Indian Dermatology Online Journal

: 2013  |  Volume : 4  |  Issue : 4  |  Page : 264--266

Progressive cribriform and zosteriform hyperpigmentation: Where are we at present?

Tarang Goyal 
 Department of Dermatology, Venereology and Leprosy, Muzaffarnagar Medical College and Hospital, Uttar Pradesh, India

Correspondence Address:
Tarang Goyal
H. No 40, Somdutt Vihar, Meerut, Uttar Pradesh - 250 004

How to cite this article:
Goyal T. Progressive cribriform and zosteriform hyperpigmentation: Where are we at present?.Indian Dermatol Online J 2013;4:264-266

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Goyal T. Progressive cribriform and zosteriform hyperpigmentation: Where are we at present?. Indian Dermatol Online J [serial online] 2013 [cited 2021 Aug 2 ];4:264-266
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It was in 1901 that Blaschko observed that many linear dermatoses followed patterns which were distinct from Langer's lines, dermatomes, embryonic clefts, pigmentary demarcation lines or even lymphatic or vascular supply lines. Known as Blaschko's lines, these followed V-shapes on posterior mid-line, S-shape on abdomen and spirals on vertex and scalp. Progressive cribriform and zosteriform hyperpigmentation (PCZH) was first described in 1978 by Rower et al.,[1] as a single area of uniformly tan, cribriform, macular pigmentation in a zosteriform distribution; histological pattern consisting of mild increase in melanin pigment in basal cell layer and complete absence of nevus cells. It had onset well after birth with no reported systemic associations.

Kalter et al.,[2] coined a term linear and whorled nevoid hypermelanosis (LWNH) in 1988 to describe a sporadic pigmentary anomaly characterized clinically by swirls and streaks of macular hyperpigmentation following Blaschko lines; no preceding vesicles, bullae or inflammation; no sex predilection; sparing of mucous membranes, eyes, palms and soles; and histologically by epidermal melanosis without dermal pigmentary incontinence. Subsequently, Di Lernia et al.,[3] suggested that PCHZ should be considered as a part of spectrum of LWNH as apart from later age of onset, there was no difference between the two disorders clinically and histopathologically. They also proposed the terms 'Diffuse LWNH' for previous reported cases of LWNH and 'Localized-form' for PCZH. [4]

Also known as zebra-like hyperpigmentation in whorls and streaks, reticulate and zosteriform hyperpigmentation, reticulate hyperpigmentation of Lijima, Naito and Uyeno, LWNH is a sporadic disorder occurring within first few weeks of life. In some patients the lesions may initially spread but stabilize by 2-3 years of age. An under-recognized disorder, it is usually a benign condition with neurological, cardiac, musculoskeletal associations been reported rarely. PCZH is a name given to an entity presenting later in life characterized clinically by swirls and streaks of macular pigmentation following the lines of Blaschko with a sharp midline cut-off and is asymptomatic in nature [Figure 1], [Figure 2] and [Figure 3]. The incidence of this rare disorder is unknown with M:F ratio of 1:1.{Figure 1}{Figure 2}{Figure 3}

Other disorders which should be kept in mind while diagnosing such cases are, early stage of epidermal nevus [Figure 4], third stage of incontinentia pigmentii, chimerism, focal dermal hypoplasia (Goltz syndrome), X-linked reticulate pigmentary anomaly, X-linked dominant variant of chondrodysplasia punctata and Cafè-au-lait macules of McCune-Albright syndrome. Some of the acquired causes to be kept as differential are lichen planus [Figure 5], linear fixed drug eruption, linear atrophoderma of Moulin and linear biphasic cutaneous amyloidosis. The histopathological features as well as the differentials have been very well elaborated in the subsequent article. The typical histopathology of PCZH has been elaborated [Figure 6].{Figure 4}{Figure 5}{Figure 6}

Due to rarity of the disorder, very few authors have reported large series of patients. In 1996 Nehal et al.,[5] studied 54 patients with both hypo- and hyperpigmentation following the lines of Blaschko and reported 31% of extracutaneous abnormalities which were similar to hypomelanosis of Ito (HI). They further reiterated Happle's postulates [6] of somatic mutation and unified these both disorders as one entity. It was postulated that developmental somatic mosaicism leading to proliferation and migration of two mixed populations of melanocytes with different potential for pigment production was the cause of these anomalies. These observations were supported by Taibjee et al.,[7] who stated that LWNH and HI are genetic mosaicism originated disorders that are pathologically related.

Also, a few cases with chromosomal anomalies have been reported, particularly mosaic trisomy 7, 14, 18, 20, 48, as well as X-chromosome mosaicism. Hartmann et al.,[8] observed that the reason of detection of fewer cases with such chromosomal anomalies may be explained by the fact that in most cases karyotype was done on blood lymphocytes only, whereas, skin fibroblasts karyotyping would have been a better investigative method. So, this area needs some research and evaluation in future.

Currently, most of the authors are of the view that term "pigmentary dysplasia with genetic mosaicism" or "pigmentary mosaicism" is a more apt term encompassing different phenotypic expressions of common pathogenic process caused by genetic mosaicism that specifically disrupt expression of pigmentary genes. Further if we accept the observations made by Taibjee et al.,[7] that HI and LWNH represent pigmentary mosaicism and same is true of localized and segmental forms of pigmentary disorders as PCZH and segmental nevus depigmentosus, many more such pigmentary anomalies may be included in this category, which needs further classification/clarifications. There seems to be a need for a revised and universally acceptable nomenclature of this pigmentary disorder group in future.


1Rower JM, Carr RD, Lowney ED. Progressive cribriform and zosteriform hyperpigmentation. Arch Dermatol 1978;114:98-9.
2Kalter DC, Griffiths WA, Atherton DJ. Linear and whorled nevoid hypermelanosis. J Am Acad Dermatol 1988;19:1037-44.
3Di Lernia V, Patrizi A, Neri I, Varotti C. Reticulate hyperpigmentation of Iijima, Naito and Uyeno and other linear hyperpigmentations of children. Acta Derm Venereol 1992;72:393.
4Di Lernia V. Linear and Whorled Hypermelanosis. Pediatr Dermatol 2007;24:205-10.
5Nehal KS, PeBenito R, Orlow SJ. Analysis of 54 cases of hypopigmentation and hyperpigmentation along lines of Blaschko. Ach Dermatol 1996;132:1167-70.
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8Hartmann A, Hofmann UB, Hoehn H, Broecker EB, Hamm H. Postnatal confirmation of prenatally diagnosed trisomy 20 mosaicism in a patient with linear and whorled nevoid hypermelanosis. Pediatr Dermatol 2004;2:636-41.