moderate to high doses of corticosteroids to controlhis disease.
Our patient gradually improved andeventually achieved complete
clinical remission withmethotrexate 15 mg weekly, which has not
previouslybeen reported. He remains in complete clinical remis-sion
22 months after methotrexate and prednisonewere discontinued.
1. Jablonska S, Chorzelski TP, Beutner EH et al. Herpeti-form
pemphigus, a variable pattern of pemphigus. Int JDermatol
2. Duarte IB, Bastazini I, Barreto JA et al.
Pemphigusherpetiformis in childhood. Pediatr Dermatol
3. Santi CG, Maruta CW, Aoki V et al. Pemphigusherpetiformis is
a rare clinical expression of nonendemicpemphigus foliaceus, fogo
selvagem, and pemphigusvulgaris. J Am Acad Dermatol
4. Moutran R, Maatouk I, Stephan F et al. Letter:pemphigus
herpetiformis of age of onset at 6 years.Dermatol Online J
5. Montgomery JR, Chan LS. An unusual clinicalpresentation of
pemphigus herpetiformis with markedresponse to dapsone. J Am Acad
Laurel A. Leithauser, M.D.Diya F. Mutasim, M.D.Department of
Dermatology, University of Cincinnati,Cincinnati, Ohio
Address correspondence to Diya F. Mutasim, M.D., Depart-ment of
Dermatology, College of Medicine, University of Cincin-nati, PO Box
670592, Cincinnati, OH 45267, USA, or e-mail:
Familial Reactive Perforating Collagenosis ina Child: Response
to Narrow-Band UVB
Abstract: A favorable response to narrow-bandultraviolet B light
treatment, a novel option, is illus-trated in familial reactive
perforating collagenosis,and its use is recommended. Its probable
mode ofaction is outlined.
Reactive perforating collagenosis (RPC) is character-ized by
umbilicated hyperkeratotic papules and nod-ules. Transepidermal
elimination of keratin andaltered dermal connective tissue are
cardinal features(1). The familial type often presents at an early
age,associated with inherited defect in collagen (1,2). The
role of narrow-band ultraviolet B light (NB-UVB) isillustrated
in a child.
An 8-year-old healthy boy with Fitzpatrick skin typeV had
progressive asymptomatic eruptions confinedto the face of 4 months
duration. Prior trauma orinsect bites were denied. His brother was
found tohave similar eruptions. Numerous skin-colored,dome-shaped,
discrete to coalescent umbilicated ker-atin-plugged nodules were
located exclusively on theforehead and temporal region (Fig. 1).
Hematoxylinand eosinstained tissue sections showed a cup-shaped
invagination filled with degenerated materialwith a narrow channel
of an altered basophiliccollagen at its base. Collagen fibers were
orientedvertically (Figs. 2 and 3).
Topical treatment failure prompted the adminis-tration of
NB-UVB, with an initial dose of 700 mJ/cm2 (minimal erythema dose
[MED] 1,000 mJ/cm2)(3). The eyes were shielded, and only the face
wasexposed. Treatment was administered three times aweek with an
increment in dose of 20% as tolerated,based on the standard
protocol for psoriasis.Improvement in the lesions was evidenced as
adecrease in induration. After 25 exposures (a totalcumulative dose
of 38,150 mJ/cm2), the lesionsregressed completely, leaving
atrophic scars andpigmentation.
Reactive perforating collagenosis, elastosis
perforansserpiginosa, perforating folliculitis, and Kyrles dis-
Figure 1. Dome-shaped, discrete to coalescent noduleswith
central umbilication containing firm keratotic plugs.
762 Pediatric Dermatology Vol. 30 No. 6 November/December
ease (4,5) are classical variants of perforating disor-ders.
Familial RPC, associated with genetic abnor-mality of collagen, may
be autosomal dominant orrecessive (5). Affliction of trauma-prone
areas andKoebners phenomenon suggest trauma as a trigger,in
addition to cold weather. Familial RPC oftenregresses spontaneously
in approximately 10 weekstime. Recurrence is frequent (2). Topical
keratolytics,tretinoin, corticosteroids, and oral drugs
includingvitamin A, methotrexate, and antibiotics have beenused.
Nevertheless, the treatment options for familialRPC are limited.
UVB, NB-UVB (6,7), and psoralenplus UVA (PUVA) (8) therapy have
successfully in acquired RPC. Improvement in pruri-tus after UV
light may precede other response (8).Neutrophils, the predominant
cells in the pathogen-esis of acquired RPC (9), release matrix
metallopro-teinases, and other serine proteases, which cross
theepithelium and may contribute to the formation oflesions by
digestion of essential extracellular matrixcomponents, elastic
fibers, anchoring fibers, andcollagen type IV (10).
The impact of NB-UVB on neutrophils may bean important mechanism
of action against familialRPC, similar to that claimed for the
acquiredform. The recurrent nature of lesions in familialRPC poses
a treatment dilemma; a safe andeffective maintenance therapy is
needed. NB-UVBmay be useful for treatment of familial RPC andmay
yield insight into RPCs pathogenesis andmechanism of action of
NB-UVB. Nonetheless,occasional photo-exacerbation of the lesions
andtheir improvement with photo-protection shouldalso be considered
1. Mehregan AH, Schwartz OD, Livingood CS. Reactiveperforating
collagenosis. Arch Dermatol 1967;96:277282.
2. Ramesh V, Sood N, Kubba A et al. Familial reactiveperforating
collagenosis: a clinical, histopathologicalstudy of 10 cases. J Eur
Acad Dermatol Venereol2007;21: 766770.
3. Fitzpatrick TB, Pathak MA, Parrish JA. Protection ofhuman
skin against the effects of the sunburn ultraviolet(290320 nm). In:
Pathak MA, Harber LC, Seiji Met al., eds. Sunlight and man-normal
and abnormalphotobiological responses. Tokyo: University of
4. Rapini RP, Herbert AA, Drucker CR. Acquired perfo-rating
dermatosis. Evidence for combined transepider-mal elimination of
both collagen and elastic fibers. ArchDermatol
5. Sehgal VN, Jain S, Thappa DM et al. Perforatingdermatoses: a
review and report of four cases. JDermatol 1993;20:329340.
6. Ohe S, Danno K, Sasaki H et al. Treatment of
acquiredperforating dermatosis with narrowband ultraviolet B. JAm
Acad Dermatol 2004;50:892894.
7. Mii S, Yotsu R, Hayashi R et al. Acquired reactiveperforating
collagenosis successfully treated with nar-row-band ultraviolet B.
Acta Derm Venereol 2009;89:530531.
8. Serrano G, Aliaga A, Lorente M. Reactive
perforatingcollagenosis responsive to PUVA. Int J
9. Zelger B, Hintner H, Aubock J et al. Acquiredperforating
dermatosis. Transepidermal elimination ofDNA material and possible
role of leukocytes inpathogenesis. Arch Dermatol
Figure 2. Section showing cup-shaped invagination withchannel at
the base and basophilic vertically orientedcollagen bundles
(hematoxylin and eosin 9100).
Figure 3. Section showing cup-shaped invagination withchannel at
the base and basophilic vertically orientedcollagen bundles
Brief Reports 763
10. Briggaman RA, Schechter NM, Fraki J et al. Degrada-tion of
the epidermaldermal junction by proteolyticenzymes from human skin
and human polymorphonu-clear leukocytes. J Exp Med
Virendra N. Sehgal, M.D.*Prashant Verma, M.D.*Sambit N.
Bhattacharya, M.D.*Sonal Sharma, M.D.*Dermato-Venereology (Skin/VD)
Center, SehgalNursing Home, Panchwati-Delhi, Department of
Dermatology and STD, and Department of Pathology,University
College of Medical Sciences, and AssociatedGuru Teg Bahadur
Hospital, Shahdara, Delhi, India
Address correspondence to Virendra N. Sehgal, M.D.,
DermatoVenerology Center, Sehgal Nursing Home, A/6 Panchwati,
Delhi110 033, India, or e-mail: [email protected]
764 Pediatric Dermatology Vol. 30 No. 6 November/December