ISSN: 0019-5154

®IJD Symposium: Integrative Dermatology

Guest Editor: S R Narahari

Highlights of the issue

• Update on cutaneous calciphylaxis

Macrophage migration inhibitory factor in

Dermatology

Fixed duration therapy in leprosy

Environmental dermatoses in Ladakh

Demodex folliculorum as a risk factor in

Diagnosing rosacea

Annular lesions in Dermatology

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Indian Journal of

DermatologyVolume 58 Issue 2 March-April 2013

Clinical and photomicrograph of Mycosis fungoides, PET-CT for staging and response assessment

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IntroductionAngiolymphoid hyperplasia with eosinophilia (ALHE) isa rare, benign disease with distinctive histopathologicalfeatures. Follicular mucinosis is not a distinct clinicalentity,but it isahistopathological reactionpattern thathasbeen described with many conditions apart from alopeciamucinosa. Concurrent occurrence of follicular mucinosisandALHEisrare.[1,2]

In this case, we describe a 49-year-old Indian male withboth these histopathological findings present in the samebiopsyspecimenleadingtodiagnosticdilemma.

Case ReportA49-year-oldmarriedIndianmalepresentedwithmultiple,pruritic hyper-pigmented papular lesions of 0.5-1 cm insize[Figure1]overthevertexandrightparietalscalpforadurationof1year.Therewasnohistoryofprecedingtraumaorsurgeryatthesite.Thelesionsarosespontaneouslywithgradual increase in size andnumber.Therewasnohistoryof bleeding, but the patient complained of occasionalpruritusinthelesions.Therewasnootherrelevantmedicalhistory.Peripheralbloodeosinophiliawasnotpresent.

A clinical differential diagnosis of prurigo nodularisand angiolymphoid hyperplasia with eosinophilia wasconsidered.

Histopathological examination from a papule revealednormal epidermis with large, thick-walled vessels in theupper and mid dermis lined by plump endothelial cellsprotruding into the lumen.Therewas adense lymphocyticinfiltrate along with many eosinophils present in the

perivascular area and other parts of the dermis. Thesefeatures were consistent with angiolymphoid hyperplasia.Inadditiontothese,therewereseveralhyperplasticfolliclesshowing plenty of bluish-gray stringy mucin collected inlarge pools within the follicular epithelium. [Figures 2and 3]Alcian blue stain at pH 2.5 confirmed presence ofmucin within dilated follicular infundibula, interstitium,andperivascularly.[Figure4]Basedonclinico-pathologicalcorrelation, afinal diagnosis of angiolymphoidhyperplasiawith eosinophilia with follicular mucinosis was made.Patient was advised intralesional bleomycin therapy;however,helosttofollow-up.

DiscussionALHEwasfirstdescribedin1969byWellsandWhimster.[2]Itisawell-establishedclinico-pathologicalentitypresumedto arise due to the development of arterio-venous (A-V)shuntsintheskin.Clinically,itpresentswitherythematous,pruritic papules and nodules of varying sizes, affectingmost commonly scalp, ears, face, and the neck regions.Histopathologically, diagnostic feature is the thick-orthin-walled vascular structures lined by protruding“hobnail”endothelialcellsandanaccompanying lymphoidinfiltrate with numerous eosinophils. The density of boththelymphoidcellsandeosinophilsishighlyvariable.[1]

Follicular mucinosis presents with solitary or groupederythematous papules, nodules, or plaques that may beindurated. Histopathologically, there is varying amountsof mucin accumulates within the follicular infundibulaand the outer root sheath epithelium, accompanied byperifollicular lympho-histiocytic infiltrate thatmay contain

Address for correspondence: Dr. Rameshwar Gutte, Department of Dermatology, Seth GS Medical College and KEM Hospital, Parel, Mumbai ‑ 400 012, India. E‑mail: drrameshwargutte@yahoo.com

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DOI: 10.4103/0019‑5154.108081

Angiolymphoid Hyperplasia with Eosinophilia with Follicular MucinosisRameshwar Gutte, Bhavana Doshi, Uday KhopkarFrom the Department of Dermatology, Seth G. S. Medical College and King Edward Memorial Hospital, Mumbai, India

AbstractFollicular mucinosis occurring along with angiolymphoid hyperplasia with eosinophils (ALHE) has been described in a 49-year-oldmale. The patient presented with pruritic hyperpigmented papules and nodules on the vertex and right parietal scalp. There was noany other complaint. Histopathological examination from one of the papule showed prominent blood vessels in the dermis lined byplump histiocytoid endothelial cells that were surrounded by a dense lymphoid infiltrate with numerous eosinophils; these findingsare typical of angiolymphoid hyperplasiawith eosinophilia. Features of follicularmucinosiswere observed in the same sectionwith 3hyperplasticfollicularinfundibulacontainingpoolsofmucinintheinfundibularepithelium.Theconcurrentoccurrenceofthese2distincthistopathologicalpatternsinthesamebiopsyspecimenhasbeenreportedrarely.

Key Words: Angiolymphoid hyperplasia, eosinophilia, follicular mucinosis, scalp

E‑Case Report

What was known?Follicular mucinosis has been described with many other conditions apart from alopecia mucinosa.

Gutte, et al.: Angiolymphoid hyperplasia with eosinophlia with follicular mucinosis

eosinophils and plasma cells. It is classified into 2 types,primary (idiopathic) and secondary variety. Primary formusuallyaffectschildrenandyoungadultsandhasashorterduration with a benign course, while secondary form ismorewidespread and almost always a disease of adults. Ithas been associated with numerous benign and malignantconditions including lymphomas, of which majority aremycosis fungoides. Other associations include Hodgkin’sdisease, cutaneous B-cell lymphoma, syringolymphoidhyperplasia with cutaneous T-cell lymphoma, andinflammatory conditions like chronic discoid lupuserythematosus,alopeciaareata,angiolymphoidhyperplasia,eosinophilicpustularfolliculitis,spongiticdermatitis,lichenstriatus, leprosy, sarcoidosis, andgrowths suchasverrucaeandmelanocyticnevi.[1,3]

The concurrent occurrence of follicular mucinosis withangiolymphoid hyperplasia is unusual; to date, only fewsuch cases have been described in the literature. Wolff,et al. reported a case of angiolymphoid hyperplasia withfollicularmucinosisin1978.[4]

BovetandDelacretazreportedsimilarcasein1979.[5]JoshiR. reported a similar case in 2007, probably a first caseof this rare association from India.[1] We report a secondsimilarcasefromIndia.

Due to rare occurrence of this association of ALHE andfollicular mucinosis, it is not clear whether such casesshould be treated differently. It is important that bothcliniciansandpathologistareawareof thisrareassociationtoavoidmisdiagnosis.

In conclusion, we report a case of rare association ofALHEwith follicularmucinosis, which shows importanceofclinico-pathologicalcorrelation.

What is new?This case highlights a rare occurrence of follicular mucinosis with angiolymphoid hyperplasia.

References1. Joshi R. Angiolymphoid hyperplasia with follicular mucinosis.

IndianJDermatolVenereolLeprol2007;73:346-7.2. Kumar JV, Guruprasad KY. Angiolymphoid hyperplasia

with eosinophilia. Indian J Dermatol Venereol Leprol1998;64:133-4.

3. Loffreda MD. Inflammatory diseases of hair follicles, sweatglandsandcartilage.In:ElderDE,editor.Lever’sHistopathology

Figure 1: Multiple hyper‑pigmented papules over scalp Figure 2: Diffuse infiltrate in dermis with vascular proliferation and dilated follicles with bluish mucin are seen. (H and E, ×40)

Figure 3: Close‑up view showing thick walled dilated vessel with plump hobnail endothelial cells and numerous eosinophils in infiltrate, also a dilated follicle with mucin is shown. (H and E, ×100)

Figure 4: Alcian blue stain showing bluish mucin deposition within dilated follicular infidibulum, interstitium and peri‑vascularly (Alcian blue, ×100)

Gutte, et al.: Angiolymphoid hyperplasia with eosinophlia with follicular mucinosis

of the skin.10th ed.NewDelhi:WolterKluwer (India) Pvt. Ltd;2009.p.459-502.

4. WolffHH,KinneyJ,AckermanAB.Angiolymphoidhyperplasiawithfollicularmucinosis.ArchDermatol1978;114:229-32.

5. BovetR,DelacretazJ.Angiolymphoidhyperplasiawithfollicularmucinosis.Dermatologic1979;158:343-7

How to cite this article: Gutte R, Doshi B, Khopkar U. Angiolymphoid hyperplasia with eosinophilia with follicular mucinosis. Indian J Dermatol 2013;58:159.

Received: July, 2011. Accepted: August, 2011.Source of support: Nil, Conflict of Interest: Nil.