Characterized by hyperpigmented and hypopigmented macules forming a reticulate pattern,
dyschromatosis universalis hereditaria (DUH) was first described by Toyamo in Japan.
Subsequent cases have been reported from other areas including Europe, China, Saudi
Arabia, Tunisia, India, and Nigeria.
A variable autosomal inheritance has been described, and a few sporadic cases have
been reported. Spontaneous regression has not been recorded. Only one case involving
treatment modality was reported in 2011.
A 20-year-old woman presented with progressive and asymptomatic mottled hyperpigmentation
involving almost the entire body since the age of 5 years. On examination, she was
of normal stature and average build. She was noted to have diffuse and symmetric mottled
hyperpigmented and hypopigmented macular dyspigmentation involving the face, neck,
trunk, back, buttocks, arms, and legs sparing palms, soles, nails, and oral mucosa.
Areas of small hypopigmented macules alternated with zones of pigmented macules of
variable sizes and shapes. The lesions were predominantly on the forearms, abdomen,
and back. There was no atrophy, erythema, or telangiectasia (Fig 1). Dermatoglyphics
pattern was preserved. Hair, teeth, and other mucosae were normal. The family history
Hyperpigmented and hypopigmented macular dyspigmentation on abdomen and forearms formed
a reticulate pattern.
Systemic examination found no abnormalities. Skin biopsy of hypopigmented lesions
on the right abdomen found a normal epidermis and perivascular lymphocytes and melanocytes
within the papillary dermis. Skin biopsy of hyperpigmented lesions on the left forearm
showed increased melanin granules and pigmentation in the basal cell layer of the
epidermis and perivascular lymphocytes and melanocytes within the papillary dermis
(Fig 2). The generalized distribution of the hyperpigmented and hypopigmented macules
and biopsy results were consistent with the diagnosis of DUH.
Increased melanin granules and pigmentation in the basal cell layer of the epidermis
(indicated by the arrows).(Hematoxylin-eosin stain; original magnification: ×200.)
We treated a 5-cm × 1-cm area on the left forearm with a 755-nm Q-switched alexandrite
laser, 3-mm spot size, 5.2-J/cm2 fluence, and 5-Hz frequency. The patient noticed
initial darkening of lentigines and then light peeling and fading over 10 to 14 days.
No erythema, edema, blister, paleness, bleeding, or ecchymosis were noticed. An ice
pack was applied for approximately 30 minutes postoperatively. The patient was instructed
to avoid sun exposure and to apply sunblock daily. Significant improvement was noted
after 1 treatment without any scarring or undesirable pigmentary changes. Compared
with the lentigines before the treatment (Fig 3, A), no recurrence was noted at the
11-month follow-up after 1 treatment session (Fig 3, B).
A, Left forearm before treatment. B, Area treated with 755-nm Q-switched alexandrite
laser (within the pink line) on the left forearm 11 months after a single laser treatment.
DUH is a rare genodermatosis characterized by hyperpigmented and hypopigmented macules
forming a generalized reticulate or mottled pattern. There are cases reported with
varying phenotypes involving the oral mucosa, tongue, palms, soles, hair, and nails.3,
4 Systemic abnormalities include renal failure,
primary ovarian failure, hypothyroidism,
and oral leukokeratosis.
Traditional therapeutic modalities for pigmentary lesions included surgical excision,
dermabrasion, electrodessication, and chemical peeling. These treatments may have
side effects such as scarring or dyspigmentation, which limit their use in generalized
pigmentary disorders such as DUH. Intense pulsed light (IPL) is found to be a cost-effective
and safe treatment modality for hyperpigmentation. We did not test treat this patient's
forearm with IPL, because IPL has been used mainly for cosmetic purposes or facial
rejuvenation in our department, and we did not have the experience of treating sites
other than the face. Also, it may require multiple treatment sessions to achieve satisfactory
results. IPL would be considered if the patient continued with treatments over other
anatomic sites, such as the face. We considered that IPL had limited effects for complicated
skin problems and combination therapies with IPL and laser or other cosmetic technologies
might be needed.
The Q-switched alexandrite laser at a 755-nm or 752-nm wavelength and 5- to 7-J/cm2
fluence, used on a bimonthly or trimonthly basis, is reported to be an effective and
safe treatment for facial and labial lentigines associated with Peutz-Jeghers syndrome,
cutaneous reticulated acropigmentation of Kitamura,
and lips, oral mucosa, and finger pigmentary disorders of Laugier-Hunziker syndrome.
The only previously reported use of the Q-switched alexandrite laser treatment for
the hyperpigmented macules of DUH produced dramatic improvement on the face and lips
of a Japanese woman with a 45-year history of DUH. We treated a test area on the forearm
using the Q-switched alexandrite laser, and the treatment proved to be effective,
well tolerated, and long lasting. We recommend that this modality be considered in
the treatment of other sites. Further study is needed to determine the optimal number
of treatment sessions and the interval between treatments.