Dermatology: Practical and Conceptual


Dermatology Practical & Conceptual

Letter | Dermatol Pract Concept. 2021;11(4):e2021072 1

An Atypical Presentation of Extragenital Lichen 
Sclerosus et Atrophicus

Sabina Vaccari1, Alessia Barisani1, Francesca Pepe1, Carlotta Baraldi1,  
Bianca Maria Piraccini1, Valeria Gaspari1

1 Dermatology, IRCCS Policlinico di Sant’Orsola, Department of Experimental, Diagnostic and Specialty Medicine, Alma Mater Studiorum 

Università di Bologna, Italy

Key words: extragenital lichen sclerosus et atrophicus, morphea, dermoscopy, histopathology

Citation: Vaccari S, Barisani A, Pepe F, Baraldi C, Piraccini BM, Gaspari V. An atypical presentation of extragenital Lichen Sclerosus et 
Atrophicus. Dermatol Pract Concept. 2021;11(4):e2021072. DOI: https://doi.org/10.5826/dpc.1104a72

Accepted: January 29, 2021; Published: October, 2021

Copyright: ©2021 Vaccari et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License 
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: Francesca Pepe, MD, Dermatology, Department of Experimental, Diagnostic and Specialty Medicine, University of 
Bologna. Email: francesca1pepe@gmail.com

Introduction

Lichen Sclerosus et Atrophicus (LSA) is a chronic inflam-

matory, invalidating disorder mainly involving the anogen-

ital skin. Extragenital lesions are uncommon, occurring as 

multiple, oval, porcelain-white macules, or papules. When 

present, they are mostly observed on the superior trunk, 

axillae, buttocks, and extremities, following Blaschko lines 

[1]. In the literature, there is a high prevalence of coexistence 

of LSA and morphea [2].

Case Presentation

We report the case of a 70-year-old woman who presented 

bilateral symmetrical itchy lesions affecting the inner thighs, 

appearing as wrinkled, porcelain-white color plaques with 

cigarette-paper-like texture and measuring 4 cm × 4 cm 

(Figure 1A). The lesions had been present for about 1 year. 

Moreover, an involvement of the vulvar mucosa, with loss of 

clitoris and fusion between the labia majora and minora, was 

observed, as it happens in genital lichen sclerosus et atrophi-

cus (LSA) (Figure 2A). 

Dermoscopy of cutaneous lesions revealed widespread 

whitish patches, comedo-like openings, and peppering pat-

terns (Figure 1B). On dermoscopy, the vulvar lesions showed 

patchy structureless milky-pinkish areas with scales and red 

purpuric blotches.

The differential diagnosis of the skin patches included 

morphea and extragenital LSA, due to their clinical morphol-

ogy and distribution and to the association with anogenital 

lesions [3]. A punch biopsy of cutaneous lesions showed a 

thick hyperkeratotic scale and an atrophic epidermis with 

flattening of the rete ridges. The basal layer cells showed 

hydropic degeneration. Beneath the epidermis there was a 

broad area of pronounced lymphedema. Within this zone, 

the collagenous fibers were swollen and homogenous and 

contained only a few nuclei. Dermal melanophages, rare lym-

phocytes, and plasma cells were observed (Figure 1C). Based 



2 Letter | Dermatol Pract Concept. 2021;11(4):e2021072

Figure 1.(A) Extragenital lichen sclerosus et atrophicus with porcelain-white color plaques. (B) Dermoscopic patterns: abundant peppering 

over white-yellowish structureless areas (magnification x 20). (C) Histological examination: thick hyperkerator scale an atrophic epidermis 

and a pale superficial dermal stroma with rare lymphocytes and plasma cells; melanophages displaced in upper dermis.

Figure 2. (A) Genital lichen sclerosus et atrophicus with patches of sclerosis and purpuric 

lesions. (B) Dermoscopic patterns: red purpuric blotches corresponding to ecchymosis in an 

intensely white background .



Letter | Dermatol Pract Concept. 2021;11(4):e2021072 3

on the clinical and histological findings, a final diagnosis of 

extragenital LSA was made. A vulvar biopsy was also per-

formed, confirming the diagnosis of genital LSA (Figure 2B).

Conclusions

Extragenital LSA affects about 15-20% of patients with 

genital LSA. It is generally asymptomatic and usually affects 

neck, shoulders, and upper trunk, presenting as flat, white 

papules or slight atrophic white plaques. The diagnosis is 

mainly clinical and is confirmed by histopathology.

Clinically, our patient showed a specific skin lesions that 

made us hypothesize an inflammatory disease, such as morphea 

or extragenital LSA, but due to symmetrical distribution and the 

peculiar size, the lesions were not pathognomonic for any disease.

The uncertainty in diagnosing extragenital LSA was due 

to the uncommon sites of the lesions, very close to anogenital 

LSA, rather than on the trunk or sites of pressure. On the 

contrary, dermoscopy added features suggesting the hypothesis 

of extragenital LSA, showing well-demarcated, whitish struc-

tureless areas, and comedo-like openings [4]. Dermoscopy also 

allowed the differential diagnosis with morphea since the latter 

usually shows fibrotic beams and linear branching vessels on 

dermoscopy (5). A peculiar dermoscopic feature in our case 

was the peppered arrangement, which is usually observed only 

in vulvar LSA [6]: it is thought to be related to melanophages 

displaced in both the upper dermis and perifollicular site. Thus, 

peppering may represent an additional dermoscopic clue in the 

differential diagnosis between morphea and extragenital LSA.

Further cases of extragenital LSA need to be reported and 

collected, to deepen the understanding of this rare disease’s 

dermoscopic features, confirming the presence of peppering 

as clue for differential diagnosis. 

References

1.  Lacarrubba F, Pellacani G, Verzì AE et al. Extragenital lichen 

sclerosus: Clinical, dermoscopic, confocal microscopy and 

histologic correlations. J Am Acad Dermatol. 2015;72(1) 

Supplement 1: 50-52. DOI:10.1016/j.jaad.2014.07.008.  

PMID: 25500042.

2. Kreuter A, Wischnewski J, Terras S et al. Coexistence of lichen 

sclerosus and morphea: A retrospective analysis of 472 patients 

with localized scleroderma from a German tertiary referral center. 

J Am Acad Dermatol. 2012;67(6):1157-1162. DOI: 10.1016/j.

jaad.2012.04.003. PMID: 22533994.

3. Shim WH, Jwa SW, Song M et al. Diagnostic usefulness of derma-

toscopy in differentiating lichen sclerous et atrophicus from mor-

phea. J Am Acad Dermatol. 2012;66(4):690-1. DOI: 10.1016/j.

jaad.2011.06.042. PMID: 22421117.

4. Garrido-Ríos AA, Álvarez-Garrido H, Sanz-Muñoz C et al.  A. 

Dermoscopy of extragenital lichen sclerosus. Arch Dermatol. 

2009;145(12):1468. DOI: 10.1001/archdermatol.2009.261. 

PMID:20026867.

5. Errichetti E, Stinco G. Dermoscopy in General Dermatolo-

gy: A Practical Overview. Dermatol Ther. 2016;6(4):471-507. 

DOI: 10.1007/s13555-016-0141-6. PMID: 27613297. PMCID: 

PMC5120630.

6. Borghi A, Corazza M, Minghetti S et al. Dermoscopic features 

of vulvar lichen sclerosus in the setting of a prospective cohort 

of patients: New observations. Dermatol. 2016;232:71-77. DOI: 

10.1159/000439198. PMID: 26574744.