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Year : 2013  |  Volume : 58  |  Issue : 2  |  Page : 161
Hyperkeratosis lenticularis perstans: Case report of a rare entity


Department of Dermatology, Venereology and Leprology, GMERS Medical College, Sola, Ahmedabad, Gujarat, India

Date of Web Publication5-Mar-2013

Correspondence Address:
Krina B Patel
Department of Dermatology, Venereology and Leprology, GMERS Medical College, Sola, Ahmedabad, Gujarat
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.108092

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How to cite this article:
Patel KB. Hyperkeratosis lenticularis perstans: Case report of a rare entity. Indian J Dermatol 2013;58:161

How to cite this URL:
Patel KB. Hyperkeratosis lenticularis perstans: Case report of a rare entity. Indian J Dermatol [serial online] 2013 [cited 2019 Sep 19];58:161. Available from: http://www.e-ijd.org/text.asp?2013/58/2/161/108092


Sir,

A 22-year-old female patient presented with complains of gradually increasing asymptomatic skin lesions all over body for last 2 years. On examination, multiple, tiny, discrete, erythrmatous, scaly, folliculo-papular, bilaterally symmetrical lesions were present all over her body, except face and scalp [Figure 1], [Figure 2], [Figure 3] and [Figure 4]. Lesions were dense on distal extremities as compared to proximal extremities and trunk. Few discrete lesions were present on palms and soles also. Skin lesions were asymptomatic, only occasional pruritus was reported by patient. Nail and mucosa were unaffected. Lesions started on distal extremities and gradually spread proximally. Patient applied various topical medicines including topical steroids, moisturizers etc., without significant improvement.
Figure 1: Discrete, follicular papular lesions on dorsa of feet

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Figure 2: Similar lesions on forearms

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Figure 3: Lesions on thighs

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Figure 4: Few lesions of similar morphology on trunk

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Patient was unmarried and healthy, had no history of any drug taken. Her menstrual periods were normal. She had history of regular hair removal only from extremities by waxing since the age of 14 years. She had no history of tuberculosis or any other major illness in past. No other family member had history of similar lesions.

Her complete blood count, blood sugar level, thyroid function tests, and X-ray chest were normal.

Skin biopsy showed dense, focal hyperkeratosis and parakeratosis, absent granular layer, acanthosis, and upper dermal lymphocytic infiltrate in a band-like manner. Changes showed sharp demarcation from normal skin, which showed basket weave hyperkeratosis [Figure 5]. Clinical and histo-pathological correlation confirmed the diagnosis of hyperkeratosis lenticularis perstans (HLP) or Flegel's disease.

Patient was prescribed oral vitamin A (5000 IU daily for 15 days), topical tretinoin cream 0.05% once-a-day application, and emollients with mild improvement in appearance noted after 3 weeks [Figure 6].
Figure 5: Histopathology showing basket - weave normal epidermis sharply demarcated by lesional hyperkeratosis, acanthosis, and band - like upper dermal infiltrate characteristic of Flegel's disease (×10 H and E)

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Figure 6: Mild improvement with treatment noted after 3 weeks

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HLP was first described by Flegel in 1958 [1] and hence also called as Flegel's disease.

Pathophysiology of HLP is unknown, but role of ultraviolet light in induction of lesions, role of cell-mediated cytotoxicity against epidermal cells are some of the mechanisms suggested. Ultrastructurally, loss of or decreased number of membrane-coating granules or Odland bodies have been reported. [2] Odland bodies are involved in normal process of desquamation. There absence may be associated with abnormal keratinisation.

Incidence and prevalence of HLP are not known. Both familial and non-familial cases have been reported. In familial cases, autosomal dominant transmission is shown. [3] Almost all reported cases are from white population. No sex predilection is seen. HLP is most common during mid to old age but has been reported in much younger patients also.

HLP is clinically characterized by small, red-brown, hyperkeratotic, 1-5 mm papules on the lower extremities. [4],[5] Involvement of the ear pinnae, arms, palms, soles, and the oral mucosa has been reported, although these reports are rare. Involvement of the trunk has been reported but remains an unusual variant. [6] Removal of the scale reveals a bright red base, often with pinpoint bleeding. A localized unilateral variant has been reported. [7] The trunk tends to be spared; absence of axial lesions is characteristic. Keratosis pilaris, Darier disease, follicular lichen planus, skin lesions of scurvy, and other follicular keratotic lesions should be differentiated from HLP.

Histopathology from a well-developed lesion shows greatly thickened, compact, strongly eosinophilic horny layer standing out in sharp contrast to the less heavily stained basket-weave keratin of the uninvolved epidermis. The underlying stratum malpighii is flattened with thin or absent granular layer. A lymphoid infiltrate with occasional histiocytes in a bandlike pattern in the papillary dermis typically is seen close to the epidermis. The lateral edge of the lesion demonstrates abrupt hyperkeratosis and a combination of epidermal atrophy and acanthosis. [8]

Various treatment options including topical 5-fluorouracil, topical and oral retinoids, oral vitamin A, topical calcipotriol, topical steroids, emollients, PUVA therapy, and dermabrasion have shown variable success rate. To our knowledge, this is the first case reported in Indian literature. Apart from rarity of this condition in Indian population, our patient also had extensive involvement with involvement of trunk, which is a rare finding. Rarity and chance of misdiagnosis, if biopsy is not done, prompted us to present this case.

 
   References Top

1.Flegel H. Hyperkeratosis lenticularis perstans. Hautarzt 1958;9:363-4.  Back to cited text no. 1
[PUBMED]    
2.Frenk E, Tapernoux B. Hyperkeratosis lenticularis perstans (Flegel). An autosomal dominant skin disease due to lack of a keratinocyte organelle. Humangenetik 1974;24:151-3.  Back to cited text no. 2
[PUBMED]    
3.Bean SF. The genetics of hyperkeratosis lenticularis perstans. Arch Dermatol 1972;106:72.  Back to cited text no. 3
[PUBMED]    
4.Humphrey S, Crawford RI, Au S. Long-standing spiny papules on the lower extremities. Flegel disease, or hyperkeratosis lenticularis perstans (HLP). Arch Dermatol 2008;144:1509-14.  Back to cited text no. 4
[PUBMED]    
5.Li TH, Hsu CK, Chiu HC, Chang CH. Multiple asymptomatic hyperkeratotic papules on the lower part of the legs. Hyperkeratosis lenticularis perstans (HLP) (Flegel disease). Arch Dermatol 1997;133:910-1.  Back to cited text no. 5
[PUBMED]    
6.Miljkovic J. An unusual generalized form of hyperkeratosis lenticularis perstans (Flegel's disease). Wien Klin Wochenschr 2004;116:78-80.  Back to cited text no. 6
    
7.Miranda-Romero A, Sanchez SP, Bajo del Pozo C, Martinez FM, Esquivias GJ, Garcia MM. Unilateral hyperkeratosis lenticularis perstans (Flegel's disease). J Am Acad Dermatol 1998;39:655-7.  Back to cited text no. 7
    
8.Miller MK, Naik NS, Nousari CH, Friedman RJ, Heilman ER. Degenerative diseases and perforating disorders. In: Elder DE, editor. Lever's Histopathology of the skin, 10th ed. New Delhi: Wolters Kluwer India; 2009. p. 389-403.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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