Progressive cribriform and zosteriform hyperpigmentation: a histologic mimicker of Dowling-Degos disease?

Progressive cribriform and zosteriform hyperpigmentation (PCZH) is a late-onset disorder of pigmentation with a zosteriform distribution [1]. It is considered to be the localized form and late onset of linear and whorled nevoid hypermelanosis (LWNH) [2]. Clinically, PCZH is characterized by cribriform macular hyperpigmentation, usually on the trunk [2].
A 24-year-old male presented with a Blaschkoid, cribriform, brown macule on the trunk for 16 years. It appeared at the age of eight and has been slowly progressing. He was otherwise in good health without other skin abnormalities. No prior injury was reported to the affected skin. His parents were nonconsanguineous and denied any history of neurofibromatosis or similar eruptions. On examination, there was discrete cribriform hyperpigmentation on the trunk with a Blaschkoid distribution (Figure 1). The lesions were pigmented homogeneously. His hair, nails, and mucosae showed no abnormality. Routine blood tests, urinalysis, liver and renal function tests, and electrolytes were all within normal limits. Skin biopsy specimens from a representative lesion showed elongated epidermal rete ridges and an accumulation of pigment at the tip of rete ridges (Figure 2). There were a few melanophages in the dermis. The plethora of clinicopathological conditions is consistent with the diagnosis of PCZH. No treatment was given, and the lesion remained unchanged until now.
PCZH is asymptomatic cribriform hyperpigmentation distributed along Blaschko’s lines. It was first reported by Rower et al. without a defined incidence [1]. PCZH has also been referred to using other terms including “zosteriform lentiginous nevus”, “reticulate hyperpigmentation of Iijima”, “zebra-like hyperpigmentation in whorls and streaks’’ and ‘‘reticulate hyperpigmentation distributed in a zosteriform fashion”. There is no sex preference, and the mean age of onset is estimated to be 14 years [3].
PCZH lesions are characterized by uniform, cribriform macular hyperpigmentation in a zosteriform or Blaschkoid distribution. It often has a late onset with gradual extension, and the trunk is the predilection site of involvement. There is typically evidence of preceding trauma or inflammation. PCZH may occur with extracutaneous abnormalities, including mental retardation, polythelia, accessory mammary tissues, kidney, and urinary tract malformation [2–4]. Skin biopsy of the lesion in PCZH reveals increased pigmentation of the basal...


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