Content » Vol 91, Issue 2

Letter to the Editor

More on ”Naevus Lentiginosus Linearis”: Reply

Daniele Torchia

Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, 1295 NW 14th Street, South Bldg, suite K, Miami, FL33125, USA. E-mail:

Accepted August 20, 2010.


Happle et al. (1) recently described two patients with a unique form of unilateral lentiginosis following strictly the lines of Blaschko. They stated that such a pattern had never been described before and named it “naevus lentiginosus linearis”. However, this picture has indeed been reported previously.

In 1978, Port et al. (2) described a 26-year-old Hispanic man with small hyperpigmented macules, present since the age of 1 and arranged in multiple Blaschkolinear streaks on the left side of the trunk. Fernández Canedo & Fernández Rodrigo (3) more recently presented a 22-year-old white woman with a 12-year history of a longitudinal band of lentigines stretching from the left breast to the adjacent axilla. A year later, Medina-Castillo et al. (4) described a 20-year-old Hispanic woman with a 2-year history of an S-shaped pattern of lentigines on the right side of the face (crossing the eye and involving the sclera). And last year, Monteagudo et al. (5) reported on a 52-year-old white man presenting with an S-shaped band of lentigines on the right side of the chest and back that had been present since childhood. Interestingly, three of these four previous cases also featured isolated café-au-lait spots within the lesional area (2, 3, 5).

These observations further support the existence of naevus lentiginosus linearis as a distinct entity within the spectrum of lentigo-associated naevi.

Rudolf Happle1, Dieter Metze2, and Ángel Vera Casaño3

Department of Dermatology, 1Philipp University of Marburg, Deutschhaus-Str. 9, DE-35033 Marburg, 2University of Münster, Münster, Germany and 3Complejo Hospitalario “Carlos Haya”, Málaga, Spain. E-mail:


We read with great interest Dr. Torchia’s comment on naevus lentiginosus linearis. The author concurs with us that this disorder represents a distinct entity.

On the other hand, we still have some doubts that the cases reported by Dr. Torchia can be considered examples of naevus lentiginosus linearis. In the case published by Port et al. (2) the linear lesions predominantly showed a rather uniform hyperpigmentation, which in our view would be more consistent with a diagnosis of “linear and whorled nevoid hypermelanosis”. The disorder described by Medina-Castillo et al. (4) likewise appears to be different because a rather large, segmentally arranged café-au-lait macule was present on the patient’s forehead. The pigmentary disorder reported by Fernández Canedo & Fernández Rodrigo (3) is reminiscent of naevus lentiginosus linearis but because of the presence of a café-au-lait macule within the lesion we cannot exclude the differential diagnosis of a mosaic manifestation of neurofibromatosis 1. On the other hand, the case of “partial unilateral lentiginosis” as reported by Monteagudo et al. (5) may indeed represent an example of naevus lentiginosus linearis, but the quality of the presented clinical photograph renders it difficult to confirm this unequivocally.

Regardless of the uncertainty surrounding these previous cases, future clinical research will certainly help improving the criteria to distinguish between naevus lentiginosus linearis and other pigmentary disorders arranged along Blaschko’s lines.

REFERENCES (for both papers)

  • Happle R, Metze D, Casaño AV. Naevus lentiginosus linearis: a distinct skin disorder. Acta Derm Venereol 2010; 90: 210–211.
  • Port M, Courniotes J, Podwal M. Zosteriform lentiginous naevus with ipsilateral rigid cavus foot. Br J Dermatol 1978; 98: 693–698.
  • Fernández Canedo I, Fernández Rodrigo I. Máculas pigmentadas de distribución segmentaria. Piel 2005; 20: 37–38.
  • Medina-Castillo DE, Maldonado-García CA, Ramos-Garibay A. Lentiginosis unilateral parcial. Estudio clínico de 6 casos. Piel 2006; 21: 14–18.
  • Monteagudo B, Cabanillas BM, Suárez-Amor O, Pérez-Valcárcel J, de la Cruz Mera A. Lentiginosis unilateral parcial siguiendo las líneas de Blaschko. Piel 2009; 24: 459–460.