Fig 2. Mid-dermal elastolysis. A, Detail of multinucleatedgiant cells in the mid dermis with occasional elastopha-gocytosis. B, Bandlike loss of elastic fibers in the middermis. (A, Hematoxylin-eosin stain; original magnifica-tion:3200.) (B, Orcein stain; original magnification:340.)
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the cause of the skin disease. In our patient,skin manifestations of mid-dermal elastolysisappeared at the same time of the immune restorationafter HAART, thus suggesting that they may beetiologically related. Indeed, our case fulfillsdiagnostic criteria of IRIS, namely: temporalassociation between initiation of HAART anddevelopment of symptoms, evidence of immunerestoration with virologic and immunologicresponse, and clinical symptoms consistent with aninflammatory process.1 The subsequent occurrenceof Crohn’s disease may support the intriguinghypothesis of a multiorgan autoimmune IRISphenomenon. In fact, IRIS-induced tissue inflamma-tion may result in an increase in local inflammatorycytokines and recognition of viral and/orself-antigens by the infiltrating T cells.4 Theinflammatory environment induced by IRIS and theloss of immune self-tolerance to tissue-associatedantigens may lead to increased susceptibility todevelop local and/or systemic pathologicalautoimmune conditions.5 This mechanism couldsupport the hypothesis that mid-dermal elastolysisis an autoimmune process against elastic fibers.
Carlo Cota, MD,a Alessandra Latini, MD,b VivianaLora, MD,b and Lorenzo Cerroni, MDc
Open access under CC BY-NC-ND license.
Dermatopathology Unita and Division ofDermatology,b San Gallicano DermatologicalInstitute, Rome, Italy; and DermatopathologyResearch Unit, Department of Dermatology,Medical University of Graz, Austriac
Funding sources: None.
Conflicts of interest: None declared.
Correspondence to: Carlo Cota, MD, Dermatopa-thology Unit, San Gallicano DermatologicalInstitute, Via Elio Chianesi 53, 00144 Rome,Italy
E-mail: [email protected]
REFERENCES
1. Lehloenya R, Meintjes G. Dermatologic manifestations of the
immune reconstitution inflammatory syndrome. Dermatol Clin
2006;24:549-70.
2. Gambichler T. Mid-dermal elastolysis revisited. Arch Dermatol
Res 2010;302:85-93.
3. Bannister MJ, Rubel DM, Kossard S. Mid-dermal elastophago-
cytosis presenting as a persistent reticulated erythema. Austral
J Dermatol 2001;42:50-4.
4. McLeod DSA, Woods ML, Kandiah DA. Immune reconstitution
inflammatory syndrome manifesting as development of
multiple autoimmune disorders and skin cancer progression.
Intern Med J 2011;41:699-703.
5. Krupica T Jr, Fry TJ, Mackall CL. Autoimmunity during
lymphopenia: a two-hit model. Clin Immunol 2006;120:121-8.
http://dx.doi.org/10.1016/j.jaad.2014.04.051
Clinicopathologic lessons in distinguishingcicatricial alopecia: 7 Cases of lichenplanopilaris misdiagnosed as discoid lupus
To the Editor: Cicatricial alopecia represents acomplex set of scarring hair loss conditions. Theypresent challenges in initial diagnostic evaluation,histopathological analysis, and long-term manage-ment.1,2 Clinically, the hallmark of cicatricialalopecia is obliteration of follicular ostia, reflectingfollicular destruction, secondary to inflammation.The common cicatricial alopecias marked bylymphocytic inflammation include discoid lupuserythematosus (DLE), lichen planopilaris (LPP),frontal fibrosing alopecia (a clinical variant of LPP),and central centrifugal cicatricial alopecia. Becauseboth clinical and histologic features of lymphocyticscarring alopecia overlap, distinguishing betweenthem can be difficult, especially the distinctionbetween DLE and LPP.3,4
Seven patients with scarring alopecia werereferred by dermatologists to 1 of 2 specialty clinicsin Boston ( focused on connective tissue diseases oralopecia). All patients were diagnosed withDLE based on clinical and histopathologic findings,
Table I. Demographic, clinical, and pathologic features of 7 cases of lichen planopilaris initially misdiagnosedas discoid lupus erythematosus
Case
Age,
y Sex
Initial clinical
impression
Initial
biopsy
diagnosis Treatments tried
History
of LP
Overall scalp
involvement Scalp findings ANA titer
CPC
diagnosis
1 61 M SA; DLE;LPP; FD
DLE TS, ILS, HCQ,MTX
None Diffuse patches;40%
PF erythema, PFscale
Negative LPP
2 66 F Tender baldpatches
DLE TS, ILS, HCQ None Several foci\25%
PF scale; PFerythema
Negative LPP
3 62 F SA; LPP; DLE DLE TS Oral LP Several foci\25%
PF erythema, PFscale
Negative LPP
4 44 M DLE; scalperythema
DLE TS, ILS, HCQ, CQ,finasteride,IVIG, MMF
None Several foci\25%
HK follicles, PFscale,PF erythema
Negative LPP
5 43 M DLE; scalperythema
DLE TS, ILS, HCQ None Diffuse patches\25%
Atrophy, PF scale,PF erythema
Negative LPP
6 47 M DLE; LPP; SA DLE TS, ILS, HCQ None Several foci\25%
Atrophy, PF scale,doll hairs;PF erythema
1:160,speckled
LPP
7 77 F FPHL DLE TS, ILS, MTX None Frontal hairline;crown
PF erythema, lossof eyebrows
1:640 FFA (LPPvariant)
ANA, Antinuclear antibody; CPC, clinicopathologic correlation; CQ, chloroquine; DLE, discoid lupus erythematosus; F, female; FD, folliculitis
decalvans; FFA, frontal fibrosing alopecia; FPHL, female pattern hair loss; HCQ, hydroxychloroquine; HK, hyperkeratotic; ILS, intralesional
steroids; IVIG, intravenous immune globulin; LP, lichen planus; LPP, lichen planopilaris; M, male; MMF, mycophenolate mofetil;
MTX, methotrexate; PF, perifollicular; SA, scarring alopecia; TS, topical steroids.
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OCTOBER 2014e136 Letters
however, upon our evaluation, these cases of cica-tricial alopecia were most consistent with LPP. Thisstudy was approved by the institutional reviewboards of Partners Healthcare and Boston MedicalCenter, both located in Boston, Massachusetts.
Patient demographics, clinical examination, andbiopsy findings are summarized in Table I.Representative clinical and pathologic images arepresented in Fig 1. Five individuals exhibited similarhair loss patterns with numerous small alopecicfoci throughout the scalp with less than 25%involvement. Five of the 7 individuals also exhibitedperifollicular scaling. All exhibited some perifollicu-lar erythema. None exhibited follicular plugging orcentral depigmentation.
Upon histopathology review, all patients wereconsidered to have LPP given the presence ofprimarily perifollicular inflammation and clinico-pathologic correlation. Features that can lead tomisdiagnosis because of their occurrence inboth conditions include interface dermatitis, peri-vascular inflammation, increased mucin, and focalbasement membrane zone thickening. Althoughinterface dermatitis is a hallmark of DLE, interfollic-ular involvement in LPP occurs uncommonly.Any accompanying thickening of the basementmembrane zone in LPP is mild, unlike the strikingthickening seen in DLE. In LPP, the inflammation ispredominantly perifollicular, unlike in DLE. The
increased dermal mucin in LPP is admixed withfibrosis in a perifollicular location (Fig 1, F ), unlikethe mucin in DLE that is interstitial in the dermis.
Our experience reinforces that LPP and DLE havedistinct, yet overlapping, clinical and histologic fea-tures and present diagnostic challenges for dermato-logists.4 The presence of perifollicular erythemaand scale, combined with a lack of follicular pluggingor central depigmentation, favors LPP over DLE.Histopathologically, perivascular inflammation shouldnot favorDLEunless it is deepanddense. Similarly, thepresence of mucin does not favor DLE when it is in aperifollicular location. Interface dermatitis can be seenin LPP, and DLE should not be favored when it ispresent, especially if the inflammation is primarilyperifollicular. Finally, the basement membranethickening in DLE is prominent, and mild thickeningshould not favor DLE. Identification of factors leadingto misdiagnosiseincluding the rarity of these condi-tions, the limited clinical experience with scarringalopecia, and the frequently subtle and overlappinghistologic features of these entitieseis the first step inmaking the correct diagnosis. Clinicians must recog-nize that clinicopathologic correlation, albeit chal-lenging, is essential in distinguishing these diseases.
Vinod E. Nambudiri, MD, MBA,a,b Ruth AnnVleugels, MD, MPH,a Alvaro C. Laga, MD,c andLynne J. Goldberg, MDd
Fig 1. Clinical and histopathologic findings of lichen planopilaris (LPP) initially misdiagnosedas discoid lupus erythematosus. Clinical findings of LPP may include numerous small foci ofalopecia throughout the scalp (A and B). A closer view demonstrates loss of follicular ostia(C) and perifollicular erythema and scaling (D). On biopsy specimen, prominent perifollicularlymphoid infiltrates are evident at the level of the isthmus (E). Additional findings includeincreased perifollicular mucin (F), a lichenoid perifollicular lymphocytic infiltrate (G), andsquamatization of the basal cell layer with mild perivascular inflammation and melanophages(H). (E, G, and H, Hematoxylin-eosin stain; original magnifications: E, 340; G, 3100;H, 3200.) (F, Colloidal iron stain; original magnification: 3100.)
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VOLUME 71, NUMBER 4Letters e137
Departments of Dermatology,a Medicine,b andPathology,c Brigham and Women’s Hospital,Boston; and Department of Dermatology, BostonUniversity School of Medicine,d Massachusetts
Supported by a Mentorship Award from theNorth American Hair Research Society toDr Nambudiri.
Conflicts of interest: None declared.
J AM ACAD DERMATOL
OCTOBER 2014e138 Letters
Correspondence to: Vinod E. Nambudiri, MD,MBA, Brigham and Women’s Hospital,75 Francis St, Boston, MA 02115
E-mail: [email protected]
REFERENCES
1. Nayar M, Shomberg K, Dawber RPR, Millard PR. A
clinicopathologic study of scarring alopecia. Br J Dermatol
1993;128:533-6.
2. Fabbri P, Amato L, Chiarini C,Moretti S, Massi D. Scarring alopecia
in discoid lupus erythematosus: a clinical, histopathologic and
immunopathologic study. Lupus 2004;13:455-62.
3. Inal€oz HS, Chowdhury MM, Motley RJ. Lupus erythematosus/
lichen planus overlap syndrome with scarring alopecia. J Eur
Acad Dermatol Venereol 2001;15:171-4.
4. Annessi G, Lombardo G, Gobello T, Puddu P. A clinicopatho-
logic study of scarring alopecia due to lichen planus:
comparison with scarring alopecia in discoid lupus erythema-
tosus and pseudopelade. Am J Dermatopathol 1999;21:324-31.
http://dx.doi.org/10.1016/j.jaad.2014.04.052
Importance of keen observation for thediagnosis of epidermal cysts: Dermoscopy canbe a useful adjuvant tool
To the Editor: Although epidermal cysts are themost common cutaneous cysts and frequentlyencountered in daily dermatologic practice,differential diagnosis of them is very broad and isoften a diagnostic challenge.1 A characteristic ofepidermal cysts is a clinically visible punctumrepresenting the orifice of an obstructed hair follicle
Fig 1. A, Dermal or subcutaneous tumor measur34-year-old man. His hair over the tumor wasC, Dermoscopic examination result of the subcutD, The cyst wall is lined by stratified epitheliulaminated keratins. The punctum is connected topunctum is approximately 0.5 mm. (D, Hematoxy
from which the cyst originates. In case punctum isnot visible, diagnosing epidermal cysts can bedifficult. Here, we report 3 cases in whichdermoscopy helped in diagnosing epidermal cystsby enabling visualization of small puncta that werehardly detected under naked-eye examination.
A 34-year-old man presented with a soft mass onthe occipital scalp. He first noticed the mass 6 yearsearlier, and it slowly enlarged. He reported nosymptom, smell, or discharge from the mass. Ourclinical differential diagnosis included lipoma, orepidermal, dermoid, pilar, or other cysts ortumors. On clinical examination, the overlying skinappeared normal (Fig 1, A and B). However,dermoscopic examination showed a punctum barelyvisible to the naked eye (Fig 1, C ). Subsequently,we successfully enucleated the cyst via a smallelliptical incision. The histology of the cyst is shownin Fig 1, D.
A 2-year-old girl presented with an asymp-tomatic nodule on the concha of her right ear(Fig 2, A and C ). The movable and nontendernodule was noticed 2 months previously. Theinitial clinical differential diagnosis includedlipoma, neurofibroma, or epidermal or dermoidcysts. Subsequent dermoscopic examination of thenodule showed a punctum hardly visible undernaked-eye examination (Fig 2, E ). The histopatho-logy of the excised cyst was consistent with that ofan epidermal cyst.
ing 2.5 3 2.5 cm in the occipital region of ashaved before surgery. B, Close-up view.aneous tumor showing a punctum (arrow).m with a granular layer and is filled withthe surface epithelium, and the size of thelin-eosin stain; original magnification: 340.)
