Progressive Cribriform and Zosteriform Hyperpigmentation: A Case Series and Review of the Literature / 대한피부과학회지

Progressive cribriform and zosteriform hyperpigmentation (PCZH) is a distinctive pigmentary disorder observed along the lines of Blaschko. Clinically, the lesions appear as uniformly tan, cribriform macular hyperpigmentation with a zosteriform distribution, without a history of rash, injury, inflammation, or other associated cutaneous or internal abnormalities. Histopathological specimens show increased melanin pigmentation in the basal cell layer with a complete absence of nevus cells. We report 8 cases of PCZH and review the literature on this peculiar disorder.

A Case of Progressive Cribriform and Zosteriform Hyperpigmentation / 대한피부과학회지

Progressive cribriform and zosteriform hyperpigmentation (PCZH) is a distinctive syndrome first described by Rower et al. in 1978. It characteristically presents as a single area of uniformly tan, cribriform macular pigmentation with zosteriform distribution. Further, it can histologically be characterized by increased melanin pigmentation in the basal cell layer with a complete absence of nevus cells, and a lack of a preceding history of skin rash, injury or inflammation or other associated cutaneous or internal abnormalities. We herein report a case of PCZH that had developed along the right abdomen, right flank area and right back area in a 26-year-old male. The histologic investigation revealed increased melanin pigmentation in the basal layer and a distinct lack of nevus cells.

Linear Hyperpigmentation Along the Blaschko's Line: A Clinicopathologic Study / 대한피부과학회지

BACKGROUND: Many pigmentary disorders can be manifested as linear streaks of hyperpigmentation, along the Blaschko's line. These include progressive cribriform and zosteriform hyperpigmentation (PCZH) and linear and whorled nevoid hyperpigmentation (LWNH). There have been debates on the universally accepted diagnostic criteria differentiating these disease entities. OBJECTIVE: To determine the clinicopathologic characteristics of hyperpigmentation along the line of Blaschko and to examine the acceptability of PCZH or LWNH criteria as a diagnostic tool for differentiating these diseases. METHODS: A retrospective study was conducted on 13 patients who presented with linear hyperpigmentation along the Blaschko's line. The patients' clinicopathologic characteristics were analyzed and matched with the PCZH/LWNH diagnostic criteria. RESULTS: Age of onset widely ranged from birth to 61 years, but predominantly before the age of 4 years. The male-female ratio was 1:1.2. Trunk was the most common site of involvement. Histologic examination commonly showed a basal layer hyperpigmentation in all patients and pigmentary incontinence was observed in 2 patients. Four patients who satisfied all the diagnostic criteria for PCZH also fulfilled the diagnostic criteria for LWNH, except for the timing of onset. An additional 4 patients satisfied all the diagnostic criteria for LWNH and also fulfilled the diagnostic criteria for PCZH, except for the timing of onset. Excluding the age of onset criteria, the other 3 patients fulfilled both diagnostic criteria for PCZH and LWNH. CONCLUSION: These results demonstrate that PCZH and LWNH should not be considered as a different disease entity and that supports the idea that these are part of the same disease spectrum.

Progressive Zosteriform Macular Pigmented Lesion / 대한피부과학회지

A progressive zosteriform macular pigmented lesion (PZMPL) is a chronic pigmentary dermatosis similar to progressive cribriform and zosteriform hyperpigmentation (PCZH), but characteristically accompanied by pruritus as a prodromal symptom and histologic findings, such as pigmentary incontinence. PZMPL was described by Simoes in 1980 and manifests a uniformly tanned macular pigmented lesion in a zosteriform distribution. PZMPL is usually preceded by multiple pruritic macular pigmentation in a part of the dermatome for a period of time. Despite the difficulty in differentiation among other pigmentary disorders with unilateral distribution, such as linear and whorled nevoid hypermelanosis, partial unilateral lentiginosis, and Becker's melanosis, PZMPL can be diagnosed by clinical symptoms and signs, the pattern of lesions, and histologic features. A 6-year-old Korean girl presented with unilaterally located brown macules and patches on the left side of the chest, arm, and back. From childhood, the pigmented macules appeared and coalesced into patches on the left back. The arm and chest lesions extended along the Blaschko's line in a zosteriform distribution. She had no history of previous skin diseases, injuries, or inflammation. The histologic findings revealed increased melanin pigment in the basal layer and focal pigmentary incontinence in the upper dermis. We report a rare case of PZMPL thought to be the same case reported by Simoes.

A Case of Progressive Cribriform and Zosteriform Hyperpigmentation Associated with Becker's Nevus and Melanonychia / 대한피부과학회지

Progressive cribriform and zosteriform hyperpigmentation (PCZH) was first described in 1978 by Rower and his colleagues as having the following clinical characteristics: a single area of uniformly-tan, cribriform, macular pigmentation in a zosteriform distribution, an histologically mild increase of melanin pigment in the basal cell layer and complete absence of nevus cells, no preceding history of skin rash, injury or inflammation, onset after birth with gradual extension - age at onset was in the second decade of life, lack of other associated cutaneous or internal abnormalities. We hereby report an atypical case of PCZH in a 16-year-old boy, who presented with multiple, cribriform and zosteriform hyperpigmented lines on his right arm and leg. This is the first ever reported case which has an association with Becker's nevus and melanonychia.

A Case of Atypical Progressive Cribriform and Zosteriform Hyperpigmentation / 대한피부과학회지

We report a case of atypical progressive cribriform and zosteriform hyperpigmentation (PCZH) in a 4-year-old boy. Progressive cribriform and zosteriform hyperpigmentation is a distinctive pigmentary disorder first described by Rower et al. in 1978. It is characterized clinically by uniformly tan cribriform macular pigmentation in a zosteriform distribution and histopathologically by increased melanin in the basal cell layer and absence of nevus cells. This case has two distinctive findings; the onset age was below 10 years and the lesions distributed on the whole body along Blaschko's lines. Histopathologic examination showed pigmentary incontinence.

Three Cases of Progressive Cribriform and Zosteriform Hyperpigmentation / 대한피부과학회지

Progressive cribriform and zosteriform hyperpigmentation(PCZH) is a pigmentary disorder of the skin which is a distinctive clinical entity first described by Rower et al. in 1978. It is characterized clinically by a single area of uniformly tan cribriform macular pigmentation in a zosteriform distribution without preceding history of skin rash, injury or inflammation and histopathologically by a mild increase of melanin pigment in the basal cell layer and complete absence of nevus cells. We report herein three cases of PCZH. The age at onset ranged from 30 to 39 years. The lesions were unilateraly distributed. The lesions of two cases were on left lower extremity and the lesions of another case were on right flank and right thigh. Histopathologic examination of the lesions showed a mild increase in melanin pigment in the basal cell layer with absence of nevus cells.