List of Abbreviations
ICD – International Classification of Diseases
Syn. - synonym
HPV – human papillomavirus
PUVA therapy is a treatment method that involves the use of a photoactive substance combined with exposure of the skin to long-wave ultraviolet radiation.
Terms and Definitions
Localized hypertrichosis is hair growth on any localized part of the body in an amount exceeding the amount normally present in individuals of the same age, race, and sex, excluding androgen-dependent hair growth [35].
1. Brief information on the disease or condition
1.1 Definition of the disease or condition
Localized hypertrichosis is hair growth on any localized part of the body in an amount exceeding the amount normally present in individuals of the same age, race, and sex, excluding androgen-dependent hair growth [35].
1.2 Etiology and pathogenesis of the disease or condition (group of diseases or conditions)
Different types of hypertrichosis differ in etiology and pathogenesis.
The main etiological factor in the development of congenital hypertrichosis is considered to be a genetic abnormality leading to hair follicle protein dysfunction [5]. Congenital localized hypertrichosis, in particular hypertrichosis in the elbow joints, palms and soles, auricle, tip of the nose, anterior or posterior surface of the neck, is an autosomal dominant disease, and eyelash trichomegaly is autosomal recessive [5]. Lumbosacral localized congenital hypertrichosis may indicate the presence of spinal defects [6]. Localized congenital hypertrichosis of the forehead, lateral surfaces of the face, shoulders, and back, and synophrys (syn.: joined eyebrows, unibrow) can be a component of a genetically inherited syndrome [5]. Limited frontotemporal hypertrichosis occurs in congenital triangular alopecia [1].
Primary nevoid hypertrichosis occurs independently and is not associated with extracutaneous manifestations [5]. Secondary nevoid hypertrichosis may be associated with lipodystrophy, hemihypertrophy, scoliosis, and underlying vascular anomalies [5].
Acquired hypertrichosis can be a consequence of the use of various drugs: systemic antibacterial agents (Streptomycin**, Benzylpenicillin**), anti-inflammatory drugs (systemic corticosteroids), heart disease drugs and peripheral vasodilator antihypertensives (Minoxidil, Alprostadil**), diuretics (Spironolactone**), antiglaucoma preparations and miotics (Acetazolamide**), antiepileptic drugs (Phenytoin**), immunosuppressants (Cyclosporine**, Mycophenolate mofetil**), systemic psoralens, penicillamine and similar drugs (Penicillamine**), monoclonal antibodies (Cetuximab**, Panitumumab**), epidermal growth factor receptor (EGFR) tyrosine kinase inhibitors (Erlotinib**, Gefitinib**), Interferon alfa**, Fenoterol, Amlodipine**, Perindopril**, Rivastigmine**, Zidovudine**, and others [2,4-7,9].
If hypertrichosis is located in areas exposed to solar radiation, porphyria should be considered as a possible cause of the condition [5]. The exact mechanism of hypertrichosis development resulting from sun exposure is not fully understood, but it is known that ultraviolet light is a potent inducer of cyclooxygenase-2 (COX-2) and causes active production of prostaglandin E2, while substances such as porphyrins and psoralens can stimulate this process [4].
Acquired localized hypertrichosis is most commonly found in areas subject to repeated trauma, friction, and irritation, as well as sites of chronic inflammation [5]. Sometimes acquired localized hypertrichosis is located at the sites of fractures, applied splints and plaster casts, vascular malformations, prior vaccination sites (diphtheria, tetanus and pertussis vaccine**, tuberculosis vaccine**), chickenpox scars, post-traumatic scars, within tattoos, wart removal and laser hair removal sites, areas of PUVA therapy, dermatological corticosteroids, tacrolimus**, antiglaucoma and miotic drugs, as well as topical agents containing mercury or iodine [4-5,8-9]. Cases of localized hypertrichosis have been noted around warts caused by human papillomavirus (HPV) type 1, within the lesions of erythrokeratoderma, as well as cases associated with prior thrombosis, osteomyelitis, systemic lupus erythematosus, and localized scleroderma [4,7,9].
Pathogenetically, hypertrichosis is the result of hair follicles transitioning into anagen (the active growth phase) or the transformation of vellus hair follicles into terminal ones [4].
1.3 Epidemiology of the Disease or Condition
There are no exact statistical data on the epidemiology of hypertrichosis at the time of writing of the clinical guidelines.
Hypertrichosis is equally likely to occur in men and women, although certain types of it (hereditary hypertrichosis of the pinna or tip of the nose) are more characteristic of the male population [5].
Prepubertal hypertrichosis is most commonly found among the populations of the Mediterranean and South Asia [5].
1.4 Features of Coding the Disease or Condition
L68.2 – Localized hypertrichosis
1.5 Classification of the disease or condition
There is no universally accepted classification.
Forms of localized hypertrichosis include congenital localized hypertrichosis (congenital nevi, Becker's melanosis/nevus, nevoid hypertrichosis, spinal dysraphism, and others), localized hypertrichosis in hereditary and acquired systemic diseases, and acquired localized hypertrichosis. [5]
1.6 Clinical presentation of the disease or condition
Hypertrichosis manifests as the appearance of coarse, long, pigmented hairs in areas where vellus hairs are normally located. [10]
Hairy elbow syndrome (hairy elbow syndrome), hairy palms and soles syndrome, hypertrichosis of the pinna, eyebrows, and tip of the nose, anterior and posterior cervical hypertrichoses manifest in the correspondingly named area from birth, in early childhood or adolescence, and are manifested by an increase in the number, thickness, and pigmentation of vellus hairs, and are most often located symmetrically [5,6,12,13].
Small complex melanocytic nevi often become more pigmented during adolescence, with coarse dark hairs potentially growing from the nevus, while hair growth in giant lesions (>20 cm) is most prominent in late childhood [6].
Becker's nevus presents as a localized unilateral area of hyperpigmentation and hypertrichosis. This disorder occurs predominantly in young men. It may be present in early childhood, but is usually first noticed in adolescence, sometimes after a sunburn, and most commonly affects the shoulders, anterior chest, or scapular region. During puberty, coarse black hairs typically appear in the affected area, which may not completely coincide with the pigmented patch [5,6,10].
Nevoid hypertrichosis represents symmetric areas of hypertrichosis of limited extent, which can occur as a developmental defect in the absence of any other skin anomalies. This likely represents a nevoid condition of the hair follicles and is characterized by single or multiple patches of terminal hair on otherwise normal skin [5,6].
Spinal dysraphism ("faun tail") is a patch over the lumbosacral region within which hypertrichosis may be present [5].
The hair collar sign is a ring of hypertrichosis surrounding aplasia cutis or ectopic brain tissue [5].
Limited hypertrichosis can be observed after repeated local friction, periodic pressure, or prolonged inflammatory changes affecting the dermis, regardless of whether clinical scars form or not. An increased growth of vellus hair is observed in the area of secondarily altered skin. [5,6]
Porphyrias, such as porphyria cutanea tarda (PCT) and hepatoerythropoietic porphyria (HEP), can present with hypertrichosis in sun-exposed areas. Patients will have other stigmata of PCT, including blisters of photosensitive rash [6,14].
Depending on the presence, amount of pigment, and the ratio of eumelanin to pheomelanin content, hair color can vary from white or gray (absence of pigment) to brown or black [2].
2. Diagnostics, medical indications and contraindications for diagnostic methods
The diagnosis of localized hypertrichosis is established based on clinical manifestations.
2.1 Complaints and Anamnesis
Patients complain of the presence, appearance, increased hair growth, or changes in hair quality (thickening, increased pigmentation) in areas of the skin where such hair growth is not typical [5,6]
When taking the medical history, the following should be considered [5,6,11]:
1. The patient's age at the onset of the disease (appearance of excessive hair growth).
2. Medication history.
3. Any concomitant anomalies.
4. Family history.
5. Patient's ethnicity and race.
6. Concomitant internal organ diseases.
Hairy elbow syndrome manifests from birth to early childhood. Hairy palms and soles syndrome manifests at birth [13]. Hypertrichosis of the pinna manifests in childhood or adolescence and is more common in males. Hypertrichosis of the eyebrows and tip of the nose appears in adolescence. Anterior cervical hypertrichosis manifests from birth to early childhood and is associated with sensory and motor neuropathy, intellectual disability, and hallux valgus. Posterior cervical hypertrichosis appears at birth and is associated with kyphoscoliosis. [5]
2.2 Physical Examination
For making a diagnosis, the primary method is a visual examination of the patient's skin. The manifestations of the disease are described in detail in the "Clinical Presentation" section.
2.3 Laboratory Diagnostics
Laboratory diagnostic tests are not used.
2.4 Instrumental Diagnostics
Instrumental diagnostic examinations are not used.
2.5 Other Procedures
In women with an increase in the number and quality of hairs in androgen-dependent skin areas, as well as in the presence of other symptoms of hyperandrogenism (acne, female-pattern hair loss, seborrhea, changes in female body contours), for the purpose of differential diagnosis with hirsutism, it is recommended to calculate the hirsutism score using the Ferriman-Gallwey scale (Appendix G1) [15,16].
GDR CLE 5Grade of recommendation C (level of evidence – 5)
Comments: the assessment is carried out using the Ferriman-Gallwey tables in 9 androgen-dependent areas of the body (upper lip, chin, chest, upper and lower back, abdomen, lower abdomen, arms, and thighs) on a scale from 0 (no hair growth) to 4 (intense visible hair growth). The sum of scores across all areas is called the hirsute score. The diagnosis of "hirsutism" is established when the hirsute score is greater than 8. According to recent guidelines, hirsutism is generally indicated by a score on the modified Ferriman-Gallwey scale of ≥4–6, although there are racial features in the assessment of hirsutism. In some Caucasian and Negroid females, a pathognomonic sign is an increase in the sum of scores on the specified scale of ≥8. In Southeast Asian females, a diagnostically significant increase in the sum of scores on this scale is ≥3. More pronounced hirsutism is characteristic of women from the Middle East, Latin America, and the Mediterranean [2,15,16].
In case of concomitant clinical signs of hormonal status disorders (abnormal testosterone levels) in a patient with localized hypertrichosis, it is recommended to schedule an appointment (examination, consultation) with an endocrinologist [2,42].
GDR CLE 5Grade of recommendation C (level of evidence – 5)
It is recommended for patients with localized hypertrichosis, if necessary to clarify the diagnosis and for differential diagnosis with other diseases, to perform a skin biopsy with a pathomorphological examination of the skin biopsy material [5,43].
GDR CLE 5Grade of recommendation C (level of evidence – 5)
Comments: upon histological examination, hypertrichosis manifests as an increase in the number of terminal or vellus hairs, depending on the etiology of the hypertrichosis [5].
3. Treatment, including drug and non-drug therapies, diet therapy, and pain management
The treatment of localized hypertrichosis is the removal of unwanted hair. The choice of method depends on the patient's clinical presentation and may be determined by the equipment available in the cosmetologist's office.
3.1 Conservative Treatment
For all hair colors, patients with localized skin hypertrichosis are recommended temporary methods of hair shaft removal without affecting the hair follicle (depilation) [17-20].
GDR CLE 5Grade of recommendation C (level of evidence – 5)
Comments: removal using a razor blade, electric shaver, tweezers, trimmer, waxing or sugaring paste, chemical depilation (potassium thioglycolate) [17-20].
Instrumental treatment methods
To remove unwanted hair of all colors in patients with localized skin hypertrichosis, it is recommended to perform electrical epilation as an individualized course depending on the clinical presentation [35-39].
GCR CLEL 5Grade of Recommendation C (Level of Evidence – 5)
To remove unwanted hair of all colors except depigmented (white, gray) in patients with localized skin hypertrichosis, it is recommended to perform epilation:
photoepilation using broadband pulsed light (IPL) (500–1200 nm) once every 4–6 weeks [3,5,6, 21-24].
GDR CLE 5Grade of recommendation C (level of evidence – 5)
Comments:. The number of procedures is individual, on average 6–10 sessions [21-24]. In each specific case, energy and pulse duration must be selected based on individual skin characteristics and hair pigmentation in the treatment area. They should be chosen in the following order: spot size (mm), pulse duration (ms), energy fluence (J/cm2) [25]. Recommended for phototypes I–IV.
or
laser epilation using a solid-state neodymium-doped yttrium aluminum garnet (Nd:YAG) dermatological laser system (1064 nm) once every 4–5 weeks [3,5,6,22,23,26-28].
GRC BLEL 2Grade of Recommendation B (Level of Evidence – 2)
Comments: The number of procedures is individual, on average 8–10 sessions [22,23,26-28]. The device of choice for dark skin phototypes and light hair due to the fact that the target chromophores for this laser are oxyhemoglobin, deoxyhemoglobin, and protein structures. Recommended for phototypes I–IV.
or
laser epilation using an alexandrite dermatological laser system (755 nm) once every 4–8 weeks depending on the epilation zone [3,5,6,28-30].
GCR CLoE 3Grade of Recommendation C (Level of Evidence – 3)
Comments: The number of procedures is individual, on average 5–8 sessions [28-30]. Due to competitive absorption of this laser radiation by melanin in the interfollicular epidermis, the procedure can only be performed on skin phototypes I–III with mandatory cooling for the prevention of burns and dyschromia.
or
laser epilation using a diode dermatological laser system (810 nm) once every 3–4 weeks [3,5,6,24,31-34].
GCR CLoE 3Grade of Recommendation C (Level of Evidence – 3)
Comments: The number of procedures is individual, on average 8–12 sessions [24,31-34]. Melanin is the target chromophore, therefore this type of laser epilation is ineffective for removing light, red, and gray hair. Recommended for phototypes I–V.
3.2 Surgical Treatment
Not applicable.
3.3 Other Treatment
Diet therapy is not used.
Pain management is not used.
4. Medical Rehabilitation and Sanatorium-Resort Treatment
Not performed.
5. Prevention and Dispensary Observation
No prevention is carried out.
6. Organization of Medical Care
Treatment is carried out on an outpatient basis by a cosmetologist.
7. Additional Information
The choice of method depends on the patient's clinical presentation and may be determined by the equipment available in the cosmetologist's office.
The number of procedures to achieve an effective result is strictly individual and may vary depending on the treatment method, number of hair follicles, and individual characteristics of the skin phototype and hair.
Criteria for Evaluating the Quality of Medical Care
№ | Quality criteria | Grade of Recommendation | Level of evidence | |
|---|---|---|---|---|
1. | Temporary methods of hair shaft removal without affecting the hair follicle (depilation) were performed and/or a course of device-based electrical epilation for hair of all colors was carried out, and/or for hair of all colors except depigmented (white, gray) a course of photoepilation using broadband pulsed light (IPL) (500–1200 nm) and/or a course of laser epilation using an alexandrite (755 nm) or diode laser (810 nm) was carried out. | A, B | 1, 2, 3 | |
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Appendix A1. Working Group Composition
Kubanov Alexey Alekseevich – Academician of the RAS, Doctor of Medical Sciences, Professor, Director of the State Research Center of Dermatovenereology and Cosmetology of the Russian Ministry of Health, President of the Russian Society of Dermatovenereologists and Cosmetologists.
Makhakova Yulia Buyandylgerovna — Candidate of Medical Sciences, Head of the Educational Department of the State Research Center of Dermatovenereology and Cosmetology of the Russian Ministry of Health, member of the Russian Society of Dermatovenereologists and Cosmetologists.
Kondrakhina Irina Nikiforovna — Doctor of Medical Sciences, Head of the Consultative and Diagnostic Center of the State Research Center of Dermatovenereology and Cosmetology of the Russian Ministry of Health, member of the Russian Society of Dermatovenereologists and Cosmetologists.
Alifanova Irina Sergeevna — Dermatocosmetologist at the Consultative and Diagnostic Center of the State Research Center of Dermatovenereology and Cosmetology of the Russian Ministry of Health, member of the Russian Society of Dermatovenereologists and Cosmetologists.
Appendix A2. Methodology for Developing Clinical Guidelines
Target audience for these clinical guidelines:
Specialist physicians: dermatovenereologists, cosmetologists.
Residents and trainees of continuing professional education programs in the specified specialty.
Table 1. Level of Evidence (LoE) grading scale for diagnostic methods (diagnostic interventions)
LoE | Definition |
|---|---|
1 | Systematic reviews of studies with reference standard control or systematic reviews of randomized controlled trials with meta-analysis |
2 | Individual studies with reference standard control or individual randomized controlled trials and systematic reviews of studies of any design, except randomized controlled trials, with meta-analysis |
3 | Studies without consecutive reference standard control or studies with a reference standard not independent of the test method, or non-randomized comparative studies, including cohort studies |
4 | Non-comparative studies, case report |
5 | Only justification of mechanism of action or expert opinion is available |
Table 2. Level of evidence (LoE) grading scale for prevention, treatment, and rehabilitation methods (preventive, therapeutic, and rehabilitative interventions)
LoE | Definitions |
|---|---|
1 | Systematic review of RCTs with meta-analysis |
2 | Individual RCTs and systematic reviews of studies of any design except RCTs, with meta-analysis |
3 | Non-randomized comparative studies, including cohort studies |
4 | Non-comparative studies, case reports or case series, case-control studies |
5 | Only justification of the mechanism of action of the intervention (preclinical studies) or expert opinion is available |
Table 3. Grade of Recommendation (GoR) scale for prevention, diagnostic, treatment, and rehabilitation methods (preventive, diagnostic, therapeutic, and rehabilitative interventions)
GoR | Definition |
|---|---|
A | Strong recommendation (all considered efficacy criteria (outcomes) are important, all studies are of high or acceptable methodological quality, and their conclusions on the outcomes of interest are consistent) |
B | Conditional recommendation (not all considered efficacy criteria (outcomes) are important, not all studies are of high or acceptable methodological quality, and/or their conclusions on the outcomes of interest are inconsistent) |
C | Weak recommendation (lack of adequate quality evidence (all considered efficacy criteria (outcomes) are unimportant, all studies are of low methodological quality, and their conclusions on the outcomes of interest are inconsistent) |
Procedure for updating clinical guidelines.
The clinical guidelines update mechanism provides for their systematic revision—at least once every three years, as well as upon the emergence of new evidence-based data regarding the diagnosis, treatment, prevention, and rehabilitation of specific diseases, or the presence of justified additions/comments to previously approved clinical guidelines, but no more frequently than once every 6 months.
Appendix A3. Reference Materials
These clinical guidelines were developed taking into account the following regulatory legal documents:
Procedure for the Provision of Medical Care in the Profile «Dermatovenereology», approved by Order of the Ministry of Health of the Russian Federation No. 924n dated November 15, 2012
Appendix B. Physician Action Algorithms
Appendix C. Patient Information
After completing a course of treatment using device-based correction methods, it is necessary to avoid excessive ultraviolet radiation (direct sunlight, visiting a tanning bed) until the skin integument is fully regenerated, and to use UVA + UVB 50+ topical sunscreens on skin areas exposed to sunlight, reapplying every 2 hours of sun exposure.
Appendix G. Assessment Scales, Questionnaires, and Other Evaluation Tools
Appendix G1. Ferriman-Gallwey Scale
The Ferriman–Gallwey score is designed to assess the presence and severity of hirsutism in women.
Original title: The Ferriman-Gallwey score
Source (official developer website, validation publication): DAVID FERRIMAN, D.M., M.R.C.P., J. D. GALLWEY, M.B., B.CH., CLINICAL ASSESSMENT OF BODY HAIR GROWTH IN WOMEN, The Journal of Clinical Endocrinology & Metabolism, Volume 21, Issue 11, 1 November 1961, Pages 1440–1447, https://doi.org/10.1210/jcem-21-11-1440
Type (underline):
- assessment scale
- index
- questionnaire
- other (specify):
Purpose: to assess the presence and severity of hirsutism in women.
Content (template): the severity of hair growth is evaluated in 9 body areas using a 4-point scale.
Key (interpretation): The sum of scores across all areas is called the hirsute score. The diagnosis of "hirsutism" is established when the hirsute score is greater than 8. Criteria for assessing the severity of hirsutism: mild (up to 15 points), moderate (16–25 points), and severe (above 25 points).
According to recent guidelines, hirsutism is generally indicated by a modified Ferriman–Gallwey score sum of ≥4–6, although there are racial characteristics in the evaluation of hirsutism. In some Caucasian and Negroid individuals, a pathognomonic finding is an increased score sum of ≥8 on the specified scale. For individuals of Southeast Asia, a diagnostically significant increase in the score sum on this scale is ≥3. More pronounced hirsutism is characteristic of women from the Middle East, Latin America, and the Mediterranean [2,15,16,40,41].