Graham and Helwig addressed warty dyskeratoma as “isolated Darier’s disease” or “follicular dyskeratoma “in 1954. The nomenclature of warty dyskeratoma was adopted by Szymanski in 1957. “Isolated dykeratosis follicularis” or warty dyskeratoma cogitates an infrequent, cutaneous proliferative lesion commonly situated on the head or neck. Warty dyskeratoma is described as an exceptional, benign skin disease which demonstrates a characteristic solitary papule or nodule with an impacted centroidal crater or comedo [1,2].
Warty dyskeratoma is exemplified by a benign, papulo-nodular lesion which typically demonstrates an endophytic pattern of proliferating squamous epithelial cells, particularly in conjunction with folliculo-sebaceous unit. Preponderant acantholytic foci are additionally enunciated. Frequently, a singular, warty, dyskeratotic papule or nodule with occasional centric crater is cogitated
Warty dyskeratoma predominantly appears in the middle aged to elderly population. The disorder is of obscure aetiology. Ultraviolet (UV) light, autoimmune attributes, exposure to chemical carcinogens and tobacco intake can be incriminated in disease pathogenesis.
Viral (human papilloma virus) infection as discerned with a polymerase chain reaction (PCR) assay is absent. However, detection of viral deoxy-ribo-nucleic acid (DNA) and ribo-nucleic acid (RNA) within the cellular epithelium layering villous projections can be cogitated as an incriminating factor in the genesis of warty dykeratoma.
Chronic actinic deterioration is devoid of implication in the genesis of warty dyskeratoma as lesions can appear in regions unexposed to sunlight [2,3].
An acquired genetic mutation of ATP2A2 gene is discerned along with an absence of SERCA2 gene. Lesions of warty dyskeratoma are engendered from hair follicles as is indicated by preponderance of warty dyskeratoma on hair bearing areas such as scalp. Head and neck are a frequent site of disease incrimination. Lesions do not recede spontaneously
Warty dyskeratoma can appear on mucosal surfaces at sites devoid of hair follicles. However, inter-relation amidst mucosal and cutaneous lesions remains obscure.
Histology of warty dyskeratoma is identical to keratotic papules of Darier’s disease and warty dyskeratoma can be contemplated as a variant of Darier’s disease.
Individuals with multiple warty dyskeratoma usually lack a family history of adjunct conditions such as Darier’s disease or acantholytic dermatosis as cogitated in Grover’s disease. Multiple warty dyskeratoma can elucidate several or an enhancing uantification of asymptomatic papules on the scalp or forehead. Innumerable, flesh coloured or greyish, hyperkeratotic papules or nodules with a istinctive, umbilicated core can be delineated [3,4].
Multiple warty dyskeratoma of the scalp depicts a female preponderance (70%), a Male: Female ratio of 1: 1.8, a mean age of appearance at 56 years with a disease extent varying from 29 years to 84 years. Lesions can enumerate from 3 to 240.
Multiple warty dyskeratoma can display associated morbidities such as cardiac dysarrythmia, hypertension, renal failure and chronic liver disease. Multiple warty dyskeratoma of the scalp is a rare variant and requires a distinction from acquired papulo-nodular lesions in adults.
Multiple warty dyskeratoma of the scalp necessitates a cogent histology, although dermatoscopy can be efficacious in diagnosis [3,5].
Majority of the lesions are solitary in warty dyskeratoma.
Characteristically flesh coloured or brown, verrucous papules,
nodules and cysts are enunciated. Exceptionally, multiple. lesions
can appear on the face, scalp or dorsum. Adjunctive sites are implicated
such as the cheeks, hands and chest.
Frequently manifested is a distinctive, discrete, cup shaped
papule or nodule situated on the head and neck or trunk and
extremities. Warty dyskeratoma can appear on mons pubis, subungual
region, oral and genital mucosa [4,5]. Lesions vary from 1
millimetre to 12 millimetre in magnitude. The disorder is essentially
asymptomatic; however, pruritus can occur accompanied by
a relapsing, offensive, cheesy effluvium from the lesions. Centric
haemorrhage can emerge in traumatic lesions. A centric zone akin
to an umbilicus or a pore with a centroidal keratin plug can be
cogitated. Multitudinous lesions of warty dyskeratoma are infrequent.
Rare, multiple lesions appearing on the scalp are exemplified
as “multiple warty dyskeratoma of the scalp” (MWDS). The
variant was initially discerned in 1993. Multiple warty dyskeratoma
of the scalp depicts an equivocal gender distribution with a
Male to Female ratio of 1:1. As denominated, lesions are discerned
on the scalp, face, neck, hand or torso. Scalp is a frequent site
(75%) followed by face (50%). Asymptomatic scalp lesions are
gradually progressive and demonstrate brownish papulo-nodular
outgrowths which are minimally pruritic, measure 0.2 centimetres
to 2.0 centimetres, evolve gradually and are discerned within
months or years. Nodules can be erythematous with a yellowish,
hyperkeratotic occlusion. Brownish or black verrucous lesions can
be discretely disseminated or clustered [5,6].
Warty dyskeratoma is represented by cup shaped invaginations
of the epidermis displaying epithelial hyperplasia and is
accompanied by supra-basal acantholysis and dyskeratosis. The
morphological characteristics are akin to Darier’s disease. Typical
lesions of warty dyskeratoma depict a well-defined, endophytic
pattern of evolution with the appearance of corps ronds and corps
grains. Additionally, a superficial covering of parakeratotic tissue
is delineated. Dyskeratosis is predominantly and characteristically
acantholytic. Aberrant linkage and keratinisation of squamous
epithelial cells are cogitated. Inverted epidermal invaginations
are congested with keratinous debris. Prominent clefts upon supra-
basal epithelium are situated inferiorly on epidermal invaginations
and configure ascending villous projections. Keratinous
deposits are abundant and articulate keratin plugs with centric
cellular proliferation [6,7].
Innumerable villous extensions upon the inferior segment of
cup shaped depression are coated with a singular layer of basaloid
epithelial cells. Invaginations can appear within an intensely
distended hair follicle. A singular lesion can implicate two to four
adjoining hair follicles. Admixture of histological features can be
enunciated. Dyskeratotic keratinocytes appear within the stratum spinosum and stratum granulosum with the elucidation of corps
ronds and corps grains, respectively. An accompanying thickened
granular layer is demonstrated. Corps ronds and corps grains can
project through the papillary dermis. Centric crater is distended
with necrotic, cornified tissue. Sequential to the frequently elucidated
cup shaped invaginations, cystic and nodular configurations
are additionally cogitated.
Cystic category depicts enlarged; well demarcated cystic articulations
confined to the dermis. Cystic arrangements are layered
with epithelial cells and impacted with abundant keratinous
substance. Nodular variant demonstrates miniature, well defined,
circumscribed, solid aggregates of epithelial cells within the dermis.
Epithelial accumulates are in continuation with superficial
epidermis which enunciates epidermal hyperplasia [7,8]. Minimal
to moderate inflammatory infiltrate comprising of lymphocytes
and histiocytes can accrue in the superficial dermis underlying the
tumefaction. Focal emergence of acantholytic dyskeratosis cannot
be ascertained as a categorical morphological parameter of warty
Focal acantholytic dyskeratosis is additionally discerned in
Darier’s disease, transient acantholytic dermatosis, acantholytic
squamous cell carcinoma, actinic keratosis and basal cell carcinoma.
Multiple warty dyskeratoma of the scalp on histology depict a
distinct, predominantly endophytic epidermal proliferation constituting
a centroidal crater with impacted keratinous substance
composed of dyskeratotic keratinocytes which additionally configure
the classic corps ronds and grains. Inferior aspect of the
principally epidermal lesion exhibits acanthosis, prominent papillomatosis
and accumulation of dyskeratotic keratinocytes which
appear submerged in acantholytic, supra-basal epithelial perforations.
Epidermal extensions simulating a “villous” arise within epidermal
clefts. Typical histology, abutting hair follicles and representative
immune histochemical reactivity to hair follicle specific
keratins (HKN) 5/17, particularly HKN-6 and HKN-7 is diagnostic.
Thus, an alternative terminology of “follicular dyskeratosis” can be
cogitated for multiple warty dyskeratoma of the scalp [8,9].
Dermoscopic manifestations of warty dyskeratoma include
whitish, homogenous zones demonstrating yellow clods interspersed
with mature, distinct hair follicles. Additionally, pale to
gray zones with a centric depression can be elucidated on dermatoscopy
Differentiation is necessitated from clinical conditions such as
verruca, seborrheic keratosis, sebaceous cyst, sebaceous hyperplasia
and hypertrophic actinic keratosis. Histological demarcation
is a pre-requisite from conditions demonstrating acantholytic
dyskerartosis on morphology such as acantholytic or dyskeratotic
acanthoma, Darier’s disease, Grover’s disease, Hailey- Hailey disease,
familial dykeratotic comedones, acantholytic actinic keratosis
and acantholytic squamous cell carcinoma.
Appearance of definitive clinical aspects and emergence of villous
epidermal hyperplasia at inferior segment of warty dyskeratoma
assists in segregation from identical morphologies [9-11].
Warty dyskeratoma can be differentiated from Darier’s disease
and transient acantholytic dermatosis on the basis of specific
clinical attributes. As the lesions are devoid of cellular atypia
and display particular morphological features, conditions such
as squamous cell carcinoma, actinic keratosis or basal cell carcinoma
can be excluded. Differentiation on histological grounds is
necessitated from conditions such as i) Benign familial pemphigusor Hailey-Hailey disease which displays excessive acantholysis
within enlarged zones of epidermis. ii) Darier’s disease which
requires clinic-pathological concordance for appropriate diagnosis.
iii) Pemphigus vegetans which demonstrates enhanced foci of
acantholysis. Eosinophilic aggregates are cogitated within the epidermis.
Surgical specimens submitted for immune fluorescence
can be utilized for discerning pemphigus vegetans [3,4] [Figures
Optimal treatment comprises of surgical extermination of the
lesions, although it is not a pre-requisite with benign histology.
Surgical eradication is a cogent form of therapy. Partial or incompletely
elucidated lesions can be suitably surgically eliminated. Apart from surgical excision, curettage, electrodessication and
irradiation with X rays can be employed to remove the lesions.
However, lesions can reoccur with the utilization of aforesaid
Administration of tazarotenic acid gel is beneficial. Systemic
administration of isotretinoin in combination with topical tretinoin
application can achieve significant alleviation. Reoccurrence
of the lesions is exceptional. Malignant transformation does not