Keywords

A 10-year-old boy, presented with hair loss of 1 year duration. It started as a small patch of hair loss appearing all of a sudden 1 year back and progressed to the present status (Fig. 81.1). There was no prior history of drugs intake, trauma or pustules/nodules. Cutaneous examination showed a diffuse non-scarring alopecia affecting the frontal area and vertex, progressing along the hairline in parietal and occipital regions (Fig. 81.1). The overlying skin was smooth and shiny and there were no similar looking lesions elsewhere in the body. Nails showed geometric pitting. KOH examination did not reveal any fungal hyphae.

Fig. 81.1
figure 1

Hair loss on vertex and along hairline, in parietal (a) and occipital area (b)

Full size image

Routine investigations and thyroid profile were within normal limits. Histopathological examination of a biopsy specimen taken from the bald patch revealed a peribulbar lymphocytic infiltrate, similar to a swarm of bees.

Q1. What is the best initial diagnosis ?

  1. A.

    Tinea capitis

  2. B.

    Trichotillomania

  3. C.

    Alopecia areata

  4. D.

    Pseudopelade of Brocq

Answer: The correct answer is C.

Alopecia areata (AA) manifests as well-demarcated patches of non-scarring alopecia. AA is usually noticed suddenly and may present as single or multiple patches. Scalp is the most common site, while nail changes can be more varied including; geometric pitting, i.e. multiple, small, superficial pits regularly distributed along transverse and longitudinal lines , geometric punctate leukonychia, i.e. multiple white spots in a grill pattern, and trachyonychia, i.e. sandpaper nails. AA may be associated with atopy, autoimmune thyroiditis, vitiligo, psoriasis, diabetes mellitus, Down’s syndrome, Addison’s disease, autosomal recessive autoimmune polyglandular syndrome, systemic lupus erythematosus, celiac disease, ulcerative colitis , and multiple sclerosis, etc. [1].

Alopecia areata is considered an autoimmune disease, and hair follicle-specific antibodies have been found in peripheral blood of AA patients [2]. Clinical presentation of AA is a myriad which has been classified:

  • Based on extent of involvement [1]:

    • Patchy alopecia: one or multiple patches of AA

    • Alopecia totalis: involvement of complete scalp

    • Alopecia universalis: loss of complete body hairs, including scalp, eyebrows, eyelashes, axillary and pubic hair

  • Based on pattern of involvement [2]:

    • Reticular: hair loss resembling a net

    • Ophiasis: hair loss along the posterior occipital and temporal margins

    • Sisaipho/Ophiasis inversus: hair loss involving the frontal, temporal, and parietal scalp but spares hair along the scalp periphery and hairline.

    • Diffuse: newly described form characterized by generalized thinning of hair. It can be diagnosed by dermoscopy and histopathology.

    • Linear

Common clinical differentials for patchy AA include tinea capitis, trichotillomania and pseudopelade of Brocq. Tinea capitis is characterized by erythema, scaling, crusting and easy pluckability of hair. Trichotillomania is characterized by loss of hair from accessible areas of scalp, and the hallmark finding is the presence of multiple broken hairs at different lengths. Pseudopelade on the other hand, is manifested as hyperpigmented atrophic patches of hair loss [1, 2].

Q2. Which of the following options is a possible dermoscopic finding in alopecia areata ?

  1. A.

    Yellow and black dots

  2. B.

    Broken and tapering hairs

  3. C.

    Short villus hairs

  4. D.

    All of the above

Answer: The correct answer is D.

Dermoscopy is an easy and handy technique of diagnosis of alopecia (including alopecia areata) and obviates the need for biopsy in many cases. Characteristic features of AA in dermoscopy include yellow dots, black dots, broken hairs, tapering hair (i.e. exclamation marks) and short vellus hairs. Presence of black dots, broken hair, and tapering hair suggest an underlying activity. The number of black dots and yellow dots are directly proportional to the severity of disease. A single feature may not be pathognomonic, but a constellation of findings leads to the corroborative diagnosis. Presence of exclamatory mark hairs at periphery, positive hair pull test (>6 hairs), daily hair loss count (>100 hairs), predominance of telogen hairs in pluck test and black dots, broken hair, and tapering hair in dermoscopy, suggest active disease [3, 4].

Q3. Which of the following is not a poor prognostic marker of alopecia areata ?

  1. A.

    Younger age of onset in the patient

  2. B.

    Alopecia involving the temporal and occipital areas and vertex and leaving the ophiasis regions intact

  3. C.

    Presence of atopy

  4. D.

    Presence of nail changes

Answer: The correct answer is B.

Poor prognostic factors of alopecia areata has been identified and includes [5, 6]:

  • Younger age of onset

  • Family history of disease

  • Presence of atopy

  • Alopecia totalis and alopecia universalis

  • Ophiasis pattern

  • Duration more than 1 year

  • Presence of nail changes

  • Associated autoimmune diseases

Q4. All of the following statements are correct regarding the histopathology of alopecia areata, except :

  1. A.

    Horizontally-sectioned scalp biopsy is helpful in confirming the diagnosis

  2. B.

    Best place to take a biopsy is at the advancing border of hair loss

  3. C.

    Mean count of less than one follicle in mm2 indicates a lower chance of regrowth

  4. D.

    Terminal to vellus hair ratio is reduced to 7:1

Answer: The correct answer is C.

Diagnosis is always made on clinical grounds. Biopsy is seldom required but is helpful when clinical diagnosis is uncertain.

Horizontal sections are usually preferred to vertical sections because they allow examination of multiple hair follicles in a single frame. Best place to take a biopsy is at the advancing border of hair loss. This helps to quantify the hair follicle density, follicle diameter, and assess the proportion of hair follicles in various stages. A mean count of less than one follicle/mm2 usually indicates lower chances of regrowth. Characteristic feature of an acute case is peribulbar lymphocytic infiltrate akin to a swarm of bees. In chronic cases, follicular miniaturization with variable inflammatory infiltrate are observed in the upper dermis. The terminal to vellus hair ratio is decreased to 1:1 in contrast to 7:1 in normal population [7, 8].

Q5. Which of the following options is used in the management of alopecia areata?

  1. A.

    Topical steroids

  2. B.

    Immunotherapy

  3. C.

    Phototherapy

  4. D.

    All of the above

Answer: The correct answer is D.

Therapeutic options available are topical agents including; corticosteroids, anthralin, minoxidil, immunotherapy, phototherapy, prostaglandin analogues; systemic therapy including; steroids, sulfasalazine, PUVA and others like cyclosporine, methotrexate, capsaicin, topical bexarotene, calcineurin inhibitors, and narrow band UVB etc. [5, 9, 10].

The treatment of choice depends on the extent of disease, co-morbidities and lifestyle of patient, previous treatment history etc. The treatment plan include:

  • For patchy alopecia areata:

    • Topical corticosteroids (Class I-III) and intralesional triamcinolone (2.5–5 mg/ml)

    • Topical retinoids

    • Topical anthralins—short contact therapy

    • Phototherapy and photochemotherapy (PUVA)

    • Immunotherapy with diphenylcyclopropenone (DPCP)

    • Topical minoxidil

    • 308-nm Excimer laser

  • For alopecia totalis or universalis, or extensive disease recalcitrant to topical therapy;

    • Oral corticosteroids (pulse therapy)

    • Other immunosuppressive agents such as methotrexate, azathioprine, and cyclosporine

    • Wigs and other hair camouflage

Recently, Topical Janus kinase inhibitor, tofacitinib and ruxolitinib, have been used successively to treat pediatric alopecia areata [11].

FormalPara Practical Points

  • Alopecia areata (AA) manifests as well-demarcated patches of non-scarring alopecia. AA is usually noticed suddenly

  • Nail changes are common and include geometric pitting, punctate leukonychia, and trachyonychia, i.e. sandpaper nails

  • AA may be associated with atopy, autoimmune thyroiditis, vitiligo, psoriasis, diabetes mellitus, autosomal recessive autoimmune polyglandular syndrome, among other autoimmune conditions

  • Dermoscopy is an easy and handy technique of diagnosis of alopecia

  • Characteristic features of AA in dermoscopy include yellow dots, black dots, broken hairs, tapering hair and short vellus hairs

  • Presence of exclamatory mark hairs at periphery, positive hair pull test (>6 hairs), daily hair loss count (>100 hairs), predominance of telogen hairs in pluck test and black dots, broken hair, and tapering hair in dermoscopy, suggest active disease