|Year : 2020 | Volume
| Issue : 2 | Page : 99-101
Adult onset progressive cribriform and zosteriform hyperpigmentation: a rare presentation
Aastha Gupta, Prekshi Bansal, Pooja Arora
Department of Dermatology, Dr Ram Manohar Lohia Hospital and PGIMER, New Delhi, India
|Date of Submission||16-Oct-2019|
|Date of Decision||25-Feb-2020|
|Date of Acceptance||16-Apr-2020|
|Date of Web Publication||03-Dec-2020|
M.B.B.S Prekshi Bansal
Junior Resident, 762-I, BRS Nagar, Ludhiana, Punjab
Source of Support: None, Conflict of Interest: None
Progressive cribriform and zosteriform hyperpigmentation (PCZH) is an uncommon pigmentary disorder characterized by the presence of asymptomatic cribriform brown macules involving a dermatome. Most cases of PCZH present in the second decade of life with gradual extension and absence of systemic involvement. Though the lesions of PCZH are usually localized, multiple lesions may be seen in some cases. We report a rare case of a 50 year old female with adult onset of this disease in fifth decade with extensive involvement of skin along multiple dermatomes.
Keywords: Adult onset, multiple dermatomes, PCZH
|How to cite this article:|
Gupta A, Bansal P, Arora P. Adult onset progressive cribriform and zosteriform hyperpigmentation: a rare presentation. Pigment Int 2020;7:99-101
Key Messages: PCZH is a distinct clinical entity known to occur in localized pattern in early life. We report a rare case of adult onset PCZH presenting at multiple sites.
| Introduction|| |
Progressive cribriform and zosteriform hyperpigmentation (PCZH) is an uncommon disorder of hyperpigmentation which manifests as cribriform brown to black macules in a dermatomal pattern. It is usually localized and occurs in the second decade. We hereby report a rare case of extensive PCZH in an adult female with onset of disease in fifth decade.
| Case history|| |
A healthy 50 year old female presented to skin outpatient department (OPD) with complaints of asymptomatic brownish black lesions on her left lower limb, left side of upper abdomen and inner aspect of left upper arm for the past 4 years. The lesions appeared initially on left lower leg and gradually progressed to involve other sites in a year. A prior history of skin eruption, trauma or topical application of any cream was absent. There was no family history of similar lesions and past medical history was unremarkable. Cutaneous examination revealed multiple coalescent brownish macules of variable sizes present in a cribriform configuration on the medial aspect of left leg (L4 dermatome) and left thigh (L1, L2 dermatome) [Figure 1]. Similar macules were present just below submammary region (T5 dermatome) and medial aspect of left arm (T1 dermatome). Rest of the mucocutaneous and systemic examination was unremarkable. The routine blood investigations were within normal limits. Lesional skin biopsy from lower leg showed focal increased basal cell pigmentation [Figure 2]. Mild perivascular chronic inflammatory infiltrate was seen in papillary dermis. No nevus cells were seen. Based on the classical clinical and histopathology findings a diagnosis of adult onset PCZH was made.
|Figure 1 (a) Cribriform pigmentation in zosteriform pattern on left lower limb, (b) hyperpigmented macules on left leg, (c) cribriform hyperpigmented macules on medial aspect of left thigh.|
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|Figure 2 Epidermis showsorthokeratosis and spongiosis with focal increased basal cell pigmentation (H & E, X40).|
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| Discussion|| |
Rower et al. initially proposed a diagnostic criteria for PCZH comprising of (1) uniform tan cribriform pigmentation in a zosteriform pattern; (2) increased basal layer pigmentation in absence of naevus cells on histology; (3) absence of preceding rash, injury or inflammation suggestive of post-inflammatory hyperpigmentation; (4) onset well after birth with slow progression; and (5) lack of other anomalies. There are only few case reports of this entity with most cases having early onset in the second decade of life,,, though Choi et al. reported a patient with onset after fourth decade. Trunk has been reported as the most common site followed by upper and lower extremities. Though the lesions of PCZH are usually localized, multiple lesions may be seen in some cases with an average of 1.57 lesions in each patient according to a study.
The possible clinical differentials include Becker’s nevus (non-hypertrichotic variant), linear lichen planus pigmentosus and linear and whorled nevoid hypermelanosis (LWNH). Becker’s nevus is distinguished by presence of smooth muscle hypertrophy on histopathology and pubertal onset. Lichen planus pigmentosus can be differentiated by the presence of bluish grey lesions over face and flexures in a non-dermatomal pattern and lichenoid infiltrate on histology. The closest mimicker LWNH can be distinguished by the features as depicted in [Table 1]. However, lesions of PCZH have been reported along the lines of Blaschko in the past, and due to a relative grey zone between zosteriform pattern and blaschkoid phenotype it may be considered to be late onset LWNH. In fact, according to many authors these entities are similar dermatosis which include various pigmentary disorders namely “zebra-like hyperpigmentation in whorls and streaks”, zosteriform lentiginous nevus”, “reticulate hyperpigmentation of Iijima” and “reticulate hyperpigmentation distributed in a zosteriform fashion” and Taibjee et al. suggested a unified nomenclature, “pigmentary mosaicism” for such disorders.
Association between LWNH and mosaicism could be understood as lines of Blaschko mirror the embryonic migration pathway of skin cells. But the role of mosaicism in PCZH has not been consistently noted. It should be conceived that mosaic skin conditions do not necessarily follow Blaschko’s lines in every case. This may be the possible cause of somatic mosaicism manifesting as zosteriform pattern seen in PCZH.
We report this case due to the rarity of this disorder and late onset, in the fifth decade, with involvement of multiple (3) sites (trunk, upper and lower extremities). There is a paucity of universal consensus in naming these disorders of pigmentation and further studies are needed to decipher the pathogenesis of pigmentary diseases which would simplify their classification and nomenclature.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]