Case Report Received: October 1, 2013 Accepted after revision: February 14, 2014 Published online: May 6, 2014
Dermatology 2014;229:65–69 DOI: 10.1159/000360759
Alopecia Universalis Associated with Cutaneous T Cell Lymphoma Mariya Miteva a Laila El Shabrawi-Caelen c Regina Fink-Puches c Christine Beham-Schmid d Paolo Romanelli a Francisco Kerdel b Antonella Tosti a
a
Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, and b University of Miami Hospital, Florida Academic Dermatology Centers, Miami, Fla., USA; Departments of c Dermatology and d Pathology, Medical University of Graz, Graz, Austria
Key Words Hair loss · Alopecia · Dermatoscopy · Dermoscopy · Trichoscopy · Mycosis fungoides · Follicular mucinosis
Abstract Background: Alopecia areata-like hair loss may occur in the context of cutaneous T cell lymphoma (CTCL) and can very rarely evolve to alopecia universalis-like presentation. The dermoscopic findings of CTCL-related alopecia have not been described. Methods: Two patients with alopecia areata universalis-like hair loss occurring in the context of preexisting, pathology-proven CTCL are presented. Results: Clinical examination showed subtotal scalp alopecia with sparse fine hair or total scalp alopecia with loss of eyebrows, eyelashes and body hair. On dermoscopy there was follicular or diffuse scaling, reduced number of follicular openings with broken hairs, short hairs or keratotic filiform spicules. Pathology confirmed the diagnosis of CTCLrelated alopecia. One patient had almost complete hair regrowth after treatment. Conclusion: CTCL-related alopecia universalis is a rare non-scarring form of hair loss which simulates alopecia areata universalis. We provide clues to distinguish both based on clinical, dermoscopic and pathologic © 2014 S. Karger AG, Basel findings.
Introduction
Alopecia areata-like hair loss may occur in the context of cutaneous T cell lymphoma (CTCL) and can very rarely evolve to an alopecia universalis-like presentation. We present here the dermoscopy features of two patients with erythrodermic CTCL associated with alopecia universalis and nail changes. Clinical Presentation
Case 1 A 71-year-old woman with CTCL, mycosis fungoides (MF) variant, in erythrodermic stage for 2 years, developed alopecia 6 months before presentation. Her treatments for CTCL included acitretin 25 mg daily with photophoresis. Clinical examination revealed subtotal alopecia and total loss of eyebrows, eyelashes and body hair (fig. 1a). Her twenty nails were thickened and showed subungual hyperkeratosis and yellow discoloration. Dermoscopy of the scalp revealed diffuse erythema and empty follicular openings filled with keratotic plugs or filiform spicules. Most were empty, but some contained hairs (fig. 1b). Treatment with clobetasol 0.05% cream under occlusion 6 days a week and pulse
© 2014 S. Karger AG, Basel 1018–8665/14/2292–0065$39.50/0 E-Mail [email protected] www.karger.com/drm
of intramuscular triamcinolone 40 mg monthly for 3 months produced almost complete regrowth, although the keratotic spicules persisted around terminal hairs on dermoscopy (fig. 2). Case 2 A 65-year-old woman with erythrodermic MF/Sézary overlap syndrome developed diffuse hair loss over the entire body resulting in alopecia universalis (fig. 3a). The hair loss started on the extremities and progressed to the rest of the body. At the beginning she had erythematosquamous plaques on the scalp, which rapidly disappeared, leaving a bald, slightly erythematous scalp. All twenty nails showed severe crumbling and yellow-brown discoloration. Her treatment for CTCL included extracorporeal photopheresis and methotrexate. Dermoscopy showed diffuse erythema with mild scale, black dots and sparse broken hairs (fig. 3b). The patient was treated with topical clobetasol without regrowth. Pathology The diagnosis of CTCL-related alopecia was established by pathology in both cases. Transverse sections showed altered follicular architecture as sebaceous glands were absent. There was slightly reduced follicu-
Mariya Miteva, MD 1600 NW 10th Avenue, RSMB, Room 2023A Miami, FL 33136 (USA) E-Mail mmiteva @ med.miami.edu
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Color version available online
lar density with predominant telogen follicles (case 1) and miniaturized follicles (cases 1 and 2). In both cases the vellus follicles showed fibrous streamers infiltrated by small lymphocytes and eosinophils. From the lower follicular level up to the isthmus, both the terminal anagen and telogen as well as the miniaturized follicles showed marked epidermotropism by small lymphocytes, forming focal granulomatous infiltrate (fig. 4a). Follicular mucinosis was focal in case 1 and prominent in case 2. The epidermotropic lymphoid cells stained highly positive for CD3 and CD4, CD8 was focally positive and CD7 was negative. In case 1, several follicular infundibula showed lamellar hyperkeratosis that occasionally surrounded vellus hairs (fig. 4b). In case 2, polymerase chain reaction of the T cell receptor gamma chain gene was performed and showed identical T cell clone in the skin biopsies and blood samples. These features were consistent with MF/Sézary syndrome and CTCL-related alopecia.
a
b
Fig. 1. Patient 1. a The scalp showed subtotal alopecia with slight erythema and multiple hyperkeratotic follicular filiform spicules before treatment. b Dermoscopy showed diffuse erythema and tiny white follicular spicules at the sites of follicular openings. There were a few terminal hairs showing fine peripilar white casts. Handyscope, ×20, FotoFinder Systems, Bad Birnbach, Germany.
66
Fig. 2. Patient 1. The scalp showed fine hair regrowth after treatment.
Dermatology 2014;229:65–69 DOI: 10.1159/000360759
Miteva et al.
Downloaded by: Carleton University Library 198.143.38.97 - 7/24/2015 5:45:44 PM
Alopecia can be a manifestation in CTCL. However, there are scant data in the literature on its prevalence and features. Diffuse alopecia is a common finding in erythrodermic MF and Sézary syndrome. On the other hand, alopecia universalistype hair loss is a very rare finding in CTCL and has been exclusively described in erythrodermic CTCL and Sézary syndrome. Patchy alopecia can be a feature of folliculotropic MF, an uncommon variant of MF, characterized histologically by atypical T lymphocytes which infiltrate preferentially the follicular epithelium and may or not be associated with follicular mucinosis [1]. These patients often have alopecic patches, which from a clinical point of view may present as (1) red and scaly inflammatory patches of scarring alopecia, (2) noninflamed smooth patches resembling alopecia areata or (3) scalp nodular lesions with alopecia [1–4]. The alopecia areatalike pattern has been described on the scalp, but it is more commonly observed in other body areas, including the limbs and pubic area [5]. In cases presenting with alopecia only, the diagnosis may be difficult as pathology shows the ‘swarm of bees’-type of peri-/intrabulbar lymphocytic infiltrate seen in alopecia areata [5]. The correct diagnosis requires evaluation of many histologic sections at serial levels as well as im-
Color version available online
Discussion
Color version available online
a
b
Fig. 3. Patient 2. a The scalp showed smooth skin without any visible hairs, resembling alopecia areata totalis. b Dermoscopy of the scalp showed slight erythema, scaling, black dots and broken hairs. Handyscope, ×20, FotoFinder Systems, Bad Birnbach, Germany.
Table 1. Clinical, dermoscopic and histologic features that allow distinguishing CTCL-related alopecia universals from alopecia areata
Distinguishing clues
CTCL-related alopecia universalis
Alopecia areata universalis
Clinical features
– alopecia with variable erythematosquamous plaques or hairless smooth skin, indistinguishable from alopecia areata (possible nail involvement: hyperkeratotic thick yellow nails)
– hairless smooth skin on scalp (possible nail involvement: nail pitting/trachyonychia)
Dermoscopic features
– follicular or diffuse scaling – reduced number of follicular openings with broken hairs, short hairs or keratotic filiform spicules
– yellow dots – dystrophic hairs: black dots, broken hairs, exclamation mark hairs
Pathology
– altered follicular architecture: loss of sebaceous glands – predominant miniaturized follicles – lichenoid/interface lymphoid epidermotropic CD4 T cell infiltrate at all follicular levels – follicular mucinosis
– preserved follicular architecture with intact sebaceous glands – predominant miniaturized follicles in chronic disease – possible peri- or intrabulbar CD4 T cell infiltrate only at this level – pigmented casts
Alopecia Universalis Associated with Cutaneous T Cell Lymphoma
Dermatology 2014;229:65–69 DOI: 10.1159/000360759
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universalis
b
Fig. 4. a The histologic specimen was bisected transversely and at the level of the mid fol-
licle and showed compound follicular structure of two terminal anagen follicles. There was interface-like epidermotropic lymphoid cell infiltrate with focal giant cells and follicular mucinosis. Note the absence of perifollicular fibrosis. Hematoxylin and eosin, ×10. b Dilated follicular infundibulum plugged by lamellar keratin (this corresponds to the follicular keratotic spicules on dermoscopy). Note the lichenoid granulomatous infiltrate. Hematoxylin and eosin, ×10.
68
Dermatology 2014;229:65–69 DOI: 10.1159/000360759
Miteva et al.
Downloaded by: Carleton University Library 198.143.38.97 - 7/24/2015 5:45:44 PM
Color version available online
a
munohistochemical and gene rearrangement studies. Alopecia in non-folliculotropic CTCL can also have different clinical presentations, including patchy areata-like lesions, erythematous patches and plaques of hair loss, ulcerated patches and plaques of hair loss, patchy hair loss with follicular prominence and follicular erythema and alopecia universalis-like hair loss [6]. The only available review on CTCL-related alopecia reported 13 cases of patchy alopecia areata-like hair loss and 5 cases of alopecia universalis-like hair loss among 38 cases with CTCL and alopecia [6]. All patients with alopecia universalis had erythroderma stage MF or Sézary syndrome. We report two additional cases of CTCL-related alopecia universalis showing dermoscopic and pathologic features of non-scarring alopecia. In CTCL-related alopecia universalis, as in alopecia areata, CD4 lymphocytes trigger anagen hair loss with dystrophic hairs in the acute stage and follicular miniaturization in the chronic stages. Scalp dermoscopy in both cases showed features of non-scarring alopecia together with features observed in alopecia areata such as broken hairs and black dots. Also, scalp pathology in both cases showed features observed in alopecia areata (decreased follicular density with a telogen shift, increased number of miniaturized follicles, intra- and perifollicular inflammatory infiltrate from the bulb to the infundibulum) in association with diagnostic features for CTCL (epidermotropism involving the outer root sheath, follicular mucinosis). A previously reported case of a patient with patch stage MF presenting also with patchy non-scaly alopecia areata-like hair loss on the scalp revealed on pathology the intra- and perifollicular infiltrate of lymphocytes, but also undifferentiated angulated basaloid structures infiltrated also by lymphocytes as in cutaneous lymphadenoma [7]. The authors called it basaloid follicular hyperplasia. It had been observed previously in follicular mucinosis [8]. In one of our cases similar findings were present, but were considered atrophic telogen structures undergoing involution, possibly secondary to the lymphocytic epidermotropism and the focal mucinosis. Table 1 summarizes the clinical, dermoscopic and histologic features that allow distinguishing CTCL-related alopecia universals from alopecia areata universalis. In one of our cases (case 1), treatment with topical and systemic steroids induced significant hair
regrowth. This patient was also taking a systemic retinoid (acitretin) for the lymphoma, which might have contributed to the regrowth. Hair regrowth after treatment of CTCL-related alopecia universals has been reported: five cases of CTCL-related patchy alopecia had hair regrowth with topical or systemic bexarotene treatment [9]. In case 1, dermoscopy showed follicular keratotic spicules corresponding to lamellar hyperkeratosis of the follicular
canal probably related to acitretin treatment, which is known to affect keratinization. We have observed similar keratotic spicules in a patient with disseminated actinic porokeratosis on acitretin (unpublished data). A possible explanation for the keratotic retention in the infundibula with acitretin is the fact that the ostia are plugged by thick-layered keratotic material which may need more time to exfoliate compared to the interfollicular epidermis. Acitretin in
a dose of 25 mg daily or more can cause telogen effluvium with diffuse alopecia, but not the alopecia totalis/universalis observed in our patients.
Vermeer MH, Wechsler J, Whittaker S, Meijer CJ: WHO-EORTC classification for cutaneous lymphomas. Blood 2005;105:3768–3785. 4 Lehman JS, Cook-Norris RH, Weed BR, Weenig RH, Gibson LE, Weaver AL, Pittelkow MR: Folliculotropic mycosis fungoides: single-center study and systematic review. Arch Dermatol 2010;146:607–613. 5 Iorizzo M, El Shabrawi Caelen L, Vincenzi C, Misciali C, Tosti A: Folliculotropic mycosis fungoides masquerading as alopecia areata. J Am Acad Dermatol 2010;63:e50–e52. 6 Bi MY, Curry JL, Christiano AM, Hordinsky MK, Norris DA, Price VH, Duvic M: The
spectrum of hair loss in patients with mycosis fungoides and Sézary syndrome. J Am Acad Dermatol 2011;64:53–63. 7 Kossard S, White A, Killingsworth M: Basaloid folliculolymphoid hyperplasia with alopecia as an expression of mycosis fungoides (CTCL). J Cutan Pathol 1995;22:466–471. 8 Pinkus H: Alopecia mucinosa; inflammatory plaques with alopecia characterized by rootsheath mucinosis. AMA Arch Derm 1957;76: 419–424; discussion 424–426. 9 Hanson M, Hill A, Duvic M: Bexarotene reverses alopecia in cutaneous T-cell lymphoma. Br J Dermatol 2003;149:193–196.
Disclosure Statement
The authors declare no conflict of interest.
1 Gerami P, Rosen S, Kuzel T, Boone SL, Guitart J: Folliculotropic mycosis fungoides: an aggressive variant of cutaneous T-cell lymphoma. Arch Dermatol 2008;144:738–746. 2 Monopoli A, Annessi G, Lombardo GA, Baliva G, Girolomoni G: Purely follicular mycosis fungoides without mucinosis: report of two cases with review of the literature. J Am Acad Dermatol 2003;48:448–452. 3 Willemze R, Jaffe ES, Burg G, Cerroni L, Berti E, Swerdlow SH, Ralfkiaer E, Chimenti S, Diaz-Perez JL, Duncan LM, Grange F, Harris NL, Kempf W, Kerl H, Kurrer M, Knobler R, Pimpinelli N, Sander C, Santucci M, Sterry W,
Alopecia Universalis Associated with Cutaneous T Cell Lymphoma
Dermatology 2014;229:65–69 DOI: 10.1159/000360759
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References
Dermatology 2014;229:65–69 DOI: 10.1159/000360759
Alopecia Universalis Associated with Cutaneous T Cell Lymphoma Mariya Miteva a Laila El Shabrawi-Caelen c Regina Fink-Puches c Christine Beham-Schmid d Paolo Romanelli a Francisco Kerdel b Antonella Tosti a
a
Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, and b University of Miami Hospital, Florida Academic Dermatology Centers, Miami, Fla., USA; Departments of c Dermatology and d Pathology, Medical University of Graz, Graz, Austria
Key Words Hair loss · Alopecia · Dermatoscopy · Dermoscopy · Trichoscopy · Mycosis fungoides · Follicular mucinosis
Abstract Background: Alopecia areata-like hair loss may occur in the context of cutaneous T cell lymphoma (CTCL) and can very rarely evolve to alopecia universalis-like presentation. The dermoscopic findings of CTCL-related alopecia have not been described. Methods: Two patients with alopecia areata universalis-like hair loss occurring in the context of preexisting, pathology-proven CTCL are presented. Results: Clinical examination showed subtotal scalp alopecia with sparse fine hair or total scalp alopecia with loss of eyebrows, eyelashes and body hair. On dermoscopy there was follicular or diffuse scaling, reduced number of follicular openings with broken hairs, short hairs or keratotic filiform spicules. Pathology confirmed the diagnosis of CTCLrelated alopecia. One patient had almost complete hair regrowth after treatment. Conclusion: CTCL-related alopecia universalis is a rare non-scarring form of hair loss which simulates alopecia areata universalis. We provide clues to distinguish both based on clinical, dermoscopic and pathologic © 2014 S. Karger AG, Basel findings.
Introduction
Alopecia areata-like hair loss may occur in the context of cutaneous T cell lymphoma (CTCL) and can very rarely evolve to an alopecia universalis-like presentation. We present here the dermoscopy features of two patients with erythrodermic CTCL associated with alopecia universalis and nail changes. Clinical Presentation
Case 1 A 71-year-old woman with CTCL, mycosis fungoides (MF) variant, in erythrodermic stage for 2 years, developed alopecia 6 months before presentation. Her treatments for CTCL included acitretin 25 mg daily with photophoresis. Clinical examination revealed subtotal alopecia and total loss of eyebrows, eyelashes and body hair (fig. 1a). Her twenty nails were thickened and showed subungual hyperkeratosis and yellow discoloration. Dermoscopy of the scalp revealed diffuse erythema and empty follicular openings filled with keratotic plugs or filiform spicules. Most were empty, but some contained hairs (fig. 1b). Treatment with clobetasol 0.05% cream under occlusion 6 days a week and pulse
© 2014 S. Karger AG, Basel 1018–8665/14/2292–0065$39.50/0 E-Mail [email protected] www.karger.com/drm
of intramuscular triamcinolone 40 mg monthly for 3 months produced almost complete regrowth, although the keratotic spicules persisted around terminal hairs on dermoscopy (fig. 2). Case 2 A 65-year-old woman with erythrodermic MF/Sézary overlap syndrome developed diffuse hair loss over the entire body resulting in alopecia universalis (fig. 3a). The hair loss started on the extremities and progressed to the rest of the body. At the beginning she had erythematosquamous plaques on the scalp, which rapidly disappeared, leaving a bald, slightly erythematous scalp. All twenty nails showed severe crumbling and yellow-brown discoloration. Her treatment for CTCL included extracorporeal photopheresis and methotrexate. Dermoscopy showed diffuse erythema with mild scale, black dots and sparse broken hairs (fig. 3b). The patient was treated with topical clobetasol without regrowth. Pathology The diagnosis of CTCL-related alopecia was established by pathology in both cases. Transverse sections showed altered follicular architecture as sebaceous glands were absent. There was slightly reduced follicu-
Mariya Miteva, MD 1600 NW 10th Avenue, RSMB, Room 2023A Miami, FL 33136 (USA) E-Mail mmiteva @ med.miami.edu
Downloaded by: Carleton University Library 198.143.38.97 - 7/24/2015 5:45:44 PM
Color version available online
lar density with predominant telogen follicles (case 1) and miniaturized follicles (cases 1 and 2). In both cases the vellus follicles showed fibrous streamers infiltrated by small lymphocytes and eosinophils. From the lower follicular level up to the isthmus, both the terminal anagen and telogen as well as the miniaturized follicles showed marked epidermotropism by small lymphocytes, forming focal granulomatous infiltrate (fig. 4a). Follicular mucinosis was focal in case 1 and prominent in case 2. The epidermotropic lymphoid cells stained highly positive for CD3 and CD4, CD8 was focally positive and CD7 was negative. In case 1, several follicular infundibula showed lamellar hyperkeratosis that occasionally surrounded vellus hairs (fig. 4b). In case 2, polymerase chain reaction of the T cell receptor gamma chain gene was performed and showed identical T cell clone in the skin biopsies and blood samples. These features were consistent with MF/Sézary syndrome and CTCL-related alopecia.
a
b
Fig. 1. Patient 1. a The scalp showed subtotal alopecia with slight erythema and multiple hyperkeratotic follicular filiform spicules before treatment. b Dermoscopy showed diffuse erythema and tiny white follicular spicules at the sites of follicular openings. There were a few terminal hairs showing fine peripilar white casts. Handyscope, ×20, FotoFinder Systems, Bad Birnbach, Germany.
66
Fig. 2. Patient 1. The scalp showed fine hair regrowth after treatment.
Dermatology 2014;229:65–69 DOI: 10.1159/000360759
Miteva et al.
Downloaded by: Carleton University Library 198.143.38.97 - 7/24/2015 5:45:44 PM
Alopecia can be a manifestation in CTCL. However, there are scant data in the literature on its prevalence and features. Diffuse alopecia is a common finding in erythrodermic MF and Sézary syndrome. On the other hand, alopecia universalistype hair loss is a very rare finding in CTCL and has been exclusively described in erythrodermic CTCL and Sézary syndrome. Patchy alopecia can be a feature of folliculotropic MF, an uncommon variant of MF, characterized histologically by atypical T lymphocytes which infiltrate preferentially the follicular epithelium and may or not be associated with follicular mucinosis [1]. These patients often have alopecic patches, which from a clinical point of view may present as (1) red and scaly inflammatory patches of scarring alopecia, (2) noninflamed smooth patches resembling alopecia areata or (3) scalp nodular lesions with alopecia [1–4]. The alopecia areatalike pattern has been described on the scalp, but it is more commonly observed in other body areas, including the limbs and pubic area [5]. In cases presenting with alopecia only, the diagnosis may be difficult as pathology shows the ‘swarm of bees’-type of peri-/intrabulbar lymphocytic infiltrate seen in alopecia areata [5]. The correct diagnosis requires evaluation of many histologic sections at serial levels as well as im-
Color version available online
Discussion
Color version available online
a
b
Fig. 3. Patient 2. a The scalp showed smooth skin without any visible hairs, resembling alopecia areata totalis. b Dermoscopy of the scalp showed slight erythema, scaling, black dots and broken hairs. Handyscope, ×20, FotoFinder Systems, Bad Birnbach, Germany.
Table 1. Clinical, dermoscopic and histologic features that allow distinguishing CTCL-related alopecia universals from alopecia areata
Distinguishing clues
CTCL-related alopecia universalis
Alopecia areata universalis
Clinical features
– alopecia with variable erythematosquamous plaques or hairless smooth skin, indistinguishable from alopecia areata (possible nail involvement: hyperkeratotic thick yellow nails)
– hairless smooth skin on scalp (possible nail involvement: nail pitting/trachyonychia)
Dermoscopic features
– follicular or diffuse scaling – reduced number of follicular openings with broken hairs, short hairs or keratotic filiform spicules
– yellow dots – dystrophic hairs: black dots, broken hairs, exclamation mark hairs
Pathology
– altered follicular architecture: loss of sebaceous glands – predominant miniaturized follicles – lichenoid/interface lymphoid epidermotropic CD4 T cell infiltrate at all follicular levels – follicular mucinosis
– preserved follicular architecture with intact sebaceous glands – predominant miniaturized follicles in chronic disease – possible peri- or intrabulbar CD4 T cell infiltrate only at this level – pigmented casts
Alopecia Universalis Associated with Cutaneous T Cell Lymphoma
Dermatology 2014;229:65–69 DOI: 10.1159/000360759
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universalis
b
Fig. 4. a The histologic specimen was bisected transversely and at the level of the mid fol-
licle and showed compound follicular structure of two terminal anagen follicles. There was interface-like epidermotropic lymphoid cell infiltrate with focal giant cells and follicular mucinosis. Note the absence of perifollicular fibrosis. Hematoxylin and eosin, ×10. b Dilated follicular infundibulum plugged by lamellar keratin (this corresponds to the follicular keratotic spicules on dermoscopy). Note the lichenoid granulomatous infiltrate. Hematoxylin and eosin, ×10.
68
Dermatology 2014;229:65–69 DOI: 10.1159/000360759
Miteva et al.
Downloaded by: Carleton University Library 198.143.38.97 - 7/24/2015 5:45:44 PM
Color version available online
a
munohistochemical and gene rearrangement studies. Alopecia in non-folliculotropic CTCL can also have different clinical presentations, including patchy areata-like lesions, erythematous patches and plaques of hair loss, ulcerated patches and plaques of hair loss, patchy hair loss with follicular prominence and follicular erythema and alopecia universalis-like hair loss [6]. The only available review on CTCL-related alopecia reported 13 cases of patchy alopecia areata-like hair loss and 5 cases of alopecia universalis-like hair loss among 38 cases with CTCL and alopecia [6]. All patients with alopecia universalis had erythroderma stage MF or Sézary syndrome. We report two additional cases of CTCL-related alopecia universalis showing dermoscopic and pathologic features of non-scarring alopecia. In CTCL-related alopecia universalis, as in alopecia areata, CD4 lymphocytes trigger anagen hair loss with dystrophic hairs in the acute stage and follicular miniaturization in the chronic stages. Scalp dermoscopy in both cases showed features of non-scarring alopecia together with features observed in alopecia areata such as broken hairs and black dots. Also, scalp pathology in both cases showed features observed in alopecia areata (decreased follicular density with a telogen shift, increased number of miniaturized follicles, intra- and perifollicular inflammatory infiltrate from the bulb to the infundibulum) in association with diagnostic features for CTCL (epidermotropism involving the outer root sheath, follicular mucinosis). A previously reported case of a patient with patch stage MF presenting also with patchy non-scaly alopecia areata-like hair loss on the scalp revealed on pathology the intra- and perifollicular infiltrate of lymphocytes, but also undifferentiated angulated basaloid structures infiltrated also by lymphocytes as in cutaneous lymphadenoma [7]. The authors called it basaloid follicular hyperplasia. It had been observed previously in follicular mucinosis [8]. In one of our cases similar findings were present, but were considered atrophic telogen structures undergoing involution, possibly secondary to the lymphocytic epidermotropism and the focal mucinosis. Table 1 summarizes the clinical, dermoscopic and histologic features that allow distinguishing CTCL-related alopecia universals from alopecia areata universalis. In one of our cases (case 1), treatment with topical and systemic steroids induced significant hair
regrowth. This patient was also taking a systemic retinoid (acitretin) for the lymphoma, which might have contributed to the regrowth. Hair regrowth after treatment of CTCL-related alopecia universals has been reported: five cases of CTCL-related patchy alopecia had hair regrowth with topical or systemic bexarotene treatment [9]. In case 1, dermoscopy showed follicular keratotic spicules corresponding to lamellar hyperkeratosis of the follicular
canal probably related to acitretin treatment, which is known to affect keratinization. We have observed similar keratotic spicules in a patient with disseminated actinic porokeratosis on acitretin (unpublished data). A possible explanation for the keratotic retention in the infundibula with acitretin is the fact that the ostia are plugged by thick-layered keratotic material which may need more time to exfoliate compared to the interfollicular epidermis. Acitretin in
a dose of 25 mg daily or more can cause telogen effluvium with diffuse alopecia, but not the alopecia totalis/universalis observed in our patients.
Vermeer MH, Wechsler J, Whittaker S, Meijer CJ: WHO-EORTC classification for cutaneous lymphomas. Blood 2005;105:3768–3785. 4 Lehman JS, Cook-Norris RH, Weed BR, Weenig RH, Gibson LE, Weaver AL, Pittelkow MR: Folliculotropic mycosis fungoides: single-center study and systematic review. Arch Dermatol 2010;146:607–613. 5 Iorizzo M, El Shabrawi Caelen L, Vincenzi C, Misciali C, Tosti A: Folliculotropic mycosis fungoides masquerading as alopecia areata. J Am Acad Dermatol 2010;63:e50–e52. 6 Bi MY, Curry JL, Christiano AM, Hordinsky MK, Norris DA, Price VH, Duvic M: The
spectrum of hair loss in patients with mycosis fungoides and Sézary syndrome. J Am Acad Dermatol 2011;64:53–63. 7 Kossard S, White A, Killingsworth M: Basaloid folliculolymphoid hyperplasia with alopecia as an expression of mycosis fungoides (CTCL). J Cutan Pathol 1995;22:466–471. 8 Pinkus H: Alopecia mucinosa; inflammatory plaques with alopecia characterized by rootsheath mucinosis. AMA Arch Derm 1957;76: 419–424; discussion 424–426. 9 Hanson M, Hill A, Duvic M: Bexarotene reverses alopecia in cutaneous T-cell lymphoma. Br J Dermatol 2003;149:193–196.
Disclosure Statement
The authors declare no conflict of interest.
1 Gerami P, Rosen S, Kuzel T, Boone SL, Guitart J: Folliculotropic mycosis fungoides: an aggressive variant of cutaneous T-cell lymphoma. Arch Dermatol 2008;144:738–746. 2 Monopoli A, Annessi G, Lombardo GA, Baliva G, Girolomoni G: Purely follicular mycosis fungoides without mucinosis: report of two cases with review of the literature. J Am Acad Dermatol 2003;48:448–452. 3 Willemze R, Jaffe ES, Burg G, Cerroni L, Berti E, Swerdlow SH, Ralfkiaer E, Chimenti S, Diaz-Perez JL, Duncan LM, Grange F, Harris NL, Kempf W, Kerl H, Kurrer M, Knobler R, Pimpinelli N, Sander C, Santucci M, Sterry W,
Alopecia Universalis Associated with Cutaneous T Cell Lymphoma
Dermatology 2014;229:65–69 DOI: 10.1159/000360759
69
Downloaded by: Carleton University Library 198.143.38.97 - 7/24/2015 5:45:44 PM
References
