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Darier's disease

Darier's disease is a genetic disorder discovered by a French dermatologist Ferdinand-Jean Darier. Darier's disease is known because of dark crusty patches on the skin, sometimes containing pus. The crusty patches are also known as keratotic papules and also called keratosis follicularisis.

The disease is hereditary and dominant. It affects both men and women and is not contagious.

With the discovery of the ATP2A2 gene, performing genetic tests to confirm the diagnosis of DD is now possible.

This is one of the three skin diseases discovered by Darier.

Dandy-Walker syndrome
Darier's disease
Demyelinating disease
Dengue fever
Dental fluorosis
Dentinogenesis imperfecta
Depersonalization disorder
Dermatitis herpetiformis
Dermatographic urticaria
Desmoplastic small round...
Diabetes insipidus
Diabetes mellitus
Diabetes, insulin dependent
Diabetic angiopathy
Diabetic nephropathy
Diabetic neuropathy
Diamond Blackfan disease
Diastrophic dysplasia
Dibasic aminoaciduria 2
DiGeorge syndrome
Dilated cardiomyopathy
Dissociative amnesia
Dissociative fugue
Dissociative identity...
Dk phocomelia syndrome
Double outlet right...
Downs Syndrome
Duane syndrome
Dubin-Johnson syndrome
Dubowitz syndrome
Duchenne muscular dystrophy
Dupuytren's contracture
Dyskeratosis congenita
Dysplastic nevus syndrome


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Puzzling palmar papules and pits
From Journal of Family Practice, 3/1/05 by Gary N. Fox

A 54-year-old African American woman came to the office with a problem on her hands that began about 10 years before: small, hard plugs that formed on her palms (Figures 1 and 2). These areas remain tender for 1 to 2 days after the plugs first form and while they "stick up." After a few days, the plugs fall out, leaving small pits. The patient experienced no other symptoms once the plugs fall out; just the appearance of her palms.


Many years ago, a physician tried freezing the lesions, believing them to be warts. That therapy provided no benefit. The patient found that filing down the plugs and lubricating them with white petrolatum helped during the symptomatic phase.

The patient was married, with no history of sexually transmitted diseases or significant occupational exposures. She did take medication to control her hypertension, hyperlipidemia, and hypothyroidism. However, the problem with her hands predated taking these medications. There was no personal or family history of skin malignancy. The remainder of the skin examination was unremarkable.



Keratosis punctata of the palmar creases (KPPC) is a benign, largely asymptomatic condition of the hands, seen almost exclusively those with African ancestry. KPPC presents as small keratotic papules (Figure 3) that evolve into discreet conical pits (Figure 4). (1,2) Although KPPC is not a novel or rare condition among African Americans, it is not found in standard dermatology texts used by primary care physicians. However, reference to KPPC may be found in ethnic dermatology texts, including reference to it as "a common normal finding in the black palm." (1)


The lesions of KPPC characteristically are 1 to 5 mm in diameter, sharply defined hyperkeratotic pits that occur in the flexural creases of the hands, both on the palms and volar surfaces of the fingers. KPPC has also referred to as keratotic pits of the palmar creases, punctate keratoses of the palmar creases, keratoderma punctata, hyperkeratosis penetrans, lenticular atrophia of the palmar creases, and hyperkeratosis punctata of the palmar creases.

Distinguishing KPPC from KPPP

KPPC has also been regarded as a variant of keratosis punctata palmaris et plantaris (KPPP). (3,4) KPPP and KPPC share some similarities with respect to the size and number of lesions per palm, probable exacerbation by trauma, and predilection for occurring in those of Afro-Caribbean descent. (1,3,4)

Historically, there has been some confusion in distinguishing KPPC from KPPP--in fact, it is possible for the 2 conditions to occur simultaneously The papular lesions of KPPP tend to occur over the entire palm, volar wrist, and medial aspects of the feet. These entities differ in age at onset, prevalence, symptoms, and prognosis.

Sources differ regarding the average age of onset for KPPP; some report onset from infancy to 70 years. (5-7) For KPPC, the age of onset generally is between 15 and 40 years. (3) Among African Americans, the prevalence of KPPC is between 1.9% and 3.1%), (3,6,8) whereas the prevalence of KPPP may be up to 11%. (3,4) While KPPP is largely asymptomatic, the lesions of KPPC tend to be noticed more often. Once present, KPPP lesions usually remain stable over time, whereas, KPPC lesions usually increase in number and size)

Demographics and causes

KPPC is rarely seen in Caucasians. Of 1001 white patients examined for palmar lesions, none fulfilled the diagnostic criteria for KPPC. (9) In a study of 534 patients, Weiss et al discovered 7 cases--all in African American patients and representing 3.1% of this racial group. (8)

The cause of KPPC is unknown. No medications have been implicated, and it has been difficult to link it to a virus. (3) Although some authors have suggested that KPPC represents flexural calluses related to manual labor, lesions also occur in patients without this history. (1,5) There is no association between KPPC and arsenical agents or syphilis. (8)

It is generally believed that KPPC does not have a recognizable heritable pattern, though there may be exceptions. (8) There may be a familial association with ichthyosis vulgaris and other disorders of keratinization. One report included 5 patients in 1 family with keratotic plugs of the palmar creases consistent with an autosomal dominant pattern of inheritance. The syndrome was associated with ichthyosis vulgaris in several family members. (10) KPPP and KPPC might be the result of abnormal callus formation in predisposed individuals, as both conditions seem to be due to an abnormal hyperproliferative response to local trauma. (3)


Punctate keratoses of the palms are fairly common frequently overlooked lesions. The differential diagnosis is extensive (Table), but there are several clinical features of KPPC that distinguish it from other hyperkeratotic conditions. The lesions of KPPC can be painful, have a predilection for joint creases, and evolve into pits. (3) KPPP is similar except not localized to the creases. (4)

Aquagenic keratoderma is a transitory condition afflicting young women and defined clinically by the appearance of palmar lesions accentuated after immersion in water. These lesions have a characteristic histological appearance (hyperkeratosis, dilated eccrine ducts). (13)

Palmoplantar pustulosis is characterized by chronically recurring sterile pustules on the palms and soles, usually found on an erythematous base, and a strong association with tobacco use. (14) Palmoplantar lichen planus may exhibit a variety of morphologic patterns including papules or plaques with pruritus, erythema, and compact hyperkeratosis. (15)

Cole disease is an uncommon disorder typified by distinctive cutaneous hyperpigmentation and punctate keratoses on the palms and soles. It is a congenital disease with an autosomal dominant inheritance pattern and phenotypic variability. (16)

Palmoplantar psoriasis is associated with manual labor in 50% of cases. Lesions are restricted to areas exposed to pressure. All patients with unilateral palmar lesions had them on their dominant hand. Biopsy may be necessary to differentiate hyperkeratotic eczema from psoriasis when just localized to the palms and soles. (17)


The mainstay of therapy is informing the patient of the benign nature of the diagnosis and avoiding unnecessary and unhelpful therapies and diagnostic modalities. Therapy with keratolytic agents or systemic retinoids may temporarily improve symptoms of KPPC.

However, lesions tend to recur when the medications are stopped or decreased. (5,7) Temporizing treatment of symptomatic keratoses, such as applying emollients and paring them, is all that is usually necessary. Systemic retinoids have far too many side effects to consider using in this completely benign condition.


The patient was reassured by the explanation of the condition and chose to try a keratolytic/emollient agent, Lac-Hydrin, for symptomatic recurrences. At her last visit for another health issue, she has reported this to be helpful.


The authors would like to acknowledge the unfailingly cheerful cooperation and expert assistance of the St. Vincent Mercy Medical Center library staff.


(1.) Rosen T, Martin S. Variants of normal skin in blacks. In: Atlas of Black Dermatology. 1st ed. Boston, Mass: Little, Brown; 1981:12-13.

(2.) Dilaimy MS, Owen WR, Sina B. Keratosis punctata of the palmar creases. Cutis 1984; 33:394-396.

(3.) Rustad OJ, Vance JC. Punctate keratoses of the palms and soles and keratotic pits of the palmar creases. J Am Acad Dermatol 1990; 22:468-476.

(4.) Kinsley-Scott TR, Young RJ 3rd, Meffert JJ. Keratosis puncrata of the instep. Curls 2003; 72:451-452.

(5.) Kong MS, Harford R, O'Neill JT. Keratosis punctata palmoplantaris controlled with topical retinoids: a case report and review of the literature. Cutis 2004; 74:173-179.

(6.) Anderson WA, Elam MD, Lambert WC. Keratosis punctata and atopy. Report of 31 cases with a prospective study of prevalence. Arch Dermatol 1984; 120:884-890.

(7.) Asadi AK. Type I hereditary punctate keratoderma. Dermatol On line J 2003; 9:38.

(8.) Weiss RM, Rasmussen JE. Keratosis punctata of the palmar creases. Arch Dermatol 1980; 116:669-671.

(9.) Penas PF, Rios-Buceta L, Sanchez-Perez J, Dorado-Bris JM, Aragues M. Keratosis punctata of the palmar creases: case report and prevalence study in Caucasians. Dermatology 1994; 188:200-202.

(10.) Del-Rio E, Vazquez-Veiga H, Aguilar A, Velez A, Sanchez Yus E. Keratosis punctata of the palmar creases. A report on three generations, demonstrating an association with ichthyosis vulgaris and evidence of involvement of the acrosyringium. Clin Exp Dermatol 1994; 19:165-167.

(11.) Habif TP. Clinical Dermatology: A Color Guide to Diagnosis and Therapy. 4th ed. St. Louis, Mo: Mosby; 2004.

(12.) Osman Y, Daly TJ, Don PC. Spiny keratoderma of the palms and soles. J Am Acad Dermatol 1992; 26:879-881.

(13.) Betlloch I, Vergara G, Albares MP, Pascual JC, Silvestre JF, Botella R. Aquagenic keratoderma. J Eur Acad Dermatol Venereol 2003; 17:306-307.

(14.) Gimenez-Garcia R, Sanchez-Ramon S, Cuellar-Olmedo LA. Palmoplantar pustulosis: a clinicoepidemiological study. The relationship between tobacco use and thyroid function. J Eur Acad Dermatol Venereol 2003; 17:276-279.

(15.) Gunduz K, Inanir I, Turkdogan P, Sacar H. Palmoplantar lichen planus presenting with vesicle-like papules. J Dermatol 2003; 30:337-340. 0

(16.) Vignale R, Yusin A, Panuncio A, Abulafia J, Reyno Z, Vaglio A. Cole disease: hypopigmentation with punctate keratosis of the palms and soles. Pediatr Dermatol 2002; 19:302-306.

(17.) Kumar B, Saraswat A, Kaur I. Palmoplantar lesions in psoriasis. Acta Derm Venereol 2002; 82:192-195.

Corresponding author: Gary N. Fox, MD, 2200 Jefferson Avenue, Toledo, OH 43624. E-mail: The authors have no conflicts of interest to report. Gary Fox is Reinventing Practice section editor for the Journal of Family Practice.

Gary N. Fox, MD Mercy Health Partners Family Practice Residency and Clinical Associate Professor of Family Medicine, Medical College of Ohio, Toledo

Susan J. Hulsemann, MD Mercy Health Partners Family Practice Residency, Toledo

COPYRIGHT 2005 Dowden Health Media, Inc.
COPYRIGHT 2005 Gale Group

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