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 Table of Contents  
CASE REPORT
Year : 2014  |  Volume : 6  |  Issue : 1  |  Page : 25-26  

The action of prostaglandins on ciliary hypertrichosis: A case report of pachydermoperiostosis


Prof. Rubem David Azulay Institute of Dermatology, Santa Casa da Misericordia, Rio de Janeiro, RJ, Brazil

Date of Web Publication15-Jul-2014

Correspondence Address:
Fabiana P Zarur
203 Guimar„es Rosa Street 705, Barra da Tijuca Zipcode: 22793-620, Rio de Janeiro, RJ
Brazil
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Source of Support: None, Conflict of Interest: None


Read associated Erratum: Erratum with this article

DOI: 10.4103/0974-7753.136756

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   Abstract 

Pachydermoperiostosis or primary hypertropic osteoarthropathy is a rare hereditary disorder that was first described in 1868. It is characterized by digital clubbing, pachydermia (thickening of the facial skin and/or scalp), and periostosis (swelling of periarticular tissue and subperiosteal new bone formation). We report a case of a patient with the complete form of the disease, and with a unique appearance of the hair shaft and eyelashes. The authors propose a possible mechanism to justify the abnormalities observed in the patient's hair shafts regarding the metabolism of prostaglandins and its relationship with the hair follicle physiological cycle.

Keywords: Cutis verticis gyrata, eyelashes, hair, pachydermoperiostosis, primary hypertrophic osteoarthropathy, prostaglandins


How to cite this article:
Zarur FP, d'Almeida LV, Novellino AC, Reis MD. The action of prostaglandins on ciliary hypertrichosis: A case report of pachydermoperiostosis. Int J Trichol 2014;6:25-6

How to cite this URL:
Zarur FP, d'Almeida LV, Novellino AC, Reis MD. The action of prostaglandins on ciliary hypertrichosis: A case report of pachydermoperiostosis. Int J Trichol [serial online] 2014 [cited 2019 Nov 16];6:25-6. Available from: http://www.ijtrichology.com/text.asp?2014/6/1/25/136756


   Introduction Top


Pachydermoperiostosis or primary hypertropic osteoarthropathy is a rare hereditary disorder that was first described in 1868. It is characterized by digital clubbing, pachydermia and periostosis. [1],[2]

Touraine et al. described three forms of pachydermoperiostosis: (1) A complete form with pachydermia and periostosis, (2) an incomplete form with evidence of bone abnormalities but lacking pachydermia, and (3) a forme frusta with prominent pachydermia and minimal-to-absent skeletal changes. [3],[4]

We describe a case of a patient with the complete form of the syndrome, focusing on the changes in the hair and eyelashes. The authors suggest a possible mechanism for the observed changes.


   Case report Top


A 22-year-old male patient, reports 5 years ago the initial development of facial skin thickening with accentuation of the ruts, pustules and cystic lesions on the face, and palmoplantar hyperhidrosis.

Physical examination revealed thickening of facial skin with accentuated skin ruts and circumvolutions mainly in the frontal region and scalp (cutis verticis gyrata), presence of dense and voluminous hair, keloid scars, hypertrophic eyelid skin, and lengthened eyelashes [Figure 1] and [Figure 2]. The joint examination revealed digital clubbing of the hands and feet, and knees with swelling and joint effusion.
Figure 1: Thickening of facial skin with accentuated skin ruts and circumvolutions mainly in the frontal region, keloid scars, hypertrophic eyelid skin and lengthened eyelashes

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He had radiographs showing diffuse periosteal thickening. Laboratorial exams were normal.

Based on clinical and radiological findings, age of onset of the disease, and absence of underlying illnesses, the diagnosis of the full form of pachydermoperiostosis was established.
Figure 2: Hypertrophic eyelid skin and lengthened eyelashes

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   Discussion Top


The pathogenesis of pachydermoperiostosis involves the Human Gene and Protein Database mutation, gene that encodes the 15-hydroxyprostaglandin dehydrogenase, a key enzyme responsible for the degradation of prostaglandins (PGs). [5],[6] Individuals with this mutation show high levels of PGs, especially PG E2 (PGE2). The clinical manifestations are due to the excessive formation of collagen by fibroblast hyperactivation promoted by PGE2.

We believe that, in this case, the changes of the scalp hair and eyelashes can also be attributed to high levels of PG.

Side-effects as cilliary hypertrichosis and eyelid hypertrophy, were observed in patients with topical PG analogues for glaucoma treatment. [7] This encouraged studies about their action in the hair follicle, and are currently approved by the Food and Drug Administration for the treatment of alopecia and cilliary hypotrichosis. The mechanism by which these drugs induce hypertrichosis is not totally elucidated, but experimental studies with rats have shown how they interfere in the hair cycle. [8],[9]

Receptors for PGE2 and F2α are described in hair follicles, especially in the follicular papilla. [10] They induce telogen follicles to enter the anagen phase early, in addition to promoting an extension of the same, delaying the onset of catagen phase. Other effects consist in follicular melanogenesis and thickening of the hair bulb, which leads to hyperpigmentation and hair thickening. [8],[9] Thus longer, pigmented and thicker hair would have been noticed, which could explain the findings in this patient.

We believe that in this case, the abnormalities found in the hair of the scalp and eyelashes can be attributed to high levels of prostaglandins present in patients with pachydermoperiostosis.

There are no studies to corroborate that this mechanism can be applied to the hair of the scalp. However the clinical findings in this case favor this hypothesis.

 
   References Top

1.
Lainetti A, Novaes F, Miranda L, Lira S. Arthralgia in primary hypertrophic osteoarthropathy: Case report and review of the treatment. Rev Bras Reumatol 2007;47:382-6.  Back to cited text no. 1
    
2.
Rimoin DL. Pachydermoperiostosis (idiopathic clubbing and periostosis): Genetic and physiologic considerations. N Engl J Med 1965;272:923-31.  Back to cited text no. 2
[PUBMED]    
3.
Poormoghim H, Hosseynian A, Javadi A. Primary hypertrophic osteoarthropathy. Rheumatol Int 2012;32:607-10.  Back to cited text no. 3
    
4.
Guerini MB, Barbato MT, Sá NB, Nunes DH, Zeni PR. Pachydermoperiostosis: The complete form of the syndrome. An Bras Dermatol 2011;86:582-4.  Back to cited text no. 4
    
5.
Martínez-Ferrer A, Peris P, Alós L, Morales-Ruiz M, Guañabens N. Prostaglandin E2 and bone turnover markers in the evaluation of primary hypertrophic osteoarthropathy (pachydermoperiostosis): A case report. Clin Rheumatol 2009;28:1229-33.  Back to cited text no. 5
    
6.
Uppal S, Diggle CP, Carr IM, Fishwick CW, Ahmed M, Ibrahim GH, et al. Mutations in 15-hydroxyprostaglandin dehydrogenase cause primary hypertrophic osteoarthropathy. Nat Genet 2008;40:789-93.  Back to cited text no. 6
    
7.
Yoshino T, Fukuchi T, Togano T, Seki M, Ikegaki H, Abe H. Eyelid and eyelash changes due to prostaglandin analog therapy in unilateral treatment cases. Jpn J Ophthalmol 2013;57:172-8.  Back to cited text no. 7
    
8.
Tauchi M, Fuchs TA, Kellenberger AJ, Woodward DF, Paus R, Lütjen-Drecoll E. Characterization of an in vivo model for the study of eyelash biology and trichomegaly: Mouse eyelash morphology, development, growth cycle, and anagen prolongation by bimatoprost. Br J Dermatol 2010;162:1186-97.  Back to cited text no. 8
    
9.
Sasaki S, Hozumi Y, Kondo S. Influence of prostaglandin F2alpha and its analogues on hair regrowth and follicular melanogenesis in a murine model. Exp Dermatol 2005;14:323-8.  Back to cited text no. 9
    
10.
Colombe L, Vindrios A, Michelet JF, Bernard BA. Prostaglandin metabolism in human hair follicle. Exp Dermatol 2007;16:762-9.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2]


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