Bazex Syndrome

Presented by: Dr. Eric Ersham

Abstract: 45 yo man with progressive hyperkeratosis on his fingers, toes and ears.

History: This 45-year-old man consulted us for a hyperkeratosis on his fingers, toes and ears which had been progressive for 6 months.

O/E: Hyperkeratosis localized on toes, fingers, and ears (helix).
The physical exam revealed a hard left cervical lymph node.

The patient was referred to an ENT specialist whose examination revealed two carcinomas: one on the left tonsil and one on the oesophagus.

Clinical Photos:






Laboratory: N/A

Histopathology: N/A

Diagnosis: Bazex's syndrome (Acrokeratosis neoplastica)

Comments: Considering the diagnosis of Bazex's syndrome lead to the discovery of the cancers of the tonsil and oesophagus in this patient. “Bazex is a rare psoriasiform dermatosis that is consistently associated with internal malignancy, usually squamous cell carcinoma (SCC) of the upper aerodigestive tract. Skin manifestations include acute onset of onychodystrophy and violaceous scaly patches on the helices, nose, and malar and acral surfaces.” (See Reference) Usually, skin manifestations precede diagnosis of the malignancy. This is an example of a paraneoplastic syndrome.

References: Acrokeratosis Neoplastica: There is a good chapter on eMedicine

Comment: This case is presented for interest. It illustrates the importance of considering paraneoplastic disorders.

Thank you,

Eric Ehrsam
Dermatologist
Graz, Austria

Pigmented Patch and Congenital Deafness

Abstract: 47 yo woman with an unusual pigmented lesion
Presented by: Mubina Nasrin and David Elpern
History: This 47 year old woman has had a slowly progressive pigmented patch on the posterior left shoulder since childhood. It has gotten darker over many years. In addition she has congenital sensoryneural hearing loss (as does a brother). His skin is reportedly normal.
O/E: There is a solitary large, somewhat "s" shaped lesion measuring 15 x 5-6 cms over the inferior aspect of the Left shoulder and upper back. The patch has speckled pigmented macules, well defined margins and a smooth surface.
Clinical Photo(s)

Lab: N/A
Histopathology: None done
Diagnosis or DDx: ? Speckled Lentiginous Nevus
Questions: Is this patient's deafness related to the pigmented lesion? Does she have the "speckled lentiginous nevus syndrome." DEafness has not been reported with this disorder in the past.
Reason(s) Presented: For discussion and ideas.
References: Rudolf Happle. Speckled lentiginous nevus syndrome: Delineation of a new distinct neurocutaneous phenotype
European Journal of Dermatology. Volume 12, Number 2, 133-5, March - April 2002
Summary : Speckled lentiginous nevus syndrome, a so far unrecognized cutaneous phenotype associated with neurological anomalies, is postulated on the basis of the following arguments. Phacomatosis pigmentokeratotica represents a twin spot phenomenon. One isolated half of this complex phenotype is observed rather often in the form of Schimmelpenning syndrome, whereas the other half of this twin nevus syndrome consists of a speckled lentiginous nevus associated with various neurological abnormalities incompatible with Schimmelpenning syndrome, such as hyperhidrosis, muscular weakness and dysesthesia. This second component of phacomatosis pigmentokeratotica may likewise occur separately. For the association of speckled lentiginous nevus with hyperhidrosis, muscular weakness, dysesthesia or other neurological abnormalities, the term "speckled lentiginous nevus syndrome" is proposed. Some case reports that may be categorized as examples of this new syndrome are reviewed. The postulated new phenotype is tentatively categorized as a paradominant trait. Future clinical studies will probably confirm the existence of speckled lentiginous nevus syndrome as a distinct neurocutaneous phenotype.

Eyelid Tumors

Submitted by Arash Abtahian from Shiraz, Iran
Abstract: 24 yo man with three year hx of eyelid lesions
History: This 24 man has come with 3 years hx of multiple translucent papule and nodules on the left upper lid. They are asymptomatic but he'd like them removed.
O/E: revealed multiple translucent papules and nodules some of which showed bluish hue.
Clinical Photo(s):



Lab: N/A
Histopathology: Apocrine Hidrocystoma
Diagnosis or DDx: Apocrine Hidrocystoma
Questions: What is the best treatment?
Reason(s) Presented: Therapeutic suggestions?
References: Apocrine Hidrocystoma eMedicine: "Apocrine hidrocystomas can be incised and drained; however, electrosurgical destruction of the cyst wall often is recommended to prevent recurrence. Punch, scissors, or elliptical excision also can remove tumors. Multiple apocrine hidrocystomas can be treated with carbon dioxide laser vaporization. Multiple apocrine hidrocystomas can also be effectively treated with trichloroacetic acid."

Black Papule in Child

Abstract: 11 yo boy with long history of black lesion left arm
History: The lesion has been present for years, little change but patient and parent are worried bout it.
O/E: 5 mm diameter papule with a peculiar serrated border
Clinical Photo(s)














Dermoscopic Image

Lab:
Pathology:

Diagnosis or DDx: Reed Nevus (aka Pigmented spindle cell nevus)
Questions: For inerest
Reason(s) Presented: When I first saw this patient, I thought it was a blue nevus. I sent the history and digital images to a few friends. Drs. Ian McColl and Stelios Minas thought it was a Reed nevus and recommended excision. Biopsy was done and confirmed their opinion.
References: The Reed Nevus is considered a variant of the Spitz nevus. It presents as a well-circumscribed deeply pigmented nodule on the extremities of young adults. Under the microscope, it resembles the Spitz nevus but is composed of spindled melanocytes with heavy melanin pigmentation. As in a Spitz nevus, this nevus is symmetrical with maturation and lack of deep dermal mitotic figures. Excision of suspected Reed nevi is recommended. For more information see: The Doctor's Doctor