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 Table of Contents  
Year : 2021  |  Volume : 5  |  Issue : 2  |  Page : 245-250

Cobblestone appearances in dermatology

Department of Dermatology, Subharti Medical College, Meerut, Uttar Pradesh, India

Date of Submission17-Jun-2020
Date of Decision08-Sep-2020
Date of Acceptance23-Nov-2020
Date of Web Publication26-Aug-2021

Correspondence Address:
Anup Kumar Tiwary
D 302, Jeevan Ashray Apartment, C58/08, Sector 62, Noida, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/CDR.CDR_95_20

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Dermatology is a visual medical discipline which demands watchful eyes, profound knowledge of different morphologic patterns of various dermatoses, and extensive clinical experience. A meticulous cutaneous examination always helps in generating the close differential diagnoses and incorporation of past visual experience leads to a provisional diagnosis, obviating the need of further investigations in many cases. Although there is a wide spectrum of morphologic presentations, some dermatoses may have characteristic appearances such as cobblestoning, Peau d' orange, umbilication, and Christmas-tree pattern. Identification of these lesional characteristics or patterns can efficiently avoid the misdiagnosis and unnecessary diagnostic workup. In this article, skin diseases showing cobblestone appearances on both clinical and dermoscopic examination have been compiled and outlined in brief.

Keywords: Cobblestone, dermoscopy, induration, peau d' orange

How to cite this article:
Tiwary AK, Kumar P. Cobblestone appearances in dermatology. Clin Dermatol Rev 2021;5:245-50

How to cite this URL:
Tiwary AK, Kumar P. Cobblestone appearances in dermatology. Clin Dermatol Rev [serial online] 2021 [cited 2021 Nov 28];5:245-50. Available from: https://www.cdriadvlkn.org/text.asp?2021/5/2/245/324584

  Introduction Top

It is an old perception among medical practitioners that diagnosis is difficult in dermatology. There are many factors, other than the experience of the examining dermatologist, which justify the above statement and responsible for diagnostic fallacy. They are color of the skin, age and immune status of the patient, duration of the skin lesion, prior use of any medicine, and wide morphologic spectrum of dermatologic conditions. Certain dermatosis may simulate the clinical picture of other dermatoses. On the bright side, there are few morphologic lesions or patterns which are so characteristic that, in corroboration with history and other clinical findings, diagnosis becomes easier. “Cobblestone” is one such example which is used to describe a characteristic morphologic pattern of some dermatoses. This term implies a three-dimensional visual appearance of multiple, equally sized, round, raised lesions resembling roads paved by multiple equal-sized “cobbled” stones [Figure 1]. It can be observed both clinically and dermoscopically. After searching databases of PubMed, Medline, Scopus, Embase, and Web of Science, dermatoses with cobblestone appearance (both clinical and dermoscopic) are compiled in [Table 1]. Cobblestone skin lesions are further subdivided into groups based on their lesions as shown in [Table 2]. For the ease of reading, all the conditions are arranged in alphabetical order.
Figure 1: Road paved by cobbled stones (cobblestone appearance)

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Table 1: Dermatologic conditions with cobblestone appearances on clinical and dermoscopic examination

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Table 2: Morphologic characteristics of cobblestone skin lesions

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  1. Acrokeratosis verruciformis of Hopf: The classic lesions are flat-topped, hyperkeratotic, skin-colored papules on the dorsal aspect of the hands and forearms [Figure 2]. On dermoscopy, it shows irregular, white, homogenous areas and cobblestone appearances[1]
  2. Bullous congenital ichthyosiform erythroderma (BCIE): Erythematous and bullous lesions of BCIE usually disappear in few months after birth and convert into ichthyotic, hyperkeratotic brown scaly lesions, and cobblestone-like keratosis on extensor surfaces such as the dorsal hands and feet[2]
  3. Cheilitis granulomatosa (also known as Miescher's cheilitis): It is a rare, idiopathic, chronic, persistent, painless, granulomatous swelling of the lips of uncertain etiology, though strongly related to Crohn's disease, sarcoidosis, and allergy. In this condition, labial mucosa gets thickened and folded like cobblestone with or without facial swelling[3]
  4. Congenital erosive and vesicular dermatosis: It characteristically heals with symmetrical reticulated supple scarring. These scars are slightly hyperpigmented and cobblestone-like especially on the forehead and scalp[4]
  5. Congenital melanocytic nevus (CMN): Surface irregularities such as nodular changes and mammillations are commonly seen in CMN [Figure 3]. On dermoscopy, a globular or cobblestone pattern is also observed.[5] Cobblestone pattern is a variation of the globular pattern, seen as larger, closely aggregated, and brown-gray globule-like structures (correspond to nevomelanocytic nests in the dermis)
  6. Connective tissue nevus: Collagenoma can present as multiple skin-colored papules and plaques in cobblestone pattern [Figure 4] and [Figure 5]. In a single case, the dermoscopic examination of shagreen patch also revealed this pattern[6]
  7. Cowden's syndrome: It is a multiple hamartoma syndrome which affects many organs such as the thyroid, breast, brain, eyes, skin, intestines, and genitourinary tract. Patients develop many distinct mucocutaneous manifestations ranging from skin-colored coalescing lichenoid papules around the eyes and mouth to sclerotic fibromas. Of note, lingual, gingival, and buccal mucosa develop cobblestone or warty lesions[7]
  8. Darier disease: White cobblestone papules with central depression on the buccal mucosa and palate are very characteristic in Darier disease [Figure 6]. Gingiva and tongue may also get involved. Lesions on the hard palate may simulate nicotinic stomatitis[8]
  9. Elephantiasis nostras verrucosa: It is a rare presentation of chronic lymphedema of the lower limb arising due to various factors affecting their lymphatic drainage. It often manifests as nonpitting edema and hyperkeratotic or verrucous papules and nodules with cobblestone-like appearance [Figure 7][9]
  10. Eosinophilic fasciitis (also known as Shulman's syndrome): It is a rare autoimmune pseudosclerodermic condition which starts with acute painful tender swelling in distal extremities and later becomes indurated due to dermal and subdermal fibrosis. Peau d' orange or cobblestone appearance of hyperpigmented skin with venous furrowing along veins (groove sign) are evocative findings[10]
  11. Erythropoietic protoporphyria (EPP): Blistering lesions of EPP on knuckles and fingers may heal with waxy and cobblestone-like induration[11]
  12. Glomuvenous malformation: It is caused by loss-of-function mutations in the GLMN gene, clinically characterized by multiple, painful, bluish-purple, hyperkeratotic nodules and plaques on extremities. These plaques are like cobblestone and noncompressible[12]
  13. Hystrix-like ichthyosis with deafness syndrome: Hyperkeratosis is more extensive, prominent, and cobblestone-like, often preceded by erythroderma[13]
  14. Herpes zoster: The classic grouped cutaneous vesicles of herpes zoster may also have cobblestone surface which are always inflamed and erythematous [Figure 8][14]
  15. Ichthyosis hystrix: Porcupine-like spiny papules and/or cobblestone plaques over large joints are diagnostic features of this condition. Palms and soles are spared in its nail-patella syndrome phenotype[15]
  16. Keratitis-ichthyosis-deafness syndrome: It presents as mildly erythematous, irregular cobblestone-like hyperkeratosis on extensors and face[13]
  17. Lichen myxedematosus: It is also known as papular mucinosis and clinically characterized by multiple, shiny, waxy, skin-colored, small, dome-shaped, firm papules coalescing to form plaques, sometimes giving a cobblestone appearance [Figure 9][16]
  18. Lipoid proteinosis (Urbach–Wiethe disease): Cobblestone appearance of oral mucosa due to hyaline deposits in the form of infiltrative yellowish papules is a common finding in this deposition disorder[17]
  19. Morphea profunda: Typically presents with bound-down, subcutaneous atrophic plaques give cobblestone feel[18]
  20. Multicentric reticulohistiocytosis: It is a multisystem disorder of histiocytic proliferation affecting the skin, joints, and viscera. The cutaneous lesions are characterized by round, translucent, and reddish-brown papules often coalescing into plaques giving a cobblestone appearance [Figure 10],[Figure 11], [Figure 12][19]
  21. Nephrogenic systemic fibrosis: A rare but serious condition caused by gadolinium exposure in patients with renal failure. It is characterized by generalized skin fibrosis predominantly affecting the limbs and trunk. Hyperpigmented fibrotic indurated plaques with cobblestone or woody appearance are very common[20]
  22. Nevus sebaceous of Jadassohn: Linear or round, yellowish, cobblestone-like hairless solitary plaque on the scalp is quite diagnostic [Figure 13]. It also shows a cobblestone pattern of yellowish globules on dermoscopy[21]
  23. Port-wine stain: It is seen at birth as irregular, reddish/purple macules which, with age, develop cobblestone-like hypertrophic papular/nodular lesions[22]
  24. Postvitiligo grafting: Cobblestoning is one of the common complications of punch grafting for vitiligo [Figure 14]
  25. Pseudoxanthoma elasticum: It is a rare genetic disease of ectopic calcification and fragmentation of elastic fibers, clinically manifested by small yellowish papules on wrinkled skin of flexures (commonly on side of the neck, axillae, groins, and abdomen) [Figure 15]. These papules may coalesce and look like cobblestone, Moroccan leather, or chicken skin[23]
  26. Systemic sclerosis: Cobblestone appearance of the skin and mucosa is uncommon in systemic sclerosis. On the skin, it is seen as shiny papules due to lymphatic obstruction by fibrosis.[24] The esophageal epithelium also looks like cobblestone because of pearly white plaques
  27. Tufted angioma: It often presents with a large erythematous plaque on the trunk with a cobblestone surface[25]
  28. Vernal conjunctivitis: Cobblestoning of the upper eyelid conjunctiva is a frequent finding in vernal conjunctivitis due to papillary hypertrophy[26]
  29. Verrucous epidermal nevus: Its dermoscopic features include large brown circles surrounding a hypopigmented area, fissures, comedo-like openings, milia-like cysts, brown globules and dots, papillomatous surface, and cobblestone appearance.[27]
Figure 2: Flat-topped, hyperkeratotic papules on hand in acrokeratosis verruciformis of Hopf

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Figure 3: A large congenital melanocytic nevus on the forehead

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Figure 4: Shagreen patch (collagenoma) with cobblestone surface on the lumbar area

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Figure 5: Another case of shagreen patch

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Figure 6: Darier disease showing white cobblestone papules on the palate

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Figure 7: Elephantiasis nostras verrucosa

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Figure 8: Herpes zoster with cobblestone appearance of blisters

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Figure 9: Small, waxy, lichenoid papules of lichen myxedematosus

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Figure 10: Multicentric reticulohistiocytosis: Brownish papules and plaques on the trunk and limbs

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Figure 11: Typical reddish brown cobblestone appearance on the scapular region in multicentric reticulohistiocytosis

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Figure 12: Cobblestone nodules on the buttocks in multicentric reticulohistiocytosis

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Figure 13: Nevus sebaceous (a) on the forehead (b) on the scalp

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Figure 14: Cobblestoning after minipunch grafting for vitiligo

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Figure 15: Pseudoxanthoma elasticum on the abdomen

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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 initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

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Alikhan A, Farshidi D, Shwayder T. Case of mistaken identity: Bullous congenital ichthyosiform erythroderma mistaken as epidermolysis bullosa simplex. Dermatol Online J 2009;15:3.  Back to cited text no. 2
Rana AP. Orofacial granulomatosis: A case report with review of literature. J Indian Soc Periodontol 2012;16:469-74.  Back to cited text no. 3
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Cohen BA, Esterly NB, Nelson PF. Congenital erosive and vesicular dermatosis healing with reticulated supple scarring. Arch Dermatol 1985;121:361-7.  Back to cited text no. 4
Cengiz FP, Emiroglu N, Ozkaya DB, Su O, Onsun N. Dermoscopic Features of Small, Medium, and Large-Sized Congenital Melanocytic Nevi. Ann Dermatol 2017;29:26-32.  Back to cited text no. 5
Gundalli S, Ankad BS, Ashwin PK, Kolekar R. Dermoscopy of shagreen patch: A first report. Our Dermatol Online 2015;6:331-3.  Back to cited text no. 6
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Kirchgesner T, Dallaudière B, Omoumi P, Malghem J, Vande Berg B, Lecouvet F, et al. Eosinophilic fasciitis: Typical abnormalities, variants and differential diagnosis of fasciae abnormalities using MR imaging. Diagn Interv Imaging 2015;96:341-8.  Back to cited text no. 10
Wang HC, Yousef E. Erythropoietic protoporphyria masquerading as angioedema in a 4-year-old female. Allergy Asthma Clin Immunol 2006;2:20-3.  Back to cited text no. 11
Boon LM, Mulliken JB, Enjolras O, Vikkula M. Glomuvenous malformation (glomangioma) and venous malformation: Distinct clinicopathologic and genetic entities. Arch Dermatol 2004;140:971-6.  Back to cited text no. 12
van Geel M, van Steensel MA, Küster W, Hennies HC, Happle R, Steijlen PM, et al. HID and KID syndromes are associated with the same connexin 26 mutation. Br J Dermatol 2002;146:938-42.  Back to cited text no. 13
Habif TP. Warts, herpes simplex and other viral infections. In: Habif TP, editor. Clinical dermatology: A color guide to diagnosis and therapy. 6th ed. Beijing: Elsevier; 2016. p. 448-86.  Back to cited text no. 14
Jairath V, Aggarwal P, Kaur S, Jindal N, Jain VK. Ichthyosis hystrix: An unusual presentation. Indian J Paediatr Dermatol 2016;17:239-41.  Back to cited text no. 15
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Ravi Prakash SM, Verma S, Sumalatha MN, Chattopadhyay S. Oral manifestations of lipoid proteinosis: A case report and literature review. Saudi Dent J 2013;25:91-4.  Back to cited text no. 17
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Kajihara I, Jinnin M, Makino T, Toihata Yonemitsu A, Sakai K, Masuguchi S, et al. Clinical significance of cobblestone appearance on the skin of patients with systemic sclerosis. J Eur Acad Dermatol Venereol 2012;26:123-5.  Back to cited text no. 24
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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11], [Figure 12], [Figure 13], [Figure 14], [Figure 15]

  [Table 1], [Table 2]


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