Reticulate postinflammatory hyperpigmentation with band-like mucin deposition.

Background: Mucinoses of the skin are a group of disorders sharing accumulation of mucin in the skin or hair follicles. Postinflammatory hyperpigmentation, with pigmentary incontinence, is due to loss of melanin from epidermal basal cells and its accumulation in dermal macrophages.

Methods: We describe clinicopathologic features of two patients presenting with the association of pigmentary incontinence with an unusual diffuse, band-like dermal deposition of mucin, clinically presenting with reticular pigmented macular lesions.

A case of dermatopathia pigmentosa reticularis.

Dermatopathia pigmentosa reticularis (DPR) is a very rare disorder with the diagnostic triad of generalized reticulate hyperpigmentation, noncicatricial alopecia, and onychodystrophy. Many other dermatologic findings have been associated with this triad, including adermatoglyphia, hypohidrosis or hyperhidrosis, palmoplantar hyperkeratosis, and nonscarring blisters on the dorsa of the hands and feet. The mode of inheritance is unclear but may be autosomally dominant.

Dermatopathia pigmentosa reticularis: a report of a family demonstrating autosomal dominant inheritance.

Dermatopathia pigmentosa reticularis is a rare heritable disorder consisting of a triad of cutaneous findings including reticulate hyperpigmentation, noncicatricial alopecia, and onychodystrophy. In addition to this triad, other variable features of dermatopathia pigmentosa reticularis have been described, including adermatoglyphia, hypohidrosis or hyperhidrosis, and palmoplantar hyperkeratosis. Only 10 cases have been described in the world literature to date, and no clear inheritance pattern has been demonstrated.

Dermatopathia pigmentosa reticularis.

Dermatopathia pigmentosa reticularis is a rare disorder that presents as reticulate pigmentation distributed widely all over the cutaneous surface. Only nine patients with this disease have been described previously. Our patient had no fingernail and toenail prints, a striking finding also noted in some, but not all, of the other nine patients.

Primary disorders of hyperpigmentation.

A classification of primary hyperpigmentation conditions is presented. The emphasis is on clinical aspects and an attempt has been made to show, when possible, a spectrum beginning with localized involvement and progressing to the more extensive involvement. Most primary hyperpigmentation conditions and syndromes are inherited by autosomal dominant genetics; notable exceptions include incontinentia pigmenti, classic dyskeratosis congenita, and xeroderma pigmentosum.

Congenital poikiloderma with traumatic bulla formation, anhidrosisi, and keratoderma.

A 14-year-old boy with congenital poikiloderma had anhidrosis, palmoplantar-pitted keratoderma, traumatic bulla formation, and defective dentition, but no abnormalities of the hair, nails, or eyes. This patient was similar in some respects to others reported as having dermatopathia pigmentosa reticularis, the Franceschetti-Jadassohn syndrome, the Mendes da Costa syndrome, and acrokeratotic poikiloderma.