A case of pityriasis rosea of vidal accompanied by neurofibromatosis type 1:

by Ahu Yorulmaz, Ferda Artuz, Sezer Kulacoglu, Elif Sen


Antonio Chuh Ass. Prof. MD FRCP FRCPCH1, Vijay Zawar Prof. MD DNB DV&D FAAD2

1School of Public Health, The Chinese University of Hong Kong and Prince of Wales Hospital, Shatin, Hong Kong2Department of Dermatology, Godavari Foundation Medical College and Research Center, DUPMCJ, India

Submission: 17.06.2015; Acceptance: 18.06.2015

The association of pityriasis circiné et marginé and neurofibromatosis type 1


We read with pleasure the case report by Yorulmaz A et al on the occurrence of pityriasis circiné et marginé which is a variant of pityriasis rosea (PR) in a 23-year-old lady with known neurofibromatosis type 1 (NF-1) [1].

We agree to both diagnoses [24]. Both diseases are uncommon, but definitely not rare. We have no data on the prevalence of NF-1 in Turkey. In the United Kingdom, the prevalence is around 1:4560 [5], with the prevalence at birth being 1:2699 [5]. The prevalences of NF-1 for six-year-old German children, and 9-11 year-old children in Cuba, are 1:2996 [6] and 1:1141 [7] respectively. The prevalence of PR is around 1:167 [8]. This means that if a clinic follows 200 patients with NF-1, and sees them once every year, around 1.20 patient with PR would be expected to be seen.

While reporting them concomitantly in one individual, we might consider exploring the mechanisms as to whether these two diseases: (1) are merely co-incidental; (2) are being innocent bystanders (NF-1); (3) are related to the same confounder(s), and (4) have underlying immunopathogenetic connections, which could be risk factors, precipitating factors, or be genuine causal relationships.

For patient with NF-1, the immunological system is compromised to various extents [912]. The processes are not comprehensively known, although it is likely that multiple immunological pathways and cellular mechanisms reduce the antigen-processing and antigen-presenting cells in NF-1 [12].

Moreover, large groups of immune function genes in human Schwann cells are down-regulated in NF-1 [13]. Acute phase reactants such as interleukins are reported to be adversely affected in NF-1 [13]. Other than systematic effects, topical immunological responses could also be compromised [11], which might facilitate the inoculation of viruses at the herald patch, a postulation not yet substantiated.

A simplified immunopathological sequence would be: primary viral infection in childhood, the body then launches a primary and non-specific immunological response, then clonal expansion of T-cells (memory cells), then life-long latent infection of the virus in the peripheral blood mononuclear cells, then physical or psychological stresses together with NF-1 weakening the immunity, then endogenous reactivation of the viruses, then secondary immunological response (mainly by cell-mediated immunity), then the visible PR rash. The immunological basis for the predilected sites of lesions in bilateral axillae and groins in pityriasis circiné et marginé, however, is completely unknown.

For other paraviral exanthems, eruptive pseudoangiomatosis was reported to be associated with hospitalisations and treatment for cancers [14]. It was postulated that relative immunocompromisation is the missing link. We have reported the association of Gianotti-Crosti syndrome – another paraviral exanthem – and hyperimmunoglobulinaemia E syndrome (Job’s syndrome), which is a congenital immunodeficiency disease [15]. Whether relative immunocompromisation is associated with other paraviral exanthems, such as asymmetric periflexural exanthem (unilateral laterothoracic exanthem) and papular-purpuric gloves and socks syndrome, is yet to be investigated.

Finally, we congratulate Yorulmaz A and his colleagues for such an outstanding piece of work which can be applied to patients immediately. We humbly recommend Yorulmaz A et al and other investigators to explore the possible associations between relative immunocompromisation and PR or other paraviral exanthems.


1. Ahu Yorulmaz, Ferda Artuz, Kulacoglu S, A case of pityriasis rosea of vidal accompanied by neurofibromatosis type 1Our Dermatol Online 2015; 6: 373-375.

2. Gutmann DH, Aylsworth A, Carey JC, Korf B, Marks J, Pyeritz RE, The diagnostic evaluation and multidisciplinary management of neurofibromatosis 1 and neurofibromatosis 2JAMA 1997; 278: 51-7.

3. Chuh AA, Diagnostic criteria for pityriasis rosea: a prospective case control study for assessment of validityJ Eur Acad Dermatol Venereol 2003; 17: 101-3.

4. Zawar V, Chuh A, Applicability of proposed diagnostic criteria of pityriasis rosea: results of a prospective case-control study in IndiaIndian J Dermatol 2013; 58: 439-42.

5. Evans DG, Howard E, Giblin C, Clancy T, Spencer H, Huson SM, Birth incidence and prevalence of tumor-prone syndromes: estimates from a UK family genetic register serviceAm J Med Genet A 2010; 152A: 327-32.

6. Lammert M, Friedman JM, Kluwe L, Mautner VF, Prevalence of neurofibromatosis 1 in German children at elementary school enrollmentArch Dermatol 2005; 141: 71-4.

7. Orraca M, Morejón G, Cabrera N, Menéndez R, Orraca O, Neurofibromatosis 1 prevalence in children aged 9-11 years, Pinar del Río Province, CubaMEDICC Rev 2014; 16: 22-6.

8. Traore A, Korsaga-Some N, Niamba P, Barro F, Sanou I, Drabo YJ, Pityriasis rosea in secondary schools in Ouagadougou, Burkina FasoAnn Dermatol Venereol 2001; 128: 605-9.

9. Koga M, Koga K, Nakayama J, Imafuku S, Anthropometric characteristics and comorbidities in Japanese patients with neurofibromatosis type 1: a single institutional case-control studyJ Dermatol 2014; 41: 885-9.

10. Gerosa PL, Bizzozero L, Fontana A, Giussani G, Spinelli M, Vai C, Immune reaction in von Recklinghausen’s neurofibromatosisMinerva Med 1991; 82: 613-25.

11. Gerosa PL1, Vai C, Bizzozero L, Local immune response in neurofibromatosis type 1 and type 2Minerva Med 1993; 84: 23-31.

12. Lee PR, Cohen JE, Fields RD, Immune system evasion by peripheral nerve sheath tumourNeurosci Lett 2006; 397: 126-9.

13. Luangwedchakarn V, Jirapongsaranuruk O, NiemeLa JE, Thepthai C, Chokephaibulkit K, Sukpanichnant S, A novel mutation of the IL12RB1 gene in a child with nocardiosis, recurrent salmonellosis and neurofibromatosis type I: first case report from ThailandAsian Pac J Allergy Immunol 2009; 27: 161-5.

14. Guillot B, Dandurand M, Eruptive pseudoangiomatosis arising in adulthood: 9 casesEur J Dermatol 2000; 10: 455-8.

15. Chuh A, The association of hyperimmunoglobulin E syndrome and Gianotti-Crosti syndrome – speculations on the roles of atopy and immunoglobulin E in a viral exanthemPediatr Infect Dis J 2005; 24: 942


Source of Support: Nil,

Conflict of Interest: None declared.



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