Generalised Reddish-Brown Papules in a Young Boy

presented by

Khalifa Sharquie MD (1)

Adil A. Al-Nuaimy, MD, FICS (2)

Nabeel O. Kadir MD (3)

Baghdad, Iraq

on December 12, 2005

(1) Professor of Dermatology, Scientific Council of Dermatology and Venereology, Iraqi Board for Medical Specializations, Baghdad, Iraq.

(2) Lecturer, Department of Dermatology, College of Medicine University of Baghdad, Baghdad, Iraq

(3) Dermatologist, Baghdad Teaching Hospital, Baghdad, Iraq.
 
Abstract This 3.5 yrs old male child presented with 9-months history of non-itchy rash mainly on the abdomen & neck. The rash consisted of yellowish-reddish or reddish- brown papules on the abdomen, neck & pubic areas.
Patient
3.5 yrs old male
Duration
9 months
Distribution
Sides of the face, neck and the whole abdomen.
History

History of 4 months duration of rash that started on the periumbilical region then over the last 5 months extended to involve the whole abdomen including the pubic area, genitalia, circumflex of the neck and sides of the face. The rash started as itchy and then over time itching disappeared. No history of the same disease in the relatives of the patients.
Physical Examination

Examination at presentation revealed a rash consisted of small yellowish-reddish or reddish-brown papules coalesced in the same place to form a sheet of rash. The rash was slightly scaly, non-tender involving the abdomen extending downward to pubic area and genitalia and upward to the neck and sides of the face Ophthalmological examination showed no angiod steaks or other abnormalities.

Images

Laboratory Data

All laboratory including hematological and biochemical tests were within normal range.
Histopathology

Using Haematoxilin & Eosin stain showed patchy basophilic calcium deposits in the upper dermis.
Diagnosis ? Pseudoxanthoma elasticum: Florid onset in early childhood.
Reasons Presented

To show the florid onset of peudoxanthoma elasticum in early childhood and to avoid and prevent the early possible complications of the disease.
Questions The question to be raised is how often our colleagues had seen early onset of the disease and any suggestions for management.
References

Glass LF, Smith DF & Gupta A. Pseudoxanthoma elasticum: last update: July 24, 2003; e-medicine: instant access to the minds of medicine.www.e-medicine.com

Comments from Faculty and Members

Robert I. Rudolph, M.D., FACP, Clinical Professor of Dermatology, University of Pennsylvania, Philadelphia. PA, USA on Dec 12, 2005

Did sectioning of the specimen show extrusion of calcium, and any calcium around vessels? This looks very peculiar, and not like PXE to me. Are radiologic studies of his vessels normal? Is his parathyroid hormone levels and urinary calcium and phosphate levels normal? Any angiod streaks in his eyes?

It looks like a calcifying version of eruptive histiocytosis!

Amira Adel MD, Egypt, on Dec 13, 2005

Clinically the lesion looks like histiocytosis. Histopathological confirmation is needed.

John Andrew Carlson MD, Professor, Divisions of Dermatopathology and Dermatology, Albany Medical College, Albany, NY, USA on Dec 18, 2005

Strange case. PXE certainly is a good bet, but that doesn't give you a large clump of basophilic material in upper dermis. The photomicrograph shows a calcinosis cutis histology. Also, instead of yellow papules and wrinkles, he has red, coalescent papules and plaques. In PXE, there are calcified and fragmented fibers in the mid dermis, not clumps of aggregated material. Could this be due to a drug or external exposure of some sort?

Lionel Bercovitch MD, Medical Director, Pseudoxanthoma Elasticum International Inc., Clinical Professor of Dermatology, Brown Medical School, Providence, RI, USA, on December 20, 2005

The distribution of the lesions and the histology do not suggest PXE. In PXE, one usually sees focal calcification of dystrophic elastic fibres in the mid-dermis and reticular deermis. The histology here suggests some form of calcinosis cutis. I assume that the lesions are not osteomas-the histology is not clear at screen resolution. The chin lesions and perhaps some of the abdominal lesions appear to perforating clinically. There is nothing in the histology shown to support a diagnosis of histiocytosis-has this been excluded histologically? Are other biopsies planned? Were calcium, phosphorus, and PTH levels drawn?
Childhood PXE, while rare, tends to present with subtle yellowish papules on the neck. Facial involvement would be extremely rare, particularly to this degree.

Joel Bamford MD, Duluth, MN, USA on December 22, 2005

Interesting case, it would be nice to see follow-up note: course and other tests ~ 3 months. Thanks for presenting this patient.

Additional comments and images from Khalifa Sharquie MD on December 25, 2005

We know the diagnosis could be labeled as calcinosis cutis. But what is the underlying cause? It is not clinically childhood dermatomyositis or histologically histiocytosis X. There are no metabolic abnormalities like hypercalcemia and the child is healthy apart from the rash. The rash on the side of the neck is suggestive of early PXE (attached). So by exclusion,we put the possibility of PXE. All sections of biopsy showed nothing apart from localised calcium deposits. Otherwise there are no inflammatory changes to suggest any inflammatory disease like histiocytosis X. Further evaluation will be carried out for the benefit of the patient and doctors. Please give a reference for any important suggestions for documentation.

 

Haitham Al-Qari MD, Department of Dermatology, Mount Sinai School of Medicine, New York, NY, USA on December 25, 2005

Great case but doesn't suggest PXE clinically or eruptive histiocytosis histopathologically, and the histopath picture is not that clear. Clinically this picture is suggestive of Darier-White disease. Suggestions: repeat the histology with specific staining.

Please Click Here To Comment and Evaluate

Back to November 29, 2005 Case