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Familial Reactive Perforating Collagenosis in a Child: Response to Narrow-Band UVB

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  • 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 1975;14:353359.

    2. Duarte IB, Bastazini I, Barreto JA et al. Pemphigusherpetiformis in childhood. Pediatr Dermatol 2010;27:488491.

    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 1996;34:4046.

    4. Moutran R, Maatouk I, Stephan F et al. Letter:pemphigus herpetiformis of age of onset at 6 years.Dermatol Online J 2011;17:10.

    5. Montgomery JR, Chan LS. An unusual clinicalpresentation of pemphigus herpetiformis with markedresponse to dapsone. J Am Acad Dermatol 2011;65:436438.

    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: [email protected]

    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 2013

  • 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 been used

    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 (2).


    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 TokyoPress, 1974:751.

    4. Rapini RP, Herbert AA, Drucker CR. Acquired perfo-rating dermatosis. Evidence for combined transepider-mal elimination of both collagen and elastic fibers. ArchDermatol 1989;125:10741078.

    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 Dermatol1988;27:118119.

    9. Zelger B, Hintner H, Aubock J et al. Acquiredperforating dermatosis. Transepidermal elimination ofDNA material and possible role of leukocytes inpathogenesis. Arch Dermatol 1991;127:695700.

    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 (Masson-trichrome 9100).

    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 1984;160:10271042.

    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 2013