Graham-Little Piccardi Lassueur Syndrome: case report

Síndrome de Graham-Little Piccardi Lassueur: relato de caso

CASE REPORT

Graham-Little Piccardi Lassueur Syndrome - Case report^*

Síndrome de Graham-Little Piccardi Lassueur - Relato de caso

Raquel Bissacotti Steglich^I; Renata Elise Tonoli^II; Giselle Martins Pinto^I; Fernanda Melo Müller^I; Isabelle Maffei Guarenti^I; Ernani Siegmann Duvelius^III

^IResident physician of the Dermatology Service, Charity Hospital of Porto Alegre (Irmandade da Santa Casa de Misericórdia de Porto Alegre) - Porto Alegre (RS), Brazil

^IIInternal medicine physician - Graduate Program - Medical Residency in Internal Medicine at the Federal University of Health Sciences (Universidade Federal de Ciência da Saúde de Porto Alegre - UFCSPA) - Porto Alegre (RS), Brazil

^IIIDermatologist of the Dermatology Service, Charity Hospital of Porto Alegre (Irmandade da Santa Casa de Misericórdia de Porto Alegre) - Preceptor of the Dermatology Service, Charity Hospital of Porto Alegre (Irmandade da Santa Casa de Misericórdia de Porto Alegre) - Porto Alegre (RS), Brazil

^{Mailing address}

ABSTRACT

A 33-year-old woman presented with a 3-year history of progressive alopecia of the scalp. Past treatment with hydroxicloroquine did not show improvement. Physical examination revealed multiple areas of alopecia with atrophic aspect of the scalp, and axillary and pubic hypotrichosis. Dermoscopy showed hyperkeratosis and accentuation of follicular ostia. Anatomopathological examination revealed decrease in the number of hair follicles, upper perifollicular infiltrate and areas with fibrosis. The Piccardi-Lassueur-Graham-Little syndrome is a rare disorder, characterized by the triad of multifocal scarring alopecia of the scalp, keratotic follicular eruption and hypotrichosis of axillary and pubic regions. Management is a challenge and many medications tried have controversial results. We report a case of this rare syndrome which improved with corticoids.

Keywords: Alopecia; Hypotrichosis; Lichen planus

RESUMO

Mulher, 33 anos, apresenta quadro de alopecia progressiva do couro cabeludo há 3 anos. Tratamento com hidroxicloroquina há 12 meses, sem apresentar melhora. Ao exame físico exibe múltiplas áreas de alopecia cicatricial no couro cabeludo, além de hipotricose axilar e pubiana. A dermatoscopia evidencia hipercetose folicular e acentuação dos óstios foliculares. O exame anatomopatológico revela diminuição do número de folículos pilosos, infiltrado perifolicular e fibrose. A síndrome de Graham-Little Piccardi Lassueur é uma dermatose rara, caracterizada pela tríade de alopecia cicatricial multifocal do couro cabeludo, ceratose folicular disseminada e hipotricose das regiões axilares e pubianas. A terapêutica desta dermatose é um desafio, muitas medicações relatadas tem resultados controversos. Relatamos o caso desta síndrome rara que apresentou melhora com corticoterapia.

Palavras-chave: Alopecia; Hipotricose; Líquen plano

INTRODUCTION

Reported initially by Piccardi, in 1913, Piccardi Lassueur Graham-Little syndrome (PLGLS) is a rare skin condition, that primarily affects women between 30 and 60 years old. ^1,2,3 This syndrome is characterized by a triad of multifocal cicatricial alopecia of the scalp, lichenoid follicular eruption and noncicatricial alopecia of the axillae and pubis. The etiology remains unknown and controversial. ^4,5

CASE REPORT

We report a case of a 33-year-old woman with progressive alopecia of the scalp of three years duration. The patient was treated with hydroxychloroquine for a year without improvement. Physical examination revealed multiple areas of alopecia with atrophic aspect of the scalp (Figure 1). Dermoscopy of the scalp showed perifollicular hyperketosis and accentuation of follicular ostia (Figure 2). There was axillary and pubic hypotrichosis without skin atrophy (Figure 3). On the upper limbs and trunk there was widespread follicular keratosis (Figure 4). The patient displayed lichenoid lesions on the pretibial region, soles of feet, shoulders, inter-mammary region and oral mucosa (Figure 5). Histopathologic examination showed overall decrease in the number of hair follicles, areas with perifollicular infiltrate and fibrosis, which was suggestive of lichen planopilaris (Figure 6). Treatment with systemic corticosteroids resulted in improvement of the lesions and stabilization of alopecia.

DISCUSSION

The essential features in PLGLS are progressively multifocal cicatricial alopecia of the scalp associated with disseminated follicular hyperkeratosis and noncicatricial axillary and pubic hypotrichosis. The clinical findings of this syndrome must be present simultaneously, even though scalp alopecia often precedes the others by months or years. ^1,2,4,6

Histology of the scalp lesions usually shows a pseudopeladic stage of cicatricial alopecia. The axillary and pubic alopecia is regularly described as noncicatricial, because there is no clinical atrophy. Histology of the papules is frequently lichenoid. ¹

It is estimated that over 50% of patients with PLGLS present at least one episode of cutaneous or mucous lichen planus in the course of the disease. PLGLS as lichen planus would stem from an immune response mediated by T cells against unknown antigens, determining destruction of keratinocytes.^1,5 The etiology of PLGLS remains unknown and controversial.^3,4,6,7 Most authors, based on clinical, histological and immunofluorescence consider PLGLS as a variant of lichen planus.^3-6 There have been cases of familial PLGLS where both patients demonstrated HLA-DR1. This HLA type is also seen in familial cases of lichen planus and provides further evidence that PLGLS is etiologically related to lichen planus. ^4,5,7

The differential diagnosis of PLGLS includes other causes of cicatricial alopecia, such as pseudopelade of Brocq, discoid lupus erythematosus, sarcoidosis, follicular mucinosis, folliculitis spinulosa decalvans and keratosis pilaris atrophicans. ^4,7

Treatment of PLGLS is difficult with respect to both the scarring alopecia and the follicular, keratotic eruption. The scalp changes tend to be refractory to established therapies. Among the first-line therapies are the high-potency topical corticosteroids, as well as intralesional injections of corticoids. The second-line therapy includes systemic glucocorticoids, retinoids, methotrexate, cycloporine, thalidomide and PUVA photochemotherapy, with inconsistent results. ^1,4

Received on 12.08.2011.

Approved by the Advisory Board and accepted for publication on 29.09.2011.

Financial Support: None

Conflict of Interests: None

• 1. Assouly P, Reygagne P. Lichen planopilaris: update on diagnosis and treatment. Semin Cutan Med Surg. 2009;28:3-10.
• 2. Ghislain PD, Van Eeckhout P, Ghislain E: Lassueur-Graham Little-Piccardi syndrome: 20-year follow-up. Dermatology. 2003,206:391-2.
• 3. Abbas O, Chedraoui A, Ghosn S: Frontal fibrosing alopecia presenting with componentes of Piccardi-Lassueur-Graham-Little syndrome. J Am Acad Dermatol. 2007;57(2 Suppl):S15-8.
• 4. Srivastava M, Mikkilineni R, Konstadt J. Lassueur-Graham-Little-Piccardi syndrome. Dermatol Online J. 2007;27:12.
• 5. Tchernev G, Nenoff P. Antigen mimicry followed by epitope spreading: A pathogenetic trigger for the clinical morphology of lichen planus and its transition to Graham Lassueur Piccardi Little Syndrome and keratosis lichenoides chronica -Medical hypotheses or reality? An Bras Dermatol. 2009;84:682-8.
• 6. Cabrera H, Rossi MA, Bech MP, Costa JA. Síndrome de Piccardi-Graham-Little. A propósito de su ubicación nosológica. An Bras Dermatol. 1982;57:169-72.
• 7. Viglizzo G, Verrini A, Rongioletti F: Familial Lassueur- Graham-Little- Piccardi syndrome. Dermatology. 2004,208:142-4.

*

Study carried out at the Charity Hospital of Porto Alegre (Irmandade da Santa Casa de Misericórdia de Porto Alegre - Porto Alegre - RS), Brazil.

Publication Dates

• Publication in this collection
  01 Oct 2012
• Date of issue
  Oct 2012

History

• Received
  12 Aug 2011
• Accepted
  29 Sept 2011