(a)
(b)
Figure 2 (a) Normal epidermis: in the superficial dermis, highly dilated vessels with eosinophilic amorphous hyaline material can be
seen in the thickened walls, and there is perivascular lymphocytic infiltration (haematoxylin and eosin, original magnification 9100). (b) Dilated vessel in the superficial dermis (periodic-acid–Schiff, original magnification 9 400.
duration. The differential diagnosis includes primary and secondary teleangectasia: generalized essential benign telangiectasia, hereditary hemorrhagic telangiectasia, hereditary benign telangiectasia, telangiectasia eruptiva macularis perstans, and lymphomas. Moreover, a careful history allows CCV to be distinguished from drug-induced benign telangiectasias. CCV is an unusual and possibly under-recognized condition; however, it should be considered in every case of acquired telangiectasia. Further studies are necessary to evaluate whether CCV may be related to systemic disease. F. Bardazzi, A. Virdi, G. Odorici, R. Balestri, S. Infusino, and A. Patrizi Department of Dermatology and Specialised, Experimental and Diagnostic Medicine, Ospedale S. Orsola-Malpighi, Universita di Bologna, Via Massarenti 1, 40138, Bologna,Italy E-mail: [email protected] Conflict of interest: none declared. Accepted for publication 31 August 2013
References 1 Salama S, Rosenthal D. Cutaneous collagenous vasculopathy with generalized telangiectasia: an immunohistochemical and ultrastructural study. J Cutan Pathol 2000; 27: 40–8. 2 Gonz alez Fern andez D, G omez Bernal S et al. Cutaneous collagenous vasculopathy: description of two new cases in elderly women and review of the literature. Dermatology 2012; 225: 1–8. 3 Burdick LM, Losher S, Somach SC, Billings SD. Cutaneous collagenous vasculopathy: a rare cutaneous microangiopathy. Cutan Pathol 2012; 39: 741–6.
230
Clinical and Experimental Dermatology (2014) 39, pp216–234
4 Perez A, Wain ME, Robson A et al. Cutaneous collagenous vasculopathy with generalized telangiectasia in two female patients. J Am Acad Dermatol 2010; 63: 882–5. 5 Bernard S, Cawet B, Theate Y et al. Cutaneous collagenous vasculopathy: a rare cause of generalized telangiectasia. Ann Dermatol Venereol 2012; 139: 381–6.
Cutaneous diffuse large B-cell lymphoma, leg type, secondary to testicular diffuse large B-cell lymphoma doi: 10.1111/ced.12255 Diffuse large B-cell lymphoma (DLBCL) is the most common testicular lymphoma in adults. It usually occurs in men older than 60 years, and is an aggressive tumour with intermediate prognosis. Testicular DLBCL can disseminate to diverse extranodal sites, with the skin being a rarely affected site.1,2 An 80-year-old man presented with 1-month history of multiple infiltrated violaceous nodules on his left leg (Fig. 1). He had previously been diagnosed as having a testicular DLBCL, and had been treated with orchidectomy, four courses of chemotherapy with R-CHOP (rituximab, cyclophosphamide, doxorubicin and prednisone), prophylactic central nervous system chemotherapy, and radiotherapy to the testis and ipsilateral inguinal lymph nodes, which had resulted in complete response. The cutaneous tumours had first appeared 2 years after resolution. On histological examination of a skin biopsy, a diffuse dermal infiltration of atypical large lymphocytic cells was seen, composed mostly of immunoblasts and centroblasts (Fig. 2). Immunohistochemical markers were positive for
ª 2013 British Association of Dermatologists
A 36-year-old man presented with a 5-day history of an itchy facial rash. His medical history was unremarkable apart from acne vulgaris as a teenager. On physical examination, striking tumid erythematous papules, plaques and nodules were seen predominantly on the lower cheeks, and scaly erythematous plaques over the central forehead, upper cheeks, chin and neck (Fig 1a). The eyebrows, beard and scalp appeared normal with no obvious hair loss, and the cutaneous findings were limited to the head and neck. The differential diagnoses considered included granulomatous rosacea, sarcoidosis and lupus erythematosus. Pending histological confirmation, twicedaily topical 0.1% tacrolimus ointment was commenced. On histological examination of an incisional biopsy taken from the left pre-auricular area, a prominent perifollicular inflammatory cell infiltrate was seen, comprised mainly of lymphocytes, with mucinous degeneration of the follicular epithelium (Fig. 2a,b). There was no significant lymphocyte atypia, and the lymphoid immunoprofile was normal. Follicular destruction and granulomatous inflammation were absent. Fungal, bacterial and mycobacterial cultures were negative, as was direct immunofluorescence. Alcian blue staining confirmed the presence of intrafollicular mucin deposits (Fig 2c). Findings were consistent with a diagnosis of benign FM. On review 10 days later, the patient’s facial eruption had dramatically improved (Fig 1b). Full blood count, erythrocyte sedimentation rate, lactate dehydrogenase, human T cell lymphotropic virus (HTLV)1 and HTLV2 serology, and lymphocyte immunophenotyping were all normal or negative. T-cell gene rearrangement studies confirmed a polyclonal population, and a final diagnosis of primary idiopathic FM was made. Treatment with topical 0.1% tacrolimus ointment was tapered over the following 4 weeks, and remission has been maintained for > 1 year. A single case of recalcitrant FM successfully treated with 1% pimecrolimus cream has previously been reported.5 The response to tacrolimus ointment in our case was unusually rapid, and spontaneous resolution of lesions cannot be excluded. The risk of activating what may be considered a premalignant condition remains a concern, and further studies evaluating the efficacy, safety and tolerability of topical tacrolimus ointment in FM are required before it can be recommended. J. Kluk,1 N. Krassilnik,2 and S. R. McBride1 Departments of 1Dermatology and 2Pathology, Royal Free London NHS Foundation Trust, Pond street, London, NW3 2QG, UK E-mail: [email protected] Conflict of interest: none declared. Accepted for publication 15 August 2013
References 1 Kim KR, Lee JY, Kim MK et al. Successful treatment of recalcitrant primary follicular mucinosis with
228
Clinical and Experimental Dermatology (2014) 39, pp216–234
2
3
4
5
indomethacin and low-dose intralesional interferon alpha. Ann Dermatol 2009; 21: 285–7. Cerroni L, Fink-Puches R, B€ ack B et al. Follicular mucinosis: a critical reappraisal of clinicopathologic features and association with mycosis fungoides and Sezary syndrome. Arch Dermatol 2002; 138: 182–9. Rongioletti F, De LD, Meyes D et al. Follicular mucinosis: a clinicopathologic, histochemical, immunohistochemical and molecular study comparing the primary benign form and the mycosis fungoides-associated follicular mucinosis. J Cutan Pathol 2010; 37: 15–19. Schneider SW, Metze D, Bonsmann G. Treatment of so-called idiopathic follicular mucinosis with hydroxychloroquine. Br J Dermatol 2010; 163: 420–3. Gorpelioglu C, Sarifakioglu E, Bayrak R. A case of follicular mucinosis treated successfully with pimecrolimus. Clin Exp Dermatol 2008; 34: 86–7.
Borderline lepromatous leprosy with type 1 (downgrading) reaction doi: 10.1111/ced.12247 In their interesting paper, describing a patient with fever, nodulobullous eruption and swollen ears, Golberg et al.1 made the diagnosis of borderline lepromatous leprosy with type 1 (reversal) reaction. However, I suggest that the diagnosis is more likely to be borderline lepromatous leprosy with type 1 (downgrading) reaction because the reaction had occurred before the appropriate therapy was started, and there were multiple acid-fast bacilli visible in the histopathological figure. Clinically, both reversal and downgrading reactions are indistinguishable without history-taking and histopathology results.2 A. Gohar Gohar’s Skin and Sexual Health Clinic, Doctors’ Building (7B), First Central Corridor, 6 October City, 12451, Greater Cairo, Egypt E-mail: [email protected] Conflict of interest: none declared. Accepted for publication 30 September 2013
References 1 Golberg O, Burd R, Sahota A, Wiselka M. Fever, nodulobullous eruption and swollen ears. Clin Exp Dermatol 2013; 38: 811–13. 2 Bryceson ADM, Pfaltzgraff RE. Leprosy, 3rd edn. Edinburgh: Churchill Livingstone, 1990; 115–26.
Cutaneous collagenous vasculopathy: report of a case doi: 10.1111/ced.12258 Cutaneous collagenous vasculopathy (CCV) is a rare syndrome described as an ‘idiopathic microangiopathy
ª 2013 British Association of Dermatologists
Correspondence
(a)
(b)
(c)
(d)
(e)
Figure 1 Cutaneous collagenous vasculopathy. Telangectasia of (a) the face, neck, presternal region, breasts, abdomen and arms, and
(c) the back, hips and buttocks. (b) Dermoscopic appearance of trunk telangectasia: straight and serpentine vessels following a branched pattern. (d) Reddish superficial telangiectatic vessels and dilated bluish deeper vessels on the legs. (e) Dermoscopic appearance of telangectasia on the legs: short, darker, more dilated serpentine vessels arranged in a less branching pattern. Dermoscopic images for each lesion were obtained with a DermLite DL3 dermoscope (3Gen LLC, Dana Point, CA, USA). Digital images were captured in JPEG format using a digital camera (Canon G12; Canon Inc. Tokyo, Japan).
involving the cutaneous blood vessels with vascular fibrosis of indeterminate etiology’.1 We report a new case of CCV with particularly extensive involvement. A 57-year-old white woman presented with a 9-year history of generalized and progressive telangiectases. The lesions had initially appeared on her legs and progressively spread over her body. On physical examination, symmetrical, widespread, reticulated and reddish lesions, which blanched with pressure, were seen on the legs, abdomen, buttocks, hips, presternal region, breasts, arms, malar region and proximal part of the fingers (Fig. 1a,c). The lesions were characterized dermoscopically by straight and serpentine vessels, following a branched pattern (Fig. 1b,e). Venular ectasias showing dilated bluish vessels were also seen on the legs (Fig. 1d). The oral mucosa, conjunctiva and nail beds were spared, and nail-fold capillaroscopy was normal. The patient was asymptomatic, denied photosensitivity, a family history of teleangectases and a personal or family history of a bleeding diathesis, but reported worsening of the lesions with heat and emotional stress, and a family history positive for venous insufficiency. Her medical history included uveitis, allergic diathesis and hypertension under treatment with losartan and hydrochlorothiazide.
ª 2014 British Association of Dermatologists
Darier sign was negative. Routine and rheumatological blood examinations were normal, and Doppler ultrasound of the legs showed no alterations. On histological examination of a skin biopsy taken from the thigh, and stained with haematoxylin and eosin, the epidermis was seen to be thin, and there was multiple dilatation of the small dermal blood vessels with thickened walls in the upper dermis, Giemsa staining showed a normal number of mast cells. Staining with periodicacid–Schiff showed that in the vessel walls, there were perivascular deposits of hyaline eosinophilic material (Fig. 2a,b), and immunohistochemical staining with collagen type IV antibody showed duplication of the basal membrane, suggesting a diagnosis of CCV. CCV is an uncommon primary cause of symmetrical generalized cutaneous telangiectasia, involving post-capillary venules of the horizontal dermal plexus and presenting with distinct histopathological features.1 It usually affects middle-aged and elderly people, but children may also be affected.2 CCV is often seen in patients with diabetes and hypertension,3 and has also been seen after pregnancy, radiotherapy4 and use of intense pulsed light.5 Telangiectasias in CCV have been described as proceeding from the distal limbs to the trunk, but facial involvement is rare, and in our opinions it should represent an advanced stage of the disease not strictly related to its
Clinical and Experimental Dermatology (2014) 39, pp216–234
229
Correspondence
(a)
(b)
Figure 2 (a) Normal epidermis: in the superficial dermis, highly dilated vessels with eosinophilic amorphous hyaline material can be
seen in the thickened walls, and there is perivascular lymphocytic infiltration (haematoxylin and eosin, original magnification 9100). (b) Dilated vessel in the superficial dermis (periodic-acid–Schiff, original magnification 9 400.
duration. The differential diagnosis includes primary and secondary teleangectasia: generalized essential benign telangiectasia, hereditary hemorrhagic telangiectasia, hereditary benign telangiectasia, telangiectasia eruptiva macularis perstans, and lymphomas. Moreover, a careful history allows CCV to be distinguished from drug-induced benign telangiectasias. CCV is an unusual and possibly under-recognized condition; however, it should be considered in every case of acquired telangiectasia. Further studies are necessary to evaluate whether CCV may be related to systemic disease. F. Bardazzi, A. Virdi, G. Odorici, R. Balestri, S. Infusino, and A. Patrizi Department of Dermatology and Specialised, Experimental and Diagnostic Medicine, Ospedale S. Orsola-Malpighi, Universita di Bologna, Via Massarenti 1, 40138, Bologna,Italy E-mail: [email protected] Conflict of interest: none declared. Accepted for publication 31 August 2013
References 1 Salama S, Rosenthal D. Cutaneous collagenous vasculopathy with generalized telangiectasia: an immunohistochemical and ultrastructural study. J Cutan Pathol 2000; 27: 40–8. 2 Gonz alez Fern andez D, G omez Bernal S et al. Cutaneous collagenous vasculopathy: description of two new cases in elderly women and review of the literature. Dermatology 2012; 225: 1–8. 3 Burdick LM, Losher S, Somach SC, Billings SD. Cutaneous collagenous vasculopathy: a rare cutaneous microangiopathy. Cutan Pathol 2012; 39: 741–6.
230
Clinical and Experimental Dermatology (2014) 39, pp216–234
4 Perez A, Wain ME, Robson A et al. Cutaneous collagenous vasculopathy with generalized telangiectasia in two female patients. J Am Acad Dermatol 2010; 63: 882–5. 5 Bernard S, Cawet B, Theate Y et al. Cutaneous collagenous vasculopathy: a rare cause of generalized telangiectasia. Ann Dermatol Venereol 2012; 139: 381–6.
Cutaneous diffuse large B-cell lymphoma, leg type, secondary to testicular diffuse large B-cell lymphoma doi: 10.1111/ced.12255 Diffuse large B-cell lymphoma (DLBCL) is the most common testicular lymphoma in adults. It usually occurs in men older than 60 years, and is an aggressive tumour with intermediate prognosis. Testicular DLBCL can disseminate to diverse extranodal sites, with the skin being a rarely affected site.1,2 An 80-year-old man presented with 1-month history of multiple infiltrated violaceous nodules on his left leg (Fig. 1). He had previously been diagnosed as having a testicular DLBCL, and had been treated with orchidectomy, four courses of chemotherapy with R-CHOP (rituximab, cyclophosphamide, doxorubicin and prednisone), prophylactic central nervous system chemotherapy, and radiotherapy to the testis and ipsilateral inguinal lymph nodes, which had resulted in complete response. The cutaneous tumours had first appeared 2 years after resolution. On histological examination of a skin biopsy, a diffuse dermal infiltration of atypical large lymphocytic cells was seen, composed mostly of immunoblasts and centroblasts (Fig. 2). Immunohistochemical markers were positive for
ª 2013 British Association of Dermatologists
