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E-IJD CORRESPONDENCE
Year : 2015  |  Volume : 60  |  Issue : 1  |  Page : 107
Unusual presentation of atrichia with papular lesions


1 Department of Dermatology, Skin Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran
2 Department of Pathology, Shahid Beheshti University of Medical Sciences, Tehran, Iran

Date of Web Publication26-Dec-2014

Correspondence Address:
Safoura Sakoei
Department of Dermatology, Skin Research Center, Shahid Beheshti University of Medical Sciences, Tehran
Iran
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.147889

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How to cite this article:
Robati RM, Zereh Posh FB, Sakoei S, Ranjbar M. Unusual presentation of atrichia with papular lesions. Indian J Dermatol 2015;60:107

How to cite this URL:
Robati RM, Zereh Posh FB, Sakoei S, Ranjbar M. Unusual presentation of atrichia with papular lesions. Indian J Dermatol [serial online] 2015 [cited 2020 Feb 21];60:107. Available from: http://www.e-ijd.org/text.asp?2015/60/1/107/147889


Sir,

Atrichia with papular lesions (APL) is a rare form of total alopecia followed by eruptions of papular lesions on the skin. [1],[2] Patients with this form of alopecia show normal hairs at birth but hair loss occurred soon after birth. At approximately 2 years of age, papular lesions of keratin-filled cysts develop on different areas of the body. [1] We report here a rare case of APL with late onset papular lesions.

A 28-year-old lady presented with complete loss of hair on the body. She had sparse hairs over the scalp at birth but gradually lost all of them [Figure 1]. She was previously treated with the diagnosis of alopecia areata. Her alopecia was unresponsive to therapies. She developed multiple skin-colored lesions on the body, which progressively increased in number from 7 months ago. Multiple discrete, skin-colored, firm, papulonodular lesions, 0.2-0.5-mm were presented on her genitalia. The lesions were treated with the diagnosis of viral wart. Papules extended to her trunk and arms gradually [Figure 2]. She revealed no history of hyperhidrosis, decrease in vision, hearing loss or seizure. Her nails, teeth and mucosa were normal. Her palms and soles were also normal. She did not have any systemic involvement or dysmorphic features. Her brother was 24-year-old and had similar symptoms.
Figure 1: Complete loss of scalp and body hair

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Figure 2: Papules over trunk and arms

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The skin biopsy from a papule on the elbow revealed multiple mid-dermal keratin cysts with granular layer and laminated keratin in the mid-dermis with sparse peri-follicular lymphocytic infiltrate [Figure 3]. Based on the clinical and pathological findings a diagnosis of APL was made.
Figure 3: Multiple mid-dermal keratin cysts with granular layer and laminated keratin in the mid-dermis with sparse peri-follicular lymphocytic infiltrate (H and E ,×40)

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APL is an autosomal recessive inheritance disease with irreversible alopecia of the scalp and body associated by development of papular lesions of keratin cysts over of the body. Scalp, axillary, and pubic hair are affected. Patients are almost devoid of eyebrows and eyelashes. [1],[2] The mutation is seen in zinc finger domain of the human hairless gene on chromosome region 8p12. [3] The hair matrix cells undergo premature and massive apoptosis and have a decline in Bcl-2 expression, so no transmitted messages between the dermal papillae and stem cells in the bulge and no further hair growth occur. [4] The patients have normal development of other organs such as hearing, teeth, nails, and sweat glands with no systemic involvement. [4],[5] APL is frequently misdiagnosed as alopecia universalis. This may be due to several factors such as the rarity of the diseases and the lack of specific criteria for diagnosis. [2]

In our patient, the papular lesions showed an adult onset in contrast to the other studies that indicated a childhood onset. In presenting case, the papular lesions was mistakenly diagnosed and treated as genital wart for a long period of time. Therefore, it would better to keep in mind the diagnosis of atrichia with papular lesion in adult patients with generalized alopecia to avoid wrong diagnosis such as viral wart.

 
   References Top

1.
Azeem Z, Wasif N, Basit S, Razak S, Waheed RA, Islam A, et al. Congenital atrichia with papular lesions resulting from novel mutations in human hairless gene in four consanguineous families. J Dermatol 2011;38:755-60.  Back to cited text no. 1
    
2.
Zlotogorski A, Panteleyev AA, Aita VM, Christiano AM. Clinical and molecular diagnostic criteria of congenital atrichia with papular lesions. J Invest Dermatol 2002;118:887-90.  Back to cited text no. 2
    
3.
Bansal M, Manchanda K, Lamba S, Pandey S. Atrichia with papular lesions. Int J Trichol 2011;3:112-4.  Back to cited text no. 3
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4.
Thomas M, Daniel S. Atrichia congenita with papular lesions. Indian J Dermatol Venereol Leprol 2011;77:70-2.  Back to cited text no. 4
[PUBMED]  Medknow Journal  
5.
Indelman M, Bergman R, Lestringant GG, Peer G, Sprecher E. Compound heterozygosity for mutations in the hairless gene causes atrichia with papular lesions. Br J Dermatol 2003;148:553-7.  Back to cited text no. 5
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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