Sir,
We are grateful for the comments of Dr. Nofal et al. and appreciate the interest they displayed in our paper entitled, “Scleromyxedema: An Atypical Case.”[1] Dr. Nofal et al. felt that our patient had cutaneous manifestations alone, with the association of eosinophilia and should not be classified as scleromyxedema. However, our patient had many systemic features in the form of hepatomegaly with a 17 cm span, mediastinal, and axillary lymphadenopathy, an elevated creatinine at 1.5 mg/dl and leukocytosis of 36,900/mm3 with an absolute eosinophil count of 31,000/mm3. We do not agree that our patient had purely cutaneous involvement and was “otherwise healthy.” We used the revised criteria proposed by Rongioletti et al.,[2] and, like them, included our patient as atypical because he did not have monoclonal gammopathy.
We used the staging proposed by Donato et al.[3] and categorized our patient as clinical Stage II, with generalized cutaneous mucinosis and/or extracutaneous manifestation(s). They had excluded the absence of thyroid disorder in their diagnostic criteria, similar to what Dr. Nofal et al., have proposed, but required the presence of a serum monoclonal gammopathy along with the histologic presence of dermal mucin deposit with increased collagen deposition and fibroblast proliferation. Donato et al.[3] also noted that monoclonal gammopathy was present in only 80% of all cases. It is recommended to follow the patient for years for development of the same.
Our reservations on a new classification are simply that an old one already exists which is different from the new one proposed by Dr. Nofal et al. The differences are displayed in the Table 1.
Table 1.
Comparison of both staging systems
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
References
- 1.Thomas E, George A, Deodhar D, John M. Scleromyxedema: An atypical case. Indian J Dermatol. 2015;60:323. doi: 10.4103/0019-5154.156456. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 2.Rongioletti F, Merlo G, Cinotti E, Fausti V, Cozzani E, Cribier B, et al. Scleromyxedema: A multicenter study of characteristics, comorbidities, course, and therapy in 30 patients. J Am Acad Dermatol. 2013;69:66–72. doi: 10.1016/j.jaad.2013.01.007. [DOI] [PubMed] [Google Scholar]
- 3.Donato ML, Feasel AM, Weber DM, Prieto VG, Giralt SA, Champlin RE, et al. Scleromyxedema: Role of high-dose melphalan with autologous stem cell transplantation. Blood. 2006;107:463–6. doi: 10.1182/blood-2004-12-4870. [DOI] [PMC free article] [PubMed] [Google Scholar]
