Hypertrichosis Lanuginosa Congenita

Treated with Diode Laser Epilation

During Infancy

Abstract: We report the case of a girl with hyper-trichosis lanuginosa congenita treated with diodelaser depilation since the age of 9 months. Thetreatment was well tolerated, and neither general norlocal anesthesia was needed. A reduction of approx-imately 80% of facial and body hair was noted, whichimproved her condition significantly.

A Mexican girl with Fitzpatrick skin type III wasborn with hypertrichosis lanuginosa congenita(HLC). There was no family history of consanguin-

ity, excessive hair, or other diseases. Her pre- andperinatal history and her karyotype and sex hor-mone levels were normal. Her entire body wascovered with hair; only the palms, soles, distalphalanges, and labia minora were spared. She had acoarse facies with prominent eyebrows and lips, anda nasal bridge with wide nasal ala and doubleeyelashes (Fig. 1).

Treatment with diode laser was initiated at theage of 9 months (LightSheer ET, Lumenis Ltd.,Yokneam, Israel), using an energy of 10 to 12 J/cm2

and a pulse duration of 30 ms in all affected areas,excluding hairy sites and genitalia. The areas treatedwere divided into face, forearms, shoulders, dorsum,abdomen, and legs. Skin that could not be coveredwith clothing was treated first and then the rest ofthe body surface by rotating sessions every 2 or3 weeks, with a rest interval of 1 month between

A B

Figure 2. (A) The patient at months old after six sessions of laser depilation. (B) The patient at 4 years old, with apractically normal appearance with a few, short, fine hairs after an average of 20 diode laser treatments.

A B

Figure 1. (A,B) A 3-month-old girl with her entire body covered with hair except the soles and palms.

Brief Reports 529

treatments. The treatment was well tolerated with-out topical or general anesthesia, and there were nocomplications. By the age of 4 years she hadreceived an average of 20 laser sessions in each area(Fig. 2), with a hair reduction of approximately80%.

DISCUSSION

HLC is a rare syndrome that can be inherited as anX-linked, dominant autosomal, or de novo pattern.Its incidence is 1 in 1 billion newborns. Children withexcessive hair may experience severe psychologicalconsequences (1). Depilatory methods include shav-ing, trimming, plucking, blanching, waxing, and laser(2).

The diode laser has been reported as the mostsuccessful for treating undesirable hair (3). In thiscase, in addition to determining whether any extra-cutaneous involvement was present, we also sought tonormalize her appearance to improve her psychoso-cial development.

The diode laser is highly effective in removingpigmented hair (with a short-term hair reductioneffect of approximately 50% and a partial long-termhair removal efficacy beyond 6 months) (4,5). Thepatient did not present any laser side effects such asburns, hypopigmentation, or scars. Periodic sessionswill be required for years to maintain a “normal”appearance. This is one of the first reports of a patientwith HLC successfully treated with diode laser (5).

ACKNOWLEDGMENTS

We would like to thank Dr. Edmundo Velazquez,pediatric dermatologist; Dr. Huicochea and Dr.Salamanca, genetist; Dr. Nishimura, endocrinologist;Alberto Castillo, histotechnologist; and Dr. SergioLozano, for his help in editing the manuscript.

REFERENCES

1. Tadin-Strapps M, Salas-Alanis JC, Moreno L et al.Congenital universal hypertrichosis with deafness anddental anomalies inhierited as an X-linked trait. ClinGenet 2003;63:418–422.

2. Rajpar SF, Hague JS, Abdullah A et al. Hair removalwith the long-pulse alexandrite and long pulse Nd:YAGlasers is safe and well tolerated in children. Clin ExpDermatol 2009;34:684–687.

3. Sadighha A, Mohaghengh Zahed G. Meta-analysis ofhair removal laser trials. Lasers Med Sci 2009;24:21–25.

4. Littler C. Laser hair removal in a patient with hypertri-chosis lanuginosa congenita. Dermatol Surg 1997;23:705–707.

5. Attia A, El Noury A, AbdAlhafezM. Intense pulsed lighthair removal in a patient with congenital hypertrichosisterminalis. Pediatr Dermatol 2012;29:219–220.

Julio C. Salas-Alanis, M.D.*Larissa D. Lopez-Cepeda, M.D.†Aurora Elizondo-Rodriguez, M.D.‡Maria Enriqueta Morales-Barrera, M.D.†Alberto R. Ramos-Garibay, M.D.†*Dermatology Service, Universidad Autonoma de NuevoLeon, Hospital Universitario Dr. Jose E. Gonzalez,Monterrey, Mexico, †Dermatological Center Dr. Ladislaode la Pascua, Mexico City, Mexico, ‡Private practice,Mexico City, Mexico

Address correspondence to Larissa D. Lopez-Cepeda, M.D.,Dr. Vertiz 464, Esq. Eje 3 Sur. Col. Buenos Aires, c.p. 06780,Delegacion Cuauhtemoc, Mexico, D.F., Mexico, or e-mail:larisslo@yahoo.com.mx.

Herlitz Junctional Epidermolysis Bullosa with

a Novel Mutation in LAMB3

Abstract: Herlitz junctional epidermolysis bullosa(H-JEB) is a rare, heritable mechanobullous diseasethat affects infants at birth and causes early death.This disease is primarily caused by compoundheterozygous or homozygous mutations in one ofthree genes affecting the function of one of the threechains of the laminin-332 (formerly laminin-5) protein.Here we report a case of H-JEB with a novel hetero-zygous mutation in LAMB3,c.1597G>A (p.Ala533Thr).These findings attest to the molecular heterogeneityof JEB and emphasize the importance of geneticanalysis to help make an accurate diagnosis, predictclinical prognosis, and identify phenotypic-genotypicrelationships that may aid in prenatal diagnosis andgenetic counseling for the future.

CASE REPORT

A newborn boy of a primigravida mother presentedupon delivery with several, large, flaccid bullae anderosions involving the right flank, back, and but-tocks (Fig. 1). Erosions involving his lips and hardpalate and nail dystrophy were also observed.Family history was significant for a maternal auntwith childhood blistering that resolved by 4 years ofage.

Electron microscopy showed small, rudimen-tary hemidesmosomes. Immunofluorescence studiesidentified staining of collagens IV, VII, and XVII, butabsence of staining of antibodies directed against thegamma-2 (GB3), alpha-3 (BM165), and beta-3 chains

530 Pediatric Dermatology Vol. 31 No. 4 July/August 2014