Journal of the
American Academy of
1122 Brief communications
Linear keratosis follicularis: A specific entity?
Report of a case responding to combined
topical retinoid and a-hydroxy acid therapy
Isabelle Thomas, MD, Joel Shockman, MD, and
J. David Epstein, MD Philadelphia, Pennsylvania
Severn reports ~,~ of patients with keratotic lesions
distributed in a localized, linear, or zosteriform pattern
and disclosing histologic features of dyskeratotic acan-
tholysis have been published, but there is still some
debate concerning the classification of this entity. Some
believe that it should be considered a variant of either
Darier's or Grover's disease, whereas others believe that
it is a form of epidermal nevus. The lesions usually
appear after the age of 20 years, tend to be persistent,
and are restricted to the skin. As in other conditions
characterized by acantholysis and dyskeratosis, sunlight
or hot humid weather may induce the lesions. Topical
retinoids have been used with temporary success in the
past? We report a case of localized keratosis follicularis
with a zosteriform distribution, which responded to
combined topical tretinoin and lactic acid therapy.
Case report. A 45-year-oldwhite man was first seen at our
clinic 9 months after the onset of a persistent asymptomatic
zosteriform eruption on the left flank. The lesions appeared
after a sunburn. He had been treated previouslywith topical
and oral acyclovirfor a provisionaldiagnosis of herpes zoster,
without improvement.His medical history and family history
were unremarkable. The physical examinationrevealed multi-
ple erythematous, keratotic, and crusted papules and plaques
in a dermatomal distribution on the left anterolateral trunk.
The rest of the examination was unremarkable. In particular,
the nails and mucous membranes wereuninvolved.The biopsy
of a keratotic lesion discloseda focal area of acantholysis with
dyskeratosis, consistent with the clinical diagnosis of a local-
ized form of Darier's or Grover's disease. The patient initially
applied 2% precipitated sulfur and 5% salicylic acid in 0.1%
triamcinolone acetonide ointment topically, without success.
Tretinoin 0.t% cream and 12% lactic acid in a neutral base
(Lachydrin lotion) topical therapy were then instituted and
induced a dramatic improvement at 8 weeks. The lesions
flattened, and only a residual erythema was observed. Com-
plete clearing was seen within 3 months, without recurrence at
the time of this writing (18 months later).
Discussion. The nature and the pathogenesis of
localized keratosis follicularis are still unclear. The
histologic features of acantholysis and dyskeratosis are
nonspecific and can be seen in Darier's or Grover's
disease, the dyskeratotic variant of an epidermal nevus,
From the Skin and CancerHospital,TempleUniversityHospital.
Reprint requests: Isabelle Thomas, MD, Apartment 313, 2201
PennsylvaniaAve,,Philadelphia,PA 19130.
Dermatology
or the isolated tumor warty dyskeratoma. Features in
favor of a separate entity include the time of onset of the
lesions, after the age of 20 years, which is late for a
linear epidermal nevus or Darier's disease; the persis-
tence of the lesions; the restriction of the lesions to the
skin; and the absence of a mendelian pattern of inheri-
tance. The onset or the aggravation of the lesions by
sweating or by sunburn 4 probably involves Koebner's
phenomenon. The term localized keratosis follicularis
may be inappropriate, as the acquired mode of onset,
clinical appearance, lack of involvement of the nails or
mucous membranes, and histologie features appear
closer to a nevoid condition or to Grover's disease than
to Darier's disease.
Retinoids are known to be beneficial in the treatment
of several disorders of keratinization, including epider-
mal nevoid, ichthyosiform, psoriasiform, and hyperkera-
totic or dyskeratotic conditions? Their precise mecha-
nism of action is still unknown, but they are thought to
act at several levels to alter epidermal differentiation. As
a rule, they modulate proliferation and increase desqua-
mation. 6
Topical and systemic retinoids have been used bene-
ficially for Darier's disease7,8 but have little place in the
treatment of a localized disorder, as the potential side
effects would be disproportionate with the limited extent
of the skin involvement. Topical tretinoin, as previously
reported in a few cases, is beneficial for localized
keratosis follicularis and may induce complete remis-
sions. Results, however, usually last only as long as the
treatment is maintained. 2 Vitamin A acid in concentra-
tions of 0.05% to 0.2% has been applied, with clearing
obtained in approximately 8 weeks. Subsequently, a
twice weekly application was generally sufficient for
maintenance. In one study a biopsy done at the time of
clearing revealed only minimal areas of acanthosis with
a mild inflammation around the dermal vessels. There
was no evidence of acantholysis or dyskeratosis. 3
Although lesions usually recur after discontinuation of
therapy, remissions may last for a period of 3 to 12
months.
In our patient both 12% lactic acid in a neutral base
(Lachydrin) and tretinoin were administered concomi-
tantly. They may be additive in their effects and
facilitate mutual percutaneous absorption, as a-hydroxy
acids, including lactic acid, have been shown to disrupt
corneocyte adhesion and decrease the thickness of the
stratum corneum. 9 In our case considerable improve-
ment was observed, with total flattening of the lesions at
8 weeks. Complete clearing was obtained within 3
months, without recurrence to date. The combination of
o~-hydroxy acids and retinoids may provide more effec-
tive therapy for this group of refractory disorders
featuring hyperkeratinization or dyskeratinization.
Volume 20
Number 6
June 1989
REFERENCES
1. Moore JA, Schosser RH. Unilateral keratosis follicularis.
Cutis 1985;35:459-6l.
2. Starink TM, Woerdeman MJ. Unilateral systematized
keratosis follicularis: a variant of Darier's disease or an
epidermal naevus (acantholytic dyskeratotic epidermal
naevus)? Br J Dermatol 1981;105:207-14.
3. Hesbasher EN. Zosteriform keratosis follicularis treated
topically with tretinoin. Arch Dermatol 1970;102:209-12.
4. Hu CH, Michel B, Farber EM. Transient acantholytic
dermatosis (Grover's disease). Arch Dermatol 1985;
121:1439-41.
5. Haas AA, Arndt KA. Selected therapeutic applications of
topical tretinoin. J AM ACAO DeRr~ATOt. 1986;15:870-7.
6. Eichner R. Epidermal effects of retinoids: in vitro studies. J
AN ACAD DEr~MA'rOt1986;15"789-97.
7. Fulton JE, Gross PR, Cornelius CE, et al. Darier's disease:
treatment with topical vitamin A acid. Arch Dermatol
1968;98:396-9.
8. Dicken CH, Bauer EA, Hazen PG, et al. Isotretinoin
treatment of Darier's disease. J AM ACAb D~r~MATOL1982;
6:721-6.
9. Van Scott E J, Yu RJ. Hyperkeratinization, corneocyte
adhesion and alpha hydroxy acids. J AM ACA9 DERMaTrOL
1984;11:86707.
Aquagenic pruritus
John N. Kalliel, M D Manchester, New Hampshire
Aquagenic pruritus is a recently described disorder of
water-induced cutaneous itching without urticaria, ecze-
ma, or other skin lesions. 1 There may be a separate
subset of aquagenic pruritus in the elderly population. 2
A l t h o u g h its pathogenesis is unknown, both increased
fibrinolytic activity 3 and activation of acetylcholinester-
ase 4 h a v e been described.
N o effective treatment for idiopathic aquagenic pru-
ritus has been described. I report a serendipitous treat-
m e n t in a patient with this condition.
Case report. I was treating a 38-year-old woman for
water-induced itching without cutaneous signs from January
1986 to July 1987. Her symptoms had been present for 9 years
and were worse in the spring and fall, despite treatment with
numerous antihistamines, moisturizing creams, and steroid
creams. Laboratory values and physical examinations were
essentially within normal limits. In March 1986, administra-
tion of the experimental antidepressant fluoxetine, which is a
serotoninergic uptake inhibitor, was initiated. Her symptoms
of aquagenic pruritus almost immediately disappeared. She
continued this therapy as per the protocol for approximately 2
months, during which time her depression improved and her
Reprint reque.~ts: John N. Kalliel, MD, 388 Wilson St., Manchester,
NPI 03103.
Brief communications 1123
symptoms of aquagenic pruritus did not return, except for mild
symptoms this past fall.
This, of course, may be coincidental or may be related to the
treatment of an underlying depression. There may, however,
also be a direct relationship between fluoxetine and this
previously untreated disorder. The drug was recently approved
by the Food and Drug Administration.
REFERENCES
I. Steinman ILK, Greaves MD. Aquagenic pruritus. J AM
ACADDERMATOL1985;13:91-6.
2. Kligman AM, Greaves MD, Steinman HI(. Water induced
itching without cutaneous signs. Arch Dermatol 1986;
122:183-6.
3. Lotti T, Cappugi P, Lattari P, et al. Increased cutaneous
fibrinolytic activity in a case of aquagenie pruritus. Int J
Dermatol 1984;23:61-2.
4. Bircher AJ, Meier-Ruge W. Aquagenic pruritus. Arch
Dermatol 1988;124:84-9.
Complete resolution of Kaposi's sarcoma with
systemic etretinate therapy in a patient with
mycosis fungoides
Richard J. Sharpe, MD, Marshall E. Kadin, MD,
David C. Harmon, MD, Michael J. Imber, MD,
PhD, and R. Pox Anderson, MD
Boston, Massachusetts
Etretinate is a retinoid with proven clinical utility in
the treatment of pustular psoriasis. 1,2 In addition, data
suggest that etretinate may be useful for certain malig-
nancies and premalignant conditions, including mycosis
fungoides? s Classic Kaposi's sarcoma is a rare, usually
chronic malignancy that responds to local radiation
therapy and to various combinations of systemic chemo-
therapy. ~ We describe a case of mycosis fungoides in
which Kaposi's sarcoma cleared with administration of
systemic etretinate.
The patient, a 75-year-old woman of Ashkenazi
Jewish ancestry, was admitted to the Massachusetts
General Hospital on Aug. 13, 1987, with erythroderma.
Her skin biopsy specimens displayed the characteristic
features of mycosis fungoides. The disease had been
present for approximately 7 years and had been treated
with systemic and topical steroids, PUVA, and metho-
trexate. During the 4 months before admission she had
From Massachusetts General Hospital, Beth Israel Hospital, and
Harvard Medical School.
Reprint requests: Richard J. Sharpe, MD, Massachusetts General
Hospital, Department of Dermatology, Wellman-2, Boston, MA
02114.