A rare presentation of keratosis follicularis spinulosa decalvans in female twins

Department of Dermatology and Venereology, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh, India

Date of Web Publication 28-Aug-2017

Correspondence Address:
Saumya Sankhwar
Department of Dermatology and Venereology, Institute of Medical Sciences, Banaras Hindu University, Varanasi – 221 005, Uttar Pradesh

How to cite this article:
Chauhan RK, Sankhwar S, Tripathi R, Pandey S S. A rare presentation of keratosis follicularis spinulosa decalvans in female twins. Indian J Dermatol Venereol Leprol 2018;84:645


How to cite this URL:
Chauhan RK, Sankhwar S, Tripathi R, Pandey S S. A rare presentation of keratosis follicularis spinulosa decalvans in female twins. Indian J Dermatol Venereol Leprol [serial online] 2018 [cited 2020 Sep 7];84:645. Available from: http://www.ijdvl.com/text.asp?2018/84/5/645/213709


A 9-year-old pair of monozygotic twin sisters, born of spontaneous, full-term normal vaginal delivery, visited the dermatology outpatient department of Sir Sunderlal Hospital, Banaras Hindu University, Varanasi, with the absence of body and scalp hair since birth. There was no history of consanguinity in parents. Parents were using various homemade remedies for the condition, on-and-off, including ayurvedic treatment, since 1 year of age. Some hairs appeared on the scalp one year later and they were persistent [Figure 1] and [Figure 2]a. Examination revealed small, discrete, spiny papules with follicular plugging, present on normal looking skin on the body [Figure 2]b. Oral mucosa, teeth, nails, palms and soles were found to be normal. There was no history of photophobia. Ophthalmic examination revealed no abnormal findings. Routine hematological and other laboratory studies were within normal limits. Hair microscopy was done to rule out hair shaft defects.

Figure 1: Female twins with follicular papules and some hair on the scalp

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Figure 2

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Histopathological examination of the hairy areas such as the scalp and extensor aspect of the left forearm showed sparse superficial perivascular and periappendageal lymphocytic infiltrates with no epidermal changes. Some of the follicular infundibula were dilated and plugged with orthokeratotic corneocytes while others showed mild spongiosis. There was dense perifollicular fibroplasia in some follicles and the terminal follicles were reduced in number on the scalp skin [Figure 3] and [Figure 4]. Based on these clinical and histopathological findings, these cases were diagnosed as keratosis follicularis spinulosa decalvans.

Figure 3: Perifollicular fibroplasia and lymphocytic infiltrate (arrowheads) in other fields on scalp histopathology (H and E, ×100)

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Figure 4: Dilated follicular infundibula plugged with orthokeratotic corneocytes and perivascular, periappendageal lymphocytic infiltrate on scalp histopathology (H and E, ×400)

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Keratosis follicularis spinulosa decalvans is a rare, X-linked, hereditary disorder of keratinization, characterized by the involvement of the skin and eyes. Keratosis follicularis spinulosa decalvans was first described as an X-linked dominant disorder.[1] However, in some cases, an autosomal dominant and sporadic inheritance have also been reported. The term was first used by Siemens in 1926, who described some individuals from a Bavarian family who presented with follicular papules on the face, trunk and extremities with partial loss of hairs; thus, the disease is also known as Siemens syndrome.[1] Genetic studies in Dutch and English families have showed connection with the Xp21.2-p22 gene.[2] When the inheritance pattern is X-linked, males are predominantly affected and females are mostly carriers. Females with severe forms of the disease have also been reported. These cases either had an autosomal dominant pattern of inheritance or some were sporadic in onset. A theory of non-random X-inactivation (process of  Lyonization More Details) was proposed in cases with sporadic onset.[3] As there is no positive family history and since the disease is rare and present in female twins, our case likely has either an autosomal dominant inheritance or heterogeneous transmission with sporadic inheritance, which is one of the four reported patterns of inheritance in literature.[4] Our patients also had the characteristic cutaneous and histological features of keratosis follicularis spinulosa decalvans. However, there were no other associated abnormalities such as ocular changes, deafness, physical and mental retardation, hypoplastic nails and palmoplantar keratoderma, as described in literature.[3],[4] Moreover, the presence of scarring alopecia on the scalp [Figure 2]a and the lack of ophthalmological abnormalities differentiate it from ichthyosis follicularis alopecia and photophobia syndrome, where there is nonscarring alopecia and photophobia that presents either at birth, in infancy or in early childhood.[3] Furthermore, in this case, while there was complete alopecia at birth, some hairs appeared on the scalp with time that are still persisting; which helped to differentiate this from the condition- congenital atrichia with papules, where there is permanent and complete alopecia by the first few months of life and no hair regrowth occurs with the passage of time.[5] Clinically, our patients did not have any bony pain or deformities and there was no decreased muscle tone and the levels of alkaline phosphatase, serum calcium and vitamin D were within normal limits, thus ruling out rickets. Other differentials such as lichen spinulosus and lichen planopilaris were excluded on the basis of histopathological findings.

There is no specific treatment for this disorder. However, drugs such as isotretinoin and dapsone have been tried; emollients, topical corticosteroids and keratolytic agents can be used for symptomatic relief.[3] Although it is a rare genodermatosis, keratosis follicularis spinulosa decalvans should always be considered in all cases of hyperkeratosis with alopecia. Genetic counseling is needed in some cases and the treatment should be started as early as possible so as to retard and minimize sequelae such as cicatrization.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his/her consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal patient identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top


Siemens HW. Keratosis follicularis spinulosa decalvans. Arch Derm Syphilol (Berlin) 1926;151:384.  Back to cited text no. 1
Oosterwijk JC, Nelen M, van Zandvoort PM, van Osch LD, Oranje AP, Wittebol-Post D, et al. Linkage analysis of keratosis follicularis spinulosa decalvans, and regional assignment to human chromosome Xp21.2-p22.2. Am J Hum Genet 1992;50:801-7.  Back to cited text no. 2
Maheswari UG, Chaitra V, Mohan SS. Keratosis follicularis spinulosa decalvans: A rare cause of scarring alopecia in two young Indian girls. Int J Trichology 2013;5:29-31.  Back to cited text no. 3
Castori M, Covaciu C, Paradisi M, Zambruno G. Clinical and genetic heterogeneity in keratosis follicularis spinulosa decalvans. Eur J Med Genet 2009;52:53-8.  Back to cited text no. 4
Yip L, Horev L, Sinclair R, Zlotogorski A. Atrichia with papular lesions: A report of three novel human hairless gene mutations and a revision of diagnostic criteria. Acta Derm Venereol 2008;88:346-9.  Back to cited text no. 5

Disseminated cutaneous fusariosis in human immunodeficiency virus-infected patient and dramatic response with oral itraconazole

1 Department of Dermatology and Venereology, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh, India
2 Department of Microbiology, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh, India

Date of Web Publication 09-Jan-2018

Correspondence Address:
Dr. Satyendra Kumar Singh
Department of Dermatology and Venereology, Institute of Medical Sciences, Varanasi, Uttar Pradesh – 221 005


Fusarium species are known to cause disseminated cutaneous lesions in immunocompromised patients. Some cases of fusariosis are reported in patients infected with the human immunodeficiency virus. There are two reports in such patients with systemic comorbidities like lymphoma, neutropenia and infective port-a-catheter. Another reported patient had systemic fusariosis, without skin involvement. Diagnosis and treatment of cutaneous fusariosis is difficult and resistance to antifungals is a problem. Our patient was at an advanced human immunodeficiency virus infection stage with disseminated cutaneous fusariosis, without any systemic involvement, who responded completely to oral itraconazole.


Keywords: Disseminated, Fusarium, human immunodeficiency virus, itraconazole


How to cite this article:
Kumari I, Singh SK, Chauhan RK, Kaushal SK. Disseminated cutaneous fusariosis in human immunodeficiency virus-infected patient and dramatic response with oral itraconazole. Indian J Dermatol Venereol Leprol 2018;84:362-8


How to cite this URL:
Kumari I, Singh SK, Chauhan RK, Kaushal SK. Disseminated cutaneous fusariosis in human immunodeficiency virus-infected patient and dramatic response with oral itraconazole. Indian J Dermatol Venereol Leprol [serial online] 2018 [cited 2020 Sep 7];84:362-8. Available from: http://www.ijdvl.com/text.asp?2018/84/3/362/222753


  Introduction Top

Fungal infections cause cutaneous lesions either directly or are metastatic in immunocompromised patients. Fusarium species are common plant pathogens that cause localized and disseminated infection (in immunocompromised and neutropenic patients). Majority of patients (72%–91%) have cutaneous lesions (primary or metastatic).[1] Diagnosis of disseminated cutaneous infection is difficult. The treatment is quite unsatisfactory due to the variable susceptibility of the pathogen to antifungals and the lack of supporting evidence. We herein report a rare case of disseminated cutaneous fusariosis, without systemic involvement, in a patient infected with the human immunodeficiency virus, who responded successfully to itraconazole.

  Case Report Top

A 40-year-old farmer presented with multiple, painless, raised skin lesions with pus discharge, which were of one-and-a-half months  duration. Lesions began to appear on the face and progressed to both his arms and thighs over a period of 1 week. He was earlier diagnosed to be infected with the human immunodeficiency virus, with CD4 T-lymphocyte count of 38 cells/μL, and was on highly active antiretroviral therapy from an antiretroviral therapy center for the past 6 months.

The patient was afebrile with no other systemic complaints. On examination, his body mass index was 18.88 kg/m [2]. Multiple erythematous and tender papules and nodules with central crusting and necrosis were present on the face, neck, and bilaterally on arms and on thighs extending to the knees [Figure 1]. Trunk, distal extremities, palms, soles and mucosae were spared. There was no history of trauma and no clinically apparent onychomycosis or any breach in the skin other than the lesions present. Clinically, the possibility of cryptococcosis (common opportunistic infections in human immunodeficiency virus patients) and ecthyma gangrenosum were considered. Lymph nodes examination revealed no significant enlargement.

Figure 1

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On hematological investigation, no significant abnormality was found. Blood culture was negative for any bacterium or fungus. All other possible systemic involvements were ruled out by appropriate tests, mainly computed tomography of the chest, x-rays of the paranasal sinuses and abdominal ultrasonography. Skin biopsy from a nodule on the arm revealed pseudoepitheliomatous hyperplasia and intraepidermal granulomas with giant cells. Periodic acid–Schiff staining was positive for spores and short fungal hyphae. An additional sample of the excised nodule was sent for microbiological examination.

A 10% potassium hydroxide wet mount of tissue revealed septate, hyaline and branched fungal hyphae. The specimen was cultured on two sets of Sabouraud dextrose agar, incubated at 37°C and 25°C. After 4 days of incubation, a heavy growth of fungal colonies was seen at 25°C in the form of a white and cotton obverse and orange reverse suggestive of Fusarium [Figure 2]. Growth was observed on successive subcultures performed on potato dextrose agar. Lactophenol cotton blue mount showed septate, branched hyphae producing microconidia (oval small 2–4 × 4–8 μm with 1–2-celled reniform conidia singly and in clusters) and many branched conidiophores with phialides producing 2–4 × 11–60 μm multiseptate (3–4), sickle or boat-shaped macroconidia typical of Fusarium solani [Figure 3]. Later, another tissue sample was sent for repeat isolation of fungus in order to confirm the species.[2]

Figure 2

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Figure 3

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Minimal inhibitory concentrations and minimal effective concentration were determined following the microdilution method recommended by Clinical and Laboratory Standards Institute document M38-A2 with minor changes.[3] Fluconazole, amphotericin-B and terbinafine were ineffective in inhibiting growth of F. solani. Among sensitive drugs, itraconazole, ketoconazole and voriconazole had minimal inhibitory concentration 90 of 2 μg/ml each [Figure 4].[4],[5],[6]

Figure 4: Minimal inhibitory concentrations by microdilution method in round-bottomed 96-well microdilution tray

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Our patient was prescribed itraconazole 200 mg twice daily and response was seen in 2 weeks, with complete subsidence of lesions in 1½ months, leaving postinflammatory hyperpigmentation and scarring [Figure 5]. Itraconazole was continued at a tapered dose of 100 mg twice daily for a further 3 months.

Figure 5

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  Discussion Top

Fusarium species are ubiquitous soil saprophytes and plant pathogens that may cause infections in humans, in localized, locally invasive and disseminated forms. Fusarium species now represent the second most common mold causing opportunistic infections in immunocompromised patients.[7]F. solani was the most frequent, followed by Fusarium oxysporumFusarium verticillioidis and Fusarium moniliforme among 12 species associated with infection.[8]

Skin involvement represents a frequent manifestation (>50%) of infection by Fusarium species and is also the most common source of diagnostic material. Distinct patterns of skin involvement exist, depending on the immune status of the host.[9] Sampathkumar and Paya classified the cutaneous lesions into three groups: violaceous nodules with central necrosis, ecthyma gangrenosum-like and target lesions.[10] In immunocompromised patients, skin lesions evolve rapidly over a short period of time and can involve any site, with predominance in the extremities.

The principal portal of entry for Fusarium species is the airways, followed by skin at the site of tissue breakdown (trauma, burns and onychomycosis) and foreign bodies (keratitis in contact lens wearers). Other sources are tap and hospital water system. The fact that we could not establish the entry portal of the fungus in our patient might be explained by the fact that skin breakdown may precede infection by up to 1 year.[9]

Disseminated infections occur when two or more noncontiguous sites are involved.[9] Immunocompromised patients with prolonged and profound neutropenia (hematologic diseases) and/or severe T-cell deficiency (allogenic hematopoietic stem cell transplantation) and high-dose corticosteroid therapy are at the highest risk.[1],[9] So far, disseminated cutaneous fusariosis in human immunodeficiency virus-positive patients is very rarely known. In a review published in 2002, out of 259 patients with fusariosis, 232 (90%) patients were immunocompromised. Skin involvement was seen in 181 (70%) patients and was more common in immunocompromised group (72% vs. 52%).[9] Out of 232 patients, only two were human immunodeficiency virus positive of which one had an infected port-a-catheter and other patient had non-Hodgkin’s lymphoma with neutropenia.[11],[12] Another case was reported in human immunodeficiency virus patient without skin involvement and neutropenia in 2013.[13] Hence, we state that this is the first reported case of isolated disseminated cutaneous fusariosis and the third case of disseminated fusariosis in a human immunodeficiency virus infected patient [Table 1].

Table 1: Cases of disseminated fusariosis in human immunodeficiency virus patients

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The most frequent pattern of disseminated disease is a combination of cutaneous lesions and positive blood cultures with or without involvement of other sites.[8] Interestingly, in a previous review among patients with positive blood culture, skin lesions preceded fungemia in 11 patients by a median of 5 days, occurred on the same day (4 patients) and did not appear until after the diagnosis of fungemia was made (3 patients).[8] In this case, the blood culture was negative for Fusarium similar to a previous review where 78 (52.7%) patients had blood cultures negative for Fusarium species.

Different patterns of susceptibility of different Fusarium species exist and there is not enough data documenting a correlation between minimal inhibitory concentrations and the clinical outcome. Amphotericin B has been used as the first-line drug but resistance to it has been reported and even our case was found to be resistant to it.[14] Newer antifungal triazole agents are available for Fusarium species including itraconazole, voriconazole and posaconazole. Susceptibility to itraconazole has been shown in some reports but at higher minimal inhibitory concentration which is even higher for F. solani.[15],[16] The results of itraconazole against localized fusariosis have been documented but rarely against disseminated cutaneous fusariosis. This variable susceptibility to these drugs poses a great problem for the physicians.

Our case of disseminated cutaneous fusariosis is being reported for the first time and is unique since the cause of immunocompromised state is only human immunodeficiency virus without any associated neutropenia, concomitant malignancy or any systemic involvement. Moreover, the excellent response of Fusarium to itraconazole is worth reporting.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initial will not be published and due efforts will be made to conceal his identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top


Boutati EI, Anaissie EJ. Fusarium, a significant emerging pathogen in patients with hematologic malignancy: Ten years’ experience at a cancer center and implications for management. Blood 1997;90:999-1008.  Back to cited text no. 1
Hafizi R, Salleh B, Latiffah Z. Morphological and molecular characterization of Fusarium Solani and F. Oxysporum associated with crown disease of oil palm. Braz J Microbiol 2014;44:959-68.  Back to cited text no. 2
Clinical and Laboratory Standards Institute. Reference Method for Broth Dilution Antifungal Susceptibility Testing of Filamentous Fungi: Approved Standard M38-A. Wayne, PA, USA: CLSI; 2008.  Back to cited text no. 3
Alastruey-Izquierdo A, Cuenca-Estrella M, Monzón A, Mellado E, Rodríguez-Tudela JL. Antifungal susceptibility profile of clinical Fusarium spp. Isolates identified by molecular methods. J Antimicrob Chemother 2008;61:805-9.  Back to cited text no. 4
Espinel-Ingroff A, Boyle K, Sheehan DJ.In vitro antifungal activities of voriconazole and reference agents as determined by NCCLS methods: Review of the literature. Mycopathologia 2001;150:101-15.  Back to cited text no. 5
Guilhermetti E, Takahachi G, Shinobu CS, Svidzinski TI. Fusarium spp. As agents of onychomycosis in immunocompetent hosts. Int J Dermatol 2007;46:822-6.  Back to cited text no. 6
Vennewald I, Wollina U. Cutaneous infections due to opportunistic molds: Uncommon presentations. Clin Dermatol 2005;23:565-71.  Back to cited text no. 7
Nucci M, Anaissie E. Fusarium infections in immunocompromised patients. Clin Microbiol Rev 2007;20:695-704.  Back to cited text no. 8
Nucci M, Anaissie E. Cutaneous infection by Fusarium species in healthy and immunocompromised hosts: Implications for diagnosis and management. Clin Infect Dis 2002;35:909-20.  Back to cited text no. 9
Sampathkumar P, Paya CV. Fusarium infection after solid-organ transplantation. Clin Infect Dis 2001;32:1237-40.  Back to cited text no. 10
Eljaschewitsch J, Sandfort J, Tintelnot K, Horbach I, Ruf B. Port-a-cath-related Fusarium oxysporum infection in an HIV-infected patient: Treatment with liposomal amphotericin B. Mycoses 1996;39:115-9.  Back to cited text no. 11
Guarro J, Nucci M, Akiti T, Gené J. Mixed infection caused by two species of Fusarium in a human immunodeficiency virus-positive patient. J Clin Microbiol 2000;38:3460-2.  Back to cited text no. 12
Esnakula AK, Summers I, Naab TJ. Fatal disseminated Fusarium infection in a human immunodeficiency virus positive patient. Case Rep Infect Dis 2013;2013:379320.  Back to cited text no. 13
Pereira GH, de Angelis DA, Brasil RA, dos Anjos Martins M, de Matos Castro e Silva D, Szeszs MW, et al. Disseminated amphotericin-resistant fusariosis in acute leukemia patients: Report of two cases. Mycopathologia 2013;175:107-14.  Back to cited text no. 14
Arikan S, Lozano-Chiu M, Paetznick V, Nangia S, Rex JH. Microdilution susceptibility testing of amphotericin B, itraconazole, and voriconazole against clinical isolates of Aspergillus and Fusarium species. J Clin Microbiol 1999;37:3946-51.  Back to cited text no. 15
Carrillo-Muñoz AJ, Quindós G, Ruesga M, Brió S, del Valle O, Rodríguez V, et al. Activity of itraconazole against clinical isolates of Aspergillus spp. and Fusarium spp. determined by the M38-P NCCLS method. Rev Esp Quimioter 2001;14:281-5.  Back to cited text no. 16

Dermoscopy of a rare case of linear Dermoscopy of a rare case of linear syringocystadenoma papilliferum with review of the literature

Dermoscopy of a rare case of linear syringocystadenoma papilliferum with a review of the literature


Syringocystadenoma papilliferum (SCAP) is a benign hamartomatous tumor arising from pluripotent cells with either apocrine or eccrine differentiation. We report a rare case of de novo linear SCAP in a 12-year-old female child with lesions over the chest along with the dermoscopic findings.

Keywords: linear syringocystadenoma papilliferum, adnexal tumor, dermoscopy


Syringocystadenoma papilliferum (SCAP) is a rare benign hamartomatous adnexal tumor that originates from the apocrine or the eccrine sweat glands. It is a relatively rare neoplasm presenting at birth in 50% of the cases. In around 15–30% of the cases, it develops around puberty. The lesions evolve either de novo or from a preexisting nevus sebaceous. There are three recognized clinical forms it i.e., plaque, solitary nodular, and linear. Most of the lesions localize over the head and neck region. We hereby present a rare case of de novo congenital linear SCAP over the chest. We also describe the dermoscopic findings observed in our case.

Case Presentation

A 12-year-old girl presented with slowly growing multiple, itchy, red, and raised lesions over her chest just below the left clavicle which were present since birth. The lesions had significantly increased in size and number in the previous two years. Serous non-foul-smelling discharge from the lesions was occasionally noticed. Associated symptoms suggestive of neurological, ocular, or skeletal abnormalities were not reported. On clinical examination, multiple grouped dome-shaped papules and nodules with central umbilication were noted to be present in a linear array over the upper part of the chest. The surface of the lesions showed central ulceration and crusting

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multiple grouped dome shaped papules and nodules with central umblication present over the chest just below the left clavicle. [Copyright: ©2018 Chauhan et al.]

Dermoscopic examination with a DermLite II Hybrid M (3Gen, San Juan Capistrano, CA, USA; 10× magnification) was performed which revealed milky red papillomatous projections with central ulceration. At places, white circles were seen over the rim of the milky red areas. Polymorphic vessels were seen within the darker ulcerated areas. Yellowish areas within the ulceration may represent sites of secretion

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Milky red papillomatous projections (black circle) with central ulceration (black arrow). White circles (red circle) over the milky red rim. Polymorphic vessels (green arrows) were also seen. [Copyright: ©2018 Chauhan et al.]

Histopathological examination of the umbilicated papule was done and showed an endophytic tumor with several papillary infoldings lined by glandular epithelium, which consisted of two rows of cells. The inner row had high columnar cells with oval nuclei and faint eosinophilic cytoplasm. The outer row was comprised of small cuboidal cells with round nuclei and scant cytoplasm. Focal areas of decapitation secretion were noted. The core of papillae had many plasma cells, lymphocytes and few polymorphic cells

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(a) Scanner view showing an endophytic tumor with several papillary infoldings in the upper dermis (H&E, ×40). (b) Histopathological examination showing the invagination of surface epithelium and irregular papillary projections protruding into the lumen (H&E, ×100). (c) Several papillary infoldings lined by glandular epithelium consisting of two rows of cells with inner row of high columnar cells having oval nuclei and faint eosinophilic cytoplasm and outer row having small cuboidal cells with round nuclei and scanty cytoplasm. (d) Higher magnification showing core of papillae having many plasma cells, lymphocytes and few polymorphic cells. Decapitation secretion is appreciated in the luminal layer (black arrow) (H&E, ×400). [Copyright: ©2018 Chauhan et al.]

The diagnosis of linear syringocystadenoma papilliferum was made on the basis of clinical, dermoscopic and histopathologic examinations. The patient was referred to the department of pediatric surgery for surgical excision.


Syringocystadenoma papilliferum (SCAP) is a rare benign cutaneous hamartoma seen to arise from the pluripotent cells with the potential to exhibit either apocrine or eccrine lineage, although apocrine differentiation is more common. It usually involves the head and neck area, taking its origin either de novo or from a preexisting nevus sebaceous in 30% of the cases. The unusual anatomical sites of involvement include arms, breast, eyelids, axilla, scrotum, and inguinal and perineal regions

Three clinical types of SCAP have been described: a) Plaque type: presenting as an alopecic patch on the scalp which may enlarge during puberty to become nodular, verrucous or crusted. b) Linear type: consisting of multiple reddish-pink firm papules or umbilicated nodules 1–10 mm in size commonly occurring over face and neck. c) Solitary nodular type: which are domed pedunculated nodules 5–10 mm in size with a predilection for the trunk, shoulder, and axillae. The linear presentation of SCAP is extremely rare with less than 20 cases reported in the literature


Summary of the histopathologically proven cases of linear SCAP

Age /Sex Age at Onset Symptom Location of the Lesions Clinical Morphology Association Author, Year of Publication Ref
10, F Since birth The upper part of the left arm Multiple yellowish-red papules, with central umbilication in several of the papules Rostan et al, 1976
16, F Since birth The left side of chest Skin-colored, firm papules and nodules
Few were ulcerated discharging a serosanguinous material
Premlatha et al, 1985
2, M Since birth Posterior neck below hairline 7 waxy erythematous oval papules Goldberg et al, 1985
12, M Since birth Occasionally mildly pruritic Upper chest Grouped shiny erythematous papules focally coalescing into plaques TAA Epstein et al, 1990
11, F Since birth Right thigh 4 plaques consisting of a dozen pea-sized pink papules and small nodules with crusted surface.
Central umbilication in some.
de Bliek et al, 1999
14, F Early childhood Pruritus Left inner thigh 6 discrete, erythematous papules Apocrine cystadenoma, hidrocystoma Patterson et al, 2001
20, F Since birth Sudden growth in lesion, recent onset of pain Nape of neck Skin-colored and erythematous warty papules arranged vertically Dawn et al, 2002
5, F 6 months of age Scalp Grouped skin-colored, umbilicated papules along with cauliflower-like moist reddish alopecia plaque Laxmisha et al, 2007
51, M early childhood Nape of neck 3 ulcerated nodules (2–3 cm) small, skin-colored and yellow papules in close proximity to the nodules Narang et al, 2008
19, F At birth Extensor site of proximal part of right upper extremity Multiple discrete, erythematous, 0.5–1 cm sized pseudovesicular papules Gönül et al, 2008
20, F At 19 years Pruritus, history of bleeding Left-sided occipital part of the scalp extending to the nape of neck Highly elevated, moist, fetid, vegetated and pinkish lesion Yaghoobi et al, 2009
40, F Since birth Right lower abdomen Multiple erythematous papules and nodules with erosion on the surface of some of the lesions Yap et al, 2010
18, F Early childhood Right inguinal & pubic region 3 large fleshy erythematous, exuberant verrucous plaques with adherent whitish slough Skin-colored, discrete papules with slight umbilication Pahwa et al, 2011
15, F Early childhood Occasionally pruritus Left side of neck 20 discrete erythematous vesicle-like papules Martorell-Calatayu et al, 2011
12, M Since birth Lower back Fleshy cauliflower-like erythematous papulonodular lesion with increase in vascularity and oozing of serosanguinous fluid Kar et al, 2012
36, M 21 years of age Right inguinal fold Multiple skin-colored, dome-shaped, firm, non-tender nodules Ghosh et al, 2012
10, F 2 months Right lower abdomen Multiple erythematous papules, coalescent plaques Chauhan et al, 2013
35, F Since 8 months Mild irritation Left breast 2 erythematous, multilobular exuberant plaques Bandopadhyay, 2014
12, M Since birth Back of the left ear extending to scalp Multiple, skin-colored verrucous papules and nodules, showing erosion on some sites NS Ekinci et al, 2016
12, F Since birth Over chest just below left clavicle Multiple grouped domeshaped papules and nodules with central umblication, ulceration and crusting Present case

Abbreviations: F, female; M, male; NS, nevus sebaceus; Ref, reference; TAA, tubular apocrine adenoma

Thirteen cases of linear SCAP were females and the majority of patients had onset at birth or in early childhood. Only two cases of late-onset linear SCAP have been reported; one at 19 years and another 21 years old. Surprisingly, the majority of the cases arose de novo. Only three cases of linear SCAP have been reported to be associated with nevus sebaceous [], tubular apocrine adenoma [], and apocrine cystadenoma and hidrocystoma []. Hitherto, only three cases of linear SCAP over the chest have been described [,,]. Of these, one case of linear SCAP in a 12-year-old male was associated with tubular apocrine adenoma[]. The remaining two were cases of de novo linear SCAP over the left side of the chest in one [] and over the left breast in the second case []. To the best of our knowledge, our patient represents the third case of de novo linear SCAP over chest.

All the published cases of linear SCAP were histopathologically proven. However, there is a lack of documentation of dermoscopic findings in linear SCAP, as none has been previously reported.

We also highlight the dermoscopic features of SCAP as the actual potential of dermoscopy has not been tapped in the diagnosis of adnexal tumors. In 2011, Bruno et al. [], described the dermoscopic features in SCAP associated with nevus sebaceous for the first time. The authors described a polymorphous vascular pattern comprised of irregular linear and glomerular vessels, some of which were surrounded by a whitish halo and others grouped in a horseshoe arrangement on a pinkish-white background. The dermoscopic findings in our case are similar and corroborate the earlier findings of Bruno et al. [] To the best of our knowledge, ours is the first case describing dermoscopic findings of de novo linear SCAP.

Dermoscopy findings in seven cases of SCAP associated with nevus sebaceous located in the head and neck regions documented by Zaballos et al. [] are noteworthy. The authors noted that the most common dermoscopic pattern associated with their SCAP cases was a symmetric erythematous lesion with “exophytic papillary structures,” followed by a central depression, ulceration and vessels (hairpin vessels, polymorphous vessels and comma vessels). Similarly, Dumen et al., [] have also documented dermoscopic features in a case of SCAP with nevus sebaceous. The dermoscopic examination in the aforementioned case revealed central yellowish-white discoloration, polymorphic vessels including irregular dotted, hairpin-like, glomerular and linear vessels with a surrounding pinkish-white rim and peripheral hairpin like vessels. These findings are also similar to that seen in our case.

The dermoscopic morphology of the other adnexal tumors of apocrine origin needs to be elucidated. Recently, dermoscopic features of 22 cases of apocrine hidrocystoma were reported []. A homogenous area that occupies the whole lesion with arborizing vessels was found to be the most common dermoscopic pattern in apocrine hidrocystoma. Similarly, tubular apocrine adenoma (TAA) is another adnexal tumor of apocrine origin whose dermoscopy is hitherto unclear. Ito et al, [] noted coexistence of short fine telangiectasias and large blue-gray ovoid nests arranged in a floriform pattern to be the specific dermoscopic finding in TAA.

The present report aims to emphasize that the dermoscopic examination can act as an extremely valuable, noninvasive and inexpensive tool in the diagnosis of SCAP especially in children when a traumatic procedure like a biopsy can raise the apprehension and discomfort of the child and the parents. However, further studies are needed to corroborate the dermoscopic findings seen in our case. Moreover, we wish to encourage the incorporation of dermoscopy as an integral part of clinical skin examination so that the dermoscopic patterns can be defined and established for benign adnexal tumors thus averting the need for biopsy as a routine procedure in these patients.


In this report, we describe clinical, histopathological features along with dermoscopic findings of SCAP in a 12-year-old girl. We wish to highlight the dermoscopic features of SCAP and emphasize that dermoscopy can contribute significantly to reaching a diagnosis of this rare entity. As far as we are aware, our patient represents the third case of de novo linear SCAP over the chest.


Funding: None.

Competing interests: The authors have no conflicts of interest to disclose.


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