Syringocystadenoma papilliferum

Syringocystadenoma papilliferum is a rare non-malignant adnexal neoplasm that develops from apocrine or eccrine sweat glands and can be identified histologically by cystic, papillary, and ductal invaginations into the dermis lined by double-layered outer cuboidal and luminal high columnar epithelium and connected to the epidermis.[1]

Syringocystadenoma papilliferum
Other namesSyringadenoma papilliferum, Fistulous vegetative verrucous hydradenoma, Naevus syringocystadenomatosus papilliferus, Papillary syringocystadenoma, and SCAP.
An example of a syringocystadenoma papilliferum
SpecialtyDermatology
Usual onsetChildhood, Neonatal, Infancy, Adolescent.[1]
CausesGenetic.[2]
Diagnostic methodSkin biopsy.[3]
Differential diagnosisHidradenoma papilliferum, Papillary eccrine adenoma, Warty dyskeratoma, Tubular apocrine adenoma, Syringocystadenocarcinoma papilliferum.[3]
TreatmentSurgical excision.[3]

Lesions have a heterogeneous, non-distinctive appearance that ranges from skin-colored to pink-colored papules or plaques. Typically, lesions form in the head and neck area.[1]

Syringocystadenoma papilliferum can develop de-novo or within a nevus sebaceous.[4] Syringocystadenoma papilliferum tends to be seen in children. It is present at birth in approximately 50% of individuals affected, and it develops before puberty in the remaining 15%-30%.[4]

Symptoms and Signs

Symptoms of Syringocystadenoma papilliferum include papules, lack of hair on the scalp, blisters, subcutaneous nodules, and nevus sebaceous.[2]

Nevus sebaceous is a congenital, hairless plaque composed of overgrown epidermis, sebaceous glands, follicles for hair, apocrine glands, and connective tissue. Sebaceous naevi are most commonly found on the scalp, however they may also be found on the face, neck, or forehead.[2]

Diagnosis

A skin biopsy is used to diagnose syringocystadenoma papilliferum. Characteristics of Syringocystadenoma papilliferum are dilated capillaries and a dense infiltration of plasma cells.[1]

Differential diagnoses include papillary eccrine adenoma, warty dyskeratoma, tubular apocrine adenoma, and hidradenoma papilliferum.[3]

See also

References
  1. "Syringocystadenoma papilliferum". Orphanet. Retrieved 5 October 2023.
  2. "Syringocystadenoma papilliferum — About the Disease — Genetic and Rare Diseases Information Center". rarediseases.info.nih.gov. Retrieved 2023-10-05.
  3. "Syringocystadenoma papilliferum". Pathology Outlines. March 10, 2022. Retrieved October 5, 2023.
  4. Sangma, Mima Maychet B. (2013). "Syringocystadenoma Papilliferum of the Scalp in an Adult Male – A Case Report". Journal of Clinical and Diagnostic Research. 7 (4): 742–743. doi:10.7860/jcdr/2013/5452.2900. ISSN 2249-782X. PMC 3644463. PMID 23730665.
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