10.5826/dpc.1201a02
text/xml
2160-9381
1713
en
Keshavamurthy Vinay
https://creativecommons.org/licenses/by-nc/4.0
Dermatology Practical & Conceptual
RPC
Crusted Umbilicated Papules in a Child 
Text.Serial.Journal
https://dpcj.org/index.php/dpc
Research Letter
2022-02-02
2021-03-10
2022-02-02
2022-02-09
e2022002
Dermatol Pract Concept
Keshavamurthy Vinay
2022/02/02
10.5826/dpc.1201a02
e2022002
Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
2160-9381
RPC
en
e2022002
Kittisak P, Tanaka M. Dermoscopic findings in a case of reactive perforating collagenosis. Dermatol Pract Concept. 2015;5(2):75-77. DOI: 10.5826/dpc.0502a13. PMID: 26114057. PMCID: PMC4462904.
Crusted Umbilicated Papules in a Child 
https://dpcj.org/index.php/dpc/article/download/1713/1504
                  Crusted Umbilicated Papules in a Child
           Hitaishi Mehta1, Sheetanshu Kumar1, Divya Aggarwal2, Debajyoti Chatterjee2,
                                     Keshavamurthy Vinay1


1 Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India



Key words: reactive perforating collagenosis, RPC, perforating disorders
Citation: Mehta H, Kumar S, Aggarwal D, Chatterjee D, Vinay K. Crusted umbilicated papules in a child.
Dermatol Pract Concept. 2022;12(1):e2022002. DOI: https://doi.org/10.5826/dpc.1201a02
Accepted: April 14, 2021; Published: January 2022
Copyright: ©2022 Mehta et al. This is an open-access article distributed under the terms of the Creative Commons Attribution-
NonCommercial License (BY-NC-4.0), https://creativecommons.org/licenses/by-nc/4.0/, which permits unrestricted noncommercial use,
distribution, and reproduction in any medium, provided the original authors and source are credited.
Funding: None.
Competing interests: None.
Authorship: All authors have contributed significantly to this publication.
Corresponding author: Keshavamurthy Vinay, MD, DNB, MNAMS, Department of Dermatology, Venereology and Leprology,
Postgraduate Institute of Medical Education and Research, Chandigarh, India. E-mail: vinay.keshavmurthy@gmail.com.




Introduction                                                             papules on both cheeks (Figure 1A) and dorsum of hands
                                                                         (Figure 1B) with central adherent keratotic plugs along with
Reactive perforating collagenosis (RPC) is a rare benign per-            few well-circumscribed areas of atrophic scars. Dermoscopy
forating disorder characterized by transepidermal elimina-               of an individual lesion is presented in Figure 2A. Routine
tion of collagen fibers. Both familial and acquired forms of             laboratory parameters including renal function tests, fast-
RPC exist, the latter in association with end-stage chronic              ing blood sugar, and urinalysis were within normal limits.
kidney disease and diabetes mellitus. The very rare child-               Histopathological examination of a facial papular lesion
hood form has slight male predilection and is commonly                   revealed compact ortho- and parakeratotic epidermis with
familial [1].                                                            a cup-shaped lesion lined by acanthotic epidermis on both
                                                                         sides along with mild perivascular and periadnexal lymph-
                                                                         omononuclear cell infiltrate mixed with collagen bundles in
Case Presentation
                                                                         the dermis (Figure 2B). Altered collagen could be seen ex-
Dermatology consultation was sought for a school-age child               truding trans epidermally through the cup-shaped depres-
with multiple mildly itchy, red, raised lesions on his face and          sion. A diagnosis of RPC was reached. The child was treated
dorsum of hands, which were first noticed by the parents                 with topical tretinoin 0.025% ointment with satisfactory
at 2 years of age. The parents also reported spontaneous                 improvement after 1 month.
healing of a few of the lesions with residual hypopigmented
scars. Preceding history of insect bites or trauma was denied.
                                                                         Conclusions
He was born out of a non-consanguineous marriage, and
no one in family had a history of similar lesions. Examina-              Familial RPC commonly presents as erythematous papules
tion revealed multiple skin-colored to erythematous discrete             with central adherent scale-crust over the extensor aspect of



Research Letter | Dermatol Pract Concept. 2022;12(1):e2022002                                                                         1
                   Figure 1. (A) Multiple skin-colored to erythematous discrete papules on left cheek with central
                   adherent keratotic plugs along with few well circumscribed areas of atrophic scars on cheek. (B)
                   Crusted umbilicated papules over dorsum of both hands.




      Figure 2. (A) Dermoscopy reveals a yellowish central homogenous area along with a white rim surrounded by erythematous
      halo at the periphery (DermLite II, Hybrid M, ×10, polarized). (B) Histopathological examination (H&E, ×40) of the punch
      biopsy taken from one of the papular lesions on the face showing orthokeratotic epidermis with a cup shaped lesion lined by
      acanthotic epidermis on both sides along with mild perivascular and periadnexal lymphomononuclear cell infiltrate mixed with
      collagen bundles in the dermis. The cup-shaped lesion was densely packed with degenerated basophilic collagen bundles.


extremities. Superficial trauma precedes most lesions. Indi-         white rim of varying thickness corresponds to the epidermal
vidual lesions heal within 6-10 weeks, but recurrences are           invagination, along with an erythematous halo which arises
common. Although the familial and acquired forms appear              due to small blood vessels around the lesion [2]. Histopathol-
similar in morphology and on histopathology, the acquired            ogy reveals a plug of keratotic debris with vertically oriented
form tends to be more persistent and shows transepidermal            collagen fibers extending into the plug, along with parakerato-
elimination of both collagen and elastin.                            sis, epithelial hyperplasia, and dyskeratotic keratinocytes [1].
    The central homogeneous yellowish area observed on der-              The diagnosis is mostly clinical and requires histopa-
moscopy corresponds to the central scale or crust, and the           thology for confirmation. Investigations other than punch



2                                                                         Research Letter | Dermatol Pract Concept. 2022;12(1):e2022002
biopsy are neither necessary nor contributory. Differential       Informed consent: Informed consent for publication of clini-
diagnosis includes papular urticaria, prurigo nodularis, per-     cal details and clinical images was obtained from the patient.
forating folliculitis, and elastosis perforans serpiginosa.
   Along with avoidance of trauma, topical therapies re-
                                                                  References
ported to be successful include retinoids, corticosteroids, and
salicylic acid. Other therapies are systemic antihistamines,      1. Millard PR, Young E, Harrison DE, Wojnarowska F. Reactive
retinoids, methotrexate, photochemotherapy, NB-UVB, and              perforating collagenosis: light, ultrastructural and immuno-
                                                                     histological studies. Histopathology. 1986;10(10):1047-1056.
cryotherapy. Most of the patients have a relapsing-remitting
                                                                     DOI:10.1111/j.1365-2559.1986.tb02541.x. PMID: 2430879.
course throughout their lifetimes.
                                                                  2. Kittisak P, Tanaka M. Dermoscopic findings in a case of reactive
   Childhood RPC is a rare entity with an obscure etio-              perforating collagenosis. Dermatol Pract Concept. 2015;5(2):
pathogenesis and usually self-limiting course. Awareness             75-77. DOI: 10.5826/dpc.0502a13. PMID: 26114057. PMCID:
among clinicians regarding this entity is imperative in order        PMC4462904.
to avoid misdiagnosis, anxiety among parents, and over-ag-
gressive management.




Research Letter | Dermatol Pract Concept. 2022;12(1):e2022002                                                                      3
