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CORRESPONDENCE |
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| Year : 2022 | Volume
: 67
| Issue : 6 | Page : 785-787 |
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| A case of hypopigmented macules in scalp of children with obvious improvement to topical retinoid: epidermodysplasia verruciformis or clear cell papulosis? |
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Zimeng You, Hongjie Liu, Zhuyu Luo, Wei Yan
Department of Dermatology; Laboratory of Dermatology, Clinical Institute of Inflammation and Immunology, Frontiers Science Center for Disease-related Molecular Network, West China Hospital, Sichuan University, Chengdu, China
| Date of Web Publication | 23-Feb-2023 |
Correspondence Address:
Wei Yan
Department of Dermatology; Laboratory of Dermatology, Clinical Institute of Inflammation and Immunology, Frontiers Science Center for Disease-related Molecular Network, West China Hospital, Sichuan University, Chengdu
China
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/ijd.ijd_501_22
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How to cite this article:
You Z, Liu H, Luo Z, Yan W. A case of hypopigmented macules in scalp of children with obvious improvement to topical retinoid: epidermodysplasia verruciformis or clear cell papulosis?. Indian J Dermatol 2022;67:785-7 |
How to cite this URL:
You Z, Liu H, Luo Z, Yan W. A case of hypopigmented macules in scalp of children with obvious improvement to topical retinoid: epidermodysplasia verruciformis or clear cell papulosis?. Indian J Dermatol [serial online] 2022 [cited 2023 Mar 29];67:785-7. Available from: https://www.e-ijd.org/text.asp?2022/67/6/785/370308 |
Sir,
Genetic epidermodysplasia verruciformis (EV) and clear cell papulosis (CCP) sometimes could be hard to differentiate because of similar clinical manifestations. Histopathology, especially immunohistochemistry could be useful in these circumstances. The treatment of EV is challenging, topical retinoids could be an effective and safe choice. Here we reported a boy of genetic EV with similar lesions to CCP showed obvious improvement to topical retinoids.
An 8-year-old boy presented with multiple white to skin-colour asymptomatic macules on his scalp, forehead and neck for 4 years [Figure 1]a. The boy was not in an immune suppression condition. The patient's father had similar lesions with negative fungus examination results.{Figure 1}
Dermatology examination showed multiple white flat hypopigmented macules with whitish scales distributed over the scalp, forehead and neck. The results of Wood's lamp and fungus examination were negative. Multiple hypopigmented macules with a few linear irregular vessels and whitish scales and irregular borders under dermoscopy (JEDA, Nanjing, China) were observed [Figure 1]b.
The skin biopsy taken from the right temporal area of the scalp showed acanthosis with enlarged greyish-blue cells. Vacuolated cells with hyperchromatic nuclei and greyish-blue cytoplasm located in the granular and upper spinous layers [Figure 2]. The diagnosis of both EV and CCP was suspected based on the clinical manifestation and histopathology (enlarged greyish-blue cells). Immunohistochemical analyses were negative for CAM 5.2, CK 7, GCDFP-15, HMB45, Mart-1, CEA, S-100, AB and PAS and positive for PCK and EMA. The diagnosis of genetic EV is based on clinical manifestation, family history, dermoscopy and immunohistochemical results.{Figure 2}
Meanwhile, 0.1% retinoid cream (used in the right temporal part of the scalp), 0.05% halometasone cream (used in the left occipital part of the scalp), 0.1% tacrolimus ointment (used in the right occipital part of the scalp) and 0.05% imiquimod cream (used in the left temporal part of the scalp) were used in a different area of scalp to observe the efficacy. Only the lesions treated with 0.1% retinoid cream showed improvement [Figure 3].{Figure 3}
EV manifests as polymorphic skin lesions, including papular lesions resembling verrucae planae, red or red-brownish papules or pityriasis versicolor-like lesions and verruca-like papillomatous or seborrheic keratosis-like lesions.[1] CCP manifests with multiple hypopigmented macules and flat-topped papules.[2] The patient presented with hypopigmented macules in the scalp without reddish or pink-red colour changes, which were not the typical lesions of EV.
Unfocused dotted and irregular vessels with whitish scales under dermoscopy raise the suspicion of EV.[3] Hypopigmented homogeneous structureless areas with irregular borders are observed in CCP.[4] The patient presented with irregular vessels, hypopigmented background, whitish scales and irregular border under dermoscopy, which cannot help make a definite diagnosis.
Typical histopathology of EV reveals characteristic large keratinocytes with blue-tinged cytoplasm and notable keratohyalin granules.[1] Although the histopathology features of CCP are clear cells within the basal cell layer, which are larger than their nearby keratinocytes and oval or round with centrally located nuclei and abundant pale cytoplasm.[4] The clear cells are consistently positive for AE1, CEA, EMA, CAM 5.2, CK-7 and mucin staining, whereas negative for S-100.[5] The diagnosis of CCP was ruled out based on the results of immunohistochemical results. The patient was diagnosed as having genetic EV eventually.
EV and CCP have similar skin lesions in some patients and positive family history could be observed. Histopathology, especially immunohistochemistry helps dermatologists differentiate EV and CCP. There is no need to treat CCP for its benign nature and spontaneous resolution tendency.[2] Although EV needs appropriate management and follow-up for its tendency to develop into skin cancers.
There is no standard treatment for EV and randomised controlled clinical trials are lacking due to its rarity. Four different species of drugs were used in this patient while improvement was observed only in skin lesions using a topical retinoid to treat.
Histopathology, especially immunohistochemistry should be conducted to make a definite diagnosis between EV and CCP. Topical retinoids show obvious efficacy and good safety in EV treatment though long-term follow-up is still needed in these patients.
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
There are no conflicts of interest.
 References | |  |
| 1. | de Jong SJ, Imahorn E, Itin P, Uitto J, Orth G, Jouanguy E, et al. Epidermodysplasiaverruciformis: Inborn rrrors of immunity to human beta-papillomaviruses. Front Microbiol 2018;9:1222.doi: 10.3389/fmicb. 2018.01222.  |
| 2. | Tseng FW, Kuo TT, Lu PH, Chan HL, Chan MJ, Hui RC. Long-term follow-up study of clear cell papulosis. J Am AcadDermatol2010;63:266-73. |
| 3. | Afra TP, Vinay K, Razmi TM, Chan HL, Chan MJ, Hui RC. Novel dermoscopic features of pityriasisversicolor-like macules in epidermodysplasiaverruciformis. PediatrDermatol 2020;37:230-2. |
| 4. | Leerunyakul K, Kanokrungsee S, Rutnin S. Clear cell papulosis: Dermatoscopicfindings and literature review. PediatrDermatol 2019;36:655-7. |
| 5. | Wysong A, Sundram U, Benjamin L. Clear-cell papulosis: A rare entity that may be misconstrued pathologically as normal skin. PediatrDermatol 2012;29:195-8. |
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