Case Report Access this article online Website: www.ijtrichology.com DOI: 10.4103/0974-7753.125618 Quick Response Code:
Address for correspondence: Prof. Abraham Zlotogorski, Department of Dermatology, Hadassah‑Hebrew University Medical Center, P.O. Box 12000, Jerusalem 9112001, Israel. E‑mail: [email protected]
Pemphigus Vulgaris with Loss of Hair on the Scalp Noa Hadayer, Yuval Ramot, Alexander Maly1, Abraham Zlotogorski Departments of Dermatology and 1Pathology, Hadassah‑Hebrew University Medical Center, Jerusalem, Israel ABSTRACT Pemphigus vulgaris is an autoimmune disease, which leads to flaccid bullae and erosions. Although the scalp is commonly involved, hair loss is rarely seen. We herein report a case of 32‑year‑old female patient with a 2‑year history of pemphigus vulgaris, involving the skin and mucosa. Although clinical improvement was achieved with oral corticosteroids and mycophenolate mofetil, the patient developed tender plaques on her scalp, accompanied by hair loss. Histopathology and direct immunofluorescence were consistent with pemphigus vulgaris. Skin swab cultured Staphylococcus aureus. Treatment with oral and topical corticosteroids combined with mycophenolate mofetil resulted in clinical remission, with regrowth of scalp hair. Alopecia is an unusual clinical manifestation of pemphigus vulgaris. Our case supports the hypothesis that hair loss is induced by antibody‑mediated outer root sheath keratinocyte acantholysis, in combination with skin infection, that together lead to the observed hair loss. Key words: Alopecia, pemphigus vulgaris, scalp
INTRODUCTION
P
emphigus vulgaris is an autoimmune disease, in which antibodies against desmogleins cause intraepidermal acantholysis, and lead to the clinical manifestation of flaccid bullae.[1] Although the scalp is frequently involved in pemphigus vulgaris,[2] associated hair loss has only rarely been described.[3‑6] We report a rare case of pemphigus vulgaris involving the scalp that leads to hair loss. CASE REPORT
We report on a 32‑year‑old female patient who presented with a 2‑year history of flaccid bullae over her upper trunk, erosions over her oral mucosa and hard palate, and several crusted erosions over her scalp. The clinical as well as the pathological and direct immunofluorescence findings supported the diagnosis of pemphigus vulgaris and the patient was treated with systemic steroids and mycophenolate mofetil as a steroid sparing agent with clinical improvement, except for the lesions on her scalp. The erosions on the scalp were treated with high potency topical steroids with good clinical response; however several months later the patient returned with scalp folliculitis, which later progressed to indurative tender plaques with loss of hair over the International Journal of Trichology / Jul-Sep 2013 / Vol-5 / Issue-3
involved areas on her scalp [Figure 1a]. Biopsy taken from the lesion was compatible with the diagnosis of pemphigus vulgaris, demonstrating acantholysis of the epidermal as well as the outer root sheath keratinocytes [Figure 1b]. Direct immunofluorescence demonstrated immunoglobulin G deposition on the intercellular spaces within the epidermis [Figure 1c]. Scalp swab taken from the lesion cultured Staphylococcus aureus. The patient was treated initially with systemic steroids, and later on the steroids were gradually replaced with mycophenolate mofetil, combined with low dose triamcinolone tablets given for her mucosal lesions. In addition, she was treated locally with high‑potency topical steroid. The above mentioned treatment led to the regression of the lesions on her scalp, and later to re‑growth of hair. DISCUSSION
We hereby describe a patient with pemphigus vulgaris, who was treated with systemic steroids combined with mycophenolate mofetil. In spite of a good mucosal reaction as well as resolution of the lesions on the trunk, the lesions on the scalp had a tendency to recur and induce local hair loss. 157
Hadayer, et al.: Pemphigus and alopecia
published case series where skin swabs were taken.[4] Indeed, also in our patient, a skin swab cultured S. aureus, which was also cultured in one previous pemphigus vulgaris and alopecia case.[4] Furthermore, acantholysis can be seen within the hair follicle [Figure 1b], an additional finding that validates the clinical diagnosis.
a
Taken together, we suggest that alopecia should be assessed for in all pemphigus vulgaris patients and our case further strengthen the hypothesis that alopecia is caused due to a secondary factor, most probably secondary infection. c
b
Figure 1: (a) Erythematous plaques accompanied with hairless patches in the area involved on the scalp. (b) A punch biopsy taken from the plaque demonstrating typical changes for pemphigus vulgaris, involving the epidermis and the hair follicle. (c) Direct immunofluorescence showing sedimentation of immunoglobulin G autoantibodies in the intercellular spaces within the epidermal layer of the scalp
Scalp erosions can be found as a clinical manifestation of pemphigus vulgaris. However, hair loss is rarely seen, even in those with scalp erosions.[4] The underlying mechanism for the pemphigus‑associated alopecia is still obscure. It has been suggested that antibody‑mediated acantholysis between outer root sheath keratinocytes is responsible for the hair loss, but the fact that alopecia is not seen in most pemphigus patients implies that an additional secondary factor is needed for hair‑loss induction.[4] One such proposed factor is secondary infection, which was evident in all cases of a recently
158
REFERENCES 1.
Ioannides D, Lazaridou E, Rigopoulos D. Pemphigus. J Eur Acad Dermatol Venereol 2008;22:1478‑96. 2. Bystryn JC, Rudolph JL. Pemphigus. Lancet 2005;366:61‑73. 3. Delmonte S, Semino MT, Parodi A, Rebora A. Normal anagen effluvium: A sign of pemphigus vulgaris. Br J Dermatol 2000;142:1244‑5. 4. Veraitch O, Ohyama M, Yamagami J, Amagai M. Alopecia as a rare but distinct manifestation of pemphigus vulgaris. J Eur Acad Dermatol Venereol 2013;27:86‑91. 5. Wilson CL, Dean D, Wojnarowska F. Pemphigus and the terminal hair follicle. J Cutan Pathol 1991;18:428‑31. 6. Zaraa I, El Euch D, Kort R, Sellami M, Mokni M, Makni S, et al. Localized pemphigus: A report of three cases. Int J Dermatol 2010;49:715‑6.
How to cite this article: Hadayer N, Ramot Y, Maly A, Zlotogorski A. Pemphigus vulgaris with loss of hair on the scalp. Int J Trichol 2013;5:157-8. Source of Support: Nil, Conflict of Interest: None declared.
International Journal of Trichology / Jul-Sep 2013 / Vol-5 / Issue-3
Copyright of International Journal of Trichology is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Address for correspondence: Prof. Abraham Zlotogorski, Department of Dermatology, Hadassah‑Hebrew University Medical Center, P.O. Box 12000, Jerusalem 9112001, Israel. E‑mail: [email protected]
Pemphigus Vulgaris with Loss of Hair on the Scalp Noa Hadayer, Yuval Ramot, Alexander Maly1, Abraham Zlotogorski Departments of Dermatology and 1Pathology, Hadassah‑Hebrew University Medical Center, Jerusalem, Israel ABSTRACT Pemphigus vulgaris is an autoimmune disease, which leads to flaccid bullae and erosions. Although the scalp is commonly involved, hair loss is rarely seen. We herein report a case of 32‑year‑old female patient with a 2‑year history of pemphigus vulgaris, involving the skin and mucosa. Although clinical improvement was achieved with oral corticosteroids and mycophenolate mofetil, the patient developed tender plaques on her scalp, accompanied by hair loss. Histopathology and direct immunofluorescence were consistent with pemphigus vulgaris. Skin swab cultured Staphylococcus aureus. Treatment with oral and topical corticosteroids combined with mycophenolate mofetil resulted in clinical remission, with regrowth of scalp hair. Alopecia is an unusual clinical manifestation of pemphigus vulgaris. Our case supports the hypothesis that hair loss is induced by antibody‑mediated outer root sheath keratinocyte acantholysis, in combination with skin infection, that together lead to the observed hair loss. Key words: Alopecia, pemphigus vulgaris, scalp
INTRODUCTION
P
emphigus vulgaris is an autoimmune disease, in which antibodies against desmogleins cause intraepidermal acantholysis, and lead to the clinical manifestation of flaccid bullae.[1] Although the scalp is frequently involved in pemphigus vulgaris,[2] associated hair loss has only rarely been described.[3‑6] We report a rare case of pemphigus vulgaris involving the scalp that leads to hair loss. CASE REPORT
We report on a 32‑year‑old female patient who presented with a 2‑year history of flaccid bullae over her upper trunk, erosions over her oral mucosa and hard palate, and several crusted erosions over her scalp. The clinical as well as the pathological and direct immunofluorescence findings supported the diagnosis of pemphigus vulgaris and the patient was treated with systemic steroids and mycophenolate mofetil as a steroid sparing agent with clinical improvement, except for the lesions on her scalp. The erosions on the scalp were treated with high potency topical steroids with good clinical response; however several months later the patient returned with scalp folliculitis, which later progressed to indurative tender plaques with loss of hair over the International Journal of Trichology / Jul-Sep 2013 / Vol-5 / Issue-3
involved areas on her scalp [Figure 1a]. Biopsy taken from the lesion was compatible with the diagnosis of pemphigus vulgaris, demonstrating acantholysis of the epidermal as well as the outer root sheath keratinocytes [Figure 1b]. Direct immunofluorescence demonstrated immunoglobulin G deposition on the intercellular spaces within the epidermis [Figure 1c]. Scalp swab taken from the lesion cultured Staphylococcus aureus. The patient was treated initially with systemic steroids, and later on the steroids were gradually replaced with mycophenolate mofetil, combined with low dose triamcinolone tablets given for her mucosal lesions. In addition, she was treated locally with high‑potency topical steroid. The above mentioned treatment led to the regression of the lesions on her scalp, and later to re‑growth of hair. DISCUSSION
We hereby describe a patient with pemphigus vulgaris, who was treated with systemic steroids combined with mycophenolate mofetil. In spite of a good mucosal reaction as well as resolution of the lesions on the trunk, the lesions on the scalp had a tendency to recur and induce local hair loss. 157
Hadayer, et al.: Pemphigus and alopecia
published case series where skin swabs were taken.[4] Indeed, also in our patient, a skin swab cultured S. aureus, which was also cultured in one previous pemphigus vulgaris and alopecia case.[4] Furthermore, acantholysis can be seen within the hair follicle [Figure 1b], an additional finding that validates the clinical diagnosis.
a
Taken together, we suggest that alopecia should be assessed for in all pemphigus vulgaris patients and our case further strengthen the hypothesis that alopecia is caused due to a secondary factor, most probably secondary infection. c
b
Figure 1: (a) Erythematous plaques accompanied with hairless patches in the area involved on the scalp. (b) A punch biopsy taken from the plaque demonstrating typical changes for pemphigus vulgaris, involving the epidermis and the hair follicle. (c) Direct immunofluorescence showing sedimentation of immunoglobulin G autoantibodies in the intercellular spaces within the epidermal layer of the scalp
Scalp erosions can be found as a clinical manifestation of pemphigus vulgaris. However, hair loss is rarely seen, even in those with scalp erosions.[4] The underlying mechanism for the pemphigus‑associated alopecia is still obscure. It has been suggested that antibody‑mediated acantholysis between outer root sheath keratinocytes is responsible for the hair loss, but the fact that alopecia is not seen in most pemphigus patients implies that an additional secondary factor is needed for hair‑loss induction.[4] One such proposed factor is secondary infection, which was evident in all cases of a recently
158
REFERENCES 1.
Ioannides D, Lazaridou E, Rigopoulos D. Pemphigus. J Eur Acad Dermatol Venereol 2008;22:1478‑96. 2. Bystryn JC, Rudolph JL. Pemphigus. Lancet 2005;366:61‑73. 3. Delmonte S, Semino MT, Parodi A, Rebora A. Normal anagen effluvium: A sign of pemphigus vulgaris. Br J Dermatol 2000;142:1244‑5. 4. Veraitch O, Ohyama M, Yamagami J, Amagai M. Alopecia as a rare but distinct manifestation of pemphigus vulgaris. J Eur Acad Dermatol Venereol 2013;27:86‑91. 5. Wilson CL, Dean D, Wojnarowska F. Pemphigus and the terminal hair follicle. J Cutan Pathol 1991;18:428‑31. 6. Zaraa I, El Euch D, Kort R, Sellami M, Mokni M, Makni S, et al. Localized pemphigus: A report of three cases. Int J Dermatol 2010;49:715‑6.
How to cite this article: Hadayer N, Ramot Y, Maly A, Zlotogorski A. Pemphigus vulgaris with loss of hair on the scalp. Int J Trichol 2013;5:157-8. Source of Support: Nil, Conflict of Interest: None declared.
International Journal of Trichology / Jul-Sep 2013 / Vol-5 / Issue-3
Copyright of International Journal of Trichology is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
