Clinical and Experimental Dermatology
Folliculocentric papules and alopecia M. S. Moye,1 R. S. Farah1 and B. L. Swick2 Departments of 1Dermatology and 2Pathology, University of Iowa Hospitals and Clinics, Iowa City, IA, USA doi: 10.1111/ced.12348
Clinical findings A 70-year-old woman presented with a 6-month history of an itchy rash on her scalp, trunk and limbs. She also reported hair loss, estimating that she had lost approximately 25% of her scalp hair over the same time period. When questioned, she reported sparse axillary and pubic hair as well. A skin biopsy taken from her right forearm 2 months previously had shown folliculocentric interface dermatitis, which was originally interpreted as lupus erythematosus. Laboratory evaluation at the time was negative for antinuclear antibodies, anti-double-stranded DNA, anti-SSA and anti-SSB. Topical steroids improved her pruritus but did not resolve the rash. On physical examination, diffuse macular erythema of the scalp was seen, with perifollicular keratotic spines and mild scale (Fig. 1). Follicular ostia were intact. Ill-defined thin, pink plaques with follicular prominence and minimal hair were present on the limbs (Fig. 2). On the axillae and pubic areas, there were few hairs with associated folliculocentric erythematous papules.
Figure 1 Scalp with scale and perifollicular keratotic spines,
Histolopathological findings A repeat biopsy of the eruption on the arm was taken (Fig. 3). Histopathological examination revealed a folliculocentric, lymphocytic, lichenoid dermatitis with sparing of the interfollicular epidermis. There was associated marked follicular plugging. What is your diagnosis? Figure 2 Forearm with pink plaques with follicular prominence
and minimal hair growth.
Correspondence: Dr Molly S. Moye, 200 Hawkins Drive, 40024 PFP, Iowa City, IA 52242, USA E-mail: [email protected] Conflict of interest: the authors declare that they have no conflicts of interest. Accepted for publication 15 January 2014
ª 2014 British Association of Dermatologists
Clinical and Experimental Dermatology (2014) 39, pp661–663
661
D CP
CED
CPD • Clinicopathological case
D
CP
Clinicopathological case
Figure 3 Skin biopsy from forearm showing folliculocentric, lym-
phocytic, lichenoid dermatitis with sparing of the interfollicular epidermis (haematoxylin and eosin, original magnification 9 40.
Diagnosis Graham-Little-Piccardi-Lassueur Syndrome (GLPLS).
Discussion GLPLS classically presents as a triad of lichen planopilaris of the scalp in addition to nonscarring alopecia of the axillae and pubic area, and a perifollicular keratotic eruption on the body.1 GLPLS is more common in women than in men and usually occurs between the ages of 30 and 60 years.1 Scarring alopecia of the scalp often precedes the eruption and tends to be progressive, even if the rash resolves. The follicular eruption has been termed ‘keratosis pilaris’, ‘lichen spinulosus’, ‘follicular lichen planus’ and ‘lichen planopilaris’.1 As of 2009, approximately 40 cases of GLPLS had been reported. The pathogenesis of GLPLS is unclear, but may be immune-mediated, with possible involvement of HLADR antigens.2 Cases are most commonly sporadic. A case of GLPLS following hepatitis B vaccination has been reported.1 The major histopathological differential diagnosis of GLPLS is lupus erythematosus (LE). Findings more suggestive of lupus include mucin deposition, interfollicular interface dermatitis, and a positive lupus band on direct immunofluorescence.3 Our patient’s histopathological results were consistent with lichen planopilaris (LPP), which is a primary cicatricial alopecia. GLPLS is a rare variant of LPP, which involves body hair in addition to scalp hair. Currently, because of the paucity of data regarding successful GLPLS treatment, GLPLS is managed much
662
Clinical and Experimental Dermatology (2014) 39, pp661–663
like LPP. Treatment guidelines for LPP are limited to case reports, case series and small retrospective studies. The risks of medications should be carefully weighed, as this disease is not life-threatening. The patient should be made aware that hair regrowth will not occur in scarred areas. High-potency topical steroids daily for at least 3 months have shown good response and are a reasonable initial regimen.3 Systemic corticosteroids tapered over 3–4 months can arrest hair loss in rapidly progressive disease.3 Ciclosporin reliably leads to improvement in > 75% of patients when used for at least 4 months.3 Patients also reported improvement with doxycycline (27% of patients) and hydroxychloroquine (41% of patients) in one small study.4 Mycophenolate mofetil led to improvement in 84% of patients who had failed other wtreatments.5 Acitretin has led to improvement but may cause telogen effluvium.3 There are anecdotal reports of efficacy with vitamin A, griseofulvin, methotrexate and thalidomide.3 Peroxisome proliferator-activated receptor (PPAR)-cisa transcription factorthatregulates inflammatory and lipid metabolic genes; it is downregulated in LPP.6 PPAR-c agonists, such as pioglitazone, are currently used to treat Type 2 diabetes mellitus, and may prove helpful in treatingLPP.6 Our patient declined immunosuppressants, and was started on topical corticosteroids and acitretin (25 mg/day). Three months later, the eruption was improved, and her scalp disease was stable. Patients with cicatricial alopecias may be referred to the Cicatricial Alopecia Research Foundation (www.carfintl. org) for additional information.
Acknowledgment We thank Dr K. Robson for her contribution to this case.
Learning points
•
GLPS is a rare variant of lichen planopilaris that affects body hair as well as the scalp. • The main histopathological differential diagnosis of GLPLS is LE. • Treatment of lichen planopilaris and GLPLS can be challenging, and risks of treatment must be weighed against potential benefits. • PPAR-c agonists are an emerging therapy that may prove effective in treating lichen planopilaris and potentially GLPLS.
ª 2014 British Association of Dermatologists
D CP Clinicopathological case
References 1 Srivastava M, Mikkilineni R, Konstadt J. Lassueur-Graham-Little-Piccardi syndrome. Dermatol Online J 2007; 13: 12. 2 L aszlo FG. Graham-Little-Piccardi-Lassueur syndrome: case report and review of the syndrome in men. Int J Dermatol 2013; doi: 10.1111/j.1365-4632.2012.05672.x. 3 Assouly P, Reygagne P. Lichen planopilaris: update on diagnosis and treatment. Semin Cutan Med Surg 2009; 28: 3–10.
ª 2014 British Association of Dermatologists
4 Spencer L, Balestreire Hawryluk E et al. Lichen planopilaris: retrospective study and stepwise therapeutic approach. Arch Dermatol 2009; 145: 333–4. 5 Cho B, Sah D, Chwalek J et al. Efficacy and safety of mycophenolate mofetil for lichen planopilaris. J Am Acad Dermatol 2010; 62: 393–7. 6 Mirmirani P, Karnik P. Lichen planopilaris treated with a peroxisome proliferator-activated receptor gamma agonist. Arch Dermatol 2009; 145: 1363–6.
Clinical and Experimental Dermatology (2014) 39, pp661–663
663
Folliculocentric papules and alopecia M. S. Moye,1 R. S. Farah1 and B. L. Swick2 Departments of 1Dermatology and 2Pathology, University of Iowa Hospitals and Clinics, Iowa City, IA, USA doi: 10.1111/ced.12348
Clinical findings A 70-year-old woman presented with a 6-month history of an itchy rash on her scalp, trunk and limbs. She also reported hair loss, estimating that she had lost approximately 25% of her scalp hair over the same time period. When questioned, she reported sparse axillary and pubic hair as well. A skin biopsy taken from her right forearm 2 months previously had shown folliculocentric interface dermatitis, which was originally interpreted as lupus erythematosus. Laboratory evaluation at the time was negative for antinuclear antibodies, anti-double-stranded DNA, anti-SSA and anti-SSB. Topical steroids improved her pruritus but did not resolve the rash. On physical examination, diffuse macular erythema of the scalp was seen, with perifollicular keratotic spines and mild scale (Fig. 1). Follicular ostia were intact. Ill-defined thin, pink plaques with follicular prominence and minimal hair were present on the limbs (Fig. 2). On the axillae and pubic areas, there were few hairs with associated folliculocentric erythematous papules.
Figure 1 Scalp with scale and perifollicular keratotic spines,
Histolopathological findings A repeat biopsy of the eruption on the arm was taken (Fig. 3). Histopathological examination revealed a folliculocentric, lymphocytic, lichenoid dermatitis with sparing of the interfollicular epidermis. There was associated marked follicular plugging. What is your diagnosis? Figure 2 Forearm with pink plaques with follicular prominence
and minimal hair growth.
Correspondence: Dr Molly S. Moye, 200 Hawkins Drive, 40024 PFP, Iowa City, IA 52242, USA E-mail: [email protected] Conflict of interest: the authors declare that they have no conflicts of interest. Accepted for publication 15 January 2014
ª 2014 British Association of Dermatologists
Clinical and Experimental Dermatology (2014) 39, pp661–663
661
D CP
CED
CPD • Clinicopathological case
D
CP
Clinicopathological case
Figure 3 Skin biopsy from forearm showing folliculocentric, lym-
phocytic, lichenoid dermatitis with sparing of the interfollicular epidermis (haematoxylin and eosin, original magnification 9 40.
Diagnosis Graham-Little-Piccardi-Lassueur Syndrome (GLPLS).
Discussion GLPLS classically presents as a triad of lichen planopilaris of the scalp in addition to nonscarring alopecia of the axillae and pubic area, and a perifollicular keratotic eruption on the body.1 GLPLS is more common in women than in men and usually occurs between the ages of 30 and 60 years.1 Scarring alopecia of the scalp often precedes the eruption and tends to be progressive, even if the rash resolves. The follicular eruption has been termed ‘keratosis pilaris’, ‘lichen spinulosus’, ‘follicular lichen planus’ and ‘lichen planopilaris’.1 As of 2009, approximately 40 cases of GLPLS had been reported. The pathogenesis of GLPLS is unclear, but may be immune-mediated, with possible involvement of HLADR antigens.2 Cases are most commonly sporadic. A case of GLPLS following hepatitis B vaccination has been reported.1 The major histopathological differential diagnosis of GLPLS is lupus erythematosus (LE). Findings more suggestive of lupus include mucin deposition, interfollicular interface dermatitis, and a positive lupus band on direct immunofluorescence.3 Our patient’s histopathological results were consistent with lichen planopilaris (LPP), which is a primary cicatricial alopecia. GLPLS is a rare variant of LPP, which involves body hair in addition to scalp hair. Currently, because of the paucity of data regarding successful GLPLS treatment, GLPLS is managed much
662
Clinical and Experimental Dermatology (2014) 39, pp661–663
like LPP. Treatment guidelines for LPP are limited to case reports, case series and small retrospective studies. The risks of medications should be carefully weighed, as this disease is not life-threatening. The patient should be made aware that hair regrowth will not occur in scarred areas. High-potency topical steroids daily for at least 3 months have shown good response and are a reasonable initial regimen.3 Systemic corticosteroids tapered over 3–4 months can arrest hair loss in rapidly progressive disease.3 Ciclosporin reliably leads to improvement in > 75% of patients when used for at least 4 months.3 Patients also reported improvement with doxycycline (27% of patients) and hydroxychloroquine (41% of patients) in one small study.4 Mycophenolate mofetil led to improvement in 84% of patients who had failed other wtreatments.5 Acitretin has led to improvement but may cause telogen effluvium.3 There are anecdotal reports of efficacy with vitamin A, griseofulvin, methotrexate and thalidomide.3 Peroxisome proliferator-activated receptor (PPAR)-cisa transcription factorthatregulates inflammatory and lipid metabolic genes; it is downregulated in LPP.6 PPAR-c agonists, such as pioglitazone, are currently used to treat Type 2 diabetes mellitus, and may prove helpful in treatingLPP.6 Our patient declined immunosuppressants, and was started on topical corticosteroids and acitretin (25 mg/day). Three months later, the eruption was improved, and her scalp disease was stable. Patients with cicatricial alopecias may be referred to the Cicatricial Alopecia Research Foundation (www.carfintl. org) for additional information.
Acknowledgment We thank Dr K. Robson for her contribution to this case.
Learning points
•
GLPS is a rare variant of lichen planopilaris that affects body hair as well as the scalp. • The main histopathological differential diagnosis of GLPLS is LE. • Treatment of lichen planopilaris and GLPLS can be challenging, and risks of treatment must be weighed against potential benefits. • PPAR-c agonists are an emerging therapy that may prove effective in treating lichen planopilaris and potentially GLPLS.
ª 2014 British Association of Dermatologists
D CP Clinicopathological case
References 1 Srivastava M, Mikkilineni R, Konstadt J. Lassueur-Graham-Little-Piccardi syndrome. Dermatol Online J 2007; 13: 12. 2 L aszlo FG. Graham-Little-Piccardi-Lassueur syndrome: case report and review of the syndrome in men. Int J Dermatol 2013; doi: 10.1111/j.1365-4632.2012.05672.x. 3 Assouly P, Reygagne P. Lichen planopilaris: update on diagnosis and treatment. Semin Cutan Med Surg 2009; 28: 3–10.
ª 2014 British Association of Dermatologists
4 Spencer L, Balestreire Hawryluk E et al. Lichen planopilaris: retrospective study and stepwise therapeutic approach. Arch Dermatol 2009; 145: 333–4. 5 Cho B, Sah D, Chwalek J et al. Efficacy and safety of mycophenolate mofetil for lichen planopilaris. J Am Acad Dermatol 2010; 62: 393–7. 6 Mirmirani P, Karnik P. Lichen planopilaris treated with a peroxisome proliferator-activated receptor gamma agonist. Arch Dermatol 2009; 145: 1363–6.
Clinical and Experimental Dermatology (2014) 39, pp661–663
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