Clinical Presentation of Nonsclerotic Epidermal Chronic Graft-Versus-Host Disease and Hair and Nail Changes

Organ or site

Diagnostic (sufficient to establish the diagnosis of chronic GVHD)

Distinctive (seen in chronic GVHD, but insufficient alone to establish a diagnosis)

Other features or unclassified entities

Skin

Poikiloderma

Lichen planus–like features

Sclerotic features

Morphea-like features

Lichen sclerosus–like features

Depigmentation

Papulosquamous lesions

Sweat impairment

Ichthyosis

Keratosis pilaris

Hypopigmentation

Hyperpigmentation

Nails

Dystrophy

Longitudinal ridging, splitting, or brittle features

Onycholysis

Pterygium unguis

Nail loss (usually symmetric, affects most nails)

Scalp and body hair

New onset of scarring or nonscarring scalp alopecia (after recovery from chemoradiotherapy)

Loss of body hair

Scaling

Thinning scalp hair, typically patchy, coarse, or dull (nor explained by endocrine or other causes)

Premature gray hair

Adapted from Jagasia et al. [1]

Table 7.2

Skin scoring of chronic graft-versus-host disease (cGVHD)

	Score 0	Score 1	Score 2	Score 3
GVHD features to be scored by BSA:	No BSA involved	1–18 % BSA	19–50 % BSA	>50 % BSA
Check all that apply: Maculopapular rash/erythema Lichen planus–like features Sclerotic features Papulosquamous lesions or ichthyosis Keratosis pilaris–like GVHD
Skin features score: Other skin GVHD features (NOT scored by BSA) Check all that apply: Hyperpigmentation Hypopigmentation Poikiloderma Severe or generalized pruritus Hair involvement Nail involvement	No sclerotic features	Superficial sclerotic features “Not hidebound” (able to pinch)		Check all that apply: Deep sclerotic features “Hidebound” (unable to pinch) Impaired mobility Ulceration
Abnormality present but explained entirely by non-GVHD documented cause (specify): _________________

Adapted from Jagasia et al. [1]

BSA body surface area

Nail changes, including dystrophy, longitudinal ridging, onycholysis, pterygium, and anychia (loss of the nail entirely) are also considered distinctive features [1]. Hair loss may be associated with cGVHD, but evaluating alopecia in the setting of HSCT is challenging, and the etiology is often multifactorial. Skin pathology can be helpful in determining the etiology and guiding the treatment.

The skin is the organ most commonly involved at the time of initial cGVHD diagnosis. The purpose of this chapter is to better acquaint the medical team with the nonsclerotic epidermal manifestations of cutaneous cGVHD, in the hope that early recognition and uniformity in grading of these clinical features will aid in diagnosis, treatment, and research.

Clinical Manifestations of cGVHD

Diagnostic Features

Diagnostic features for cGVHD (requiring no further testing to establish the presence of cutaneous cGVHD) include lichen planus–like lesions, lichen sclerosus, and poikilodermatous skin changes [1]. Each diagnostic feature may be seen alone, but they often appear in combination, along with other epidermal or sclerotic features of cutaneous GVHD.

Lichen Planus–like GVHD

Lichen planus is characterized by purple-hued, polygonal papules and plaques, sometimes with a fine white scale and an overlying network of white lines (Wickham striae). Lichen planus of the hair follicle is termed lichen planopilaris (Figs. 7.1, 7.2, 7.3, 7.4, 7.5, 7.6, 7.7, 7.8, 7.9, 7.10 and 7.11).

Fig. 7.1

The purple, polygonal lichen planus–like papules and plaques may be solitary (a, b) or may become confluent (c, d)

Fig. 7.2

(a, b) Lichen planus-like GVHD may koebnerize, following lines of pressure or trauma. (c) An unusual sporotrichoid pattern (arrows) is present without known antecedent. Trauma

Fig. 7.3

Inflammation may lead to significant postinflammatory pigment changes, including the hypopigmentation and hyperpigmentation seen in the beard area of this patient

Fig. 7.4

Unusual presentations of lichen planus–like GVHD may occur, including prominent lesions in distribution that may mimic tinea pedis (a) and tinea cruris (b). This patient had been treated with topical antifungal agents

Fig. 7.5

(a, b) Lichen planus–like GVHD may become hypertrophic, mimicking prurigo nodularis or squamous cell carcinoma

Fig. 7.6

Lichen planus–like changes may cause scarring. These scalp (a) and facial (b) lesions are difficult to distinguish from those of chronic cutaneous lupus

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