Umbilicated Lesions in Dermatology : Clinical Dermatology Review

Secondary Logo

Journal Logo

Advanced Search
Resident Page

Umbilicated Lesions in Dermatology

Bubna, Aditya Kumar

Author Information

Department of Dermatology, IQ City Medical College, Durgapur, West Bengal, India

Address for correspondence: Dr. Aditya Kumar Bubna, Department of Dermatology, IQ City Medical College, Sovapur, Bijra Road, Jemua, Durgapur - 713 206, West Bengal, India. E-mail: [email protected]

This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.

Clinical Dermatology Review 3(1):p 99-103, Jan–Jun 2019. | DOI: 10.4103/CDR.CDR_52_18
  • Open

Introduction

The word “umbilicated” is derived from the Latin word “umbilicatus” which means depressed in the middle like a naval. In dermatology, there are a variety of conditions which may present in this manner. They range from infections at one end of the spectrum to tumors on the other end. This review will orient to the discussion of these lesions.

Classification

  1. Infections
    • Viral
    • Molluscum contagiosum (MC)
      • Cowpox
      • Orf
      • Milker's nodule (MN)
      • Eczema herpeticum.
    • Fungal
      • Disseminated cryptococcosis in HIV
      • Histoplasmosis
      • Penicillinosis.
    • Bacterial
      • Histoid leprosy
      • Methicillin-resistant Staphylococcus aureus (MRSA) septicemia.
  2. Noninfective granulomas
    • Granuloma annulare (GA)
    • Sarcoidosis.
  3. Perforating disorders
    • Reactive perforating collagenosis (RPC)
    • Perforating lichen nitidus (LN)
    • Perforating folliculitis.
  4. Soft-tissue tumors
    • Keratoacanthoma
    • Pilar sheath acanthoma (PSA)
    • Trichofolliculoma
    • Desmoplastic trichoepithelioma (DT)
    • Sebaceous gland hyperplasia (SGH) and sebaceous adenoma.
  5. Malignancies
    • Leukemia cutis (LC).
  6. Others
    • Acrokeratoelastoidosis of Costa
    • Vesicular pityriasis rosea (PR).

Viral infections

Molluscum contagiosum

This is a common cutaneous viral infection observed mainly in children. The hallmark feature of MC lesions are discrete, dome-shaped waxy papules with central umbilication [Figure 1].[1] The average size of these papules is 2–5 mm.[2] In immune-competent individuals, these lesions tend to involute, often with mild inflammation and tenderness. However, in the immune-compromised, an atypical and florid manifestation of the disease is seen.

F1-19
Figure 1:
Umbilicated lesions in molluscum contagiosum

Cowpox

Cowpox is a viral infection caused by orthopox virus, usually acquired from domestic cats and occasionally from rodents.[34] Clinically, it is characterized by a painful papule which evolves rapidly to form a vesicle and then a pustule which may become umbilicated. Ulceration occurs in the 2nd week with crusting and eschar formation. After 1–2 months, healing occurs.[5]

Orf

Orf is a cutaneous viral infection caused by orf virus. Following a 3–6 days incubation period, a firm papule forms which progresses to form a hemorrhagic pustule or bulla with an umbilicated center and an overlying crust.[67]

Milker's nodule

MN is a paravaccinia virus-associated cutaneous infection. Clinically, it is characterized by red papules which are flat initially, but over time progresses to form slightly tender nodules with a depressed center. Overlying the depression is a crust.[8]

Eczema herpeticum

Eczema herpeticum or Kaposi's varicelliform eruption (KVE) is a distinct clinical condition caused by herpes simplex virus, coxsackievirus A16, and vaccinia virus. It is characterized clinically by clusters of umbilicated vesiculopustules, seen in areas affected by the preexisting dermatosis. Some of the dermatological conditions which herald KVE include atopic dermatitis, mycosis fungoides, pityriasis rubra pilaris, pemphigus foliaceus, Hailey–Hailey disease, and Darier's disease.[9] In KVE, there is a rapid dissemination of viral infection. The vesicles and pustules progress to form punched-out erosions with hemorrhagic crusting which has a disseminated pattern. Constitutional features such as myalgia, fever, malaise, and lymphadenopathy are commonly encountered. Complications such as superinfection with S. aureus and Streptococcus pyogenes as well as herpetic keratoconjunctivitis and meningoencephalitis have been reported.[10]

Fungal infections

Disseminated cryptococcosis in HIV

Cryptococcosis is a potentially fatal fungal disease caused by variants of Cryptococcus neoformans species.[11] Disseminated infection can have cutaneous manifestations. Cutaneous lesions occur in 10%–15% of patients with disseminated infection.[12] Cutaneous lesions present as MC like lesions characterized by typical pedunculated dome-shaped papules with an umbilicated center [Figure 2].[13]

F2-19
Figure 2:
Umbilicated lesions in disseminated cryptococcosis

Histoplasmosis

Histoplasmosis is an opportunistic fungal infection caused by inhalation of the dimorphic fungus Histoplasma capsulatum.[14] It occurs mainly in immunocompromised individuals, more so in HIV-infected persons and usually with CD4 counts <75 cells/mcl. Clinical manifestations of histoplasmosis are of three main types, namely, pulmonary, progressive disseminated, and chronic cavitary forms.[15] Cutaneous lesions may occur in all three forms of histoplasmosis or rarely as primary cutaneous histoplasmosis. Cutaneous lesions occur in up to 17% of patients with disseminated histoplasmosis and can manifest as papules, pustules, ulcers, and molluscoid centrally umbilicated papular lesions.[161718]

Penicillinosis

Penicillinosis is an AIDS-defining disease caused by the fungus Penicillium marneffei. It is a fatal opportunistic infection in AIDS patients and is the third most common infection only after tuberculosis and cryptococcosis. The typical cutaneous lesions are umbilicated papules with a central necrotic core.[19] Other morphologies include ecthyma-like lesions, folliculitis, subcutaneous nodules, and morbilliform eruptions.[20]

Bacterial infections

Histoid leprosy

Histoid leprosy is a type of lepromatous leprosy characterized by small nodules. Molluscoid skin lesions have been described here with the characteristic central umbilication.[21]

Methicillin-resistant Staphylococcus aureus septicemia

Septicemia secondary to MRSA can present as a diffuse cutaneous eruption. However, vesiculation with central umbilication is a rarity. It has been described in a critically ill child with MRSA septicemia.[22]

Noninfective granulomas

Granuloma annulare

GA is a necrobiotic disorder with many clinical variants. Some of them include the classical annular form, disseminated GA, actinic GA, subcutaneous GA, papular type, and the perforating variant.[23] One specific pattern of GA presenting as umbilicated papules has been described by Chaudhary et al.,[24] Lucky et al.,[25] and Cho et al.[26]

Sarcoidosis

Sarcoidosis is a chronic granulomatous disorder and may have diverse clinical presentations. One clinical variant of sarcoidosis presents as umbilicated papules.[27]

Perforating disorders

Reactive perforating collagenosis

RPC is a rare familial disorder. It manifests first during early childhood following superficial trauma and is characterized by skin colored papules over the elbows, knees, and dorsa of hands which gradually enlarge and then umbilicates overlying which is a keratinous plug.[28]

Perforating lichen nitidus

LN is a chronic inflammatory condition affecting the skin, characterized by flesh colored papules. The perforating type of LN is one of the clinical ways in which the disease can present. The papules here present with central umbilication.[29]

Perforating folliculitis

Perforating folliculitis is a folliculocentric perforating dermatosis. The lesions consist of keratotic papules mainly confined to hair bearing areas of the extremities with a central depressed center containing keratin and fragments of coiled hair.[303132]

Soft-tissue tumors

Keratoacanthoma

Keratoacanthoma, also known as molluscum sebaceum, is a squamoproliferative tumor of non-glabarous skin, that rarely can progress to squamous cell carcinoma.[33] Clinically, a solitary keratoacanthoma presents as a smooth, rounded papule which grows in size over a few weeks to produce a flesh-colored umbilicated nodule with a central, keratin-containing crater [Figure 3].[34]

F3-19
Figure 3:
Umbilicated nodule of keratoacanthoma with a keratin-plugged crater

Pilar sheath acanthoma

PSA is a hair follicle tumor commonly seen over the face. It consists of skin-colored papules and nodules with a central depression plugged with keratin.[35]

Trichfolliculoma

Trichfolliculoma is a rare hair follicle tumor. It is located primarily over the face and is characterized by a nodule with an umbilicated center from which a tuft of hairs protrude.[36]

Desmoplastic trichoepithelioma

DT is a variant of trichoblastoma, characterized by extensive sclerotic changes within the stroma. It presents classically as a firm skin-colored annular plaque with a central dell or depression. It is more commonly observed in women, affecting mainly the upper cheek.[37]

Sebaceous gland hyperplasia and sebaceous adenoma

SGH represents a benign enlargement of sebaceous glands. It is a commonly witnessed entity, characterized by one to multiple yellowish papules over the central and upper face, and sometimes on the upper trunk. These lesions demonstrate central umbilication which corresponds to a central follicular infundibular ostium.

Sebaceous adenoma is a benign neoplasm characterized by sebaceous glandular differentiation on histopathology. These lesions present as painless, yellow, round papules, or subcutaneous nodules, some of which demonstrate central umbilication as seen in MC.[38]

Malignancies

Leukemia cutis

LC is encountered in various forms of leukemia, predominantly in adult T-cell leukemia and pursued by acute myelogenous leukemia.[39] LC may present in myriad forms dermatologically, with skin colored to violaceous macules, papules, plaques, and nodules at one end of the spectrum to hemorrhagic bullae, purpura fulminans, lupus-like butterfly rash,[40] and Sweet's syndrome-like lesions at the other end. Umbilicated papules in LC is a rarity but has been described by Verma et al.[41]

Others

Acrokeratoelastoidosis of Costa

This disorder also referred to as marginal papular keratoderma is characterized by skin colored papules around 2–4 mm occurring circumferentially over the hands, feet, and digits. Some of the papules may be umbilicated.[42] This disorder was first reported by Costa[43] in a series of 13 cases.

Vesicular pityriasis rosea

PR is a common dermatosis with a suspected viral etiology, characterized usually by plaques which demonstrate a peripheral collarette of scales, situated mainly over the trunk in a Christmas tree pattern. However, atypical presentations in the form of umbilicated vesicles have been documented in literature, though rare.[44]

Conclusion

We see here that there are a number of cutaneous disorders which could present as umbilicated papules, nodules, or plaques. Thus, as a dermatologist it would be imperative to know how various disorders could present along with atypical presentations to arrive at a conclusive diagnosis, and thus a better therapeutic outcome.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

REFERENCES

1. Diven DG. An overview of poxviruses J Am Acad Dermatol. 2001;44:1–6
2. Elder DE, Elenitsas R, Johnson BL, Murphy GF. Diseases caused by virus Lever's Histopathology of Skin. 20059th Philadelphia Lippincott Williams and Wilkins:662
3. Wolfs TF, Wagenaar JA, Niesters HG, Osterhaus AD. Rat-to-human transmission of cowpox infection Emerg Infect Dis. 2002;8:1495–6
4. Baxby D, Bennett M, Getty B. Human cowpox 1969-93: A review based on 54 cases Br J Dermatol. 1994;131:598–607
5. Laurance B. Cowpox in man and its relationship with Milker's nodules Lancet. 1955;268:764–6
6. Gill MJ, Arlette J, Buchan KA, Barber K. Human orf. A diagnostic consideration? Arch Dermatol. 1990;126:356–8
7. Leavell UW Jr., McNamara MJ, Muelling R, Talbert WM, Rucker RC, Dalton AJ, et al Orf. Report of 19 human cases with clinical and pathological observations JAMA. 1968;204:657–64
8. Nomland R, McKee AP. Milkers' nodules; report of ten cases AMA Arch Derm Syphilol. 1952;65:663–74
9. Shenoy MM, Suchitra U. Kaposi's varicelliform eruption Indian J Dermatol Venereol Leprol. 2007;73:65
10. Wollenberg A, Wetzel S, Burgdorf WH, Haas J. Viral infections in atopic dermatitis: Pathogenic aspects and clinical management J Allergy Clin Immunol. 2003;112:667–74
11. Tabassum S, Rahman A, Herekar F, Masood S. Cryptococcal meningitis with secondary cutaneous involvement in an immunocompetent host J Infect Dev Ctries. 2013;7:680–5
12. Blauvelt AFreedberg IM, Eisen AZ, Wolff K, Austen KF, Goldsmith LA, Katz SI. Mucocutaneous manifestations of the non HIV infected immune-suppressed host Fitzpatrick's Dermatology in General Medicine. 2003;Vol 16th New York McGraw Hill:1154–64
13. Lacaz Cda S, Heins-Vaccari EM, Hernandez-Arriagada GL, Martins EL, Prearo CA, Corim SM, et al Primary cutaneous cryptococcosis due to Cryptococcus neoformans var. Gattii serotype B, in an immunocompetent patient Rev Inst Med Trop Sao Paulo. 2002;44:225–8
14. Vasudevan B, Ashish B, Amitabh S, Mohanthy AP. Primary cutaneous histoplasmosis in a HIV-positive individual J Glob Infect Dis. 2010;2:112–5
15. Cirillo-Hyland VA, Gross P. Disseminated histoplasmosis in a patient with acquired immunodeficiency syndrome Cutis. 1995;55:161–4
16. Sayal SK, Prasad PS, Mehta A, Sanghi S. Disseminated histoplasmosis: Cutaneous presentation Indian J Dermatol Venerol Leprol. 2003;69:90–1
17. Wheat LJ, Connolly-Stringfield PA, Baker RL, Curfman MF, Eads ME, Israel KS, et al Disseminated histoplasmosis in the acquired immune deficiency syndrome: Clinical findings, diagnosis and treatment, and review of the literature Medicine (Baltimore). 1990;69:361–74
18. Nair PS, Vijayadharam M, Vincent M. Primary cutaneous histoplasmosis Indian J Dermatol Venerol Leprol. 2002;66:151–3
19. Hogan MT. Cutaneous infections associated with HIV/AIDS Dermatol Clin. 2006;24:473–95 vi
20. Varghese GM, Pise G, Michael SJ, Jacob M, George R. Disseminated Penicillium marneffei infection in a human immunodeficiency virus-infected individual J Postgrad Med. 2004;50:235–6
21. Ghorpade A. Molluscoid skin lesions in histoid leprosy with pseudo-isomorphic Koebner phenomenon Int J Dermatol. 2008;47:1278–80
22. Chang L, Cassarino D, Sosa V. Diffuse umbilicated vesicles in a critically ill child JAMA Dermatol. 2013;149:641–2
23. Burns T, Breathnach S, Cox N, Griffiths C. Metabolic and nutritional disorders Rooks Textbook of Dermatology. 2004;Vol. 37th New York Wiley Blackwell:57.1–124
24. Chaudhary A, Malkani R, Aswani V. Generalized umbilicated granuloma annulare Indian J Dermatol Venereol Leprol. 1998;64:305–6
25. Lucky AW, Prose NS, Bove K, White WL, Jorizzo JL. Papular umbilicated granuloma annulare. A report of four pediatric cases Arch Dermatol. 1992;128:1375–8
26. Cho E, Cho SH, Lee JD. Unusual presentation of papularumbilicated granuloma annulare J Dermatol. 2001;38:402–4
27. Kashyap S, Kumar M, Thami GP, Saini V. Umbilicated papular sarcoidosis Clin Exp Dermatol. 1996;21:395–6
28. Cerio R, Calnan CD, Wilson-Jones E. A clinicopathologic study of reactive perforating collagenosis: Report of 10 cases Br J Dermatol. 1987;117(Suppl 32):16–7
29. Yoon TY, Kim JW, Kim MK. Two cases of perforating lichen nitidus J Dermatol. 2006;33:278–80
30. Sehgal VN, Jain S, Thappa DM, Bhattacharya SN, Logani K. Perforating dermatoses: A review and report of four cases J Dermatol. 1993;20:329–40
31. Patterson JW. The perforating disorders J Am Acad Dermatol. 1984;10:561–81
32. Mehregan AH, Coskey RJ. Perforating folliculitis Arch Dermatol. 1968;97:394–9
33. Schwartz RA. Keratoacanthoma: A clinico-pathologic enigma Dermatol Surg. 2004;30:326–33
34. Choi YS, Park SH, Bang D. Pilar sheath acanthoma – Report of a case with review of the literature Yonsei Med J. 1989;30:392–5
35. Calonje E, Brenn T, Lazar A, Mckee PH. Tumours of the surface epithelium Mckee's Pathology of Skin with Clinical Correlation. 2012;Vol. 24th St. Louis Elsevier Saunders:1076–149
36. Weedon D. Tumours of cutaneous appendages Weedons Skin Pathology. 20103rd London Churchill Livingstone Elsevier:758
37. Brownstein MH, Shapiro L. Desmoplastic trichoepithelioma Cancer. 1977;40:2979–86
38. Bhaijee F, Brown AS. Muir-Torre syndrome Arch Pathol Lab Med. 2014;138:1685–9
39. Baer MR, Barcos M, Farrell H, Raza A, Preisler HD. Acute myelogenous leukemia with leukemia cutis. Eighteen cases seen between 1969 and 1986 Cancer. 1989;63:2192–200
40. Misri R, Khopkar U, Kharkar V, Mahajan S. Different faces of leukemia cutis: Presenting as purpura fulminans and lupus like butterfly rash Indian J Dermatol Venereol Leprol. 2010;76:710–2
41. Verma P, Sharma S, Sharma R, Yadav P. Leukemia cutis manifesting umbilicated cutaneous lesions Indian J Dermatol Venereol Leprol. 2012;78:752–4
42. Dowd PM, Harman RR, Black MM. Focal acral hyperkeratosis Br J Dermatol. 1983;109:97–103
43. Costa OG. Acrokeratoelastoidosis AMA Arch Derm Syphilol. 1954;70:228–31
44. Zehou O, Revet J, Ducret J, Jainer M. Vesicular pityriasis rosea in an adult: A case report and review of literature Internet J Dermatol. 2012;9:1–4
© 2019 Clinical Dermatology Review | Published by Wolters Kluwer – Medknow