PoikilodermaEdit

Poikiloderma is a descriptive pattern seen on the skin rather than a single disease. It combines a netlike or reticulate hyperpigmentation with erythema, telangiectasia (dvisible small blood vessels), and dermal/epidermal atrophy. Because poikiloderma appears in a range of conditions, its identification prompts careful evaluation for underlying causes, which can span chronic sun exposure, radiation or drug effects, inflammatory diseases, and several hereditary syndromes. In some hereditary forms, poikiloderma is a hallmark feature and carries implications for cancer risk and genetic counseling. Recognizing the pattern and its context helps clinicians determine appropriate management and surveillance strategies.

Presentation

Visual pattern: A reticulated network of light to dark hyperpigmentation, often mottled with red or violaceous areas. Erythema and visible telangiectasia may be prominent, and the skin may appear thin or atrophic in affected regions.
Common distributions: Poikiloderma frequently involves sun-exposed areas such as the face, neck, and chest, but the exact distribution varies with the underlying cause. Certain forms, such as the facial and neck pattern seen in Poikiloderma of Civatte, have characteristic localization.
Symptoms: In many cases poikiloderma is asymptomatic, though irritation, pruritus, or burning can occur with ongoing sun exposure or in association with inflammatory skin conditions.
Course: Some etiologies cause transient poikiloderma that improves with treatment of the underlying trigger, while hereditary syndromes may show persistent changes from childhood and require ongoing monitoring.

Etiology

Poikiloderma can arise from multiple, distinct processes. Broadly, it is categorized as acquired (developed after birth) or hereditary (present from birth or early childhood).

Acquired poikiloderma

Chronic actinic damage and photoaging: Long-term sun exposure, especially in fair-skinned individuals, can produce the classic reticulate hyperpigmentation with telangiectasia and atrophy. This pattern can be accentuated by cosmetics or irritants and is sometimes described under the umbrella of actinic damage or photoaging. For sun-related cases, sun protection and avoidance of further ultraviolet exposure are central to management. See also Ultraviolet radiation and Photoaging.
Radiation-induced poikiloderma: Exposure to ionizing radiation, such as that used in radiotherapy, can leave a lasting poikilodermic pattern in treated skin. Related concepts include Radiation dermatitis and long-term sequelae after Radiation therapy.
Drug-induced poikiloderma: Certain medications can trigger poikiloderma as a cutaneous reaction. A well-known example is poikiloderma associated with Bleomycin therapy, which may appear during or after treatment and can persist for months.
Graft-versus-host disease: After bone marrow or stem cell transplantation, cutaneous poikiloderma may be part of the spectrum of Graft-versus-host disease.
Inflammatory and misciritic dermatoses: A range of inflammatory skin disorders and chronic dermatitis can produce poikilodermatous changes as part of the chronic inflammatory milieu.

Hereditary poikiloderma

Rothmund-Thomson syndrome: A hereditary disorder in which poikiloderma is a defining early sign, often accompanied by sparse hair, sparse eyelashes, cataracts later in life, and growth impairment. See Rothmund-Thomson syndrome.
Bloom syndrome: Another autosomal recessive condition in which poikiloderma with telangiectasia is part of the syndrome, alongside growth abnormalities and a predisposition to cancer. See Bloom syndrome.
Poikiloderma with neutropenia: A hereditary syndrome characterized by poikiloderma with persistent neutropenia and increased infection risk. See Poikiloderma with neutropenia.
Other genetic etiologies: Several rare genetic disorders feature poikiloderma as part of a broader syndrome, and genetic testing can aid in diagnosis and family counseling. See Histopathology and Genetic testing for diagnostic considerations.

Pathophysiology

The poikilodermic pattern results from a combination of epidermal atrophy, superficial dermal changes, capillary fragility, and pigmentary alteration. In acquired cases linked to sun exposure or radiation, cumulative damage to collagen and elastic fibers and dermal vasculature contributes to atrophy and telangiectasia, with pigmentary changes reflecting melanocyte response to injury. In hereditary forms, genetic defects disrupt DNA stability, replication, or repair, leading to early and persistent surface changes and, in some syndromes, a heightened risk of malignancy. See also Histopathology for microscopic correlates of poikiloderma and how biopsy findings can help distinguish etiologies.

Diagnosis

Clinical assessment: A clinician evaluates the pattern, distribution, and evolution of the skin changes, along with patient history regarding sun exposure, prior radiation, medications, and family history.
Laboratory and genetic testing: For suspected hereditary syndromes, targeted genetic testing (for example, testing associated gene panels) can confirm a diagnosis and guide surveillance for associated risks. See Genetic testing and the pages for specific syndromes such as Rothmund-Thomson syndrome and Bloom syndrome.
Skin biopsy: Histopathology can support the diagnosis by showing epidermal atrophy, dermal thinning, telangiectasia, and pigmentary alteration; results help distinguish poikiloderma from other conditions that mimic it. See Biopsy and Histopathology.

Differential diagnosis

Other reticulated or patchy pigmentary disorders
Scleroderma-like changes due to autoimmune or inflammatory disease
Dermal atrophy without pigment alteration in aging or chronic sun damage
CTCL variants with poikilodermic patterns, including poikiloderma vasculare atrophicans; appropriate oncologic evaluation is essential if lymphoma is suspected
Radiation or drug-related dermatitis without a lasting poikiloderma pattern

Management

Address underlying causes: Remove or minimize exposure to the inciting factor (sun exposure, radiation, or an offending medication, when possible), and treat associated inflammatory skin disease.
Skin protection and care: Regular use of broad-spectrum sun protection, gentle skin care, and avoidance of irritants can help prevent progression in acquired cases.
Surveillance for hereditary forms: In syndromes with cancer risk, structured surveillance and genetic counseling are important. See Cancer risk management in hereditary poikiloderma and related syndromes.
Symptomatic and cosmetic options: If warranted, treatments may include topical therapies to even skin tone, laser or other vascular therapies for telangiectasia, and management of pruritus.