Initial Presentation

6 months later

Laboratory Data

1. Palisaded neutrophilic and granulomatous dermatitis (a.k.a."interstitial granulomatous dermatitis," which is a pattern that can be seen in association with connective tissue disease, rheumatoid arthritis, systemic vasculitis, infection, lymphoproliferative disorders, inflammatory bowel disease, and drug reaction, among others).
2. Annular elastolytic granuloma (actinic granuloma), which many experts consider to be the same as granuloma annulare occurring on sun-damaged skin (and would fit well with the photodistribution and facial involvement).
3. Disseminated granuloma annulare.
We do not think that this is xanthoma disseminatum or generalize eruptive histiocytosis, although these are still in the differential diagnosis.

Interstitial Granulomatous Dermatitis

Granulomatous dermatitis initially in a photodistribution but with extensive involvement of the trunk. Extensive work-up was done to rule out a deep fungal process, BCG reaction

Drug reaction was considered and his enalapril September, 2006. Although he has been off of enalapril, he continues to develop new lesions on the trunk.

Alternate diagnosis suggested when path was read again by Dr. Walter Burgdorf.

Xanthoma disseminatum...

Started on methotrexate by his rheumatologists for joint pain. Some mild improvement of the rash and joint pain throughout the winter.

Now flaring with more erythema, papules and itching.

David Elpern MD, Williamstown, Massachusetts, USA on September 7, 2007

Kudos to Dr. Dintiman and her colleague for an extraordinary case presentation. Lucidly presented and challenging. I have seen three cases of interstitial granulomatous dermatitis (IGD) in the past year and have been struck by the fact that they looked quite dissimilar to each other. This may be a histopathologic diagnosis, like sarcoidosis which has protean clinical manifestations. The cause of IGD, like that of sarcoidal reactions of the skin may be varied, too. My cases were idiopathic, although we suspected drug in at least one. The process in my cases seemed to wax and wane. Clinically, I agree this looked like xanthoma disseminatum, but the path did not support that. It would be instructive to have a few dermatopathologists weigh in on this case.

Many case of IGD in the literature have been associated with arthritis. What was the rheumatologist's working dx that led to Mtx therapy?

Nick Mousdicas MD, Clinical Associate Professor, Department of Dermatology, Indiana University, Indianopolis, Indiana, USA on September 7, 2007

I would consider doing appropriate age and sex cancer screening and strongly consider stopping whichever of the listed drugs that had been prescribed prior to the onset of the dermatosis. I have been burned too many a time ignoring prescribed drugs as a cause of unusual skin presentations.

Khalid Al Aboud M.D., Medical Director, Makkah, Saudi Arabia on September 7, 2007

What about leprosy ?Mycosis fungoids? What about CBC , and peripheral blood smear? Is there any atypical cell?

Omid Zargari MD, Rasht, Iran on September 7, 2007

I think this is a case of disseminated granuloma annulare. Immunohistochemical staining (CD68/PGM1) may aid in making a definite diagnosis. A short course of cyclosporine might be helpful in treating this man. Nice case, thanks for sharing.

Bushan Kumar MD, Former Head of Dermatology, Postgraduate Institute of Medical Education & Research, Chandigarh, India on September 7, 2009

Langerhans cell histiocytosis. It will heal of its own but it will take many months. No treatment is required – slight response to methotrexate is expected

Stephen Glinick MD, Clinical Assoc Prof, Brown University,Providence, Rhode Island, USA on September 8, 2007

Consider Multicentric Reticulocytic Histiocytosis given the arthritis, leonine facies and consistent histologic findings.

Goh Chee Leok MD FRCP, Professor, National Skin Centre, Singapore on September 9, 2007

An interesting case indeed. The case will have to depend heavily on the dermpath report. They seem to have several differential diagnoses. The dermatopath needs to do some immunohistochemistry study to ascertain the cell type.

I hope they have excluded leprosy - histoid leprosy with the relevant Fite stain. I would seriously remove/replace the drugs that he is on to exclude a drug eruption.

If it is disseminated GA, phototherapy has been reported to be helpful (provided the lesions are not photosensitive).

Update: Brenda J Dintiman M.D., Fairfax, VA, USA on Feb 26, 2008

He has been on methotrexate 15mg a week for 1 year and a little dermazinc . Methotrexate given to him by rheumatologist. Still diagnosis of skin unclear but cleared.