|Year : 2015 | Volume
| Issue : 1 | Page : 41-43
Progressive cribriform and zosteriform hyperpigmentation: Need for change in nomenclature
Sneha Ghunawat, Rashmi Sarkar, Vijay Kumar Garg
Department of Dermatology, Venereology and Leprosy, Maulana Azad Medical College, New Delhi, India
|Date of Web Publication||26-Jun-2015|
Dr. Rashmi Sarkar
Department of Dermatology, Venereology and Lepreology, Maulana Azad Medical College and Associated Lok Nayak Hospital, New Delhi
Source of Support: None, Conflict of Interest: None
Progressive cribriform and zosteriform hyperpigmentation is a distinct clinical entity presenting with cribriform macules present in a dermatomal pattern. Much conflict is present in the literature regarding the nomenclature of this disorder. A plethora of pigmentary anomalies have been described in the literature, which demand proper classification. This article provides an insight into the conflict, which surround these entities.
Keywords: Cribriform and zosteriform hyperpigmentation, dermatomal pigmentation, linear pigmentation
|How to cite this article:|
Ghunawat S, Sarkar R, Garg VK. Progressive cribriform and zosteriform hyperpigmentation: Need for change in nomenclature. Pigment Int 2015;2:41-3
| Introduction|| |
Progressive cribriform and zosteriform hyperpigmentation (PCZH) is a distinct clinicopathological entity characterized by brown-black pigmented macules present in a cribriform pattern along a dermatome. To the best of our knowledge, only five reports have been published in the English literature describing this disorder. We hereby report a case of PCZH followed by an insight into the conflicts that surround the nomenclature of this entity.
| Case Report|| |
A healthy 19-year-old male presented to the skin out-patient department with chief complaints of brownish macules present in a linear pattern over his right arm and forearm since the past 2 years. The lesions had first appeared over the arm and within a span of 6 months had progressed to involve the forearm. There was no history of any preceding eruption or trauma to the site. No history of similar complaints in the family members was noted.
Cutaneous examination revealed multiple brown macules present in a cribriform pattern over the medial aspect of right arm. Coalescent brownish macules with well-defined borders were present over the forearm in a dermatomal pattern [Figure 1]. Rest of the mucocutaneous examination was within normal limits. All routine biochemical investigations were within normal limits. Biopsy from the lesion revealed elongation of rete ridges, prominent pigmentation of the basal layer and occasional melanophage in the dermis [Figure 2]a and b. No nevus cells were noted. Based on these findings a diagnosis of PCZH was made.
|Figure 2: (a) Biopsy showing elongation of rete ridges and increased pigmentation of the basal layer (H and E; ×100). (b) Biopsy showing pigment incontinence (H and E; ×400)|
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| Discussion|| |
Progressive cribriform and zosteriform hyperpigmentation was first described in 1978 by Rower et al.  The following diagnostic criteria have been proposed for this entity. (1) Uniform tan cribriform pigmentation present in a zosteriform distribution. (2) Histological finding of increased pigmentation of basal layer and absence of nevus cells. (3) No history of rash, injury, inflammation suggestive of postinflammatory hyperpigmentation. (4) Onset after birth with gradual extension. (5) Lack of other associated cutaneous or internal abnormalities. The incidence of this disorder is unknown and has M: F ratio 1:1.  To the best of our knowledge, only five cases of PCZH have been reported in the English literature. ,,,, In a clinicopathologic study by Cho et al.  13 (43.3%) patients demonstrated pigment incontinence. Pigment incontinence has also been reported previously in cases of PCZH. 
Clinical differential diagnosis of this disorder includes non-hypertrichotic variant of Becker's nevus, the third stage of incontinentia pigmenti, linear lichen planus pigmentosus and linear and whorled nevoid hypermelanosis (LWNH). Clinical and histological findings help in differentiating among these clinical entities. Becker's nevus can be differentiated by the presence of smooth muscle hypertrophy in the dermis. Lesions of lichen planus are characterized clinically by a violaceous hue, and also by the presence of lichenoid infiltrate on histopathology. The pigmentary stage of incontinentia pigmenti is preceded by vesicular and verrucous lesions appearing since birth.
However, the closest differential diagnosis of PCZH is LWNH. This entity was first described by Kalter et al. in 1988.  It presents clinically with diffuse streaks and whorls of hypermelanosis present in a blaschkoid pattern. The lesions are early in the onset compared to PCZH. Extra cutaneous anomalies such as neurological, cardiac and musculoskeletal have been associated with it. Since the two disorders have similar clinical and histopathological findings with only minor difference being the age of presentation, extent of involvement and presence of extracutaneous features. Choi et al.  suggested that PCZH can be regarded as a late onset variant of LWNH. Happle  suggested somatic mosaicism to be the cause of pigmentary anomalies present along blaschko's lines. Although, cases of LWNH have been reported with chromosomal mosaicism, , PCZH with associated mosaicism has not been reported.
Blaschko's lines are a form of mosaicism followed by linear dermatosis and are distinct from pigmentary demarcation lines, Langer's lines and embryonic clefts. These follow the spiral pattern on the scalp, S-shaped on the abdomen and V-shaped on the midline on the back.  Lesions of PCZH described by Rower et al.  were distributed in a zosteriform (dermatomal) pattern. Blaskho's lines have often been confused with zosteriform pattern, and lesion of PCZH has been described along blaschkoid pattern. Schepis et al.  and Cho et al.  reported cases of PCZH present along blaschko's lines. Gutte  suggested that the term "progressive cribriform and blaschkoid hyperpigmentation" should be used for disorders in the PCZH-LWNH spectrum. These reports have led to the confusion regarding the terminology that is used to describe these entities.
A plethora of pigmentary anomalies have been described in the literature by different names such as "zosteriform lentiginous nevus," "zebra-like hyperpigmentation in whorls and streaks," "reticulate hyperpigmentation distributed in a zosteriform fashion" and "reticulate pigmentation of Iijima," these have also been hypothesized to represent LWNH-PCZH spectrum.  Taibjee et al.  advised use of the umbrella-term "pigmentary mosaicism" for these disorders. Thus, further clinicopathological studies are required for better understanding of these pigmentary anomalies and also their proper nomenclature.
| References|| |
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Choi JC, Yang JH, Lee UH, Park HS, Chun DK. Progressive cribriform and zosteriform hyperpigmentation - The late onset linear and whorled nevoid hypermelanosis. J Eur Acad Dermatol Venereol 2005;19:638-9.
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Kubota Y, Shimura Y, Shimada S, Tamaki K, Amamiya S. Linear and whorled nevoid hypermelanosis in a child with chromosomal mosaicism. Int J Dermatol 1992;31:345-7.
Lal K, Di Lernia V. Linear and whorled naevoid hypermelanosis in a patient with trisomy 4 mosaicism. Clin Exp Dermatol 2015;40:45-7.
Taibjee SM, Bennett DC, Moss C. Abnormal pigmentation in hypomelanosis of Ito and pigmentary mosaicism: The role of pigmentary genes. Br J Dermatol 2004;151:269-82.
[Figure 1], [Figure 2]