Keratosis punctata of the palmar creases: report of two casesassociated with ichthyosis vulgaris

M.JUST, M.RIBERA,* I.BIELSA,* A.CALATRAVA² AND C.FERRAÂ NDIZ*

Department of Dermatology, Hospital de Figueres, Figueres, Spain, Departments of *Dermatology and ²Pathology, Hospital Germans

Trias i Pujol, 08916 Badalona, Spain

Accepted for publication 8 March 1999

Summary Two patients with keratosis punctata of the palmar creases are described. The association with

ichthyosis vulgaris and other disorders of keratinization is discussed. In both cases, histopathologyrevealed a close relation between the keratotic plug and the sweat glands. The role of genetic factors

and manual activity in the pathogenesis is discussed. Treatment with oral etretinate resulted in a

good improvement in the ®rst patient, but prolonged low-dose maintenance therapy was required toprevent recurrence.

Key words: eccrine sweat ducts, ichthyosis vulgaris, keratosis punctata, palmar pits

Keratosis punctata of the palmar creases (KPPC) is a

variant of punctate keratoderma in which the lesionsare con®ned to palmar and digital creases. Associated

®ndings are unusual.1 We report two Caucasian

patients with KPPC associated with ichthyosis vulgaris.

Case reports

Patient 1

A 32-year-old Caucasian man gave a 5-year history of

painful keratotic papules con®ned to the palms. He wasa manual worker with no history of arsenic exposure,

and had noticed that work exacerbated the symptoms.

He had also had ichthyosis vulgaris since the age of2 years, and had ®ve affected relatives (Fig. 1). Exam-

ination showed more than 20 painful hard keratotic

plugs (1±4 mm in size), localized on the palmar creasesof the digits and palms of both hands (Fig. 2). These

lesions left tiny crater-like depressions when removed. In

addition, he had generalized non-in¯ammatory scalingthat spared the ¯exures, consistent with ichthyosis

vulgaris. No lesions were apparent on the soles. Exam-

ination of close relatives showed no similar palmarlesions. Microscopic examination of a biopsy from a

keratotic plug showed an inverted cone-shaped ortho-

keratotic plug depressing the epidermis, with a normalgranular layer, a mild peripheral acanthosis of the

Malpighian layer and ¯attened central papillae (Fig. 3).

The dermis was normal. When serial sections were

examined, an eccrine gland duct was observed crossingthe epidermis in the middle portion of the depression.

Treatment with oral etretinate 50 mg/day in addition to

topical emollients and keratolytics was started, whichimproved the skin lesions. Slow tapering of etretinate

was started 4 months later. Relapse of the lesions

required a dose increment on three occasions. To date,5 years later, he is still being treated with acitretin

10 mg/48 h plus topical emollient, and only discrete

asymptomatic pits are present.

Patient 2

A 35-year-old Caucasian man had developed ichthyosis

vulgaris when he was 4 years old, and had had pro-gressive asymptomatic small keratotic papules on his

palms for some years. These began insidiously but

became increasingly prominent when he changed hisjob from accountant to gardener. The lesions character-

istically showed a partial improvement while on holiday.

Family history revealed one brother with ichthyosisvulgaris (Fig. 1). There was no history of arsenic

intake. Examination showed palmar lesions and a

slightly scaly skin similar to those seen in the ®rstpatient. No similar palmar lesions were seen in close

relatives. Histopathology revealed features similar to

those seen in the ®rst patient. Owing to the lack ofsymptoms, only topical treatment with keratolytics

and tretinoin was started, resulting in minimal and

transient improvement.

British Journal of Dermatology 1999; 141: 551±553.

551q 1999 British Association of Dermatologists

Correspondence: Dr Miquel Just, c/ sta. Llogaia 61, A60 ®gueres,Girona, Spain

Discussion

KPPC was ®rst described by Arnold2 in 1947 as apossible variant of Kyrle disease. Thirty years later, its

clinical and pathological characteristics were recog-

nized and the disease was classi®ed within the groupof palmar keratodermas.3 The condition affects young

and middle-aged adults without sex predominance, and

is more often seen in black people.4 Although KPPC isusually considered as a sporadic disorder, some familial

cases with an autosomal dominant pattern of inheri-

tance have been reported.1,5 Clinically, KPPC appears assmall hyperkeratotic plugs con®ned to the palmar and

digital creases; it is less common on soles.6 In most

cases, the condition is an asymptomatic, incidental®nding, but some patients experience tenderness or

pain when pressure is applied to the lesions. Certain

occupations can aggravate the disease, leading to pro-fessional incapacity, as in our ®rst patient.

KPPC has been reported to occur in association with

Dupuytren's contractures,7 dermatitis herpetiformisand psoriasis,8 pterygium inversum unguis,9 striate

keratoderma and knuckle pads.1 KPPC has also been

associated with other disorders of keratinization such asichthyosis vulgaris5 and punctate keratoses.10 In asso-

ciation with ichthyosis vulgaris, inheritance of KPPC is

consistent with an autosomal dominant pattern.Although our patients and some of their relatives had

ichthyosis vulgaris, no additional cases of KPPC among

the relatives were detected. Therefore, we believe thatother elements, in addition to genetic factors, are

involved in the clinical expression of KPPC. In this

sense, we agree with Smith and Jetton10 that therepeated microtrauma of manual activity could play

an important part. This occurred in our patients as well

as in most previous reports.1,5,10,11 The onset in adultlife, sometimes related to the beginning of hard manual

activity,12 and the improvement when on holiday,

support this hypothesis. As opposed to the report of

Rustad and Vance,1 who found a predominance oflesions on the non-dominant hand (usually the left),

our patients were right-handed and both had more

lesions on the dominant hand.The pathology of KPPC shows a characteristic cone-

shaped hyperkeratotic plug depressing the epidermis, in

contrast to punctate keratoses. As shown in ourpatients, the hyperkeratosis is usually orthokerato-

tic,1,5,9,13 although other authors have observed para-

keratosis and a mild dermal mononuclear in®ltrate aswell.6 The keratotic plugs have been found to be clearly

related to the acrosyringium of an eccrine sweat gland

in some cases.1,4,5,14 We believe this relationship couldbe merely casual due to the palmar location of the

lesions, and it is possible that most of the describedpatients would have shown a central acrosyringium if

serial sections had been performed, as seen in our two

patients. The differential diagnosis1 includes punctatekeratoderma, basal cell naevus syndrome, palmoplantar

552 M.JUST et al.

q 1999 British Association of Dermatologists, British Journal of Dermatology, 141, 551±553

Figure 2. Hyperkeratotic plugs are seen to be con®ned to the palmar

and digital creases.

Figure 1. Family pedigrees. Grey shadingdenotes individuals with ichthyosis and

black shading denotes those with ichthyosis

and keratosis punctata of the palmar

creases.

porokeratosis, arsenic exposure, acrokeratoelastoidosisand focal acral hyperkeratosis. Owing to the lack of

symptoms associated with KPPC, treatment other than

simple emollients is seldom required. As shown in oursecond patient, the use of topical tretinoin is usually

ineffective or results in minimal improvement.1 For

those with painful or tender lesions, oral retinoidshave been used.13 As shown in our ®rst patient, sys-

temic etretinate usually improves the condition, but

prolonged low-dose maintenance therapy is requiredto prevent recurrence. Surgical treatment has also been

used for those with severe and localized disease.6

In conclusion, we report two new patients with KPPCand ichthyosis vulgaris. In contrast to the kindred

reported by Del Rio et al.5 our patients had no family

history of KPPC. Thus, we believe KPPC could be due toan abnormal localized, hyperproliferative response in

predisposed persons induced possibly by trauma. On theother hand, the relationship between the keratotic plug

and the sweat gland could be merely casual, due to the

palmar location of the lesions. Finally, this associationmust be kept in mind so as to examine carefully the

hands of all patients with ichthyosis and the skin of

those with KPPC.

References

1 Rustad OJ, Vance JC. Punctate keratoses of the palms and soles and

keratotic pits of the palmar creases. J Am Acad Dermatol 1990; 22:

468±76.

2 Arnold HL. Hyperkeratosis penetrans: report of a case of a prob-

able variant of Kyrle disease. Arch Dermatol 1947; 55: 633±8.

3 Dupre A, Christol B, Sorbara AM, Ghrenassia S. HyperkeÂratosepunctueÂe des plis palmaires. Ann Dermatol Venereol 1977; 104:

195±9.

4 PenÄ as PF, Rios-Bruceta L, SaÂnchez-PeÂrez J et al. Keratosis punctata

of the palmar creases: case report and prevalence study incaucasians. Dermatology 1994; 188: 200±2.

5 Del Rio E, VaÂzquez-Veiga H, Aguilar A et al. Keratosis punctata of

the palmar creases. A report on three generations, demonstratingan association with ichthyosis vulgaris and evidence of

involvement of the acrosyringium. Clin Exp Dermatol 1994; 19:

165±7.

6 Weiss R, Rasmussen J. Keratosis punctata of the palmar creases.Arch Dermatol 1980; 116: 669±71.

7 Schepart BS, Schwartz RA, Sothwick GJ. Dupuytren's disease

presenting as palmar pits. J Med 1978; 9: 347±50.

8 Thankappan TP, Harilal KR, Zacharia J. Keratosis punctata ofpalmar creases (case report). Indian J Dermatol Venerol Lepr 1982;

48: 176±8.

9 Dupre A, Christol B, Bonafe JL. Pterygium inversum unguis et

atrophie punctueÂe des plis palmaires. Dermatologica 1981; 162:209±12.

10 Smith EB, Jetton RL. Punctate pits and keratoses of the palmar

creases. South Med J 1970; 63: 1291±3.11 Anderson WA, Elam MD, Lambert WC. Keratosis punctata and

atopy. Arch Dermatol 1984; 120: 884±90.

12 Phillips B. A case of keratodermia punctata. Br Med J 1947; ii:

689.13 Ortega M, Quintana J, Camacho F. Keratosis punctata of the

palmar creases. J Am Acad Dermatol 1985; 13: 381±2.

14 Ortega M, Sanchez-Conejo-Mir J, Quintana del Olmo J, Camacho F.

Keratosis punctata of the palmar creases. J Cutan Pathol 1989; 16:109±11.

KERATOSIS PUNCTATA AND ICHTHYOSIS VULGARIS 553

q 1999 British Association of Dermatologists, British Journal of Dermatology, 141, 551±553

Figure 3. Photomicrograph showing a

hyperkeratotic plug invaginated in theepithelium and giving a cup-like

appearance (patient 1: haematoxylin and

eosin; original magni®cation, ´ 100).