Correspondence
5 Farkas K, Nagy N, Kiny o A, et al. A newly identified missense mutation of the HR gene is associated with a novel, unusual phenotype of Marie Unna hereditary hypotrichosis 1 including limb deformities. Arch Dermatol Res 2011; 304: 679–81.
(a)
Follicular mucinosis treated with topical 0.1% tacrolimus ointment doi: 10.1111/ced.12244 Follicular mucinosis (FM) is characterized histologically by deposition of mucin in the epithelium of the follicular outer root sheath and sebaceous glands.1 FM may occur as a benign primary idiopathic disorder or in the presence of malignant lymphoproliferative disease, usually mycosis fungoides or Sezary syndrome.2 Distinction between benign and lymphoma-associated FM is challenging, as considerable overlap exists. Generalized eruptions, age > 40 years, lymphocyte atypia, epidermotropism and monoclonal T-cell receptor gene rearrangements confer a worse outcome.2,3 There is speculation that the so-called ‘benign’ form may actually be a precursor to a nonaggressive cutaneous lymphoma with a prolonged course and favourable outcome.2,3 FM most commonly presents as pale follicular papules or scaling erythematous infiltrated plaques. Treatment of lymphoma-associated FM is directed at the underlying lymphoproliferative disorder. Primary FM is said to resolve spontaneously, often within 2–24 months of onset, but a more persistent variant may be encountered. Management is largely based on anecdotal reports.4 We describe a case of primary FM responding to topical 0.1% tacrolimus ointment.
(a)
(b)
(b)
(c)
Figure 2 (a) Perifollicular inflammation and widespread mucinFigure 1 (a) Striking erythematous nodules and plaques on the
face and neck at presentation; (b) dramatically improved facial eruption 10 days after commencing topical 0.1% tacrolimus ointment.
ª 2013 British Association of Dermatologists
ous degeneration of the follicular and sebaceous epithelium; (b) mucinous degeneration of the follicular epithelium. Haematoxylin and eosin; original magnification (a) 9 10; (b) 9 40. (c) Confirmation of intrafollicular mucin (Alcian blue diastase periodicacid-Schiff; original magnification 9 20.
Clinical and Experimental Dermatology (2014) 39, pp216–234
227
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
5 Farkas K, Nagy N, Kiny o A, et al. A newly identified missense mutation of the HR gene is associated with a novel, unusual phenotype of Marie Unna hereditary hypotrichosis 1 including limb deformities. Arch Dermatol Res 2011; 304: 679–81.
(a)
Follicular mucinosis treated with topical 0.1% tacrolimus ointment doi: 10.1111/ced.12244 Follicular mucinosis (FM) is characterized histologically by deposition of mucin in the epithelium of the follicular outer root sheath and sebaceous glands.1 FM may occur as a benign primary idiopathic disorder or in the presence of malignant lymphoproliferative disease, usually mycosis fungoides or Sezary syndrome.2 Distinction between benign and lymphoma-associated FM is challenging, as considerable overlap exists. Generalized eruptions, age > 40 years, lymphocyte atypia, epidermotropism and monoclonal T-cell receptor gene rearrangements confer a worse outcome.2,3 There is speculation that the so-called ‘benign’ form may actually be a precursor to a nonaggressive cutaneous lymphoma with a prolonged course and favourable outcome.2,3 FM most commonly presents as pale follicular papules or scaling erythematous infiltrated plaques. Treatment of lymphoma-associated FM is directed at the underlying lymphoproliferative disorder. Primary FM is said to resolve spontaneously, often within 2–24 months of onset, but a more persistent variant may be encountered. Management is largely based on anecdotal reports.4 We describe a case of primary FM responding to topical 0.1% tacrolimus ointment.
(a)
(b)
(b)
(c)
Figure 2 (a) Perifollicular inflammation and widespread mucinFigure 1 (a) Striking erythematous nodules and plaques on the
face and neck at presentation; (b) dramatically improved facial eruption 10 days after commencing topical 0.1% tacrolimus ointment.
ª 2013 British Association of Dermatologists
ous degeneration of the follicular and sebaceous epithelium; (b) mucinous degeneration of the follicular epithelium. Haematoxylin and eosin; original magnification (a) 9 10; (b) 9 40. (c) Confirmation of intrafollicular mucin (Alcian blue diastase periodicacid-Schiff; original magnification 9 20.
Clinical and Experimental Dermatology (2014) 39, pp216–234
227
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
