tumour of the scalp’’ associated ‘‘with osteomyelitis ofthe frontal bone and intracranial infection.’’ It usuallyoccurs in children, most commonly in adolescent malepatients. It evolves from frontal and ethmoid sinusitis,and is usually caused by Streptococcus sp., Staphylococ-cus sp., and anaerobes.

Headache, fever, and a forehead mass following afrontal sinusitis are a classical presentation for Pott’spuffy tumor. Differential diagnosis of swelling on theforehead includes skin and soft-tissue infection, infectedhematoma, as well as benign and malignant tumorsof the skin, soft-tissue, bone, and frontal sinuses (1)(Table 1).

The diagnosis is based on clinical examination, andappropriate imaging performed to evaluate the possi-ble complications (epidural, subdural, and brainabscesses) (2). Intracranial complications of Pott’spuffy tumor result from the direct extension of theinfection or more commonly from secondary septicthrombophlebitis.

Treatment consists of surgical drainage of the abscessand a long-term culture-directed intravenous and oralantibiotics treatment course to prevent further purulentcomplications (3).

In conclusion, Pott’s puffy tumor is usually seen as acomplication of frontal sinusitis and it is easily confusedwith neoplasm, skin and soft-tissue infection. So in thecase of an atypic mass of the forehead, clinical exami-nation and imaging are necessary to exclude intracranialinvolvement.

REFERENCES

1. Bellaney GJ, Ryan TJ. Pott’s puffy tumour. Br J Dermatol1997;136:145–147.

2. Eufinger H, Machtens E. Purulent pansinusitis, orbital cel-lulitis and rhinogenic intracranial complications. J Cranio-maxillofac Surg 2001;29:111–117.

3. Maheshwar AA, Harris DA, Al-Mokhthar N et al. Pott’spuffy tumour: an unusual presentation and management.J Laryngol Otol 2001;115:497–499.

M. SABATIELLO, M.D.*,�O. VANHOOTEGHEM, M.D.*,�S. MOSTINCKX, M.D.*M. DE LA BRASSINNE, M.D.�*Department of Dermatology – St Elisabeth Hospital,5000 Namur, Belgium, �Department of Dermatology –University Hospital Sart-Tilman, 4000 Liege, Belgium

LOOSE ANAGEN HAIR SYNDROME: AN

UNDERDIAGNOSED CONDITION IN MALES

Abstract: Loose anagen hair syndrome (LAHS) is acondition of sparse, short hair that is easily and pain-lessly extracted from the scalp. Since it was first de-scribed in the 1980s, it is considered a rare, sporadiccondition found predominantly in females. Since then,there have been multiple reports of LAHS occurringin families, suggesting an autosomal dominant in-heritance pattern may also be present. An autosomaldominant trait would suggest a more equal sex ratio. Wereport a case of a boy diagnosed with LAHS and pro-pose that it may be underdiagnosed in males simplybecause of hairstyle differences between boys and girls.

Loose anagen hair syndrome (LAHS) is a condition ofsparse, short hair that is easily and painlessly extractedfrom the scalp. Since it was first described in the 1980s, itis considered a rare, sporadic condition found predomi-nantly in females. Since then, there have been multiplereports of LAHS occurring in families, suggesting anautosomal dominant inheritance pattern may also bepresent (1–5). The incidence is estimated at 2 to 2.5 casesper million per year, with the sex ratio of six male to 37females (2).However, an autosomal dominant pattern ofinheritance would suggest a more equal sex ratio. Wepropose that LAHS may be underdiagnosed in malessimply because of hairstyle differences between boysand girls. As opposed to females, most males do notgrow their hair long; therefore, the diagnosis may goundetected.

We report the case of a boy who presented at 3 yearsof age with slow-growing, easily plucked hair. The pa-tient was essentially hairless at birth, and at 1 year of agehad short, sparse, light blond hair. The boy’s father andmaternal grandmother had curly hair and as the child’shair grew, it became curly. Themother noted it was oftenunruly in the occipital region. At 2 years of age, thepatient’s hair was pulled out in clumps by another childat day care, leaving a bald patch. The area regrew, buttook nearly 1 year. The patient’s mother reported infre-quent haircuts; approximately 1 cm, two times per year.His past medical history was otherwise unremarkable.

On examination, he was noted to have curly, lightblonde, shoulder length hair (Fig. 1). Eyebrows, teeth,and nails were normal. The hair pull test resulted in

Address correspondence to O. Vanhooteghem, M.D., Depart-ment of Dermatology, University Hospital Sart-Tilman, CHU B35,4000 – Liege – Belgium, or e-mail: ovanhooteghem@hotmail.com.

408 Pediatric Dermatology Vol. 27 No. 4 July ⁄August 2010

multiple loose anagen hairs. Light microscopy showedmisshapen anagen bulbs, absent internal root sheath,and ruffled cuticles distal to the twisted bulbs (Fig. 2).With this evidence, the patient was diagnosed with looseanagen hair syndrome. The parents were reassured, andno treatment was undertaken.

The case reports of males with LAHS include a3-year-old boy (6) and two unrelated boys, aged 4 and9 years (7). It is of interest that the 4-year-old boy wasnoted to have shoulder-length hair. There have also beentwo instances where LAHS was found in a male whoseolder female sibling was diagnosed first: a 2-year-old girl

whose 1-year-old brother was also affected (5), and a6-year-old girl whose younger brother was similarlyaffected (4).

An autosomal dominant inheritance pattern ofLAHS was first suggested by Baden et al (3). A total of17 cases have been reported where both parent and childhad LAHS (1–5). Chapalain et al identified a keratingene mutation, E337K in K6HF, which is possiblycausative for LAHS in three of nine families (1). Adominant inheritance pattern was confirmed in thesefamilies because the mutation was present in both thechildren and the father, although LAHS was not clini-cally evident in every father (1). The authors suggestedthis could be attributed to variable penetrance or spon-taneous resolution of the condition.

Our case features LAHS in a male, which was likelydiagnosed because he has shoulder length hair. Becauseof societal norms and convenience, most young maleskeep their hair short. Added to the spontaneousimprovement of the condition, LAHS in males may re-solve before it is diagnosed.

REFERENCES

1. Chapalain V, Winter H, Langbein L et al. Is the looseanagen syndrome a keratin disorder? Arch Dermatol2002;138:501–506.

2. Sinclair R, Cargnello J, Chow CW. Loose anagen syn-drome. Exp Dermatol 1999;8:297–298.

3. Baden HP, Kvedar JC,Magro CM. Loose anagen hair as acause of hereditary hair loss in children. Arch Dermatol1992;128:1349–1353.

4. ChongA, SinclairR.Loose anagen syndrome: a prospectivestudy of three families. Australas J Dermatol 2002;43:120–124.

5. Price VH, Gummer CL. Loose anagen syndrome. J AmAcad Dermatol 1989;20:249–256.

6. O’Donnell BP, Sperling LC, JamesWD. Loose anagen hairsyndrome. Int J Dermatol 1992;31:107–109.

7. Hamm H, Traupe H. Loose anagen hair in childhood: thephenomenon of easily pluckable hair. J AmAcadDermatol1989;20:242–248.

CATHERINEM. PHAM, M.D.JENNIFER KREJCI-MANWARING, M.D.Division of Dermatology and Cutaneous Surgery,University of TexasHealth ScienceCenter – SanAntonio,San Antonio, Texas

Figure 1. Patient with loose anagen hair syndrome. Note thesparse, curly, light blonde, shoulder length hair.

Figure 2. Light micrograph of a loose anagen hair withmisshapen anagen bulb, absent internal root sheath, andruffled cuticle distal to the twisted bulb.

Address correspondence to Jennifer Krejci-Manwaring, M.D.,Division of Dermatology, University of Texas Health ScienceCenter – San Antonio, 7703 Floyd Curl Drive MC 7876, SanAntonio, TX 78229-3900, or e-mail: krejcimanwar@uthscsa.edu.

Conflict of Interest: Dr. Krejci-Manwaring has received con-sulting support from, Abbott Laboratories, Connetics Corpora-tion, and Bioform Medical.

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