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Thick, adherent, asbestos-like and overlapping scales encircling the tufts of hair shafts in a 13-year-old girl


 Department of Dermatology, Venereology and Leprosy, Dr. R. P. Govt. Medical College, Kangra (Tanda), Himachal Pradesh, India

Date of Web Publication24-Jan-2020

Correspondence Address:
Vikram K Mahajan,
Department of Dermatology, Venereology and Leprosy, Dr. R. P. Govt. Medical College, Kangra (Tanda) - 176 001, Himachal Pradesh
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/idoj.IDOJ_278_19



How to cite this URL:
Kumar P, Chowdhary B, Mahajan VK. Thick, adherent, asbestos-like and overlapping scales encircling the tufts of hair shafts in a 13-year-old girl. Indian Dermatol Online J [Epub ahead of print] [cited 2020 Aug 10]. Available from: http://www.idoj.in/preprintarticle.asp?id=276570



A 13-year-old girl presented with thick, adherent and overlapping yellowish scales encircling the tufts of hair shafts for the last 4 years. Her lesions had started initially over the vertex and progressed to involve the whole scalp [Figure 1]a. It was associated with fissuring, itching, pain and mousy smell on occasions especially during summers and rainy seasons. It remained relentlessly progressive and repeated treatments in the form of shampoos and topical lotions did no benefit. During last 2 years, she also noticed asymptomatic, dry, hyperkeratotic, brownish-red asymptomatic papular lesions over left supraclavicular area, and over left [Figure 1]b and right [Figure 1]c fronto-tempo-parietal scalp margins. Examination for mucous membranes, nails, palms and soles was normal. Her other medical history and systemic examination were unremarkable. She was the only child born to non-consanguineous otherwise healthy parents after an uneventful pregnancy. No other family members had similar problem. Microscopy showed no hair shaft abnormality or fungal elements in KOH mounts. The findings of hematoxylin-eosin-stained histology sections of a skin lesion are shown in [Figure 2]a and [Figure 2]b.
Figure 1:(a) Hyperkeratotic scalp lesions with classic matted hair and overlapping crusts in a tiled appearance. (b) Multiple brown hyperkeratoticpapular lesions over left temporal, and (c) right temporal hair margins

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Figure 2: (a) Extensive hyperkeratosis (HK), irregular dermal papillae lined by single layer of basal cells (P) projecting into the suprabasalacantholyticunilocular cleft (C) within the central epidermis with no acantholytic cells. Arrows indicate dyskeratotic cells in the stratum granulosum (stain H and E, ×40). (b) Dyskeratotic cells, corps ronds with small pyknotic nuclei, a perinuclear clear halo and eosinophilic cytoplasm (arrows) and grains, compressed cells with elongated nuclei (arrow heads) are visualized in stratum ganuosum. Extensive hyperkeratosis (HK) and suprabasalacantholytic cleft (C) are also seen (stain H and E, ×100)

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What is your diagnosis?

Diagnosis: Darier's disease presenting as pityriasis amiantacea


   Microscopic Findings Top


Histopathology revealed features consistent with Darier's disease comprising extensive hyperkeratosis, papillomatosis, suprabasal acantholysis and cleft formation, and villus-like dermal papillae lined by single layer of basal cells projecting into it [Figure 2]a. Corp ronds and grains were visualized in the stratum granulosum [Figure 2]b. Absence of acantholytic cells and minimal dermal infiltrate were other notable features.


   Treatment Top


Her skin lesions resolved and scaling from scalp decreased 4 weeks after treatment with oral isotretinoin 20mg once daily, ketoconazole (2%) shampoo for alternate day scalp washing and topical clobetasol propionate 0.05%+salicylic acid 3% lotion for once daily scalp application. However, the isotretinoin dose had to be decreased due to severe cheilitis. Subsequent flare up was treated successfully with oral cefadroxil 500mg twice daily given for 10 days. She did not continue follow-up.


   Comments Top


Pityriasis amiantacea is a distinct and usually localized inflammatory scalp disorder affecting young adults, adolescents and children. Clinically, adherent, thick and overlapping, asbestos-like silvery or yellowish scales encircling the hair shafts and binding down tufts of hair and occasional temporary alopecia are characteristic. Its etiopathogenesis remains unclear and is thought to represent a reaction pattern to inflammatory skin disease. Scalp psoriasis was confirmed in 35.3% while features of seborrheic and atopic dermatitis were observed in 34.2% in series of 85 cases.[1] Lichen planus, lichen simplex chronicus, pityriasis rubra pilaris, and superficial fungal or pyogenic infections were other notable causes. However, it is considered a manifestation of psoriasis by most workers. Darier's disease (syn. Keratosis follicularis, Darier-White disease) is an uncommon disorder of autosomal dominant inheritance with reported incidence of 1:30,000 to 100,000 population. A mutation in ATP2A2 gene has been mapped to chromosome 12q23-24.1 that encodes sarcoplasmic/endoplasmic reticulum Ca2+-ATP isoform 2 protein (SERCA2) which impairs intracellular Ca2+ signaling and affects cell adhesion. [2,3] However, genetic studies are rarely needed for diagnosis. Clinically, it usually presents at a mean age of 6-20 (range 4-70) years affecting both genders equally. Exacerbations occur from sunlight, heat, sweat, and humidity, and secondary bacterial infections. There is increased susceptibility to infections while short stature, mental retardation, epilepsy, mood disorders, schizophrenia occur rarely.[4] Pemphigus foliaceous, Hailey-Hailey disease, acrokeratosis verruciformis of Hopf, and transient acantholytic dermatosis remain major differentials.

Darier's disease as comedonal lesions or localized to scalp as coalescing hyperkeratotic papules has been reported infrequently in adults.[5] However, Darier's disease presenting as pityriasis amiantacea as in our patient is extremely rare.[6] Their causal relationship is not clear, but an exaggerated inflammatory response to the Darier's disease or repeated pyogenic infection seems most plausible explanation.

The treatment of Darier's disease remains unsatisfactory and it tends to persist for life with partial remissions. Most cases have been treated with topical emollients, keratolytics, steroid, and/or retinoid creams during exacerbations. Systemic retinoids (isotretinoin, acitretin) are effective to resolve keratotic lesions but recurrences follow after treatment cessation and long-term use remains problematic due to various adverse effects and in child bearing age.[7] Dermabrasion, electrosurgery, CO2, Er: Yag, pulse-dye lasers or other fractional resurfacing devices, electron beam therapy, photodynamic therapy, and surgical excision have been used with variable success. The therapeutic efficacy of miglustat, an α-glucosidase inhibitor that increases adhesion strength by restoring mature adherens junctions and desmosomes in dyskeratotic keratinocytes, remains unevaluated.[8] Oral vitamin A appears an attractive safe and low-cost therapeutic option.[9]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest



 
   References Top

1.
Abdel-Hamid IA, Agha SA, Moustafa YM, El-Labban AM. Pityriasisamiantacea: A clinical and etiopathologic study of 85 patients. Int J Dermatol 2003;42:260-4.  Back to cited text no. 1
    
2.
Sakuntabhai A, Ruiz-Perez V, Carter S, Jacobsen N, Burge S, Monk S, et al. Mutations in ATP2A2, encoding a Ca2+ pump, cause Darier disease. Nat Genet 1999;21:271-7.  Back to cited text no. 2
    
3.
Dhitavat J, Fairclough RJ, Hovnanian A, Burge SM. Calcium pumps and keratinocytes: Lessons from Darier's disease and Hailey-Hailey disease. Br J Dermatol 2004;150:821-8.  Back to cited text no. 3
    
4.
Tang C, Chan M, Lee J, Hariram J. Darier's disease and schizophrenia. East Asian Arch Psychiatry 2010;20:190-2.  Back to cited text no. 4
    
5.
Chung J, Kim JY, Gye J, Namkoong S, Hong SP, Park BC, et al. A case of familial comedonal Darier's disease. Ann Dermatol 2011;23(Suppl 3):S398-S401.  Back to cited text no. 5
    
6.
Hussain W, Coulson IH, Salman WD. Pityriasisamiantacea as the sole manifestation of Darier's disease. Clin Exp Dermatol 2009;34:554-6.  Back to cited text no. 6
    
7.
Suzuki K, Aoki M, Kawana S. Localized Darier's disease of the scalp: Successful treatment with oral etretinate. Dermatology 2004;208:83-4.  Back to cited text no. 7
    
8.
Savignac M, Simon M, Edir A, Guibbal L, Hovnanian A. SERCA2 dysfunction in Darier disease causes endoplasmic reticulum stress and impaired cell-to-cell adhesion strength: Rescue by miglustat. J Invest Dermatol 2014;134:1961-70.  Back to cited text no. 8
    
9.
Sondhi M, Chowdhary B, Mahajan VK. Darier's disease- response to oral vitamin A: Report of a case and brief review. Indian Dermatol Online J [In press].  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2]



 

 
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